MS- Course not thought to be related to relapse rate
- EBV
- Prevalence 90% in healthy population
- Prevalence 100% in MS population
- Mimicry?
- 2 disease processes?
- Inflammation
- Neurodegeneration
- Relapse rate 0.6-0.8 episodes per year
- Subtype
- RRMS
- 85% of cases
- No progression between relapses
- PPMS
- 10% of cases
- No relapses
- Continuous progression
- PRMS
- Clearly defined relapses
- Disease progression between relapses
- SPMS
- Initial diagnosis is RRMS
- But then continuous disease progression
- DMT
- IFNb
- Blocks T-cell proliferation and tumor necrosis factor production
- 3 different types
- Glatiramer acetate
- Immunomodulator
- Inhibits T-cell activity
- Mitoxantrone
- Inhibits T-cell, B-cell, and macrophage proliferation
- Decreases the secretion of pro-inflammatory cytokines and increases an anti-inflammatory response via promotion of the
T-cell suppressor function
- Inhibits macrophage-mediated myelin degradation
- Natalizumab
- Anti-alpha4-integrin
- Inhibits the adhesion of leukocytes to their counter-receptor
- Prevents cells crossing BBB
- Fingolimod
- First oral disease modifying drug
- Sphingosine 1-phosphate receptor modulator
- Sequesters lymphocytes in lymph nodes
Synamet- Combination of carbidopa and levodopa
- Carbidopa is polar and so cannot cross the blood brain barrier
- But prevents peripheral
conversion of levodopa to dopamine
- Thereby reduces the unwanted
peripheral side effects of levodopa
- Also increases the quantity of levodopa in the bloodstream that is available to enter the brain
Entacapone - Catechol-O-methyl transferase (COMT) inhibitor
- Prevents COMT from metabolizing L-DOPA into
3-methoxy-4-hydroxy-L-phenylalanine (3-OMD) in the periphery, which does
not easily cross the blood brain barrier (BBB)
- May cause urine to turn reddish-brown
Stalevo
- Anti-parkinsonian dopaminergic combination medication
- Contains carbidopa, levodopa, and entacapone
Myotonic dystrophy
- Army drink-drive guy
- Chronic, slowly progressing, highly variable, inherited multisystemic disease
- Trinucleotide expansion
- Characterized by muscular dystrophy, cataracts, heart conduction defects, endocrine changes, and myotonia
- Two types:
- DM1, also called Steinert disease
- Has a severe congenital form and a milder childhood-onset form
- DM2, also called proximal myotonic myopathy (PROMM) or adult-onset form
- Rarer than DM1 and generally manifests with milder signs and symptoms
- Autosomal dominant inheritance
- Presentation
- Muscle wasting
- Slow relaxation
- Thenar tap test
- Frontal hair loss
- Hyporeflexia
- Sunken face
- A useful clinical clue for diagnosis is the failure of spontaneous
letting go of the hands following strong handshakes due to myotonia
Notes
- Leprosy is the most common global cause of neuritis
- Sarcoidosis can have neurological manifestations
- Neurologic findings are observed in about 5% of patients
- Known as neurosarcoidosis
- Cranial
nerves are predominantly affected
- Peripheral facial nerve palsy,
often bilateral, is the most common neurological manifestation
- Sildenafil-associated anterior ischemic optic neuropathy
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