331 - Wiskott-Aldrich syndrome is X-linked characterized by triad of eczema - thrombocytopenia (from autoantibodies) - and repeated infections; small platelets (3-5 fL); failure to express sialic acid-rich glycoprotein - sialophorin or CD15 - ending in early death.
332 - Bowen’s disease is squamous cacrinoma in situ - seen generally on sun-exposed areas.
333 - Lennox-Gastaut syndrome is childhood seizure disorder associated with multiple seizure types (including drop attacks - atypical absences general tonic clonic seizures - and myoclonus) - slow generalized spike-and-slow-wave EEG - mental retardation - and resistant to standard anti-epileptic medications.
334 - de Musset’s sign is in aortic regurgitation - head bobbing - named after 19th century French poet who had luetic aortic insufficiency.
335 - Lassa fever is hemorrhagic fever caused by Lassa virus - first isolated in 1969 in Nigeria - an arenavirus - spread through contact with secretions (e.g. - urine) from infected rats; fatality 15-20% of hospitalized patients; can be treated with ribavirin.
336 - Froin’s syndrome is spinal block from tumor or inflammatory conditions - etc. causing increased protein in CSF - resulting in xanthochromia and increased coagulability in CSF.
337 - Pautrier’s microabcesses is q.v. Sézary-Lutzner cells.
338 - Markesbery distal myopathy is distal myopathy associated with rimmed vacuoles.
339 - Alexander’s law is peripheral vestibular nystagmus increases in intensity when the gaze is in the direction of the fast phase - and decreases in intensity when the gaze is away from the fast phase.
340 - Kocher’s maneuver is dissection of the duodenum from the right-sided peritoneal attachment to allow mobilization and visualization of the back of the duodenum/pancreas.(2nd part).
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