A neurologic symptom or symptom complex caused by cerebral ischemia or hemorrhage is commonly called a cerebrovascular accident (CVA), or stroke. 

Classify Cerebrovascular diseases.

Subdivided into:

  • Ischemic events: Further classified according to whether symptoms occurred in the carotidor vertebrobasilar distribution and by the duration of symptoms.
  • Cerebral hemorrhages


  • Extracranial atherosclerosis.
  • Intracranial atherosclerosis.
  • Embolism from heart.
  • Intracranial vasculitis (several types).
  • Increased blood viscosity or coagulability.
  • Complicated migraine.
  • Hypertensive arteriolar disease (lacunar stroke).
  • Fibromuscular dysplasia.
  • Carotid dissection.


  • Hypertension (primary hypertensive hematoma)
  • Saccular aneurysm
  • Arteriovenous malformation
  • Anticoagulant therapy
  • Bleeding dyscrasias
  • Amyloid angiopathy
  • Hemorrhage due to brain tumor
  • Mycotic aneurysm
  • Idiopathic

Carotid distribution

  • Hemiparesis or monoparesis
  • Facial weakness
  • Hemisensory numbness or neglect
  • Aphasia
  • Dysarthria
  • Amaurosis fugax (fleeting blindness of one eye)

Vertebrobasilar distribution

  • Vertigo
  • Dysarthria
  • Dysphagia
  • Diplopia
  • Homonymous hemianopsia
  • Total blindness (cortical blindness)
  • Alternating or bilateral weakness
  • Alternating or bilateral numbness
  • “Crossed” weakness or numbness (ipsilateral face and contralateral body)
  • Gait ataxia
  • Limb dysmetria 

TIA is a transient episode of neurological dysfunction caused by focal brain, spinal cord, or retinal ischemia without acute infarction.

Defined as focal or non-focal cerebrovascular symptoms lasting less than 24 hours and patients with LI on diffusion-weighted images (DWI) or fluid-attenuated inversion recovery (FLAIR)

PSI Defined as focal or non-focal cerebrovascular symptoms lasting more than 24 hours and patients with LI on DWI or FLAIR.

Defined as bilateral, symmetrical, and diffuse hyperintensities, which were located in the subcortical and periventricular white matter with T2-weighted images and prone density image.

VSE defined as TIAs, transient non-focal cerebrovascular symptoms or deterioration of cerebrovascular symptoms or recurrent infarction, and plus a modified Rankin scale (mRS) ≥2 scores during a follow-up.

  • The term lacuna, or cerebral infarct, refers to a well-defined, subcortical ischemic lesion at the level of a single perforating artery, determined by primary disease of the latter.
  • Lacunar infarcts are small lesions (usually < 5 mm in diameter).
  • It occurs in the distribution of:
  1. Short penetrating arterioles in the basal ganglia,
  2. Circle of Willis.
  3. Pons
  4. Cerebellum
  5. Anterior limb of the internal capsule.
  6. Deep cerebral white matter(rare).
  • Associated with poorly controlled hypertension or diabetes.
  • Symptoms depend on the region of the brain damaged by the lacunar stroke, with different regions of the brain controlling different aspects of the body.
  • Slurring of speech.
  • Inability to raise the arm up over the head.
  • Facial drooping on one side.
  • Numbness on one side of the body.
  • Problems ambulating
  • Confusion and altered awareness
  • Memory difficulties
  • Problems with speaking fluently
  • Persistent headaches
  • Loss of consciousness
  • CT scan or MRI scan in order to get a detailed image of the brain.
  • Doppler ultrasound to measure blood flowing through veins and arteries.
  • Heart function test.
  • Kidney test.
  • Blood tests.
  • Clot-dissolving medication (tissue plasminogen activator) either by mouth or by IV.
  • Aspirin within 48 hours to reduce the chance for additional clotting.
  • Physiotherapy program to improve any ability that was lost or compromised.
  • Physical therapy includes relearning speech, language, and motor skills.

Transient ischemic attack (TIA) is a transient episode of neurologic dysfunction caused due to loss of blood flow to the brain or spinal cord without acute infarction.

  • In an ischemic stroke, a clot blocks the blood supply to part of brain.
  • In a transient ischemic attack, unlike a stroke, the blockage is brief, and there is usually no permanent damage.
  • The underlying cause is a buildup of cholesterol-containing fatty deposits called plaques (atherosclerosis) in an artery or one of its branches that supplies oxygen and nutrients to brain.

 Predisposing factors:

  • Diabetes
  • Hypertension
  • Age
  • Smoking
  • Obesity
  • Alcoholism
  • Unhealthy diet
  • Psychosocial stress
  • Regular physical activity
  • Transient ischemic attacks usually last a few minutes.
  • Most signs and symptoms disappear within an hour.
  • The signs and symptoms of a TIA may include sudden onset of:
    • Weakness, numbness or paralysis in face, arm or leg, typically on one side of body.
    • Slurred or garbled speech or difficulty understanding others.
    • Blindness in one or both eyes or double vision.
    • Dizziness or loss of balance or coordination.
    • Sudden, severe headache with no known cause.
  • Should be directed toward the correction of the immediate pathologic problem (eg, embolism).
  • Control the primary underlying problem (eg, hypertension or coagulopathy) Anticoagulant therapy with either heparin or Coumadin.
  • Aspirin
  • Vascular surgical endarterectomy.
  • Patient is often on anticoagulant therapy.
  • A thorough medical history with an accurate medication with dosages.
  • Refer the patient to physician to obtain current coagulation values (ie, PT, INR).
  • Xerostomia is a side effect of the medications used for management of cerebrovascular disease.
  • Patient susceptible to a higher caries rate.
  • Meticulous oral hygiene, more frequent recalls, saliva substitutes, and fluoride application are preventive measures.
  • Patients with hemiplegia or hemiparesis need additional help in home care. Weakness in the muscles of the orofacial area may have poor control of oral secretions, a reduced gag reflex, and changes in their ability to masticate, leading to poor nutrition.
  • Patients with apraxia of orofacial region may have impaired voluntary movements, such as protruding the tongue, expectorating, and lip puckering.
  • Dental treatment is not contraindicated for most post-stroke patients.
  • Following points should be kept in the mind:
  1. Careful history taking.
  2. Checking of blood pressure prior to treatment.
  3. Avoidance of lengthy appointments.
  4. General reassurance.
  • Cavernous sinus is a dural venous sinus situated lateral to the sella turcica, between two layers of cranial dura in the middle cranial fossa.
  • Structures present within the cavernous sinus include the third, fourth, and sixth cranial nerves; ophthalmic and maxillary divisions of the fifth cranial nerve; and internal carotid artery with its surrounding sympathetic plexus.

The cavernous sinus has been coined the term “anatomic jewel box” because it shares an intimate relationship with several important structures.

Cavernous sinus thrombosis (CST) was first described by Bright in 1831 as a complication of epidural and subdural contamination.

CST may be septic or aseptic.

  1. Septic cases follow central facial infections, especially within the danger triangle of the face (from the corners of the mouth to the bridge of the nose). These include:
  • Abscess or cellulitis.
  • Sinusitis (especially sphenoiditis and ethmoiditis).
  • Dental infections.
  • Extractions or procedures (even a posterior superior alveolar nerve block entering the pterygoid plexus).
  • Maxillofacial surgery.
  • Otitis media.
  • Mastoiditis


  1. Aseptic causes are trauma, surgery, or pregnancy.

Infectious organisms: 

  • Staphylococcus aureus. 
  • Streptococcus species.
  • Pneumococcus (5%).
  • Gram-negative species such as Proteus, Hemophilus, Pseudomonas, Fusobacterium, Bacteroides.
  • Gram-positive species such as Corynebacterium and Actinomyces.

Risk factors:

  • Uncontrolled diabetes.
  • Steroid use.
  • Cancer
  • Chemotherapy
  • Thrombophilia
  • Women who are pregnant, post-partum, or receiving oral contraceptives or hormone replacement therapy.

CST leads to decreased drainage from the facial vein and superior and inferior ophthalmic veins resulting in:

  • Facial and periorbital edema.
  • Ptosis
  • Proptosis
  • Chemosis
  • Discomfort
  • Pain with eye muscle movement.
  • Papilledema
  • Retinal venous distention.
  • Loss of vision.

Communication between the right and left cavernous sinuses via the inter-cavernous sinuses, anterior and posterior to the sella, allows spread of thrombus and infection from one side to the other.

Eagleton’s six criteria for establishment of diagnosis:

  • A known site of infection.
  • An infection of the blood stream.
  • Early signs of venous obstruction.
  • Involvement of nerves in the sinus.
  • A neighborhood abscess of the soft parts.
  • Symptoms of complicating disease.

Carotid angiography, MRI, and CT can visualize the narrowing and/or obstruction of the carotid artery.


  • CT scan:
  1. CST visualized as an area of increased density.
  2. Exophthalmos
  3. Soft tissue edema.
  4. Dilation of the superior ophthalmic vein.
  • Magnetic resonance venography shows the absence of venous flow in the affected cavernous sinus.
  • CT venogram(CTV) and enhanced-MRV can detect dilation of the cavernous sinus, enhancement, and convexity of the lateral wall (which is normally concave) on coronal views, heterogeneous and asymmetric filling defects after contrast, increased density of orbital fat, thrombosis in the superior ophthalmic vein or veins and tributaries leading to the cavernous sinus.
  • Antibiotics
  • Maintaining the partial thromboplastin time or thrombin clot time at 1.5 to 2 times that of the control.
  • Intravenous heparin can be given at a dose greater than 24,000–30,000 U/day.
  • Heparin counteracts the conversion of fibrinogen to fibrin and thus inhibits further thrombogenesis.
  • In pituitary insufficiency, corticosteroids indicated (lessens inflammation, edema and prevent residual cranial nerve dysfunction)
  • It is a chronic autoimmune, inflammatory neurological disease of the central nervous system (CNS).
  • MS attacks the myelinated axons in the CNS, destroying the myelin and the axons to varying degrees.


Disease course

Relapsing/Remitting Multiple Sclerosis (RRMS)

Most common type, accounts for approximate 85% of cases. Characterized by discrete attacks that evolve over days to weeks followed by some decree of recovery over weeks to months. In between attacks, the patient has no worsening neurological function.

Secondary Progressive Multiple Sclerosis (SPMS)

Characterized by initial relapses, followed by gradual neurological deterioration not associated with acute attacks.

