Thursday, February 14, 2008

72 - eponyms in medicine - part 4

151 - Kernig sign is first flex patient’s leg at both hip and knee - and then straighten knee; positive sign is pain and increased resistance to extending knee - suggesting meningeal irritation.

152 - Lisker’s sign is in thrombophlebitis or deep vein thrombosis - tenderness to percussion of the medial anterior tibial surface.

153 - Flatbush diabetes is GAD antibody negative diabetes in adult black subjects with diabetic ketoacidosis and increased frequency of human leukocyte antigen DR3 and DR4.

154 - Canavan disease is autosomal recessive infantile spongy degeneration of the brain with Alzheimer type II cells - increased prevalence among Ashkenazi from mutation in aspartoacylase leading to increased levels of N-acetylaspartic acid - described in 1931.

155 - Marie’s sign is fine tremor in hyperthyroidism.

156 - Wright’s stain is stain used for blood and bone marrow films.

157 - Morgagni - hydatids of is fallopian tube cysts found near the fimbriated end or in the broad ligaments.

158 - Zieve’s syndrome is jaundice - hyperlipemia - and hemolytic anemia in alcoholic liver disease; described in 1958.

159 - Cushing’s triad is signs of increased ICP 1. hypertension 2. bradycardia 3. irregular respirations.

160 - Meissner’s plexus is submucous plexus; innervates glandular epithelium - muscularis mucosa - intestinal endocrine cells - and submucosal blood vessels.

161 - Auerbach’s plexus is myenteric plexus - between the longitudinal and circular layers of muscle; provides motor innervation to the two muscle layers and secretomotor innervation to the mucosa.

162 - Magendie - foramen of is midline foramen exiting out of fourth ventricle.

163 - Bertin - renal columns of is the spaces between adjacent pyramids where cortical tissue extends into .

164 - Hutchinson-Guilford syndrome is aka progeria a condition in which the normal development of the first year is followed by gross retardation of growth - with a senile appearance characterized by dry wrinkled skin - total alopecia - and bird-like facies; genetics unclear.

165 - Stein-Leventhal syndrome is polycystic ovarian disease.

166 - Maroni sign is erythema of the skin overlying thyroid in hyperthyroidism.

167 - Lhermitte-Duclos disease is dysplastic cerebellar gangliocytoma; associated with germ line PTEN mutations; felt to be a part of multiple hamartoma syndrome or Cowden’s syndrome.

168 - Ball’s disease is intracerebral leukocytostasis - potentially fatal complication of acute leukemia (especially AML) when peripheral blast cell count >100 -000/uL; leukemic cells capable of invading through endothelium and causing hemorrhage into brain; not generally seen with CLL or CML.

169 - Costen’s syndrome is ear pain - tinnitus - impaired hearing - and dizziness from temporomandibular joint dysfunction.

170 - Bohr equation is Vd/Vt - for determining ratio of physiologic dead space.

171 - Gorlin-Goltz syndrome is focal dermal hypoplasia characterzied by widespread lesions of dermal hypoplsia with herniation of adipose tissue - streaks of pigmentary disturbance following lines of Blaschko - and severe absence deformities of bone - mental retardation - defects of optic nerve.

172 - Doi’s sign is elicitation of diminished deep tendon reflexes after maximal voluntary contraction in Lambert-Eaton syndrome.

173 - Kussmaul-Maier disease is old name for polyarteritis nodosa.

174 - Lafora’s disease is progressive myoclonus epilepsy - autosomal recessive - onset teenage years - characterized by seizures and cumulative neurological deterioration - death usually within ten years of first symptoms; associated with intracellular inclusions (Lafora bodies); associated with mutation in gene EPM2A.

175 - Kveim antigen is saline suspension of human sarcoid tissue prepared from spleen of an individual with active sarcoidosis; used in Kveim-Sitzbach test.

176 - Schick’s test is for testing immune status to Cornyebacterium diphtheriae - intradermal injection of 0.1 mL of purified standardized toxin; if no inflammation - antitoxin present.

