Journal of neurology
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Journal of neurology · Aug 2012
Case ReportsGlycine receptor antibodies are detected in progressive encephalomyelitis with rigidity and myoclonus (PERM) but not in saccadic oscillations.
Glycine receptor (GlyR) antibodies were recently identified in a few patients with progressive encephalomyelitis with rigidity and myoclonus (PERM); none of these patients had antibodies against glutamic acid decarboxylase (GAD). An inhibitory glycinergic transmission defect has also been implicated in the mechanism underlying saccadic oscillations, including ocular flutter or opsoclonus; GlyR antibodies have not been reported in these patients. The purpose was to determine whether GlyR antibodies are found in patients with PERM, ocular flutter syndrome (OFS), and opsoclonus-myoclonus syndrome (OMS). ⋯ GlyR antibodies were not found at significant levels in 13 patients with saccadic oscillations. GlyR and GAD antibodies can co-exist in PERM and follow the clinical course. Although saccadic oscillations are a feature of this condition, GlyR antibodies are not commonly found in patients with isolated saccadic oscillations.
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Journal of neurology · Jul 2012
Multicenter StudyIs adjunctive corticosteroid beneficial in pneumococcal meningitis in a region with high rates of resistance to penicillin and ceftriaxone?
The role of adjunctive corticosteroids remains controversial in meningitis by penicillin-resistant pneumococci. We determined the effect of adjunctive corticosteroids in adults with pneumococcal meningitis in a region with a high rate of penicillin resistance. A multicenter, retrospective cohort study was conducted between 1998 and 2008 in Korea. ⋯ Multivariate analysis showed that adequate corticosteroids did not reduce mortality (HR 0.773, 95% CI 0.293-2.040) and neurologic sequelae (HR 0.604, CI 0.262-1.393). Propensity-adjusted analysis showed that adjunctive corticosteroid was not associated with time to death (HR 0.949, CI 0.374-2.408), however, a decreasing tendency was shown in neurologic sequelae in the adequate corticosteroid group (HR 0.479, CI 0.207-1.110). In conclusion, adjunctive corticosteroids did not affect mortality in adults with pneumococcal meningitis in a region with high rates of resistance to penicillin and ceftriaxone; however, the patients receiving adequate corticosteroid therapy tended to develop neurologic sequelae less frequently.
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Journal of neurology · Jun 2012
Deficient sustained attention to response task and P300 characteristics in early Huntington's disease.
Evidence for the extent and nature of attentional impairment in premanifest and manifest Huntington's disease (HD) is inconsistent. Understanding such impairments may help to better understand early functional changes in HD and could have consequences concerning care for HD patients. We investigated attentional control in both early and premanifest HD. ⋯ Subjects with early HD showed a reduced capacity to effectively control attention. They proved unable to resume the task directly after having made an error, and need more time to return to pre-error performance levels. No attentional control deficits were found for the premanifest HD group.