Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0027066 (myoclonus)
 4,275 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Subacute sclerosing panencephalitis is an almost universally fatal late complication of measles infection for which there is no established treatment. We report a patient with subacute sclerosing panencephalitis who was bed-bound and ataxic and had a left hemiparesis and frequent myoclonus. He was started on a new regimen consisting of intraventricular interferon-alpha (starting at 100,000 U/m2/day, building up to 1 million U/m2/day), ribavirin (60 mg/kg/day intravenously), and inosiplex (3 g/day) and improved markedly. At 10 weeks, the intraventricular reservoir was removed because of bacterial infection, and he was discharged home on oral ribavirin (1200 mg/kg/day) and inosiplex. He continued to improve as judged by neurologic examination, functional independence measurement, neuropsychometry and single photon emission computed tomography (SPECT) imaging. However, after 10 months, he deteriorated suddenly and died before further intraventricular treatment could be instituted. Further trials are needed to evaluate long-term combination therapy in subacute sclerosing panencephalitis.
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PMID:Treatment of subacute sclerosing panencephalitis with interferon-alpha, ribavirin, and inosiplex. 1250 50

From 1972 to 2002, we diagnosed and treated 22 cases of subacute sclerosing panencephalitis. We report on two pediatric patients with fulminant subacute sclerosing panencephalitis who had atypical clinical manifestations. In both patients diagnosis was confirmed by elevated titers of CSF and serum antimeasles antibodies. Patient 1 presented with behavioral disorder, dysarthria, and drop attacks, while Patient 2 presented with partial complex seizures. Mental difficulties, personality changes, or myoclonus were not noticed in Patient 2. In both our patients stage I was not prominent, and stage II was of shortened duration. In spite of treatment with isoprinosine and interferon-alpha, both our patients deteriorated rapidly and died 2.5 and 4 months, respectively, after the onset of neurologic symptoms. Both atypical presentation and rapid clinical course observed in our patients could cause problems in making final diagnosis of subacute sclerosing panencephalitis. Therefore, subacute sclerosing panencephalitis should be included in differential diagnosis of acute unexplained encephalopathic diseases.
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PMID:Fulminant subacute sclerosing panencephalitis:two cases with atypical presentation. 1367 25

We report on a man who received interferon-alpha 2a therapy for kidney cancer and who subsequently developed propriospinal myoclonus. The myoclonus was noted at rest and during movement. The jerks were reinforced by cutaneous stimuli and tendon taps and spread to the spinal cord via polysynaptic propriospinal pathways. Cerebrospinal fluid analysis, spinal cord magnetic resonance imaging, electroencephalogram with back-averaging, and somatosensory-evoked potentials were normal. No antineuronal antibodies were found. Although the mechanism of interferon neurotoxicity remains unclear, the possible responsibility of interferon was considered, as no focal lesion or paraneoplastic pathology were disclosed.
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PMID:Atypical propriospinal myoclonus with possible relationship to alpha interferon therapy. 1467 3