eponyms in medicine - part 39

381 - Parinaud’s oculoglandular syndrome is preauricular node enlargement associated with chronic granulomatous conjunctivitis; atypical form of cat scratch disease from inoculation near eye.

382 - Reinke’s edema is vocal cord polyposis in female smokers - 50s-70s.

383 - Rosenthal fibers is inclusions that develop in astrocytes in chronic reactive and neoplastic proliferations; abundant in Alexander’s disease.

384 - Fontan procedure is for treating tricuspid atresia - pulmonary atresia - or hypoplastic left heart syndrome - procedure developed in 1968 whereby IVC is connected to pulmonary artery; long term complications include arrhythmias and protein losing enteropathy because of edema in liver and small bowel.

385 - Gleason score is prostate cancers stratified into 5 grades on basis of glandular patterns and degree of differentiation; grade 1 most differentiated and grade 5 no glandular differentiation; primary score and secondary score (for second most common histology) added together for score; therefore most differentiated tumor score of 2.

386 - Gianotti-Crosti syndrome is reactive exanthem - first described in association with hepatitis B infection (papular acrodermatitis of childhood - papular eruption on face and limbs); also associated with EBV - CMV infection.

387 - Naxos disease is initially described on Greek island of Naxos - synrome of arrhythmogenic right ventricular cardiomyopathy - non-epidermolytic palmoplantar keratoderma - and woolly hair - due to deletion in plakoglobin - protein in adherens and desmosomal junctions.

388 - Carney complex is AD complex of cardiac myxomas with aggressive biologic behavior - spotty pigmentation - and association with endocrine tumors (causing e.g. - Cushing syndrome); mutations in PRKAR1alpha gene (a protein kinase) account for half of the cases; unrelated to Carney syndrome.

389 - Klinger disease is Wegener’s disease; first described by Heinz Klinger in 1931 as a medical student and then described by Friederic Wegener in 1936.

390 - Paget’s disease of breast is breast cancer involving nipple and areola - appears as eczematous rash over breast with crusting - scaling - or erosion.

eponyms in medicine - part 28

271 - Rosenthal fibers is inclusions that develop in astrocytes in chronic reactive and neoplastic proliferations; abundant in Alexander’s disease.

272 - Klein-Waardenburg syndrome is Waardenburg syndrome type III.

273 - Boas’s sign is right subscapular pain due to cholelithiasis - <7%>

274 - Behçet’s disease is triad of aphthous ulcers - genital ulcerations - and ocular inflammation (posterior uveitis). Associated with erythema nodosum - cutaneous pustular vasculitis - also synovitis - CNS vasculitis involving brain stem - thrombophlebitis - and positive pathergy response. Described by Turkish dermatologist Behçet in 1937; also known as Silk Road disease due to clustering of cases along the Silk Road.

275 - Kaposi’s sarcoma is low-grade vascular tumor associated with HHV-8 - four forms - classic (older men of Mediterranean or Jewish extraction) - African - organ transplant-associated - and AIDS; differential for skin lesions includes bacillary angiomatosis from Bartonella.

276 - Charcot’s joints is neurogenic joint degeneration - can be secondary to syphilis - peripheral neuropathy.

277 - There are two sets of Charcot's triads, both of which are sets of clinical signs relating to quite separate diseases. One pertains to multiple sclerosis while the other refers to ascending cholangitis. Charcot's triads are named for Jean-Martin Charcot (1825-1893), the French neurologist who first described these combinations of signs in relation to these diseases.

Charcot's triad 1: The combination of nystagmus, intention tremor, and scanning or staccato speech. This triad is sometimes associated with multiple sclerosis but is not, however, as previously considered by some authors, pathognomonic for multiple sclerosis.

Charcot's triad 2: The combination of jaundice; fever, usually with rigors; and right upper quadrant abdominal pain. Occurs as a result of ascending cholangitis. When the presentation also includes hypotension and mental status changes, it is known as Reynolds' pentad.

278 - Rebuck skin window is dermal abrasion technique for testing tissue penetration of neutrophils - scraping forearm - then putting coverslip over it - checking glass for neutrophils.

279 - Mustard procedure is for treating transposition of great vessels - now rarely used; an atrial inversion procedure which connects RA to LV - which pumps out to pulmonary arteries - and connects LA to RV - which becomes systemic pump to aorta; variant uses pericardial or prosthetic intraatrial baffles.

280 - Stransky’s sign is involuntary dorsiflexion of the toes after firmly abducting the 5th digit for 2 seconds - and then acutely letting it go in upper motor neuron defects.