Primary Progressive Multiple Sclerosis (PPMS)

Characterized by steady functional decline from the onset of the disease. No relapses ever.

Progressive Relapsing Multiple Sclerosis (PRMS)

Characterized by steady functional decline from onset of the disease with later superimposed acute attacks. PRMS and PPMS cannot be distinguished during early stages, until the relapses occur

  • Most common in northern Europe, Canada, and New Zealand.
  • Etiology is unknown.
  • MS is a T-cell mediated autoimmune attack on the central nervous system. Concomitant with the myelin destruction, there is also damage to the underlying axon, which leads to further disability.


  1. Measles virus.
  2. Rubella virus.
  3. Mumps virus.
  4. Epstein-Barr virus.
  5. Herpes simplex viruses 1 and 2.
  6. Human herpesvirus 6 (HHV-6).

First symptoms:

  • Vision problems such as blurred or double vision or optic neuritis, which causes pain in the eye and a rapid loss of vision.
  • Weak, stiff muscles, often with painful muscle spasms.
  • Tingling or numbness in the arms, legs, trunk of the body, or face.
  • Clumsiness, particularly difficulty in staying balanced while walking.
  • Bladder control problems, either inability to control the bladder or urgency dizziness that doesn’t go away.

Later symptoms:

  • Mental or physical fatigue which accompanies the above symptoms during an attack.
  • Mood changes such as depression or euphoria.
  • Changes in the ability to concentrate or to multitask effectively.
  • Difficulty making decisions, planning, or prioritizing at work or in private life.
  • Found in MS.
  • Characterized by rapid vision loss following a body temperature increase that is associated with strenuous exercise.
  • Sign of ocular disturbance in MS
  • Elicited in patients with unilateral optic neuritis in the following manner: A bright light is shown into each eye separately; when this light is moved from the normal to the affected eye, the pupil of the latter dilates rather than constricts.
  • Spinal fluid protein and white blood cell counts are occasionally mildly elevated.
  • Increases immunoglobulin G (IgG) level and synthesis rate.
  • Immunoglobulins in the spinal fluid, presumably reflecting the underlying autoimmune activation, appear as distinct oligoclonal bands on CSF electrophoresis.

Type of Multiple Sclerosis


Non-symptomatic Multiple Sclerosis

Interferon beta -1a and 1b, glatiramer acetate, intravenous immunoglobulin

Relapsing Remitting Multiple Sclerosis and Secondary Progressive Multiple Sclerosis

Interferon beta-1a and 1b, glatiramer acetate, natalizumab, intravenous immunoglobulin, pulse methylprednisolone (for acute relapses). 
If poor response: mitoxantrone, cyclosphosphamide, mycophenolate, azathioprine, methotrexate, methylprednisolone, intravenous immunoglobulin, cyclosporine.

Primary Progressive Multiple Sclerosis and Progressive Relapsing Multiple Sclerosis

No specific treatment has been shown to be effective. 
Ineffective agents: interferon beta, mitoxantrone, rituximab

Clinical manifestations of MS affect the orofacial region:

  • Trigeminal neuralgia (2%)
  • Sensory neuropathy of the trigeminal nerve.
  • Facial palsy.

When MS is associated with TGN, it appears:

  • Earlier age of onset
  • Symptoms are commonly bilateral.
  • Pain is normally severe and lancinating, but trigger zones may be absent.
  • Pain often becomes less severe but more continuous.

Treatment includes: Carbamazepine, baclofen, gabapentin, or phenytoin, thermocoagulation or alcohol injection.

Sensory neuropathy:

  • Affects the second and third divisions of the trigeminal nerve.
  • Sudden onset and is painful.
  • Numbness of lower lip and chin (due to neuropathy of mental nerve)
  • Facial paralysis: occurs in later stages.
  • ALS was first described in 1869 by French neurologist Jean-Martin Charcot.
  • Also known as Lou Gehrig disease.
  • It is a fatal motor neuron disorder that is, characterized by progressive loss of the upper and lower motor neurons (LMNs) at the spinal or bulbar level.

ALS is categorized in two forms:

  1. Sporadic (90–95%):
  • Most common form.
  • It has no obvious genetically inherited component.
  1. Familial-type ALS (FALS):
  • 5–10% of the cases are familial-type.
  • Associated with genetic dominant inheritance factor.
  • Etiology is unknown
  • Exposure to lead, pesticides, and other environmental toxins.
  1. Sporadic ALS

The majority of the cases are considered sporadic, means the disease seems to occur randomly with no clear associated risk factors and no family history of the disease.

  1. Familial (Genetic) ALS
  • Means an individual inherits the disease from his or her parents.
  • Mutations in more than a dozen genes occurs in familial ALS.

Most of the cases are caused by a defect in a gene known as “chromosome 9 open reading frame 72,” or C9ORF72. Same mutation can be associated with atrophy of frontal-temporal lobes of the brain causing frontal-temporal lobe dementia. 

  1. UMN signs:
  • Hyperreflexia
  • Extensor plantar response.
  • Increased muscle tone.
  • Weakness in a topographic representation.
  1. LMN signs:
  • Weakness
  • Muscle wasting.
  • Hyporeflexia
  • Muscle cramps.
  • Fasciculations
  1. Orofacial region:
  • Difficulties in mastication (dysphagia) and speech(dysarthria).
  • Dysfunction of the temporomandibular joint.
  • Development of malocclusion.

When symptoms begin in the arms or legs, it is referred to as “limb onset” ALS. 

Other individuals first notice speech or swallowing problems, termed “bulbar onset” ALS.

  • Till date there no single test for definitive diagnosis of ALS.
  • Diagnosis is based on detailed history of the signs and symptoms

Muscle and imaging tests

  • Electromyography (EMG), a special recording technique that detects electrical activity of muscle fibers.
  • Nerve conduction study (NCS): Measures electrical activity of the nerves and muscles by assessing the nerve’s ability to send a signal along the nerve or to the muscle.
  • Magnetic Resonance Imaging (MRI): For spinal cord tumor, a herniated disk in the neck that compresses the spinal cord, syringomyelia (a cyst in the spinal cord), or cervical spondylosis (abnormal wear affecting the spine in the neck).

Tests for other diseases and disorders

  • Infectious diseases such as human immunodeficiency virus (HIV), human T-cell leukemia virus (HTLV), polio, and West Nile virus.

Multiple sclerosis, post-polio syndrome, multifocal motor neuropathy, and spinal and bulbar muscular atrophy (Kennedy’s disease)


  • Modulates glutamatergic activity suppressing excitotoxicity
  • 100 mg/day in 2 dosages of 50 mg


  • Carbamazepine
  • Phenytoin
  • Quinine

Other treatments:

  • Physiotherapy
  • Physical exercise
  • Massage
  • Hydrotherapy


  • Baclofen
  • Tizanidine
  • Dantrolene
  • Botulinum toxin type A

Other treatments

  • Physiotherapy
  • Hydrotherapy
  • Cryotherapy


Excessive watery saliva

  • Atropine
  • Hyoscine hydrobromide
  • Hyoscine butylbromide
  • Hyoscine scopoderm

Other treatments

  • Home suction device
  • Dark grape juice
  • Nebulisation
  • Steam inhalation

Emotional lability        

  • Tricyclic antidepressant
  • Selective serotonin-reuptake inhibitors
  • Levodopa
  • Dextrometorphan and quinidine

Communication difficulties           

  • Speaking techniques
  • Low-tech augmentative and alternative communication tools
  • Voice amplifiers
  • Light writers


  • Amitriptyline
  • Psychological support, counselling
  • Citalopram


  • Modafinil

Dental Treatment Modifications in ALS

ALS symptom

Treatment Modification

Advanced disease

Care provider to learn oral home care


Semi-supine treatment position


Nutritional counseling

Excessive saliva

Additional practitioner to assist with HVE(high volume evacuation)

Muscle weakness/spasticity

Shorter appointment time or frequent breaks during appointment

Muscle weakness/spasticity

Bite block/assistance with holding mouth open

  • Decreased muscular activity of the tongue and the perioral musculature may lead to poor oral hygiene.
  • Mechanical toothbrushes, fluoride rinses, and more frequent periodontal recall may be beneficial.

Parkinson’s disease was first described by Dr. James Parkinson in 1817 as a “shaking palsy.”

It is a chronic, progressive neurodegenerative disease characterized by both motor and non-motor features.

Numerous risk factors and genetic mutations are associated with PD.

Risk factors:

  1. Elevated cholesterol
  2. Environmental toxins
  • Carbon disulfide
  • Cyanide
  • Herbicides
  • Methanol and organic solvents
  • Pesticides
  1. Head trauma
  2. High caloric intake
  3. Increased body mass index
  4. Inflammation associated with activation of microglia
  5. Methcathinone (manganese content)
  6. Methamphetamine/amphetamine abuse
  7. Mitochondrial dysfunction
  8. Nitric oxide toxicity
  9. Oxidative stress:
  • Formation of free radicals (e.g., hydrogen peroxide)
  • Potent neurotoxins (e.g., 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine)
  1. Post-infection states
  2. Signal-mediated apoptosis
  • Alpha-synuclein gene (SNCA)
  • Eukaryotic translation initiation factor 4 gamma 1 gene (EIF4G1)
  • Glucocerebrosidase gene (GBA)
  • Leucine-rich repeat kinase 2 (LRRK2) gene loci
  • PTEN-induced putative kinase 1 (PINK1) gene loci
  • Superoxide dismutase 2 gene (SOD2)
  • Vacuolar protein sorting 35 homolog gene (VPS35)
  • Parkinson disease is a neurodegenerative syndrome involving multiple motor and non-motor neural circuits.
  • It is characterized by two major pathologic processes:

(a) Premature selective loss of dopamine neurons.

(b) The accumulation of Lewy bodies, composed of α-synuclein, which become  misfolded and accumulate in multiple systems of patients with PD.

Affect the age group 55–75 years.

  1. Cardinal Motor Features (“Classical Triad”)
  • Bradykinesia
  1. Occurs in 80% to 90% of patients
  2. Slowness of movement
  3. Decreased amplitude of movement
  • Rigidity
  1. Occurs in 80% to 90% of patients
  2. Resistance to passive movement in both flexor and extensor muscles with limb relaxed.
  3. Often accompanied by “cogwheel” phenomenon
  • Tremor at rest
  1. Common initial symptom (70% to 90% of patients).
  2. Often resolves with action or during sleep.
  3. Primarily distal, involving hands.
  4. May also involve jaw, tongue, lips, chin, or legs.


