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Ans. 260 Benedikt’s syndrome
Benedikt's syndrome is a clinical picture from paramedian midbrain infarction fromocclusion of the paramedian penetrating branches of the basilar artery affecting the third nerve root fiber, red nucleus, cerebral peduncle resulting in ipsilateral medial rectus palsy with a fixed dilated pupil and contralateral tremor, chorea, and athetosis.
Ans. 259. Bordet-Gengou medium.
It's used for identifying Bordetella pertussis , medium contains high percentage of rabbit blood (20-30%) to inactivate inhibitors in blood; also has potato and glycerol.
Ans 258. Chuvash polycythemia
Autosomal recessive disorder with features of both primary and secondary polycythemia, endemic to the mid-Volga River region of Russia (Chuvas); associated with homozygous mutation Arg200Trp in VHL gene (gene associated with von Hippel-Lindau syndrome); mutation impairs interaction of VHL with hypoxia-inducible factor 1 protein (HIF), allowing HIF to avoid destruction.
257. What is Churg-Strauss syndrome ?
Allergic angiitis and granulomatosis involvement in the lung, associated with eosinophilia. Asthma is the cardinal feature (occurs in 95%) and precedes vasculitic phase by 8-10 years; 2/3 of patients have skin lesions which can appear as subcutaneous nodules on extensor surfaces; 70% have P- ANCA; 4/6 following criteria 85% sensitive and 99.7% specific: asthma; eosinophilia>10%; neuropathy; pulmonary opacities; paranasal sinusabnormality; biopsy of blood vessel showing eosinophils in extravasculararea.
266. What are Siderocytes ?
They are red cells containing granules of nonhaem iron. Originally described by Gruneberg. The granules are formed of a water insoluble complex of ferric iron, lipid, protein, and carbohydrate.
255. What is Carabello’s sign ?
In severe aortic stenosis, during catheterization across aortic valve, an augmentation of the peripheral systolic pressure of more than 5 mm Hg during aortic pullback; from catheter further increasing the severity of the stenosis and removal resulting in a transient increase in stroke volume and systolic pressure.
254. What are Calabar swellings ?
They are seen in loiasis, swellings 5-10 cm in diameter from localized areas of angioedema associated with migration of adult Loa loa worms through subcutaneous tissues.
253. What are Brushfield’s spots ?
Brushfield's spots are seen in Down's syndrome, small white spots on the periphery of the iris.
252.What is Birt-Hogg-Dube syndrome ?
Genodermatosis characterized by hair follicle hamartomas, renal cell cancer, and spontaneous pneumothorax; caused by mutation in gene encoding folliculin; also associated with trichodiscomas and acrochordons.
251. What are Birbeck’s granules ?
They are also known as Langerhans's granules, a small tennis racket-shaped membrane-bound granule with characteristic cross-striated internal ultrastructure seen in Langerhans cell histiocytosis.
250. What is Bernstein test ?
To test for gastroesophageal reflux disorder, acid perfusion test of esophagus with 0.1 N HCl and see if reproduces chest pain; limited sensitivity and specificity though.
249. What is Benedikt’s syndrome ?
Clinical picture from paramedian midbrain infarction fromocclusion of the paramedian penetrating branches of the basilar artery affecting the third nerve root fiber, red nucleus, cerebral peduncle resulting in ipsilateral medial rectus palsy with a fixed dilated pupil and contralateral tremor, chorea, and athetosis.
248. What is Bazex’s syndrome ?
Acrokeratosis paraneoplastica, consists of erythematous to violaceous psoriaform plaques occurring predominantly in acral areas; associated with Hodgkin's disease and squamous cell carcinomas of digestive tract.
247. What is Barre - Lieou syndrome ?
Cervicosympathetic syndrome, characterized by neck pain and dizziness due to arthritic or traumaticdamage to the cervical spine.
245. What is Ball’s disease ?
Intracerebral leukocytostasis, a 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. Condition not generally seen with CLLor CML.
245. What is Austin Flint murmur ?
Austin Flint murmur is a Diastolic rumble in aortic regurgitation heard at cardiac apex, thought to be due to aortic jet impinging on the mitral valve, causing vibrations and also from simultaneous diastolic filling of the left ventricle from the left atrium and aorta closing the mitral valve in diastole, producing
physiologic stenosis; named after Austin Flint (1812-1886).
physiologic stenosis; named after Austin Flint (1812-1886).
243. What is Happy puppet syndrome ?
Angelman syndrome or Happy puppet syndrome is a disorder with severe developmental delay, frequent laughing, easily excitable personality; from maternal deletion of 15q11-13; associated with mutation in maternally- imprinted ATP10C, a putative aminophospholipid translocase.
243. What is TRALI syndrome ?
Transfusion Related Acute Lung Injury; transfusion reaction occurring within 6 hours after transfusion of blood product, characterized by pulmonary edema; due to anti-granulocyte antibodies in donor's plasma causing pulmonary sequestration of recipient leukocytes in susceptible patient.
242. Perlia's nucleus
Cell group located between motor neuron groups innervating left and right medical rectus muscles; felt to be involved in ocular convergence.
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