177 - Swyer-James syndrome is in pediatrics - acquired hypoplastic lung that develops after severe obliterative bronchiolitis associated with bronchiolar obstruction - bronchiectasis - and distal air-space destruction.

178 - Kernohan notch is focal impression against the cerebral peduncle - pressure against notch in uncal herniation.

179 - stiff-man syndrome is chronic disorder of involuntary stiffness - painful muscle spasms - and rigidity - predominantly in the axial muscles; often occurs in conjunction with autoimmune diseases - particularly type I diabetes - associated with circulating antibodies to glutamic acid decarboxylase.

180 - Steele-Richardson-Olszewski syndrome is aka progressive supranuclear palsy.

181 - Hollenhorst plaques is cholesterol emboli visible as small bright flecks lodged in arterial bifurcations in retina.

182 - Reinke crystals is in 25% of Leydig cell tumors - intracytoplasmic rod-shaped crystalloids.

183 - Heyde’s syndrome is association between bleeding from gastrointestinal angiodysplasia and severe aortic stenosis which may resolve with aortic valve replacement; may be explained by acquired type IIA von Willebrand’s syndrome; first described by Hyde in N Engl J Med 1958;259:196.

184 - Bannayan-Ruvalcaba-Riley syndrome is Bannayan-Zonana syndrome

185 - Brodman area is neuro anatomical classification of the cortex based on its cyto architecture into 52 areas; e.g. precentral gyrus Brodman 4 - striate cortex Brodman 17

186 - Dressler’s beat is fusion beat seen in ventricular tachycardia.

187 - Leydig cell tumors is testicular tumor derived from the stroma - may find Reinke crystals (q.v.).

188 - Jatene’s arterial switch is for treating transposition of great vessels - pulmonary artery and aorta are transected above valves and switched - and coronary arteries are moved from old aortic root to new aorta (former pulmonary root).

189 - Kartagener’s syndrome is a triad of sinusitis - bronchiectasis - and situs inversus; also associated with ciliary dysfunction; Manes Kartagener - Swiss physician - 1897-1975.

190 - Hippocratic fingers is clubbing.

191 - Oliver’s sign is in aortic aneurysm - pulsation of aorta felt through cricoid cartilage when chin is elevated.

192 - Brodie’s abscess is small - intraosseus abscess that frequently involves the cortex and is walled off by reactive bone.

193 - Spurling’s test is for diagnosing cervical radiculopathy - exerting downward pressure on the head while rotating the head towards the symptomatic side creating pain radiating into affected extremity.

194 - Parkland formula is total body surface area % burned x kg x 4; 1/2 in first 8 hours - second 1/2 given next 16 hours.

195 - farmer’s lung is hypersensitivity pneumonitis from exposure to antigens of microorganisms which colonize equipment used in farming - including from moldy hay - grain - silage ( e.g. thermophilic actinomycetes - fungus such as Aspergillus umbrosus).

196 - Drummond - marginal artery of is vessel which lies along mesenteric border of colon and supplies vasa recta; arises from right and left branches of middle colic artery and left and right colic arteries; also supplies collateral connection between superior and inferior mesenteric arteries.

197 - Heimlich maneuver is abdominal thrust maneuver for clearing airway (blocked by foreign object - e.g. food) described in 1974 by Henry Heimlich.

198 - Möbius’s sign is in Graves’s ophthalmopathy - failure of ocular convergence following close accommodation at a distance of 5 inches.

199 - Cheyne-Stokes breathing is regularly irregular pattern characterized by a progressive increase in the depth and at times frequency of respiration with a crescendo-decrescendo shape that eventually culminates in an apneic phase; seen in CHF but also in meningitis - CVA - pontine damage.

200 - Sly’s syndrome is mucopolysaccharidosis (VII) - from beta-glucoronidase deficiency - resulting in hepatosplenomegaly - physical deformity from defect in degradation of dermatan sulfate and heparan sulfate.

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