Thursday, February 21, 2008
eponyms in medicine - part 26
251 - Duncan’s disease is X-linked lymphoproliferative syndrome - normal response to childhood infections but develop fatal lymphoproliferative disorders after infection with EBV; most patients with this syndrome die of infectious mononucleosis.
252 - Gerstmann syndrome is finger agnosia - agraphia - right-left disorientation - and dyscalculia.
253 - Jamaican vomiting sickness is poisoning from hypoglycin A in unripe ackee fruit; hypoglycin A is potent hypoglycemic agent that decreases rate of fatty-acid beta oxidation probably by inhibition of acyl dehydrogenase flavin-dependent oxidation; causes liver damage indistinguishable from Reye’s syndrome.
254 - Adie syndrome, sometimes known as Holmes-Adie's syndrome or Adie's Tonic Pupil, is a neurological disorder which affects the pupil of the eye and the autonomic nervous system. It is caused by damage to the postganglionic fibers of the parasympathetic innervation of the eye, usually by a viral or bacterial infection which causes inflammation, and characterized by a tonically dilated pupil.Adie syndrome presents with three hallmark symptoms, namely abnormal pupil size, loss of deep tendon reflexes and diaphoresis. Other signs may include hyperopia due to accommodative paresis, photophobia and difficulty reading.
255 - clay shoveler’s fracture is fracture of spinous process of C7.
256 - jacksonian march is in simple partial seizures - clonic movements of a single muscle group spreading to involve contiguous regions of the motor cortex.
257 - Goldenhar syndrome is syndrome of oculoauriculovertebral dysplasia characterized by unilateral deformity of the external ear and small ipsilateral half of the face with epibulbar lipodermoid and vertebral anomalies; autosomal dominant.
258 - Schwartz’s dictum is no acid - no ulcer.
259 - Young’s syndrome is clinical features similar to cystic fibrosis - including bronchiectasis - sinusitis - and obstructive azoospermia - but don’t have increased sweat chloride values - nor pancreatic insufficiency - nor abnormal nasal potential differences - nor the CF delta F508 mutation; affected individuals are often middle-aged males identified during evaluation for infertility.
260 - Mayer-Rokitansky-Küster-Hauser syndrome is absence of vagina from abnormality of müllerian development.