  • Postural instability
  1. Predisposes patients to falls and injuries
  2. Occurs in later stages of Parkinson’s disease
  3. Results from loss of postural reflexes
  • Dysarthria
  • Dystonia

Autonomic Dysfunction

  • Constipation (parasympathetic nervous system cholinergic)
  • Orthostatic hypotension (sympathetic nervous system noradrenergic)
  • Sexual dysfunction (parasympathetic nervous system cholinergic)
  • Sweating (sympathetic nervous system cholinergic)
  • Urinary retention (parasympathetic nervous system cholinergic)

Neuropsychiatric Symptoms

  • Anxiety
  • Cognitive impairment (mild)
  • Dementia
  • Depression (e.g., dysphoria, suicidal ideation, apathy)
  • Impulse-control disorders (e.g., pre-occupations, hypersexuality, compulsive shopping, binge eating).
  • Panic disorder
  • Psychosis (e.g., hallucinations, delusions)

Sensory Symptoms

  • Olfactory dysfunction (hyposmia).
  • Paresthesias
  • Pain

Sleep Disturbance

  • Daytime somnolence.
  • Insomnia
  • Rapid eye movement disorder.
  • Restless legs syndrome.
  • Sleep attacks.
  • Sleep apnea.


  • Fatigue
  • Sialorrhea
  • Weight loss.

The typical hand tremor is often called a “pill-rolling” movement, characterized by the rubbing of the thumb against the fingers, and is particularly pronounced when the patient is otherwise at rest.

The typical “masklike” facial appearance with infrequent blinking and lack of expression is caused by bradykinesis.

There are four cardinal features of PD that can be grouped under the acronym TRAP:

  • Tremor at rest.
  • Rigidity
  • Akinesia (or bradykinesia).
  • Postural instability.

Degenerative disorders

  • Multiple system atrophy
  • Progressive supranuclear palsy
  • Corticobasal degeneration
  • Dementia with Lewy bodies
  • Alzheimer’s disease

 Non-degenerative disorders

  • Essential tremor
  • Dystonic tremor
  • Cerebrovascular disease
  • Drug-induced parkinsonism

Autosomal-dominant (AD) PD:

  • PARK1/PARK4 (gene SNCA, α-synuclein).
  • PARK3 (gene unknown).
  • PARK5 (gene UCHL1, ubiquitin carboxy-terminal hydrolase 1).
  • PARK8 (gene LRRK2, leucine-rich repeat kinase 2 or dardarin).

Autosomal recessive (AR) PD:

  • PARK2 (gene Parkin)
  • PARK6 (gene PINK1, PTEN-induced putative kinase 1)
  • PARK7 (gene DJ-1)
  • Parkinson disease is a clinical diagnosis
  • Following neuroimaging modalities differentiates idiopathic Parkinson disease from other parkinsonian disorders:
  1. Magnetic resonance imaging (MRI).
  2. Transcranial Doppler ultrasonography.
  3. Positron emission tomography (PET).
  4. Single-photon emission computed tomography (SPECT).
  5. Morphometric MRI studies.
  6. Tractography
  7. Functional MRI.
  8. Perfusion imaging.
  • According to National Institute of Neurological Disorders and Stroke (NINDS) diagnostic criteria for Parkinson’s disease (PD) is as follows:

Group A features (characteristic of PD)

  1. Resting tremor.
  2. Bradykinesia
  3. Rigidity
  4. Asymmetric onset.

Group B features (suggestive of alternative diagnoses)

  1. Features unusual early in the clinical course.
  2. Prominent postural instability in the first 3 years after symptom onset.
  3. Freezing phenomenon in the first 3 years.
  4. Hallucinations unrelated to medications in the first 3 years.
  5. Dementia preceding motor symptoms or in the first year.
  6. Supranuclear gaze palsy (other than restriction of upward gaze) or slowing of vertical saccades, severe, symptomatic dysautonomia unrelated to medications.
  7. Documentation of condition known to produce parkinsonism and plausibly connected to the patient’s symptoms (such as suitably located focal brain lesions or neuroleptic use within the past 6 months).

Criteria for definite PD

  1. All criteria for probable Parkinson’s are met.
  2. Histopathological confirmation of the diagnosis is obtained at autopsy.

Criteria for probable PD

  1. At least three of the four features in group A are present


  1. None of the features in group B is present (note: symptom duration ≥3 years is necessary to meet this requirement)


  1. Substantial and sustained response to levodopa or a dopamine agonist has been documented.

 Criteria for possible PD

  1. At least two of the four features in group A are present; at least one of these is tremor or bradykinesia


  1. Either none of the features in group B is present or symptoms have been present (≤3 years and none of the features in group B is present


  1. Either substantial and sustained response to levodopa or a dopamine agonist has been documented or the patient has not had an adequate trial of levodopa or a dopamine agonist.
  • Dental caries and periodontal disease (Due to general motor impairment and hypokinesia).
  • Sialorrhea and drooling.
  • Xerostomia (due to medication).
  • Orofacial pain.
  • Burning mouth syndrome (depressive symptoms).
  • Mastication disorders (Advanced PD).
  • Bruxism
  • Subjective taste impairment.


Differentiating features


Paucity of vocal inflection and facial movement

Essential tremor

High-frequency tremor with limbs held against gravity, head tremor, improves with alcohol

Normal-pressure hydrocephalus

Apraxic gait, urinary incontinence, and dementia

Wilson’s disease

Early age of onset, Kayser–Fleischer rings, and low serum copper, low ceruloplasmin

Huntington’s disease

Family history, chorea, dementia

Multiple system atrophy

Early urinary incontinence, orthostatic hypotension, and dysarthria

Dementia with Lewy bodies

Early hallucinations, behavioral disturbances

Progressive supranuclear palsy

Early imbalance and falls, downgaze paresis

Treatment of Motor Symptoms of Parkinson Disease

Medication Class



Levodopa-PDDI (Peripheral dopa decarboxylase inhibitor)


Titrate to initial dose of 100/25 mg thrice daily; max, 1500/375 mg/d or more based on symptoms

All motor symptoms


Titrate to initial dose of 100/25 mg thrice daily; max, 1500/375 mg/d or more based on symptoms

All motor symptoms

Dopamine agonists


Start 0.125 mg thrice daily; max, 4.5 mg/d

All motor symptoms

Pramipexole extended release

0.26 mg, 0.52 mg, 1.05 mg, 2.1 mg, or 3.15 mg once daily

All motor symptoms


Start 0.25 mg thrice daily; max, 24 mg/d

All motor symptoms

Ropinirole prolonged release 

6-24 mg once daily

All motor symptoms


Start 2 mg/24 h; max, 16 mg/24 h

All motor symptoms

MAOBIs (monoamine oxidase type B inhibitors)


2.5 mg once daily; max, 5 mg twice daily

Early, mild symptoms, and MF(motor fluctuations)


1 mg once daily

Early, mild symptoms, and MF(motor fluctuations)

COMTIs (catechol-O methyltransferase inhibitors)


200 mg with each dose of levodopa; max, 8/d

MF(motor fluctuations)


100-200 mg thrice daily




Start 100 mg once daily; max, 4 times daily (thrice daily is typical)

Gait dysfunction and dyskinesia



Start 40 mg twice daily; max, 320 mg/d




Start 1 mg once daily; typical maintenance dose 2 mg thrice daily



Start 0.5-1 mg once daily; usual dose 1-2 mg thrice daily




Start 6.25-12.5 mg at bedtime; max, 150 mg/d

Tremor and dyskinesia


Treatment of Non-motor Symptoms of Parkinson Disease

Nonmotor Symptom





10 mg thrice daily; max, 20 mg 4 times daily

RBD (Rapid eye movement sleep behavior disorder)


0.25-2 mg at bedtime


3-15 mg at bedtime



10-20 mg once daily


10-50 mg once daily


20-40 mg once daily


25-150 mg/d single or divided



6.25-150 mg at bedtime or divided (often effective in very low doses)


12.5-400 mg at bedtime or divided


1.5-6 mg twice daily; transdermal patch, 4.5-9.8 mg/24 h

Parkinson disease mild cognitive impairment 


Target dose, 80 mg once daily

Parkinson disease dementia


1.5-6 mg twice daily; transdermal patch, 4.5-9.8 mg/24 h


5-10 mg once daily


4-12 mg twice daily

Orthostatic Hypotension


2.5-10 mg thrice daily


50 mg thrice daily


300 mg thrice daily



1 mg thrice daily


1-2 drops of 1% concentration up to 4 times daily

Ipratropium bromide

1-2 sprays (21 μg); max, 4 times daily

  • Surgery
  • Deep Brain Stimulation.
  • Patients must be treated in a relatively upright position, making complex dental procedures in the maxillary arch or posterior oral cavity a challenge.
  • Resting tremors and drug-related dyskinesia can complicate procedures, and behavioral techniques to reduce anxiety as well as gentle cradling techniques can help.
  • Dysphagia and impaired gag reflex increase the risk for aspiration of oral and irrigation fluids, so high-speed evacuation of fluids is important.
  • Patients with sialorrhea, making maintenance of a dry field difficult for some operative and surgical procedures. Chewing gum may modify certain swallowing parameters and reduce drooling.
  • Schedule the patient with PD 60 to 90 min after their medications have been taken, as medications tend to be most effective in that time period.
  • Careful consideration and management include monitoring of blood pressure; correct positioning and repositioning during and after treatment.
  • Xerostomia and caries risk reduction through hygiene, sealants, and fluorides.
  • It is a rare neurodegenerative disorder of the central nervous system characterized by unwanted choreatic movements, behavioral and psychiatric disturbances and dementia. 
  • First described by Waters in 1842.
  • Autosomal dominantly inherited disease.
  • Caused by an elongated CAG repeat on the short arm of chromosome 4p16.3 in the Huntingtin gene.
  • This gene codes for the huntingtin protein and, on exon 1, contains the CAG tract.
  • The wild-type contains a CAG repeat, coding for a polyglutamine stretch in the protein at that site in the range 6 to 26.
  • Huntington’s disease is associated with 36 repeats or more.
  • Mean age at onset is between 30 and 50 years (range 2 to 85 years).
  • The mean duration of the disease is 17-20 years
  • The nuclear symptoms and signs of Huntington’s disease (HD) consist of:
  1. Motor disturbances. 
  2. Cognitive disturbances. 
  3. psychiatric disturbances. 

