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1/4. Laminar cortical necrosis in central nervous system lupus: sequential changes in MR images.

    A 44-year-old woman with systemic lupus erythematosus developed central nervous system lupus presenting with headache, fever, cloudiness of consciousness, and psychotic symptoms. T1-weighted and proton MR images showed laminar high intensity lesions in the parietal and temporal cortex bilaterally. T2-weighted images of the lesions showed low signal intensity. Treatment with corticosteroids alleviated the clinical symptoms within 7 months. The low T2 and high T1 signal abnormalities disappeared in a year and in 5 years, respectively. The laminar cortical lesions on MR images were suggested to represent cortical necrosis associated with central nervous system lupus.
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ranking = 1
keywords = nervous system, central nervous system
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2/4. Neuro-Behcet's disease.

    Behcet's disease (BD) is a multifocal disorder with an immunogenetic basis, which persists over many years. Initial descriptions mentioned oral and genital ulcers with uveitis. Later a number of other manifestations were added, like skin, joint and neurological. The involvement of nervous system (Neuro-Behcet's) is reportedly uncommon. We hereby report four cases of Neuro-Behcet's, i.e.; two cases of strokes involving multiple areas of the central nervous system and two cases had features of benign intracranial hypertension. All cases had mucocutaneous lesions or other system involvement. Cases satisfied the international study group criteria for diagnosis of BD. All cases were pathergy test positive. In comparison with the literature from turkey and greece, which reports a high pathergy positivity, reports from india have shown only few cases to be positive. The prognosis of Neuro-Behcet used to be poor but has recently been improved with reduced mortality, although whether this can be attributed to treatment with steroids and/or cytotoxic agents remains uncertain.
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ranking = 0.28959569765897
keywords = nervous system, central nervous system
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3/4. Neuro-Behcet disease with predominant involvement of the brainstem.

    We report a patient with relapsing-remitting meningoencephalitis secondary to neuro-Behcet disease which resulted in recurrent brainstem encephalitis. MRI revealed increased signal intensity on the proton density and T2-weighted images and gadolinium enhancement during relapses. autopsy revealed acute on chronic meningoencephalomyelitis involving the entire CNS, which was most marked in the brainstem.
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ranking = 0.045836091924652
keywords = brain
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4/4. Sequential antibodies to potassium channels and glutamic acid decarboxylase in neuromyotonia.

    A patient with thymoma-associated neuromyotonia and voltage-gated potassium channel (Kv1.2 and Kv1.6) antibodies by immunoprecipitation and rat brain immunolabeling was treated successfully with immunoadsorption and cyclophosphamide. Curiously, glutamic acid decarboxylase antibodies, absent at onset, appeared later. stiff-person syndrome was absent, but fast blink reflex recovery suggested enhanced brainstem excitability. The range of antibodies produced in thymoma-associated neuromyotonia is richer, and the timing of antibody appearance more complex, than previously suspected.
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ranking = 0.015278697308217
keywords = brain
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