| Dejerine's disease | A familial type of demyelinating sensorimotor polyneuropathy that begins in early childhood and is slowly progressive; clinically characterised by foot pain and paresthesias, followed by symmetrical weakness and wasting of the distal limbs; one of the causes of stork legs; patients are wheelchair bound at an early age; peripheral nerves are palpably enlarged and non-tender; pathologically, onion bulb formation is seen in the nerves: whorls of overlapping, intertwined Schwann cell processes that encircle bare axons; usually autosomal recessive inheritance. Synonym: Dejerine's disease, progressive hypertrophic polyneuropathy. (05 Mar 2000) |
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| Dejerine-Sottas disease | A familial type of demyelinating sensorimotor polyneuropathy that begins in early childhood and is slowly progressive; clinically characterised by foot pain and paresthesias, followed by symmetrical weakness and wasting of the distal limbs; one of the causes of stork legs; patients are wheelchair bound at an early age; peripheral nerves are palpably enlarged and non-tender; pathologically, onion bulb formation is seen in the nerves: whorls of overlapping, intertwined Schwann cell processes that encircle bare axons; usually autosomal recessive inheritance. Synonym: Dejerine's disease, progressive hypertrophic polyneuropathy. (05 Mar 2000) |
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| Dejerine | Joseph J., Paris neurologist, 1849-1917. See: Dejerine's disease, Dejerine's hand phenomenon, Dejerine's reflex, Dejerine's sign, Dejerine-Lichtheim phenomenon, Dejerine-Roussy syndrome, Dejerine-Sottas disease, Dejerine-Klumpke syndrome, Landouzy-Dejerine dystrophy. (05 Mar 2000) |
| Dejerine-Klumpke | Augusta, French neurologist (born in the U.S.), 1859-1927. See: Klumpke palsy, Klumpke's paralysis, Dejerine-Klumpke palsy, Dejerine-Klumpke syndrome. (05 Mar 2000) |
| Dejerine-Klumpke palsy | A type of brachial birth palsy in which there is paralysis of the muscles of the distal forearm and hand (all ulnar innervated muscles, plus more distal radial and median-innervated muscles), due to a lesion of the lower trunk of the brachial plexus, or of the C8 and T1 cervical roots. Synonym: Dejerine-Klumpke palsy, Dejerine-Klumpke syndrome, Klumpke's paralysis. (05 Mar 2000) |
| Dejerine-Klumpke syndrome | A type of brachial birth palsy in which there is paralysis of the muscles of the distal forearm and hand (all ulnar innervated muscles, plus more distal radial and median-innervated muscles), due to a lesion of the lower trunk of the brachial plexus, or of the C8 and T1 cervical roots. Synonym: Dejerine-Klumpke palsy, Dejerine-Klumpke syndrome, Klumpke's paralysis. (05 Mar 2000) |
| Dejerine-Lichtheim phenomenon | <clinical sign> In subcortical aphasia, the patient can indicate by use of the fingers the number of syllables of a word he has in mind but cannot speak. Synonym: Dejerine-Lichtheim phenomenon. (05 Mar 2000) |
| Dejerine-Roussy syndrome | <syndrome> A syndrome produced by infarction of the postero-inferior thalamus causing transient hemiparesis, severe loss of superficial and deep sensation with preservation of crude pain in the hypalgic limbs which frequently have vasomotor or trophic disturbances. Synonym: Dejerine-Roussy syndrome. (05 Mar 2000) |
| Dejerine's hand phenomenon | Clonic contractions of the flexors of the hand (wrist) on tapping the dorsum of the hand or the volar side of the forearm near the wrist; occurs in normal persons but is exaggerated in pyramidal tract lesions. Synonym: Dejerine's reflex. (05 Mar 2000) |
| Dejerine's reflex | Clonic contractions of the flexors of the hand (wrist) on tapping the dorsum of the hand or the volar side of the forearm near the wrist; occurs in normal persons but is exaggerated in pyramidal tract lesions. Synonym: Dejerine's reflex. (05 Mar 2000) |
| Dejerine's sign | <clinical sign> Aggravation of symptoms of radiculitis by the acts of coughing, sneezing, or straining to defecate. (05 Mar 2000) |
| Landouzy-Dejerine dystrophy | A relatively benign type of muscular dystrophy commencing in childhood and slowly progressive; characterised by wasting and weakness, sometimes asymmetrical, mainly of the muscles of the face, shoulder girdle, and arms; autosomal dominant inheritance. Synonym: facioscapulohumeral atrophy, Landouzy-Dejerine dystrophy. (05 Mar 2000) |
| aaa disease | Endemic anaemia of ancient Egypt, ascribed in the Papyrus Ebers to intestinal infestation with ancylostoma; now called ancylostomiasis. (05 Mar 2000) |
| ABO haemolytic disease of the newborn | Erythroblastosis foetalis due to maternal-foetal incompatibility with respect to an antigen of the ABO blood group; the foetus possesses A or B antigen which is lacking in the mother, and the mother produces immune antibody which causes haemolysis of foetal erythrocytes. (05 Mar 2000) |
| accumulation disease | A disease characterised by abnormal accumulation of a metabolic product in certain cells and tissues; examples include the mucopolysaccharidoses, lipoidoses. (05 Mar 2000) |
| Acosta's disease | A condition that results from prolonged exposure to high altitude. Symptoms include a continuous dry cough, shortness of breath, poor exercise tolerance, dizziness, headache, sleep difficulty, anorexia, confusion, fatigue and a rapid pulse. Treatment includes the immediate movement to a lower altitude. Prophylaxis has been accomplished successfully with the use of acetazolamide (Diamox). (27 Sep 1997) |