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56 Cards in this Set

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Definition of MS
Multifocal demyelinating disease - causes the destruction of the myelin sheath of nerve fibers
Sclerotic Plaques
Scar-like lesions that form in the areas where the demyelination has occurred and block or distort the normal transmission of nerve impulses
Diagnosis of MS
Requires the occurrence of 2 or more attacks each lasting a minimum of 24 hours and separated by at least a month
Diagnosis of MS
The attacks must also be caused by lesions in 2 distinct areas of the CNS
Diagnosis of MS
Diagnosis rests on clinical grounds
Diagnosis of MS
MRI’s can be used to detect demyelinating plaques in the brain - BUT other disease can cause similar imaging patterns – e.g., AIDS and Lyme disease
 Plaques have predilection for periventricular white matter of brain (L/R hemisphere equally affected)
 Extent of plaques and total lesion area is related to cognitive fxing, but in general location of plaques is not predictive
• CSF analyses – can be used to detect oligoclonal abnormalities
Etiology of MS
Unknown. But data implicates genetic variables
Etiology of MS - Genetic Factors
1st degree relatives of affected individuals are 6-8 times more at risk than the general population, with siblings being most at risk; women:men = 2:1
Etiology of MS - Genetic X Environmental Variables
Siblings who develop MS do so in the same calendar year rather than at the same age
Etiology of MS - Environmental Variables
Prevalence rates of MS decreases systematically as latitude of habitation nears the equator
Etiology of MS - Environmental Variables
Those who move from high-risk latitude to a low-risk latitude (e.g. Europeans moving to South Africa) or vice-versa carry with them some of the risk from their place of origin if their movement occurs after the age of 15 --- disease acquisition is believed to occur before puberty
Etiology of MS - Immunologic Variables
Proposed explanations include a slow acting virus, a delayed reaction to a common virus, or an autoimmune reaction in which the body attacks its own tissues
Treatment of MS
Corticosteriods, adrenocorticotropic hormone (ACTH) and other anti-inflammatory agents may be used during exacerbation to hasten remission
Treatment of MS - Side Effects
These drugs are known to produce mood changes
Treatment of MS - Newer Methods
Newer immunomodulators (e.g., interferon B-1-a) to treat, but even these still can’t arrest or cure
Course of MS
Deficits partially resolve as inflammation subsides; also can be suppressed with steroids.
Course of MS
As more attacks occur more plaques develop and permanent deficits occur
MS - Mixed or Generalized Type
Affects approximately 50% and involves the optic nerve, the brain, the cerebellum, and the spinal cord
MS - Age of Onset
2/3 of people are diagnosed between the ages of 20-40 (average 30). Onset before age 15 is extremely rare
MS - Age of Onset
Onset after 40 is usually characterized by a faster progression of the degeneration process and shorter length of survival
MS - First Episode
After first episode, complete remission often occurs and most pts go years before recurrence
MS - Delayed Diagnosis
D/T the transient and variable nature of MS symptoms, mean delay of 3.5 –4 years from time of onset to diagnosis has been documented
MS - Life Expectancy
after onset of MS symptoms is generally estimated to be more than 30 years
MS - 4 Types
1. Relapsing/Remitting
2. Primary progressive
3. Secondary progressive
4. Progressive Relapsing
MS - Relapsing/Remitting
Most common in early years – about 90%
MS - Primary Progressive Type
Primary Progressive
MS - Secondary Progressive Type
Initially relapsing-remitting course followed by progression with or without occasional relapses, minor remissions and plateaus
MS - Progressive Relapsing Type
Rarest – progressive disease from onset with clear acute relapses, periods between relapses characterized by continuing progression
MS Symptoms - General
- Weakness in one or more limbs
- Bladder dysfunction - includes urinary urgency, frequency, dysuria, nocturia, and incontinence
MS Symptoms - Optic Neuritis
Inflammation, demyelination or degeneration of the optic nerve - Temporary or total loss of vision, usually occurring over several hours or days
MS Symptoms - Retrobulbar Neuritis
Optic neuritis that occurs far enough behind the optic disk that no early changes of the optic disk are visible by opthalmascope
MS Symptoms - Acute Myelitis (Transverse Myelitis)
Acutely evolving inflammatory-demyelinative lesion of the spinal cord,
MS Symptoms - Other Neurological Signs
vertigo, seizures, nystagmus, ataxia, diplopia, hemiplegia, deafness, neuropathy, aphasia and emotional changes
MS Symptoms - Charcot’s Triad
Nstagmus, dysarthria, and tremor
MS Symptoms - Spinal Cord (3 I's)
Incontinence, impotence, impairment of gait
MS - Other common symptoms
Spasticity
MS Symptoms - Fatigue
One of the most common and debilitating complaints, affecting up to 90% of patients
MS Symptoms - Psychiatric Changes
Affective disturbances, psychoses and personality changes - believed to be due to the disease process rather than (only) a psychological response to the illness
MS - Cognitive Changes - General
Cognitive symptoms have been reported in the early stages of the disease. More evident as more white matter is affected
MS - Cognitive Changes - Prevalence
40-70%
MS - Cognitive Changes - Pattern
Pattern of impairments more likely to resemble patients with frontal and subcortical lesions
MS - Cognitive Changes - Language
Receptive and expressive language skills are generally spared, although mild deficits in naming and word generation are common
MS - Cognitive Changes - Secondary Memory
Impairment in retrieval from long-term memory, but normal recognition, immediate recall, and rates of forgetting
MS - Cognitive Changes - Executive Functions
Impaired abstract and conceptual reasoning, slowed rates of information processing, impaired attention
MS - Cognitive Changes -Intellectual Functioning
VIQ > PIQ (likely reflects motor impairments)
MS - Cognitive Changes - Speed of Information Processing
While exaggerated by physical impairment, it also appears to cause an overall slowed speed of processing
MS - Cognitive Changes - Attention/Tracking/Motor Speed
Reduced span, impaired mental tracking, slowed complex visuomotor tracking, and Motor Slowing
MS - Cognitive Changes - Executive Functions
Impairments similar to frontal lobe patients (e.g., problems with conceptual flexibility) - Most common w/ chronic-progressive form
MS - Intact Cognition
Storage, encoding, recogniton, simple auditory span; motor skill and implicit learing, language skills (aside from fluency) visualspatial functions
MS - Affective Functioning
Depression is common and it is sometimes the first or most prominent symptom
MS - Affective Functioning
Also, rarely, includes euphoria
MS - Etiology of Affective/Personality Changes
May be related to periventricular and frontal white matter lesions
MS - Differential DX - Guillain-Barre Syndrome
Demyelinating disease of PNS
characterized by monophasic attack, lasting several weeks to months; no cog impairment
MS - Differential DX - Leukodystrophies
Group of genetically transmitted illnesses that usually manifest in children or adolescents, but sometimes later. Causes unremitting physical and mental deterioration
- Two well-known: adrenoleukodystrophy (ALD) and metachromatic leukodystrophy (MLD)
MS - Differential DX - Toxins
Numerous toxins preferentially attack CNS myelin
- Marchiafava-Bignami: possibly caused by substance in homemade red wine and results degeneration of CC
- Chronic Toluene exposure
MS - Differential DX - Lupus:
Produces CNS changes in only about 5% of cases initially, but later involves as many as 75%