The motor symptoms and signs

  • Involuntary, unwanted movements occur in the distal extremities (fingers and toes) but also in small facial muscles.
  • Unwanted movements spread to all other muscles from distal to more proximal and axial.
  • Choreatic movements are present all the time the patient is awake.
  • Facial choreatic movements can lead to a continuous movement of facial muscles where for instance an eyebrow is lifted, an eye closed, the head is bent or turned while the tongue is protruded with the lips pouting.
  • Dysarthria
  • Dysphagia
  • Hypokinesia
  • Akinesia
  • Rigidity leading to a slower pace of all activities.
  • Dystonia
  • Walking is often described as ‘drunk’ or ‘cerebellar ataxia’-like.
  • Daily activities become more and more difficult.

Behaviour and psychiatric symptoms and signs

  • Present in the early stage of the disease
  • Depression
  • Low self-esteem.
  • Feelings of guilt.
  • Anxiety
  • Irritability and aggression.
  • Suicide occurs more frequently in early symptomatic individuals. 
  • Pychosis


  • Cognitive decline is other main sign.
  • Can be present long before the first motor symptoms appear.
  • Patients are no longer able to organise their life or to plan things.
  • Loss of flexibility of mind
  • Language is relatively spared.
  • Memory becomes impaired
  • If the first symptoms and signs start before the age of 20 years, the disease is called Juvenile Huntington’s disease (JHD).
  • Behaviour disturbances and learning difficulties at school are often the first signs.
  • Motor behaviour is often hypokinetic and bradykinetic with dystonic components.
  • Chorea is seldom seen in the first decade.
  • Epileptic fits are frequently seen. 
  • Based on the clinical symptoms and signs.
  • DNA determination: shows a CAG-repeat of at least 36 on the huntingtin gene on chromosome 4.
  • In most cases a combination of the three main signs is present.
  • The combination with the family history is sufficient for diagnosis.
  • No imaging, general blood tests or other diagnostic tools are helpful.
  • Changes in brain imaging (MRI): Brain volume and brain connections show changes several years before any clinical manifestation is present.
  1. Hyperkinesia, or chorea, is treated with dopamine receptor blocking or depleting agents:
  • Tiapride: max 600 mg
  • Olanzapine: max 20 mg
  • Pimozide: max 6 mg
  • Risperidone: max 16 mg
  • Fluphenazine: max 10 mg
  1. Drug Treatment for depression
  • Citalopram: max 60 mg
  • Fluoxetine: max 60 mg
  • Mirtazapine: max 45 mg
  • Valproic acid: max 2000 mg
  • Carbamazepine: max 1600 mg
  1. Drug Treatment for aggression
  • Citalopram: max 60 mg
  • Sertraline: max 200 mg
  • Olanzapine :max 20 mg
  • Dipiperon: max 360 mg
  1. During the course of the disease, the patient requires more care, which can also help his/her partner.
  2. Dysphagia and choreic movement of the face and tongue make dental treatment difficult.
  3. Sedation with diazepam may be useful.
  4. Dentures should be avoided because of the danger of fracture or the accidental swallowing of the dentures.
  • It is a condition caused by damage to the brain, usually occurring before, during or shortly after birth.
  • ‘Cerebral’ refers to the brain and ‘palsy’ refers to a disorder of movement or posture.
  • Cerebral palsy is a central nervous system (CNS) disorder of movement, coordination, and posture, reflecting a nonprogressive abnormality or insult to the immature brain.
  • William John Little gave the first description of CP in 1843, he coined the term ‘cerebral palsy’ in 1889.

Condition or risk factors associated with CP:

  • Prenatal time.
  • Perinatal time.
  • Postnatal time period.

The prenatal risk factors associated with CP are:

  • Hypoxia
  • Genetic
  • Metabolic disorders.
  • Multiple gestation.
  • Intrauterine infections.
  • Thrombophilic disorders.
  • Teratogenic exposure.
  • Chorioamnionitis
  • Maternal fever.
  • Exposure to toxins.
  • Malformations of brain structures.
  • Intrauterine growth restriction.
  • Abdominal trauma and vascular insults.

Perinatal risk factors associated with CP are:

  • Asphyxia
  • Premature birth (<32 weeks or <2500 gm).
  • Blood incompatibility.
  • Infection
  • Abnormal fetal presentation.
  • Placental abruption.
  • Instrument delivery.

The postnatal risk factors:

  • Asphyxia
  • Seizures in postnatal period.
  • Cerebral infarction.
  • Hyperbilirubinemia
  • Sepsis
  • Respiratory distress syndrome.
  • Chronic lung disease.
  • Meningitis
  • Postnatal steroids.
  • Intraventricular hemorrhage.
  • Periventricular leukomalacia.
  • Shaken baby syndrome.
  • Head injury.
  • Cerebral palsy is classified according to the Gross Motor Function Classification System (GMFCS).

The system focuses on:

  • The ability to sit.
  • The capability for movement and mobility.
  • Charting independence.
  • The use of adaptive technology.

The five levels of the GMFCS increase with decreasing mobility:

Level 1 cerebral palsy

  • Level 1 CP is characterized by being able to walk without limitations.

Level 2 cerebral palsy

  • A person can walk long distances without limitations, but they can’t run or jump.
  • They may need assistive devices, such as leg and arm braces, when they first learn to walk. They also may need to use a wheelchairto get around outside of their home.

Level 3 cerebral palsy

  • A person with level 3 CP can sit with little support and stand without any support.
  • They need handheld assistive devices, such as a walker or cane, while walking indoors. They also need a wheelchair to get around outside of the home.

Level 4 cerebral palsy

  • A person with level 4 CP can walk with the use of assistive devices.
  • They’re able to move independently in a wheelchair, and they need some support when they’re sitting.

Level 5 cerebral palsy

  • A person with level 5 CP needs support to maintain their head and neck position.
  • They need support to sit and stand, and they may be able to control a motorized wheelchair.
  • Majority of children with cerebral palsy are born with it, although it may not be detected until months or years later. 
  • Early signs usually appear before a child reaches 3 years of age.

The most common are:

  • Lack of muscle coordination when performing voluntary movements (ataxia)
  • Stiff or tight muscles and exaggerated reflexes (spasticity)
  • Walking with one foot or leg dragging
  • Walking on the toes, a crouched gait, or a “scissored” gait.
  • Muscle tone that is either too stiff or too floppy. 

Neurological symptoms:

  • Seizures
  • Hearing loss.
  • Impaired vision.
  • Bladder
  • Bowel control issues.
  • Pain and abnormal sensations.
  • Bacterial meningitis
  • Viral encephalitis.
  • Head injury from a motor vehicle accident, a fall, or child abuse.
  • Poor oral hygiene. 
  • Dental Erosion.
  • Enamel Defects.
  • TMJ Disorders.
  • Sialorrhea
  • Dental caries.
  • Malocclusion
  • Angles Class II with increased overjet and overbite.
  • Open bite.
  • Hypotonia of the orofacial muscles with resultant forward tongue posture
  • Poor swallow reflex.
  • Frequent mouth breathing.

Traumatic Dental Injuries

Factors predispose the individual to dental trauma are:

  1. Class II malocclusion with prominent maxillary incisors.
  2. Incompetent lips.
  3. Difficulty in ambulation.
  4. Increased incidence of seizures.


  • Habitual grinding of teeth is common.
  • Bruxism leads to tooth abrasion and fat biting surfaces.

Other habits: Pacifier-sucking, finger sucking, habit of biting objects and tongue inter-positioning is also common.

  • Diagnosis of CP is not always straightforward.
  • It is not possible to diagnose CP in infants less than 6 months.
  • The major signs that collectively can lead to a CP diagnosis are:
  1. Delayed motor milestones.
  2. Abnormal neurological examination.
  3. Persistence of primitive reflexes.
  4. Abnormal postural actions.
  • Diagnosis of CP may not be possible till 12 months.
  • Diagnosis often is easier if there is known brain damage documented by:
  1. Cranial ultrasound.
  2. Computed tomography (CT).
  3. Magnetic resonance imaging (MRI).
  4. Genetic and metabolic tests.
  5. Tests to rule out coagulopathy.
  • Complete evaluation of a child with CP should include:
  1. Assessment of associated deficits like vision, speech and hearing, sensory profile, oromotor evaluation, epilepsy and cognitive functioning.
  2. Orthopedic evaluation is must as muscle imbalance and spasticity cause subluxation/ dislocation of the hips, equinus deformities, contractures and scoliosis.
  1. Apprehension: Many of these children are not used to meet strangers.
  2. Difficulty of communication: If there is an auditory, visual or speech defect, chairside communication must be modified accordingly.
  3. Low intelligence: This can contribute to difficulty of cooperation.
  4. Poor concentration: This may be an inherent aspect of the cerebral dysfunction, trivial things distracting the attention.
  5. Convulsions: These are not common in the dental surgery as the child will be receiving drugs to control such episodes.
  6. Posture: Ataxic patients need to have the dental chair tipped well back to give stability and support, while the spastic and athetoid may need more manual support and control in the chair.
  7. Ability to cooperate: If the patient can sit in the chair and open his/her mouth, he/she can be treated as a normal patient. Those with less physical control need further help.

First dental visit:

The first visit or visits must be used primarily for the dentist and patient to become acquainted with each other and for the establishment of mutual confidence.

Radiographic examination:

  • Occasionally, assistance from the parent and dental auxiliaries.
  • Use of immobilization devices to obtain the films.
  • An 18″ (46 cm) length of floss is attached through a hole made in the tab, to facilitate retrieval of the film if it falls towards the pharynx.

Patient positioning:

Assistive stabilization and postural maintenance can be achieved through the following techniques:

  • Head position maintained in the midline by one of the dental staff over a head support (position device) located at the occipital level.
  • Maintenance of bent and juxtaposed upper members in the midline, with the help of Velcro straps.
  • Maintenance of bent lower members decreasing the hip angle to 120° in relation to the trunk using soft foam rolls as positioning devices, such as for support under the knees.
  • Maintenance of an open mouth with the use of mouth props.

