| Westphal's disease | <gastroenterology, neurology> An inherited (autosomal recessive) disorder where there is excessive quantities of copper in the tissues, particularly the liver and central nervous system. Wilson's disease causes the body to absorb and retain copper. The copper deposits in the liver, brain, kidneys and eyes. Complications include dementia and liver failure. Symptoms include jaundice, vomiting, tremors, weakness and slow stiff movements. Blood tests show serum ceruloplasmin is low. Medications are given to remove the excess copper from the body. Even with life-long treatment, disabling (and life-threatening) side effects are common. Inheritance: autosomal recessive. (27 Sep 1997) |
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| Strumpell-Westphal disease | <gastroenterology, neurology> An inherited (autosomal recessive) disorder where there is excessive quantities of copper in the tissues, particularly the liver and central nervous system. Wilson's disease causes the body to absorb and retain copper. The copper deposits in the liver, brain, kidneys and eyes. Complications include dementia and liver failure. Symptoms include jaundice, vomiting, tremors, weakness and slow stiff movements. Blood tests show serum ceruloplasmin is low. Medications are given to remove the excess copper from the body. Even with life-long treatment, disabling (and life-threatening) side effects are common. Inheritance: autosomal recessive. (27 Sep 1997) |
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| Westphal-Erb sign | <clinical sign> Abolition of the patellar tendon reflex, in tabes and certain other diseases of the spinal cord, and occasionally also in brain disease. Synonym: Erb sign, Westphal's phenomenon, Westphal's sign, Westphal-Erb sign. (05 Mar 2000) |
| Westphal, Karl | <person> German neurologist, 1833-1890. See: Westphal's disease, Westphal's phenomenon, Westphal's pseudosclerosis, Westphal's pupillary reflex, Westphal's sign, Westphal-Erb sign, Westphal-Piltz phenomenon, Westphal-Strumpell pseudosclerosis, Edinger-Westphal nucleus, Erb-Westphal sign, Strumpell-Westphal disease. (05 Mar 2000) |
| Westphal-Piltz phenomenon | A constriction of both pupils when an effort is made to close eyelids forcibly held apart. A variant of the pupil response to near vision. Synonym: Galassi's pupillary phenomenon, Gifford's reflex, lid-closure reaction, orbicularis phenomenon, orbicularis pupillary reflex, Piltz sign, Westphal's pupillary reflex, Westphal-Piltz phenomenon. (05 Mar 2000) |
| Westphal's phenomenon | <clinical sign> Abolition of the patellar tendon reflex, in tabes and certain other diseases of the spinal cord, and occasionally also in brain disease. Synonym: Erb sign, Westphal's phenomenon, Westphal's sign, Westphal-Erb sign. (05 Mar 2000) |
| Westphal's pseudosclerosis | <gastroenterology, neurology> An inherited (autosomal recessive) disorder where there is excessive quantities of copper in the tissues, particularly the liver and central nervous system. Wilson's disease causes the body to absorb and retain copper. The copper deposits in the liver, brain, kidneys and eyes. Complications include dementia and liver failure. Symptoms include jaundice, vomiting, tremors, weakness and slow stiff movements. Blood tests show serum ceruloplasmin is low. Medications are given to remove the excess copper from the body. Even with life-long treatment, disabling (and life-threatening) side effects are common. Inheritance: autosomal recessive. (27 Sep 1997) |
| Westphal's pupillary reflex | A constriction of both pupils when an effort is made to close eyelids forcibly held apart. A variant of the pupil response to near vision. Synonym: Galassi's pupillary phenomenon, Gifford's reflex, lid-closure reaction, orbicularis phenomenon, orbicularis pupillary reflex, Piltz sign, Westphal's pupillary reflex, Westphal-Piltz phenomenon. (05 Mar 2000) |
| Westphal's sign | <clinical sign> Abolition of the patellar tendon reflex, in tabes and certain other diseases of the spinal cord, and occasionally also in brain disease. Synonym: Erb sign, Westphal's phenomenon, Westphal's sign, Westphal-Erb sign. (05 Mar 2000) |
| Westphal-Strumpell pseudosclerosis | <gastroenterology, neurology> An inherited (autosomal recessive) disorder where there is excessive quantities of copper in the tissues, particularly the liver and central nervous system. Wilson's disease causes the body to absorb and retain copper. The copper deposits in the liver, brain, kidneys and eyes. Complications include dementia and liver failure. Symptoms include jaundice, vomiting, tremors, weakness and slow stiff movements. Blood tests show serum ceruloplasmin is low. Medications are given to remove the excess copper from the body. Even with life-long treatment, disabling (and life-threatening) side effects are common. Inheritance: autosomal recessive. (27 Sep 1997) |
| Edinger-Westphal nucleus | A small group of preganglionic parasympathetic motor neurons in the midline near the rostral pole of the oculomotor nucleus of the midbrain; the axons of these motor neurons leave the brain with the oculomotor nerve and synapse on the cells of the ciliary ganglion which in turn innervate the sphincter muscle of the pupil and ciliary muscle. Destruction of this nucleus or its efferent fibres causes maximal paralytic dilation of the pupil; also demonstrated to project fibres to lower levels of the brainstem and all spinal levels. (05 Mar 2000) |
| Erb-Westphal sign | <clinical sign> Abolition of the patellar tendon reflex, in tabes and certain other diseases of the spinal cord, and occasionally also in brain disease. Synonym: Erb sign, Westphal's phenomenon, Westphal's sign, Westphal-Erb sign. (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) |