Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0018991 (hemiplegia)
3,997 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Tapia's syndrome, first described in 1904 by A.G. Tapia, is considered to be a syndrome consisting of ipsilateral hemiplegia of larynx and tongue with spared movement of soft palate. A 61-year-old Japanese woman had been in good health until August 1991, when she developed hoarseness and atrophy of the left side of her tongue. Although she also showed mild disturbance of elevation of bilateral soft palates and loss of taste in the posterior third of her tongue as well, the main symptoms were the paralyses of the ipsilateral larynx and tongue without involvement of the ipsilateral sternocleidomastoid and trapezius muscles. We concluded that she had cranial polyneuropathy similar to that of Tapia's syndrome. A carotid angiography revealed that she has a large aneurysm, which originated from the extracranial internal carotid artery in the region near the skull base. No other abnormal findings were detected by any computed tomography, magnetic resonance imaging or 67Ga-scintigraphy. The patient's lower cranial polyneuropathy was considered to be caused by the aneurysm. A survey of the literature indicates that extracranial carotid aneurysm is an extremely rare cause of Tapia's syndrome. In this case, the location of the aneurysm, which was present considerably distant from the skull base, seems to be the reason for the sparing of the accessory nerve.
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PMID:[A patient with aneurysm of extracranial internal carotid artery presenting lower cranial polyneuropathy similar to Tapia's syndrome]. 795 29

A Portuguese female, aged 47 years, who had emigrated to Spain, was admitted to the hospital in 1991 for pontine haematoma. The patient, four siblings and her father were affected by a peripheral neuropathy, indicating autosomal dominant inheritance. The patient presented in the 2nd decade with sensory and motor neuropathy beginning in the lower extremities. Alternating constipation and diarrhoea, and urinary incontinence became uncontrollable. She had to be colostomised, and, eventually, confined to a wheelchair from the age of 43. Neurological examination showed bilateral facial involvement, and severe signs of sensory and motor peripheral neuropathy, and later right hemiplegia. There were abnormalities of atrial rhythm and left bundle branch block. Computerised axial tomography and magnetic resonance images demonstrated left-sided pontine haemorrhage. Nerve conduction studies revealed severe diminution of motor conduction velocity and absence or reduction of amplitude of sensory and motor action potentials. Inanition and a respiratory infection led to her death. Clinical diagnosis was type I familial amyloid polyneuropathy (FAP). Postmortem examination demonstrated amyloid deposits in peripheral nerves, including spinal roots and cranial nerves, leptomeninges, thyroid, breasts, heart, adrenal glands, kidneys, intestines, pancreas, and meningeal and some pontine vascular structures. Advanced pontine haematoma was verified. Cerebral haemorrhage usually occurs with cerebrovascular amyloidosis, but exceptionally with FAP. A minority of patients presenting with CNS haemorrhage showed arteriovenous malformation or embolism [Da Silva Horta and Dias Coelho (1960) Arch 'de Vecchi' Anal Patol Med Clin 31 = 163-172].(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Type I familial amyloid polyneuropathy and pontine haemorrhage. 831 Aug 8

Hypereosinophilic syndrome is a rare disorder of the hematopoietic system. The disease is characterized by continuously high number of eosinophils (>1.5 x 10(9)/L) for more than six months. Other possible causes of hypereosinophilia, such as allergic and parasitic diseases, malignant disease, Churg-Strauss disease and infection should be eliminated. The most common manifestations of hypereosinophilic syndrome are pulmonary, skin, gastrointestinal, cardiac difficulties and neurologic lesions. Numerous neurologic lesions have been described, in particular of the central and peripheral nervous systems. Review of the literature revealed the following to have been recorded so far: mononeuritis multiplex, sensory polyneuropathy, radiculopathy, myalgia, myositis and perimyositis, neuropathy, ataxia, paraplegia, ophthalmologic abnormalities, optic neuritis, hemiplegia-hemiparesis, spasmodic quadriplegia, seizures, meningitis, cerebral infarction, organic psychosyndrome, other mental changes, stroke, temporal arteritis, leptomeningeal dissemination, memory deficits and dysarthria.
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PMID:Neurologic manifestations of hypereosinophilic syndrome--review of the literature. 2292 4