Demyelinating Disease Flashcards
Destruction of the myelin in the CNS w/ relative sparing of the axon
Demyelinating Disease
White matter Disease
Demyelinating Disease
Areas of Destruction (or demyelination) are called
Plaques
On actual specimen appeared hard, called
Sclerosis
If multiple areas, it is called
Multiple Sclerosis
Classification of the Demyelinative Diseases
- Multiple Sclerosis (Disseminated or Insular Sclerosis)
- Diffuse Cerebral Sclerosis (Encephalitis Periaxalis Diffusa
- Acute Disseminated Encephalomyelitis
- Acute and Subacute Necrotizing Hemorrhagic Encephalitis (rarest)
- Chronic Relapsing Encephalomyelopathic Form
- Acute Multiple Sclerosis
- Neuromyelitis Optica (Devic’s Disease)
Multiple Sclerosis (Disseminated or Insular Sclerosis)
Damage either to parts of cerebral hemisphere and spinal cord,
lasts long periods and have episodic attacks
Chronic Relapsing Encephalomyelopathic Form
Spinal cord and optic pathways affected
Neuromyelitis Optica (Devic’s Disease
- Schilder’s Disease- If seen before 18 years old
- Concentric Sclerosis of Balo
- If seen in adults
Diffuse Cerebral Sclerosis (Encephalitis Periaxalis Diffusa)
- Post-infectious
- Following rabies, smallpox vaccination
Acute Disseminated Encephalomyelitis
- Acute Encephalopathic Form
- Subactue Necrotic Myelopathy
- Acute Brain Purpura
Acute and Subacute Necrotizing Hemorrhagic Encephalitis
- 2 Lesions separated by space or time
- Clinical lesions or laboratory supported lesions
- History of Remission & Relapse
Multiple Sclerosis
Laboratory Aids in MS
- MRI
- Double Contrasted CT Scan
- CSFIgGandOligoclonal Antibodies (Oligodendrocyte Antibodies)
- Evoke Potentials: Visual, Brainstem, Auditory, Somatosensory
2 or moreclinically focal lesions
Clinically Definite MS
1 clinical lesion and 1 laboratory lesion
Laboratory Supported Definite MS
1 Clinical Lesion
Clinically Probable MS
1 laboratory lesion
Laboratory Supported Probable MS
Precipitating Factors of Exacerbation of MS
- Infection
- Trauma
- Pregnancy
- Heat
- Fatigue
Initial Clinical Manifestation of MS
- 25% initial presentation is Optic Neuritis (partial or total loss of vision)
- 50%haveinitial symptoms of weakness or numbness in extremities
- Tingling sensation, tightband like sensation around trunk & limbs
- Lhermitte Sign: Passive flexion of the neck induces a tingling sensation down
the back
- May manifest as scotomas (blind spots) & papillitis
- ⅓will recover completely–
- Others improve significantly w/in 2 wks
- ½will eventually develop signs of MS
Optic Neuritis
Other CommonInitial Signs of MS
- Unsteadiness in walking
- Brainstem symptoms (diplopia, vertigo, vomiting)
- DIsorders of micturition
- Nostupor or coma
Charcot’s Triad
- Nystagmus
- Scanning Speech
- Intention Tremor
- Seen in advanced stages
- Not a common mode of presentation
Diseases Not Under Demyelinating Disease but with Myelin Destruction
- Subacute Combined Degeneration of the Cord (SCD)
- Marchiafava-Bignami Disease-