Some of the positions most commonly used for children requiring oral care assistance are as follows:

  • The standing or sitting child is placed in front of the adult so that the adult can cradle the child’s head with one hand while using the other hand to brush the teeth.
  • The child reclines on a sofa or bed with the head angled backward on the parent’s lap. Again, the child’s head is stabilized with one hand, while the teeth are brushed with the other hand.
  • The parents face each other with their knees touching. The child’s buttocks are placed on one parent’s lap, with the child facing that parent, while the child’s head and shoulders lie on the other parent’s knees; this allows the first parent to brush the teeth.
  • The extremely difficult patient is isolated in an open area and reclined in the brusher’s lap. The patient is then immobilized by an extra attendant while the brusher institutes proper oral care. If a child cannot be adequately immobilized by one person, then both parents and perhaps siblings may be required to complete the home dental care procedures.
  • The standing and resistive child are placed in front of the caregiver so that the adult can wrap his or her legs around the child to support the torso while using the hands to support the head and brush the teeth.
  • Proper perinatal care, both anoxia and perinatal infections.
  • Management of CP is teamwork and a planned approach to the individual child’s problem.
  • Many children with CP have normal intelligence and should not be penalized because of dysarthria or involuntary movements.
  • Physiotherapy to prevent contractures and orthopedic surgery.
  • For seizures, appropriate drug should be given.

Babinski sign is elicited by gentle stroking the lateral sole of the foot with a slightly blunted object (eg., cotton-tip swab) and drawing the stimulus slightly medially across the metatarsal area.

A positive response has two components:

  • Dorsiflexion of the great toe, and slight abduction (fanning) of the remaining toes.
  • A positive sign represents disinhibition of the normal spinal reflex because of damage to descending inhibitory pathways from the brain or spinal cord.

A positive babinski sign however is normal in neonates because of immaturity in the myelination of these pathways.

Can be seen in:

  1. Upper motor neuron lesion.
  2. Cerebral palsy.
  3. Strokes
  4. Brain injury or brain tumors.
  5. Spinal cord tumor or injury.
  6. Multiple sclerosis (MS).
  7. Meningitis.
  • Bell’s palsy, named after the Scottish anatomist, Sir Charles Bell.
  • Bell’s palsy is the most common peripheral paralysis of the seventh cranial nerve with an onset that is rapid and unilateral.
  • Most common cause of acute facial paralysis.
  • Caused by inflammation of the facial nerve at the geniculate ganglion.
  • Due to the narrow opening of the facial canal, inflammation causes compression and ischemia of the nerve. 
  • Viral illnesses such as:
  1. Herpes simplex virus.
  2. Varicella-zoster virus.
  3. Epstein-Barr virus.
  • Weakness or paralysis of the upper and lower facial muscles of the affected side.
  • Drooping of ipsilateral eyelids.
  • Inability to close the eye completely.
  • Dry eyes due to inability to close eyes completely.
  • Excessive tearing of the eye (epiphora).
  • Drooping of the corner of the mouth.
  • Ipsilateral impaired/loss of taste sensation.
  • Difficulty with eating due to ipsilateral muscle weakness causing food to be trapped on the affected side of the mouth.
  • Dribbling of saliva.
  • Altered sensation on the affected side of the face.
  • Pain in or behind the ear.
  • Increased sensitivity to sound (hyperacusis) on affected side if stapedius muscle is involved.

On attempted closure of the eyelid, the eye rolls upward that is known as Bell’s phenomenon.

It is a decreased sensation along the medial posterior or superior areas of external acoustic meatus caused by acoustic neuroma that is pressing against facial nerve.

  1. History and physical examination.
  2. The House-Brackmann Facial Nerve Grading System can be used to describe the degree of facial nerve weakness.
  3. Electrodiagnostic test to stimulate the facial nerve to assess the level of facial nerve insult.
  4. Serologic studies to test for infectious causes.
  5. Hearing test to determine if the cochlear nerve or inner ear has been affected.
  6. Vestibular testing to determine if the vestibular nerve is involved.
  7. Schirmer tear testing to measure the eye’s ability to produce tears.
  8. Imaging: Computed tomography (CT) or magnetic resonance imaging (MRI) to identify infection inflammation, tumor, fractures, or other potential causes for facial nerve involvement.
  1. Grade I – Normal
  • Normal facial function in all areas
  1. Grade II – Slight Dysfunction
  • Gross: Slight weakness noticeable on close inspection; may have very slight synkinesis.
  • At rest: Normal symmetry and tone
  • Motion: Forehead – moderate to good function; Eye – complete closure with minimum effort; Mouth – slight asymmetry.
  1. Grade III – Moderate Dysfunction
  • Gross: obvious but not disfiguring difference between two sides; noticeable but not severe synkinesis, contracture, and/or hemi-facial spasm.
  • At rest: Normal symmetry and tone.
  • Motion: Forehead – slight to moderate movement; Eye – complete closure with effort; Mouth – slightly weak with maximum effort.
  1. Grade IV – Moderate Severe Dysfunction
  • Gross: Obvious weakness and/or disfiguring asymmetry
  • At rest: Normal symmetry and tone
  • Motion: Forehead – none; Eye – incomplete closure; Mouth – asymmetric with maximum effort.
  1. Grade V – Severe Dysfunction
  • Gross: Only barely perceptible motion
  • At rest: Asymmetry
  • Motion: Forehead – none; Eye – Incomplete closure; Mouth – slight movement
  1. Grade VI – Total Paralysis
  • No movement
  1. Bell’s palsy is rapid in onset (<72 hours).
  2. Bell’s palsy is diagnosed when no other medical etiology is identified as a cause of the facial weakness.
  3. Bilateral Bell’s palsy is rare.
  4. Currently, no cause for Bell’s palsy has been identified.
  5. Other conditions may cause facial paralysis, including stroke, brain tumors, tumors of the parotid gland or infratemporal fossa, cancer involving the facial nerve, and systemic and infectious diseases, including zoster, sarcoidosis, and Lyme disease.
  6. Bell’s palsy is typically self-limited.
  7. Bell’s palsy may occur in men, women, and children but is more common in those 15 to 45 years old; those with diabetes, upper respiratory ailments, or compromised immune systems; or during pregnancy
  1. Drug therapy:
  • There is no optimum regimen.
  • In adults 50–60 mg prednisolone daily for 10 days
  • Doses of more than 120 mg/day have been used safely in patients with diabetes
  1. Antiviral drugs
  • Acyclovir (400 mg five times daily for five days)
  • Valaciclovir (1000 mg/day for five days).
  1. Combination therapy

Prednisolone reduced the chances of incomplete recovery but using an antiviral drug had an additional benefit.

  1. Non-drug therapy

Physical therapies:

  • Tailored facial exercises.
  • Acupuncture to affected muscles.
  • Massage
  • Thermotherapy
  • Electrical stimulation.

5. Hyperbaric oxygen may improve time to recovery and the proportion of people who make a full recovery compared with corticosteroids.

6. Facial retraining may improve recovery of facial motor function scores including stiffness and lip mobility, and may reduce the risk of motor synkinesis

Lyme disease, sarcoidosis, meningitis (neoplastic or infectious), brain stem encephalitis, benign intracranial hypertension, leukemia, Melkersson-Rosenthal syndrome

  • Bilateral facial palsy is extremely rare, comprising 0.3% to 2% of all facial paralysis cases.
  • Most common infectious cause is Lyme disease, caused by spirochete Borrelia burgdorferi, whose carrier is a common tick.
  • Guillain-Barre syndrome.
  • Lyme’s disease.
  • Sarcoidosis
  • Brain stem encephalitis.
  • Benign intracranial hypertension.
  • Leukaemia
  • Melkersson-Rosenthal syndrome.
  • Diabetes mellitus.
  • HIV
  • Syphilis
  • Leprosy
  • Infectious mononucleosis.
  • Bilateral neurofibromas.
  • Trauma

Diagnostic workup for a patient with bilateral facial paralysis depends greatly on a meticulous history.

Diagnostic blood work should include:

  • Complete blood cell count.
  • Fluorescent treponemal antibody test.
  • Human immunodeficiency virus test.
  • Fasting glucose level.
  • Erythrocyte sedimentation rate.
  • Lyme (Borrelia) IgM.
  • Epstein-Barr virus capsid Ag IgM antibody tests.
  • Antinuclear antibody level.
  • Traumatic skull fractures and cerebello-pontine angle tumors were excluded by CT and MRI of the brain.
  • Hilar adenopathy on chest X-ray(for sarcoidosis)
  • A lumbar puncture to evaluate cell counts, as well as bacterial or viral content.
  • Also known as acute idiopathic polyneuropathy, Landry-Guillain-Barré-Strohl syndrome, acute inflammatory demyelinating polyneuropathy (AIDP).
  • Guillain-Barré syndrome (GBS) was first described in 1916.
  • Guillain–Barré syndrome (GBS) is an acute demyelinating polyneuropathy characterized by progressive muscle weakness and areflexia.
  • Etiology is unknown.
  • Autoimmune cause triggered by infection stimulating anti-ganglioside antibodies production.
  • GBS occur 1–3 weeks after an acute infectious process.

The organisms involved:

  • Campylobacter jejuni(diarrhea).
  • Mycoplasma pneumonia
  • Haemophilus influenza.
  • Cytomegalovirus
  • Epstein-Barr virus.
  • Influenza
  • Sensory symptoms in the legs followed by rapidly progressive distal weakness that soon spreads proximally.
  • Lumbar pain.
  • The weakness of GBS is typically “pyramidal in distribution” with ankle dorsiflexion and knee and hip flexion.
  • Weakness in the arms is usually more severe in shoulder abduction and elbow extension.
  • Facial nerve involvement (70%).
  • Bilateral facial weakness.
  • Dysphagia in 40%.
  • Ophthalmoplegia, ptosis, or both (5%).

Respiratory involvement:

  • May be sudden, vital capacity falls steadily and intubation and ventilation are required at the level of approximately 1 litre.
  • Papilloedema
  • Bradyarrhythmias
  • Affects the feet or hands first, before spreading to other parts of the body.
  • Elevation of creatine kinase /or transaminases.
  • Marked vomiting, delayed hair loss or Mee’s lines may support the need for heavy metal testing.
  • CSF analysis reveals albuminocytologic dissociation; that is an elevated protein up to 1,800 mg/dl.
  • Half of GBS cases may have a normal CSF protein in the first week but that proportion declines to 10% if the test is repeated a week later.
  • Pleocytosis of 10–20 cells/mm3, if there are more than 50 cell/per mm3 particularly two weeks of after the onset of symptoms early HIV infection, leptomeningeal carcinomatosis, CMV poly-radiculitis and sarcoidosis should be considered.

