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

A 20-year-old man who had developed involuntary movement of his left hand and memorial disturbance visited our hospital in December, 1991. On admission, myoclonus, dementia and speech disturbance were recognized. He was diagnosed as subacute sclerosing panencephalitis (SSPE) based on a high titer of serum anti-measles antibody (1/256), serum anti-measles-IgG antibody (> 1/4,800) and typical EEG fiding of periodic synchronus discharge (PSD). Inosine pranobex was administrated orally (4,800mg per day). Serial cranial magnetic resonance imagings (MRI) were taken since January, 1992 to June, 1994. No abnormal finding was demonstrated until April 16, 1992 in MRI, but 123I-IMP SPECT detected decreased accumulation in parietal to occipital lobes on early image in February 5, 1992. Marked high signal area on T2 weighted image in right temporal lobe and parieto-occipital lobe were firstly demonstrated in June 22, 1992 on MRI. These high signal lesions alternated the areas and locations, but the changes were not related to his clinical symptom. These findings may suggest ischemic changes after demyelination. His symptoms have been improving gradually since June, 1994. To our knowledge, 42 cases of adult-onset SSPE were reported so far (5 were in Japan). This case is the first report in the world on adult-onset SSPE serially observed with MRI and SPECT since early stage.
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PMID:[Serial changes of MRI and SPECT findings in a case of adult-onset SSPE]. 872 Mar 31

We studied the value of long video-split electroencephalographic monitoring (VSEEG) in detecting myoclonus in nearly SSPE and evaluated the natural history and outcome-affecting factors. The 32 newly diagnosed patients had VSEEG to detect myoclonus and its correlations with EEG periodic complexes. Disease progression was monitored by a special "outcome score'; the chi-square test and multi-variable statistics analysed the outcome score in relation to different variables, such as age at onset, sex, duration of symptoms at presentation, CSF measles antibody titre, type and interval between periodic complexes (EEG discharges). Myoclonus or atonia occurred in all patients and was time-related to the EEG periodic complexes; in 32% of patients, myoclonus or atonia were not clinically evident. The EEG periodic complexes were of 3 types: Type I (16 patients) periodic giant delta waves; Type II (10 patients) periodic giant delta waves intermixed with rapid spikes or fast activity; and Type III (6 patients), long spike-wave discharges interrupted by giant delta waves. Outcome score was associated with symptoms duration (P < 0.01) and EEG periodic complexes (P < 0.05). Symptom duration was inversely related to final outcome (multi-variable analysis). Long VSEEG monitoring greatly improves early diagnosis and detection of subtle atonia or segmental myoclonus. Prognostic factors were the type of EEG periodic complexes and duration of symptoms at presentation.
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PMID:Subacute sclerosing panencephalitis (SSPE): early diagnosis, prognostic factors and natural history. 885 57

Subacute sclerosing panencephalitis is characterized by the insidious onset of diffuse cerebral dysfunction associated later with myoclonus and typical electroencephalographic changes. The disease progresses relentlessly to coma and death within 2 years. We report a case of acute onset and rapid course associated with atypical CSF, EEG and MRI features simulating acute disseminated encephalomyelitis. Brief review of relevant literature is presented.
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PMID:Subacute sclerosing panencephalitis presenting as acute disseminated encephalomyelitis: a case report. 907 90

Subacute sclerosing panencephalitis (SSPE) is a persistent viral infection of the central nervous system due to a defective measles virus. Beginning with progressive cognitive malfunctioning, myoclonus and other less specific neurologic signs and symptoms, it usually evolves to a vegetative, decorticated state and death. Disease course and characteristics can be highly variable. Since the widespread measles vaccination in the Western countries started in the late seventies and early eighties, the incidence of SSPE has dropped significantly. Also adult onset is more frequently seen which changes the field of differential diagnosis. In this article 3 atypical cases of SSPE are described with peculiarities such as adult onset, pronounced extrapyramidal involvement, remission and prolonged disease course. Common features of the disease are emphasized to enable accurate diagnosis which is based on clinical features, presence of high titers of serum and cerebrospinal fluid (CSE) measles antibodies, MRI and EEG.
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PMID:Prolonged and atypical course in some cases of subacute sclerosing panencephalitis. 910 44

