Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0004134 (ataxia)
 15,886 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

It is aimed to recover, considering its historical value, a semiological sign described in 1931 by an eminent neurologist of Rio de Janeiro, together with a master of the French neurology. In the article by Alajouanine and Akerman, named "Attitude of the hand in an astereognostic monobrachial crisis of multiple esclerosis", a semiologic alteration was described which was characterized by "an instability in the attitude of the fingers, which is observed mainly with the hand extended in the attitude of swearing". This attitude of hand worsened a lot with the eyes closed and was accompanied by sensory ataxia, astereognosis, and impaired deep sensation in the affected member. From the original article, it is possible to consider at the present time the described semiologic alteration as a form of pseudoathetosis localized in the hand.
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PMID:[Ataxic instable Alajouanine-Akerman's hand: recovery of a semiologic sign]. 1041 40

A 41-year-old, right-handed man was admitted to our hospital on September 12, 2002, due to progressive clumsiness in both hands. The patient had been diagnosed as having multiple sclerosis three years prior to admission. He noticed difficulty in manipulating objects three months before admission. Cervical T2-weighted MRI showed a high signal intensity at the level of C3-4 which was enhanced on T1-weighted image with gadolinium. On admission, neurological examinations revealed impairment of dexterity, deep sensory disturbance, and astereognosis in both hands. The clumsiness of complex finger movements was predominant on the left side, and was exaggerated with the eyes closed in association with pseudoathetosis. After steroid therapy, his clumsy hands improved gradually. This type of clumsiness in multiple sclerosis had been described as useless hand syndrome by Oppenheim. In accordance with our case, useless hand syndrome has been reported to arise from high cervical (C2-4) lesions, mainly involving the posterior cord ipsilateral to the clumsy hand. Although the majority of reported cases with useless hand syndrome had other neurological complications, such as hemiparesis, tetraparesis, and truncal ataxia, our patient exhibited a pure form of useless hand syndrome. In addition, useless hand syndrome is usually unilateral, and bilateral useless hand syndrome is very rare. Clumsiness of fine finger movements with astereognosis in our patient is similar to numb clumsy hands or limb-kinetic apraxia due to cervical spondylosis or postcentral gyrus lesion, respectively. This indicates an important role of the high cervical posterior cord in conveying a kinesthetic sense necessary to guide fine finger movements. It should be kept in mind that high cervical lesions in multiple sclerosis causes clumsy hands mimicking limb-kinetic apraxia.
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PMID:[A case of multiple sclerosis with bilateral useless hand syndrome as a main clinical feature]. 1583 90