Nerve tests

Two tests:

  1. Electromyography (EMG) – Tiny needles are inserted into the muscles and electrical recordings are taken to see how they react when nearby nerves are activated.
  2. Nerve conduction studies – Small discs (electrodes) are stuck on skin and minor electric shocks are used to activate the nerves and measure how quickly these signals travel along them.

In people with Guillain-Barré syndrome, these tests will usually show that signals aren’t travelling along the nerves properly.

  • Marked radicular back pain or neuropathic pain refractory to acetaminophen or NSAIDS, treatment with pain modulating drugs such as antidepressants, gabapentin, pregabalin, carbamazepine, tramadol and mexiletine is indicated.
  • Treatment with plasmapheresis or IVIG is indicated for patients with weakness impairing function or any respiratory involvement.

Intravenous immunoglobulin (IVIG)

  • IVIG is a treatment made from donated blood that contains healthy antibodies. These are given to help stop the harmful antibodies damaging nerves.
  • IVIG is given directly into a vein. Most people need treatment once a day for around five days.

Plasma exchange (plasmapheresis)

  • A plasma exchange, also called plasmapheresis, is sometimes used instead of IVIG.
  • This involves being attached to a machine that removes blood from a vein and filters out the harmful antibodies that are attacking nerves before returning the blood to body.
  • Most people need treatment every other day for a week or two.

Myasthenia gravis (MG) is an autoimmune antibody-mediated disorder of neuromuscular synaptic transmission.

  • Caused by autoimmune attack against components of the neuromuscular junction (NMJ), on the postsynaptic membrane of striated skeletal muscles.
  • Autoimmune response is mediated by antibodies against the acetylcholine receptor (AChR), which reduce the number of functional AChRs through three possible mechanisms:
  1. Direct destruction of the receptors,
  2. Blockade of the acetylcholine binding sites,
  3. Complement-mediated damage

Subtypes of MG are broadly classified as follows:

  1. Early-onset MG: age at onset <50 years. Thymic hyperplasia, usually females.
  2. Late-onset MG: age at onset >50 years. Thymic atrophy, mainly males,
  3. Thymoma-associated MG (10%–15%)
  4. MG with anti-MUSK antibodies.
  5. Ocular MG (oMG): Symptoms only affecting extraocular muscles.
  6. MG with no detectable AChR and muscle-specific tyrosine kinase (MuSK) antibodies.
  • MG patients with Thymoma almost always have detectable AChR antibodies in serum.
  • Thymoma-associated MG may also have additional paraneoplasia-associated antibodies (e.g., anti-voltage-gated K+and Ca++channels, anti-Hu, anti-dihydropyrimidinase-related protein 5, and anti-glutamic acid decarboxylase antibodies
  • Fluctuating fatigability and weakness affecting ocular, bulbar and (proximal) limb skeletal muscle groups.
  • Ocular myasthenia affects outer ocular muscles including the M. levator palpebrae that leads to:
  1. Ptosis
  2. Double vision.
  3. Ptosis and double vision may be transient, fluctuating or progressive during the day.
  • Facial and masticatory muscle weakness leading to dysphagia, dysarthria and expression less face.
  • Changes to phonation with a nasal quality (due to soft palate muscle weakness and impaired lip movement).
  • Proximal limb muscles, diaphragm and neck extensors are also affected.
  • The severity of muscular weakness tends to fluctuate during the day, being less severe in the morning, and gradually worsening as the day progresses, especially after prolonged use of the affected muscles.
  1. Emotional stress.
  2. Systemic illness (especially viral respiratory infections).
  3. Hypothyroidism
  4. Hyperthyroidism
  5. Pregnancy
  6. The menstrual cycle.
  7. Increased body temperature.
  8. Drugs and medications affecting neuromuscular transmission.
  • Severe involvement of the respiratory muscles leads to myasthenic crisis.
  • It is a life-threatening respiratory collapse requiring immediate treatment with mechanical ventilation.
  • Occurs in about 15-20% of patients amongst which 4-8% of cases are fatal.
  • From a clinical viewpoint, demonstration of weakness and fatigability of skeletal muscles, with improvement following rest is by itself diagnostic of MG.
  • Detection of serum AChR antibodies is important.
  1. Tensilon (Edrophonium Chloride) Test
  • Edrophonium chloride is a short-acting acetylcholinesterase inhibitor that prolongs the duration of action of acetylcholine at the NMJ.
  • Edrophonium is administered intravenously and the patient is observed for objective improvement in muscle strength particularly the eyelid ptosis and/or extraocular muscle movement.

2.   Ice Pack Test

  • Considered in patients with ptosis when Edrophonium test is contraindicated.
  • It is performed by placing an ice pack over the eye for 2–5 minutes and assessed by for improvement in ptosis

3.   Electrophysiological Tests

The two principal electrophysiologic tests for the diagnosis of MG:

  • Repetitive nerve stimulation study.
  • Single fiber electromyography.

Repetitive nerve stimulation tests neuromuscular transmission. It is performed by stimulating the nerve supramaximally at 2-3 Hz. A 10% decrement between the first and the fifth evoked muscle action potential is diagnostic for MG.

Single-fiber electromyography (SFEMG) is done by using a special needle electrode that allows identification of action potentials from individual muscle fibers. It allows simultaneous recording of the action potentials of two muscle fibers innervated by the same motor axon. The variability in time of the second action potential relative to the first is called “jitter.” In MG, the jitter will increase because the safety factor of transmission at the neuromuscular junction is reduced. 

4. Patients with gMG (Generalized myasthenia gravis) who are anti-AChR antibody(Acetylcholine receptor) negative should be tested for anti-MuSK antibodies 

5. Chest CT or MRI is done in all patients with confirmed MG to exclude the presence of a thymoma.

  1. Symptomatic treatment with acetylcholinesterase inhibitors.
  2. Rapid short-term immunomodulating treatment with plasmapheresis and intravenous immunoglobulin.
  3. Chronic long-term immunomodulating treatment with glucocorticoids and other immunosuppressive drugs.
  4. Surgical treatment (Thymectomy).

1. Acetylcholinesterase Inhibitors:

  • First-line treatment.
  • Used as a symptomatic therapy
  • Act by increasing the amount of available acetylcholine at the NMJ.
  • Pyridostigmine is used:

a. Has a rapid onset of action within 15 to 30 minutes reaching peak activity in about two hours.

b. Effect lasts for about three to four hours.

c. Initial oral dose is 15–30 mg every 4–6 hours and is titrated upwards depending on the patient’s response.

2. Short-Term Immunomodulating Therapies:

A. Plasma exchange.

B. Intravenous immunoglobulin.

Used in myasthenic crisis and preoperatively before thymectomy or other surgical procedures.


A. Plasmapheresis

Typically one exchange is done every other day for a total of four to six times. Adverse effects: hypotension, paresthesias, infections, thrombotic complications related to venous access, and bleeding tendencies due to decreased coagulation factors.

  1. Intravenous Immunoglobulin Therapy (IVIg):
  • It involves isolating immunoglobulins isolated from pooled human plasma by ethanol cryoprecipitation.
  • Administered for 5 days at a dose of 0.4 g/kg/day, fewer infusions at higher

doses are also used.

  • Mechanism of action of IVIg is complex.
  • Factors include inhibition of cytokines competition with autoantibodies, and inhibition of complement deposition.
  • Interference with the binding of Fc receptor on macrophages, Ig receptor on B cells, and interference with antigen recognition by sensitized T cells are other mechanisms.
  1. Corticosteroids:
  • Prednisone 1 mg/kg/day.
  • Steroid-induced clinical improvement usually begins within 2 to 4 weeks.

4. Nonsteroidal Immunosuppressive Agents:
1. Azathioprine (AZA)

  • It effects T cell replication, with evidence supporting a steroid-sparing effect.
  • The daily dose is 2–3 mg/kg/day.

