Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0018991 (hemiplegia)
 3,997 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 59-year-old male patient presented in 1991 with left-sided hemiplegia and hemihypesthesia and left homonymous hemianopsia, which had developed for the preceding 12 months. The cerebrospinal fluid (CSF) level of protein was mildly elevated, oligoclonal IgG band was initially negative, but finally positive and myelin basic protein was absent. Magnetic resonance imaging and X-ray computed tomography (CT) of the brain revealed a unilateral demarcated lesion extending around the posterior horn and triagular part of the right lateral ventricle, apparently capped by remarkably gadolinium enhancement at its front, which had no mass effect, and several small isolated plaques were also observed in other areas of the brain. All lesions appeared as high intensity areas on T2 weighted images and hypodense areas on CT. The JC virus hemagglutination-inhibition antibody titers were 1:32 in serum and 1:8 in CSF; the antibody titer ratio and index both suggested an intrathecal antibody production. Pathological examination of brain biopsy specimen taken from the large lesion demonstrated profound demyelination with preservation of axons, a large number of macrophages and spotted perivascular lymphocyte cuffings. Based on this experience, we discussed the features in this case which differentiated it from multiple sclerosis, leukodystrophy and typical progressive multifocal leukoencephalopathy (PML). Diffuse-disseminated sclerosis or transitional sclerosis was most likely so far as the clinical picture, imaging and histology were concerned. However, we implicated the possible involvement of JC virus infection to the etio-pathogenesis of the demyelinating lesions in view of the intrathecal JC virus antibody production in CSF observed in this case.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[A unilateral large demyelinating lesion in the cerebral white matter with slowly progressive hemiplegia and intrathecal JC virus antibody production]. 839 19

A previously healthy 50-year-old man developed aseptic meningoencephalitis with clinical manifestations including fever, headache, seizure, Wernicke aphasia, right hemiplegia, and blindness in the left eye. One and one-half months after remission of meningoencephalitis, marked ataxia and psychiatric symptoms became apparent. Magnetic resonance imaging revealed multiple new lesions involving the basal ganglia, thalamus, white matter, and cerebellum. Despite these developments, cerebrospinal fluid findings continued to improve except for excessive content of myelin basic protein. Within 2 weeks, steroid therapy dramatically resolved the ataxic symptoms and disseminated lesions.
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PMID:Acute disseminated encephalomyelitis following aseptic meningoencephalitis. 1153 55

We report a 47-year-old woman with progressive multifocal leukoencephalopathy (PML). She was a carrier of HTLV-I virus, and developed subacute right hemiparesis and marked motor aphasia. She had a malignant lymphoma in the left neck and basal cell carcinoma in the right inguinal region. Three months after the onset, she became unable to walk because of the right leg weakness or to speak because of motor aphasia. Magnetic resonance imaging (MRI) revealed multifocal T2-high lesions in the white matter of the left frontal lobe, and a brain biopsy revealed demyelinating pathology. A biopsy of the left parotid gland revealed a diffuse pleomorphic type large B cell lymphoma. Although anti-HTLV-I antibody was positive in the serum and cerebrospinal fluid (CSF), no adult T-cell leukemia (ATL) cells were found in the blood or CSF. The patient was then admitted to our hospital. Neurological examinations revealed severe motor aphasia, mild sensory aphasia/cognitive impairment, right hemiplegia, mild right hemihypesthesia, limb-kinetic apraxia in the left hand, idiomotor apraxia, agraphia, perseveration, marked spasticity and brisk tendon reflex in four extremities, and positive bilateral pathological reflexes. MRI showed multifocal T2-high lesions mainly in the cerebral white matter, predominantly in the left hemisphere, and partly in the cerebral cortex. No gadolinium enhancement was found. In addition, 99mTcECD-SPECT showed a broad decrease in cerebral blood flow (CBF) in the cortex. Anti-HTLV-I antibody was positive but anti-HIV antibody was negative in serum. ATL cells were found in 1-3% of the peripheral white blood cells after admission. CSF examination revealed that the cell count (1/microl), protein level (24 mg/dl), and IgG index (0.4) were all normal. However, the myelin basic protein level (321 pg/ml; normal < 102) was increased, JC virus DNA was detected by PCR, and anti-HTLV-I antibody (x 8) was detected in CSF. The regulatory region of the JC virus DNA in the CSF was partly deleted; immunostaining with anti-JC virus protein antibodies revealed the existence of JC virus in biopsied brain specimens, and these findings were consistent with PML. Her symptoms such as motor aphasia, cognitive dysfunction and left hemiparesis were subacutely progressive, and she developed akinetic mutism two weeks after admission. Since the efficacy of cytosine arabinoside for PML has been reported, she was administered 80 mg/day of the drug for five days. After treatment, her communication function was mildly improved but the efficacy was transient. Since it has been reported that HTLV-I, as well as HIV, activates the JC virus promoter and its proliferation, the latent infection of HTLV-I in the central nervous system (CNS) in this case might have stimulated the JC virus proliferation, promoting lesion extension over the cerebral cortex. There have been only a few reports of broad decreases in CBF by SPECT in PML patients. Further MRI and SPECT studies on PML patients are therefore necessary to evaluate the significance of HTLV-I in promoting the JC virus infiltration into the CNS.
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PMID:[A case of progressive multifocal leukoencephalopathy presenting white matter MRI lesions extending over the cerebral cortex and a marked decrease in cerebral blood flow on SPECT, and associated with HTLV-I infection]. 1602 67