12.11.05 Clinic notes

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

        • Nausea + vomiting

      • 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