2. Cyclosporin A (CyA)

  • Cyclosporin A inhibits the production of interleukin (IL)-2 by helper T cells. 
  • The recommended daily dosage is 5 mg/kg/day twice daily.
  1. Mycophenolate mofetil
  • Selectively blocks purine synthesis, thereby suppressing both T-cell and B-cell proliferation.
  • The standard dose used in MG is 1000 mg twice daily, but doses up to 3000 mg daily can be used.
  • Routine dental treatments and minor procedures (root canals, fillings etc.) can be performed safely within the setting of a private dental office.
  • If the patient’s recent medical history is consistent with frequent exacerbations, severe bulbar and respiratory symptoms and generalized weakness, the dentist should consult the patient’s physician and a neurologist, as additional therapy may be recommended.
  • Prior to treatment, multiple and short early morning appointments are preferable, in order to avoid cumulative muscle weakness,
  • Oral anticholinesterase drugs should be preferably administered 1.5 h before dental treatment to achieve maximum effectiveness during the dental session.
  • Emotional stress is a known risk factor for myasthenic crisis. In order to avoid it, the patient should be allowed to arrive and rest for a while prior to dental appointment, possibly in a peaceful and relaxing environment.
  • A comfortable, semi-upright position rather than a deep recline is preferred, in order to reduce the risk of closing the throat or regurgitating saliva and other fluids.
  • Isolation of the working field with a rubber dam is recommended, and good oral evacuation suction is imperative to rapidly remove all dental debris and avoid aspiration.
  • Resting periods should be allowed during the dental session, use of benzodiazepines, hypnotics and barbiturates should be avoided since these drugs may lead to respiratory depression, worsening of myasthenic symptoms and myasthenic crisis.
  • Nitrous oxide-oxygen sedation is safe.
  • Amide-type anesthetics (lidocaine, mepivacaine) are instead metabolized by the liver and should be considered the option of choice.
  • Certain antibiotics may cause muscle weakness due to their ability to produce partial neuromuscular blockade by inhibiting the release of acetylcholine from the presynaptic membrane.
  • Aminoglycosides (gentamicin, streptomycin, amicacin, neomycin, kanamycin) must be avoided.
  • Penicillins, cephalosporins, sulfonamides and carbopenems have instead been widely used in MG.
  • Patients should be encouraged to perform regular brushing using an electric toothbrush or a manual toothbrush with a modified handle to reduce muscle fatigue.
  • Chlorhexidine or a fluoride mouth rinse to prevent decay and periodontal disease can also be used safely by MG patients.
  • It is a genetic disease characterized by muscle atrophy that causes severe progressive weakness.
  • The word dystrophy is derived from the Greek dys, which means “difficult” or “faulty,” and troph, or “nourish.”
  • These disorders vary in age of onset, severity, and pattern of affected muscles.
  • All forms of MD grow worse as muscles progressively degenerate and weaken. Many individuals eventually lose the ability to walk.
  • All of the muscular dystrophies are inherited and involve a mutation in one of the thousands of genes that program proteins critical to muscle integrity. The body’s cells don’t work properly when a protein is altered or produced in insufficient quantity.
  • Sometimes MD occur from spontaneous mutations that are not found in the genes of either parent, and this defect can be passed to the next generation.
  • Muscular dystrophies can be inherited in three ways:
  1. Autosomal dominantinheritance:
  • Occurs when a child receives a normal gene from one parent and a defective gene from the other parent.
  • Autosomal means the genetic mutation can occur on any of the 22 non-sex chromosomes in each of the body’s cells.
  • Dominant means only one parent needs to pass along the abnormal gene in order to produce the disorder. 
  1. Autosomal recessiveinheritance
  • Means that both parents must carry and pass on the faulty gene. The parents each have one defective gene but are not affected by the disorder.
  • Children in these families have a 25 percent chance of inheriting both copies of the defective gene and a 50 percent chance of inheriting one gene and therefore becoming a carrier, able to pass along the defect to their children. 
  1. X-linked(or sex-linked) recessive inheritance
  • Occurs when a mother carries the affected gene on one of her two X chromosomes and passes it to her son (males always inherit an X chromosome from their mother and a Y chromosome from their father, while daughters inherit an X chromosome from each parent).
  • Muscles are activated when an impulse is sent from the brain through the spinal cord and peripheral nerves to the neuromuscular junction.
  • There is release of the chemical acetylcholine that triggers a series of events that cause the muscle to contract.
  • The muscle fiber membranes contains a group of proteins—called the dystrophin-glycoproteincomplex—which prevents damage as muscle fibers contract and relax.
  • When this protective membrane is damaged, muscle fibers begin to leak the protein creatine kinase(needed for the chemical reactions that produce energy for muscle contractions) and take on excess calcium, which causes further harm. Affected muscle fibers eventually die from this damage, leading to progressive muscle degeneration.
  • MD most prominently affects the integrity of muscle fibers.
  • The disease causes:
  1. Muscle degeneration.
  2. Progressive weakness.
  3. Fiber death.
  4. Fiber branching.
  5. Splitting, phagocytosis (in which muscle fiber material is broken down and destroyed by scavenger cells).
  6. Chronic or permanent shortening of tendons and muscles.
  7. Overall muscle strength and tendon reflexes are usually lessened or lost due to replacement of muscle by connective tissue and fat.
  • There are nine major groups of the muscular dystrophies.
  • The disorders are classified by the extent and distribution of muscle weakness, age of onset, rate of progression, severity of symptoms, and family history.
  1. Duchenne MD
  2. Becker MD
  3. Congenital MD.
  4. Distal MD.
  5. Emery-Dreifuss MD.
  6. Facioscapulohumeral MD(FSHD).
  7. Limb-girdle MD(LGMD).
  8. Myotonic dystrophy (DM1).
  9. Oculopharyngeal MD (OPMD)
  • Results from an absence of the muscle protein dystrophin. 
  • Most common muscular dystrophy.
  • Inheritance is X-linked recessive (caused by a mutation on the X, or sex, chromosome).
  • Primarily affects boys.
  • Becomes apparent during the toddler years.
  • Progressive weakness and muscle wasting (a decrease in muscle strength and size) caused by degenerating muscle fibers begins in the upper legs and pelvis before spreading into the upper arms.

Other symptoms:

  • Loss of some reflexes.
  • Waddling gait.
  • Frequent falls and clumsiness (especially when running).
  • Difficulty when rising from a sitting or lying position or when climbing stairs.
  • Changes to overall posture.
  • Impaired breathing.
  • Lung weakness.
  • Cardiomyopathy
  • The Wasting muscles, in particular the calf muscles may be enlarged by an accumulation of fat and connective tissue, causing them to look larger and healthier than they actually are (called pseudohypertrophy).
  • Muscles in the diaphragm may weaken.
  • Breathing difficulties, respiratory infections, and swallowing problems.
  • Bone thinning and scoliosis (curving of the spine).
  • Cognitive and behavioral impairments.
  • There is partial but insufficient function of the protein dystrophin.
  • Usually appears around age 11 but may occur as late as age 25.
  • Affected individuals generally live into middle age or later.
  • Most individuals are able to walk until they are in their mid-thirties or later, while others are unable to walk past their teens.
  • Muscle weakness noticed first in the upper arms and shoulders, upper legs, and pelvis.

Early symptoms:

  • Walking on one’s toes.
  • Frequent falls.
  • Difficulty rising from the floor.
  • CALF muscles may appear large and healthy as deteriorating muscle fibers are replaced by fat, and muscle activity may cause cramps.
  • Refers to a group of autosomal recessive muscular dystrophies that are either present at birth or become evident before age 2.
  • They affect both boys and girls.
  • Weakness may be first noted when children fail to meet landmarks in motor function and muscle control.
  • Muscle degeneration is restricted primarily to skeletal muscle.
  • The majority of individuals are unable to sit or stand without support, and some affected children may never learn to walk.

 There are three groups of congenital MD:

  1. Merosin-negative disorders, where the protein merosin(found in the connective tissue that surrounds muscle fibers) is missing.
  2. Merosin-positive disorders, in which merosin is present but other needed proteins are missing.
  3. Neuronal migration disorders, in which very early in the development of the fetal nervous system the migration of nerve cells (neurons) to their proper location is disrupted.
  • Defects in the protein merosin cause nearly half of all cases of congenital MD.
  • Development of contractures(chronic shortening of muscles or tendons around joints, which prevents the joints from moving freely).
  • Scoliosis,
  • Respiratory and swallowing difficulties,
  • Foot deformities.
  • Weakness in diaphragm muscles (cause respiratory failure).

CNS symptoms:

  • Vision and speech problems.
  • Seizures
  • Structural changes in the brain.
  • Also called distal myopathy,
  • Primarily affect distal muscles (those farthest away from the shoulders and hips) in the forearms, hands, lower legs, and feet.
  • Distal dystrophies are typically less severe, progress more slowly, and involve fewer muscles.
  • Can affect the heart and respiratory muscles.
  • Patient unable to perform fine hand movement and have difficulty extending the fingers.
  • As leg muscles are involved there is difficulty in walking, climbing stairs and inability to stand on the heels.
  • Affects both men and women, is typically between the ages of 40 and 60 years.
  • In one form of distal MD, a muscle membrane protein complex called dysferlin is known to be lacking.
  • Primarily affects boys.
  • It has two forms: X-linked recessive and autosomal dominant.
  • Usually apparent by age 10, but symptoms can appear as late as the mid-twenties.
  • Progressive wasting of the upper arm and lower leg muscles and symmetric weakness.
  • Contractures in the spine, ankles, knees, elbows.
  • Back of the neck usually precede significant muscle weakness.
  • Contractures may cause the elbows to become locked in a flexed position.
  • The entire spine may become rigid as the disease progresses.
  • Other symptoms: shoulder deterioration, toe-walking, and mild facial weakness.
  • Serum creatine kinase levels moderately elevated.
  • Complains of heart problem by age 30.
  • Female carriers often have cardiac complications without muscle weakness.
  • Affects muscles of the face (facio), shoulders (scapulo), and upper arms (humera) with progressive weakness.
  • Also known as Landouzy-Dejerine disease.
  • Autosomal dominant disorder.
  • Disease progression is typically very slow, with intermittent spurts of rapid muscle deterioration.
  • Onset is usually in the teenage years but may occur as early as childhood or as late as age 40.
  • Asymmetric weakness. 
  • Muscles around the eyes and mouth are affected first, followed by weakness around the shoulders, chest, and upper arms.
  • Particular pattern of muscle wasting causes the shoulders to appear to be slanted and the shoulder blades to appear winged.
  • Muscles in the lower extremities become weakened.

Facial appearance:

  • Crooked smile.
  • A pouting look.
  • Flattened facial features or mask-like appearance.
  • Patient cannot pucker their lips or whistle.
  • Difficulty swallowing, chewing, or speaking.
  • Muscle weakness of diaphragm.
  • Hearing loss (particularly at high frequencies)
  • Lordosis, an abnormal swayback curve in the spine.
  • Affecting both males and females.
  • Different types of limb-girdle are caused by different gene mutations.
  • Patients with limb-girdle inherit a defective gene from either parent, or, in the more severe form, the same defective gene from both parents.
  • Appear in childhood but most often appears in adolescence or young adulthood. Limb-girdle can progress quickly or slowly, but most patients become severely disabled
  • Limb-girdle MD affects both males and females.
  • Weakness is noticed first around the hips before spreading to the shoulders, legs, and neck.
  • Waddling gait.
  • Difficulty in rising from chair, climbing stairs, or carrying heavy objects.
  • Patient fall frequently and are unable to run.
  • Contractures in the back muscles gives appearance of a rigid spine.
  • Proximal reflexes (closest to the center of the body) are impaired.
  • Cardiomyopathy and respiratory complications.
  • Intelligence remains normal.
  • Also known as Steinert’s disease and dystrophia myotonica
  • The most common adult form of MD
  • Appears in two forms, type 1 and type 2.
  • Type 1 is more common and is caused by an abnormally large number of repeats of a three-letter “word” (CTG) in genetic code.
  • Myotonic dystrophy type 2 (DM2) is characterized by myotonia and muscle dysfunction (weakness, pain, and stiffness), and less commonly by cardiac conduction defects, iridescent posterior subcapsular cataracts, insulin-insensitive type 2 diabetes mellitus, and testicular failure.
  • Myotonic MD affects both men and women between ages 20 and 30.
  • Myotonic MD causes an inability to relax muscles following a sudden contraction.

