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Query: UMLS:C0004134 (ataxia)
 15,886 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Twenty patients with multiple sclerosis (MS), 19 women and 1 man, with acute proprioceptive sensory disturbances related to the presence of plaques on the posterior columns (posterior column syndrome) at the cervical or thoracic levels of the spinal cord, were selected among 138 new patients with MS assisted in our neurological unit over the past five years. In 17 of these patients, the acute posterior cordonal syndrome was responsible for the first clinical manifestations of the disease. The other 3 patients had a history suggestive of MS. These 20 patients were followed with a minute analysis of neurological function with repeated clinical evaluation combined with repeated MRI study of the spinal cord. Brain MRI (strongly suggestive of MS in 15 patients), evoked potentials (EP) and cerebrospinal fluid electrophoresis analysis (with oligoclonal bands present in all patients were it was performed) were also obtained at least once in each patient. Spinal cord MRI demonstrated more lesions in the cervical region (90 p.100) than in the thoracic regions (10 p.100). Eighty percent of the cervical lesions were located high, between C1 and C4. The most characteristic clinical expression was the deafferentation of one upper limb, preferentially the "useless hand" (Oppenheim) or even a pseudoathetosic or dystonic limb. Propioceptive ataxia or spontaneous cervical or brachial pain were other forms of clinical expression. No major motor deficit or sphincter disorders were noted at any time in the clinical course in any of the patients. There was a good correlation between localization and morphology of the plaques detected by spinal cord MRI and clinical signs. Intrinsic medullary lesions were seen as high intensity signals on T2-weighted images which were enlarged more than the same lesion visualized on T1-weighted images after injection of paramagnetic contrast agents. This reflected the presence of edema extending beyond the main inflammatory lesion. There was also a good correlation between improvement of clinical symptoms and total or, mor frequently, partial reduction of the plaques, analyzed morphologically by successive spinal cord MRI series. The diagnosis of MS was clinically definitive in 60 p.100 of cases and laboratory-supported definitive in 40 p.100. During the follow-up period (average 36 months), 15 patients (75 p.100) presented one or more exacerbations, all of them presenting a favorable course: at last follow-up, 9 patients were asymptomatic, EDSS was 1 in 6 patients, 1.5 in 4 patients and 2 in 1 patient. This study confirms the contribution of serial spinal cord MR studies to understanding the natural history and pathophysiology of medullary forms of MS presenting as a cordonal posterior syndrome. It also shows a good relationship between the clinical manifestations and course of this form of MS and the localization and variable morphology of plaques. Finally, our results suggest the predictive benign course for this medullary form of MS that seems to be almost exclusively restricted to the female gender.
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PMID:[Acute posterior cord lesions in multiple sclerosis. An MRI study of the clinical course in 20 cases]. 1113 29

The aim of this study was to characterize the epidemiology and clinical features of tick-borne encephalitis in north-eastern Poland. Clinical and epidemiological data were analysed of patients hospitalized with the diagnosis in the Department of the Infectious Diseases and Neuroinfections of the Medical University in Bialystok in 1997-2001. Tick-borne encephalitis was diagnosed in 152 patients: 51 (34%) presented with meningitis, 89 (59%) with meningoencephalitis and 12 (8%) with meningoencephalomyelitis. Headache (84%) and fever (81%) were the most common symptoms. Meningeal signs were present in 137 patients (90%). Most common neurological abnormalities were: Oppenheim and Babinski signs (74 patients, 49%), ataxia (37, 24%), impaired consciousness (37, 24%) and pareses (16, 10%). Of patients examined, 146 (96%) had raised pleiocytosis, frequently accompanied by high cerebrospinal fluid protein concentration (90%), raised erythrocyte sedimentation rate (65%), peripheral blood leucocytosis (26%) and increased aminotransferase activity (16%). There was only 1 forest worker among the patients. Tick-borne encephalitis remains common in north-eastern Poland but, possibly because of effective vaccination, it has virtually disappeared among forest employees. The diagnosis appears difficult in some cases, as meningeal signs may not be present and laboratory findings may not be suggestive of a viral infection.
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PMID:Tick-borne encephalitis in north-eastern Poland in 1997-2001: a retrospective study. 1258 23

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