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)

A review of the literature shows that long-lasting neurological complications of eclampsia are rare, but that, when present, they frequently involve visual function. Two patients are reported, whose symptomatology suggested damage to the posterior region of both cerebral hemispheres. The first patient showed a Balint's syndrome with complete inability to perceive more than one object at a time, erratic gaze movements and optic ataxia. After a year and half a marked improvement was observed. The second patient presented with severe of dyscalculia, spatial memory disorders, constructional apraxia and mild aphasia. She recovered completely in one year. This symptomatology might result from ischemic lesions in the watershed area between the territories of the middle and posterior cerebral arteries, the underlying mechanism being the compression of the posterior cerebral artery against the tentorium, caused by edema.
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PMID:[Neuropsychological study of long-term cerebral complications of eclampsia (author's transl)]. 103 34

A 29-year-old immunocompetent woman was admitted in 2006 with ataxia, limb weakness, generalized dystonia, and vertical diplopia that developed after a febrile episode. Brain magnetic resonance imaging (MRI) revealed the presence of extensive periventricular white matter lesions that did not enhance after gadolinium injection. As low titers of cytomegalovirus-IgM antibodies were found in the serum, a presumed diagnosis of postviral acute disseminated encephalomyelitis (ADEM) was made, and the patient received a 5-day course of 1 g methylprednisolone. The clinical and radiological outcome was very rapidly favorable, and subsequent brain MRIs in 2007 and 2008 were normal. In March 2011, the patient was readmitted with the complaints of abnormal fatigue, imbalance, and speech disorder. The neurological examination showed fluctuating spatiotemporal disorientation with dyscalculia, verbal deafness, gait ataxia, right hemianopia, and pyramidal signs in the four limbs. The brain MRI demonstrated extensive T(2) hyperintense white matter lesions predominating in the left temporal and parieto-occipital lobes, with a pseudotumoral aspect enhancing with gadolinium contrast. A clinical improvement was transiently noted after pulse steroid therapy, but after relapse and radiological worsening, the diagnosis of recurrent ADEM was challenged. The brain biopsy confirmed the presence of primary central nervous system lymphoma (PCNSL) under the variant form of lymphomatosis cerebri. Despite a partial response to chemotherapy, the patient died 8 months after the diagnosis. We discuss the role of sentinel lesions that may precede PCNSL for several years and insist on the importance to consider early brain biopsy in the presence of extensive, non-enhancing white matter lesions, even in a young and immunocompetent patient.
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PMID:Lymphomatosis cerebri Presenting as a Recurrent Leukoencephalopathy. 2318 72