Other symptoms include

  • Long, thin face and neck.
  • Swallowing difficulties.
  • Drooping eyelids, cataracts, and other vision problems.
  • Baldness at the front of the scalp.
  • Weight loss.
  • Increased sweating.
  • Drowsiness
  • Heart problems that may lead to death during the 30s or 40s.
  • Irregular menstrual periods.
  • Infertility
  • Impotence.
  • Occurs in both men and women
  • It can be mild or severe.
  • Caused by a defect in a protein that binds to molecules that help make other proteins.
  • Common among Americans of French-Canadian descent, Jewish Ashkenazi, and Hispanics from the Southwest region.
  • Appears in 40s or 50s, some people will eventually lose their ability to walk.
  • Drooping eyelids and other vision problems.
  • Swallowing problems.
  • Muscle wasting and weakness in the neck, shoulders, and sometimes limbs.
  • Heart problems.
  • Elevated creatine kinase levels.
  • High serum aldolase, an enzyme involved in the breakdown of glucose, is measured to confirm a diagnosis of skeletal muscle disease.
  • High blood levels of myoglobin.
  • Polymerase chain reaction (PCR) can detect some mutations in the dystrophin gene.
  • Serum electrophoresis is a test to determine quantities of various proteins in a person’s DNA. A blood sample is placed on specially treated paper and exposed to an electric current. The charge forces the different proteins to form bands that indicate the relative proportion of each protein fragment.
  • Exercise tests: They are performed when the person is relaxed and in the proper position to allow technicians to measure muscle function against gravity and detect even slight muscle weakness.
  • Genetic testing: Genetic linkage studies can identify whether a specific genetic marker on a chromosome and a disease are inherited together.
  • Immunofluorescence testing can detect specific proteins such as dystrophin within muscle fibers.
  • Electron microscopy can identify changes in subcellular components of muscle fibers.
  • Neurophysiology studies can identify physical and/or chemical changes in the nervous system.
  • Muscle biopsies.

There is no specific treatment that can stop or reverse the progression of any form of MD. All forms of MD are genetic and cannot be prevented at this time, besides the use of prenatal screening interventions.

Assisted ventilation: needed to treat respiratory muscle weakness, especially in the later stages. Air that includes supplemental oxygen is fed through a flexible mask to help the lungs inflate fully.

Drug therapy:

  • Delay muscle degeneration.
  • Corticosteroids can slow the rate of muscle deterioration.
  • Immunosuppressive drugs such as cyclosporine and azathioprine can delay some damage to dying muscle cells.

Drugs that may provide short-term relief from myotonia (muscle spasms and weakness) include mexiletine; phenytoin; baclofen, which blocks signals sent from the spinal cord to contract the muscles.
Drug Exondys 51 (eteplirsen) treat individuals who have a confirmed mutation of the dystrophin gene amenable to exon 15 skipping.

Physical therapy: Passive stretching, postural correction, and exercise.
Corrective surgery is often performed to ease complications from MD.

  • In muscular dystrophy, the upper teeth and the lower teeth do not meet properly due to weakness of the muscles or by the tongue pushing the teeth outward to the lips.
  • Patient can have trouble with their jaw joints or their temporomandibular joints that lead to trouble with chewing, headaches, clicking of the joints, and tenderness of the muscles.
  • Patient may have a hard time brushing their teeth, flossing, and rinsing so advise electric toothbrushes.
  • Dentist should collaborate with the physician to so that they can provide the best dental and medical treatment possible.
  • Dental appointments should be as short as possible.
  • Long dental treatment makes the patient stressed.
  • Difficulty in controlling tongue movements and keeping mouth open for a long time, use a rubber bite block between teeth.
  • Use a tongue retractor in order to keep tongue from moving around.
  • Use a rubber dam that prevents inhaling any foreign substances.

Epilepsy is defined by the World Health Organization (WHO), as a chronic affection of multiple etiologies, characterized by recurring episodes of paroxysmal brain dysfunction caused by a sudden disorderly and excessive neuronal discharge.

In epilepsy, certain areas of the brain or all areas of the brain are overactive, sending too many signals. This results in seizures, sometimes also referred to as epileptic fits.

  • Epileptic seizures can take different forms. They may cause only a few muscles to twitch, for instance, or they may cause your whole body to convulse (shake uncontrollably) and lead to loss of consciousness.
  • Epileptic seizures do not usually last very long.
  • If a seizure lasts longer than five minutes, it is referred to as ‘status epilepticus.’

There are two main categories of epileptic seizures:

  1. Generalized seizures.
  2. Partial (focal) seizures.
  • Generalized seizures affect the whole brain.
  • They aren’t necessarily worse than partial seizures.
  • Generalized seizures are more likely to lead to loss of consciousness and make whole body convulse.
  • Tonic seizures: The person’s arms and legs become rigid and stiff. This kind of seizure usually passes quite quickly and doesn’t always affect your state of consciousness.
  • Atonic seizures (“drop attacks”): Here the muscles in one part of body suddenly become limp. As a result, chin might drop down onto chest, or legs might give way, for instance. patient may also briefly become unconscious and fall.
  • Clonic seizures: Large muscle groups – for instance in the arms or legs – jerk in a slow rhythm. This is usually accompanied by loss of consciousness.
  • Myoclonic seizures: Individual muscle groups twitch rapidly. State of consciousness is usually not affected.
  • Tonic-clonic seizures (sometimes called “grand mal seizures”): Whole body convulses and twitches, and patient become unconscious.
  • Absence seizures (sometimes called “petit mal seizures”): In this mild type of seizure, people suddenly lose awareness for a brief moment.
  • Partial seizures arise in a certain part of the brain.
  • Symptoms depends on the function of that part of the brain, and may include things like twitching of the arm (motor disturbances), abnormal sensations (sensory disturbances) or changes in vision (visual disturbances).
  • Patient may experience abnormal sensations, behave oddly, lose awareness, or hear, see or smell things differently.
  • They may also feel dizzy, feel anxious or hallucinate. This is known as an aura.
  • Some people smack their lips, grimace, stammer, walk around aimlessly or fiddle with things.
  • Partial seizures can be accompanied by twitching and/or convulsions.
  • Partial seizures may spread across the whole brain, resulting in what is known as a generalized seizure.
  • People with epilepsy usually don’t have any physical symptoms in between seizures.
  • Certain areas of the brainor all areas of the brain become overly active and fire off too many signals.
  • The resulting “storm in the brain” has noticeable effects, such as convulsions, in the rest of the body. 

Epilepsy can be caused by:

  • Injuries.
  • Inflammations of the meningesor brain.
  • Strokesor tumors.
  • If there is a known cause for someone’s epilepsy, it is referred to as “symptomatic epilepsy.”
  • Temporary problems: Forgetfulness, speech, or paralysis.
  • Epileptic seizures may lead to injury(in cases of generalized seizures).
  • Person accidentally hurt themselves or bites their tongue.
  • Certain types of epilepsy are associated with a greater risk of accidents and falls.
  • Fear of having a seizure can be mentally distressing
  • Epilepsy can itself lead to death in the following situations:
  1. If someone has a seizure that results in a fatal accident.
  2. If a severe, long-lasting seizure(status epilepticus) prevents the brain from getting enough oxygen, and that leads to heart and lung failure.
  3. If people with epilepsy die suddenly and unexpectedly. This is known as “sudden unexpected death in epilepsy” (SUDEP). It is extremely rare, though.

Someone is considered to have epilepsy if the seizures keep recurring. Epilepsy is usually diagnosed if

  • They have had at least two seizures.
  • There was a period of at least 24 hours between the seizures.
  • There is nothing to suggest that the seizures were one-off events.
  1. Blood test: For blood sugar and electrolyte balance
  2. Etectroencephalography (EEG): Provides information concerning the location and nature of any abnormal electrical activities in brain
  3. Computenzed tomography (CT): Detect sizable tumors, malformations involving blood vessels, birth related malformations, strokes, brain abscesses.
  4. Magnetic Resonance Imaging (MRI): detect small vascular malformations and scar tissue
  5. Positron-emission tomography (PET): use to locate the area in brain causing seizure.
  6. Neurosonography: identify the excessive spinal fluid(hydrocephalus) or blood(hemorrhage) in the brain.
  7. Sample of cerebrospinal fluid is taken from the lumbar (lower back) region of the spine too, using a needle. This procedure is known as a lumbar puncture or spinal tap.



Indications (seizure type)


Most common oral side effects and dental considerations


Partial and secondarily generalized

Drowsiness/sedation, osteopenia/osteomalacia


Partial and secondarily generalized

Xerostomia, stomatitis, gingival bleeding, rash, osteopenia/osteomalacia


Partial and secondarily generalized

Gingival hyperplasia, gingival bleeding, osteopenia/osteomalacia

Valproate or valproic acid

Partial and generalized

Gingival bleeding, petechiae, decreased platelet aggregation


Partial and generalized



Partial and generalized




Partial and generalized


Mild cognitive side effects


  • Usually occur within 2 to 18 months after starting medication.
  • The etiology is still unknown, but may be due to increase in the number of fibroblasts in the connective tissues.
  • Occur at any age, but younger are affected more than adults.
  • Men and women are equally affected.
  • It starts in the interdental papillae.
  • Occurs only where teeth are present.
  • Papillae enlarge buccally and lingually.
  • The enlarged areas are firm, pink, and covered with normal mucosa.
  • Patients have increased risk of fracture because enzyme-inducing antiepileptic drugs (e.g., phenytoin, phenobarbital, carbamazepine) alter the metabolism and clearance of vitamin D that leads to osteopenia and osteomalacia.
  • Adequate calcium and vitamin D supplementation (a minimum of 1,000 mg and 400 IU daily, respectively) especially in pa­tients taking phenobarbital, phenytoin or primidone.
  • Gingival overgrowth is a complication of phenytoin, use of chlorhexidine, folic acid rinses or both, excellent oral hygiene probably decreases the severity of the condi­tion.
  • Xerostomia and stomatitis are rare side effects of carbamazepine.
  • Valproic acid can cause direct bone marrow suppres­sion, which can impair wound healing and increase post-operative bleeding and infections.
  • Decreased platelet count is the most common hema­tologic effect of valproic acid, for elective surgery, laboratory evaluation — including bleeding time, fibrinogen level, prothrombin time, par­tial thromboplastin time and von Willebrand factor level — is needed to assess the risk of peri and postoperative bleeding.
  • Ensure medications have been taken properly in relation to dental appointments to minimize risk of seizure.
  • Ensure proper dental lighting (no light directly in eyes)
  • Treatment plan and design restorations to minimize risk of damaging or displacing dental restorations or prosthesis during epileptic seizure.
  • Quickly remove all foreign material from the patient’s mouth.
  • Chair should be placed in supine position.
  • Turn the patient to their side in order to minimize aspiration of foreign bodies or secretions.

Use passive restraint only to prevent injury that may occur by patient hitting nearby objects or to prevent them from falling out of the chair.

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