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 21-year-old man with acute cerebellar ataxia and sympathotonic orthostatic hypotension, following Epstein-Barr (EB) virus infection, was reported. He noticed unsteady gait 2 weeks after the development of cough, nausea and vomiting. On admission, he was unable to sit and walk due to truncal ataxia and orthostatic hypotension with marked tachycardia. Limb ataxia of moderate degree was also noted. The blood pressure was 112/42 mmHg, and the pulse rate was 64/min in supine position, and 5 minutes after standing, they were 82/42 mmHg and 128/min. In laboratory studies, no atypical lymphocytes were detected in the peripheral blood. However, the titers of antibodies, VCA-IgM, against EB virus, were x80 and x160 in serum, respectively. And the titer of VCA-IgM subsequently decreased to the normal level in two months. They were negative in the cerebrospinal fluid. The results of the autonomic function studies revealed dysfunctions of the sympathetic post-ganglionic nerves, especially of alpha-adrenergic system, with preservation of beta-adrenergic system. He recovered from cerebellar ataxia and from sympathotonic orthostatic hypotension 3 and 8 months after the onset, respectively, without residuals.
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PMID:[Acute cerebellar ataxia with sympathotonic orthostatic hypotension following Epstein-Barr virus infection--a case report]. 839 87

We studied clinical manifestations of sixteen patients with cerebellar infarcts diagnosed by MRI. In fourteen of them, the stroke developed abruptly with vertigo, which continued for several days. At the early stage of illness, ataxia was obscure. But after vertigo and nausea disappeared, nine cases showed truncal ataxia, while limb ataxia was found in only five. Their vertigo was rotatory and aggravated by head movement. Gaze-evoked nystagmus was observed in only 5 cases. Four patients preferred to take unilateral posture since they experienced less vertigo. The side of their lesions was the lower side of their posture. Limb ataxia was more frequent in SCA-involving cases than in SCA-non involving cases (3 out of 6 vs 2 out of 10, respectively). On the other hand, headache was more frequent in PICA-involving cases than in PICA-non-involving cases (6 out of 11 vs 1 out of 5, respectively). Ataxic gait was seen more in medial branch-involving cases than medial branch non-involving cases (5 out of 6 vs 4 out of 10, respectively). One patient died due to obstructive hydrocephalus.
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PMID:[A study of MRI and clinical neurology in acute cerebellar infarcts]. 939 54

A 28-year-old woman initially suffered high fever and headache (day 1). Aseptic meningitis was diagnosed on day 3. Limb ataxia, however, appeared on day 7, and external ophthalmoplegia and drowsiness were recognized on day 8. Urinary disturbance and orthostatic hypotension appeared on day 13. Cerebrospinal fluid showed moderately high cell counts(mononuclear cells, 51/microliter; polynuclear cells, 9/microliter). MRI T2-weighted images showed high intensity lesions in the pons and mesencephalon. No serum anti-GQ1b IgG antibody was detected on day 4. Epstein-Barr virus (EBV) viral capsid antigen-IgG antibody was positive, and EBV determined nuclear antigen antibody was seroconverted. EBV-DNA was detected in the CSF by PCR. These findings indicate prior infection by EBV. After intravenous dexamethasone therapy, these symptoms rapidly disappeared. Our patient showed external ophthalmoplegia, ataxia, and disturbance of consciousness, which are the cardinal signs in Bickerstaff's brainstem encephalitis. The time course of her neurological symptoms, the presence of meningitis, and the MRI findings, however, indicated the pathogenesis of acute disseminated encephalomyelitis rather than Bickerstaff's brainstem encephalitis. We diagnosed this patient as a brainstem encephalitis associated with EBV infection.
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PMID:[A case of brainstem encephalitis associated with Epstein-Barr virus infection: differentiation of acute disseminated encephalomyelitis and Bickerstaff's brainstem encephalitis]. 1172 5

The aim of the present study was to examine somatotopy in the cerebellar cortex and a possible differential role of the cerebellar cortex and nuclei in functional outcome. Clinical findings and 3D MRI-based cerebellar lesions site were compared in a group of 90 patients with focal cerebellar lesion using International Cooperative Ataxia Rating Scale (ICARS) and voxel-based lesion-symptom mapping (VLSM). Separate analysis was performed in patients with acute and chronic ischemic lesions (n=43) and patients with acute and chronic surgical lesions (n=47). Thirty-eight patients were included after resection of a cerebellar tumor in childhood or adolescence. The most significant lesion symptom correlations were observed in the subgroup with acute ischemic lesions. Limb ataxia was significantly correlated with lesions of the interposed (NI) and part of the dentate nuclei (ND), ataxia of posture and gait with lesions of the fastigial nuclei (NF) including NI. Correlations with cortical lesions were less significant and present in the superior cerebellum only. Upper limb ataxia was correlated with lesions of vermal, paravermal and hemispheral lobules IV-V and VI, lower limb ataxia with lesions of vermal, paravermal and hemispheral lobules III and VI, dysarthria with lesions of paravermal and hemispheral lobules V and VI and ataxia of posture and gait with lesions of vermal and paravermal lobules II, III and IV. In the subgroups with chronic focal lesions, similar correlations were observed with lesions of the cerebellar nuclei, but significantly less correlations with lesions of the cerebellar cortex. Functional localization based on VLSM backs findings in previous animal and functional brain images studies in healthy human subjects. The lesion site appears to be critical for motor recovery. Lesions affecting the cerebellar nuclei are not fully compensated at any age and independent of the pathology in humans.
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PMID:Functional localization in the human cerebellum based on voxelwise statistical analysis: a study of 90 patients. 1625 26

Limb ataxia of sudden onset is due to a vascular lesion in either the cerebellum or the brainstem (posterior circulation, PC, territory). This sign can involve both the upper and the lower limb (hemiataxia) or only one limb (monoataxia). The topographical correlates of limb ataxia have been studied only in brainstem strokes. Therefore, it is not yet known whether this sign is useful to localize the lesion within the entire cerebellar system, both the cerebellar hemisphere and the cerebellar brainstem pathways. Limb ataxia was semi-quantified according to the International Cooperative Ataxia Rating Scale in 92 consecutive patients with acute PC stroke. Limb ataxia was present in 70 patients. Four topographical patterns based on magnetic resonance imaging findings were identified: picaCH pattern (posterior inferior cerebellar artery infarct); scaCH pattern (superior cerebellar artery infarct); CH/CP pattern (infarct involving both the cerebellum and the brainstem cerebellar pathways); and CP pattern (infarct involving the brainstem cerebellar pathways). Hemiataxia was present in (47/70; 67.1%) and monoataxia in (23/70; 32.9%) of patients. Monoataxia involved the upper limb in (19/70; 27.1%) and the lower limb in (4/70; 5.7%) of patients. Limb ataxia usually localized the lesion ipsilaterally (picaCH, scaCH, CH/CP, and CP patterns involving the medulla and sometimes the pons) (53/70; 75.7%), but it might be due also to contralateral (CP pattern involving the pons or midbrain) (16/70; 22.9%) or bilateral lesions (1/70). Limb ataxia usually localizes the lesion ipsilaterally but the infarct might be sometimes contralateral. The occurrence of monoataxia may suggest that the cerebellar system is somatotopically organized.
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PMID:Hemi- and monoataxia in cerebellar hemispheres and peduncles stroke lesions: topographical correlations. 2235 51