Subacute sclerosing panencephalitis (SSPE) is mainly thought of as a disorder of childhood and adolescence and may not be readily recognized when presenting later in life. Prior reports have suggested that adult-onset SSPE may have atypical features. We have added two cases to the existing literature on adult-onset SSPE, compared them with a more classic juvenile presentation, and extensively reviewed those reports that were published after the etiological link with the measles virus had been established. Adult-onset SSPE patients present at a mean age of 25.4 years (range 20-35 years). They have a higher proportion of either negative history of measles exposure or undocumented history by the reporting authors. Those with available history of measles exposure tend to have it either earlier (younger than 3 years old) or later (after 9 years) than the usual childhood measles infection. Where the primary infection is known, unusually long measles-to-SSPE intervals have been documented, ranging from 14 to 22 years. None of the cases followed measles vaccination. Visual symptomatology was very frequent, with 8 of the 13 cases reviewed having a purely ophthalmological presentation; only 2 patients presented with behavioral changes. Although the course of the disease was progressive and fatal in the majority, there appeared to be a higher rate of spontaneous remission as compared with childhood-onset SSPE. Myoclonus, spastic hemiparesis, bradykinesia, and rigidity were the predominant motor manifestations. Neuropathology revealed cortical and subcortical gray matter involvement preferentially of the occipital lobes, thalamus, and putamen. The importance of recognizing the spectrum of potential presentations of SSPE and providing an early diagnosis will increase as more effective treatments become available.
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PMID:Adult-onset subacute sclerosing panencephalitis: case reports and review of the literature. 915 29

Subacute sclerosing panencephalitis (SSPE) is an inflammatory neurodegenerative disease related to the persistence of measles virus. Although its frequency is declining because of measles eradication, we still have some cases being diagnosed. With the aim to describe epidemiological aspects of SSPE in Brazil, we sent a protocol to Child Neurologists around the country, 48 patients were registered, 27 (56%) were from the southeast region, 34 (71%) were male and 35 (73%) white, 27 (56%) had measles, 9 (19%) had measles and were also immunized, 7 (14%) received only immunization, 1 patient had a probable neonatal form. Mean time between first symptoms and diagnosis was 12 months (22 started with myoclonus or tonic-clonic seizures, 7 (14%) with behavioral disturbances); 36 patients (75%) had EEG with pseudoperiodic complexes. Follow up performed in 28 (58%) patients showed: 12 died, 2 had complete remission and the others had variable neurological disability. Our data shows endemic regions in the country, a high incidence of post-immunization SSPE and a delay between first symptom and diagnosis.
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PMID:Subacute sclerosing panencephalitis. Clinical aspects and prognosis. The Brazilian registry. 1041 14

A 5-year-old boy presented with an acute ataxia and altered mental status. Although he initially recovered from these symptoms, he presented a second time with myoclonus and seizures and rapidly became vegetative. Cerebrospinal fluid studies, magnetic resonance imaging, and brain biopsy all confirmed the presence of subacute sclerosing panencephalitis. Despite courses of therapy with cimetidine, amantadine, ribavirin, and inosine, no clinical improvement has been seen. Clinicians need to be alert to the possibility of subacute sclerosing panencephalitis even in the vaccinated child in the appropriate clinical setting.
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PMID:Subacute encephalopathy in a 5-year-old boy. 1052 34

To clarify the mechanism of periodic dystonic myoclonus in subacute sclerosing panencephalitis (SSPE), a 22-year-old patient with a clinical diagnosis of SSPE was electrophysiologically investigated. Involuntary movements consisted of generalized dystonic posturing which occurred quasiperiodically once every 4 to 8 seconds. Effects of sensory stimuli and voluntary movements were studied by means of polygraphic recording of surface electromyogram (EMG), scalp electroencephalogram (EEG), and magnetoencephalogram (MEG). EEG showed quasi-periodic, generalized, transient complexes synchronous to each dystonic myoclonus, which were preceded by a slow negative EEG shift at the parietal region by approximately 5 seconds. Neither external stimuli nor self-paced movements alone influenced the periodicity of dystonic myoclonus or EEG complexes. In the reaction time task, however, the external stimuli given as an imperative cue to execute a motor task elicited dystonic myoclonus and generalized EEG complexes only if they were presented in the latter segment of the interval between the two successive EEG complexes while the slow negative EEG shift appeared. These findings suggest that EEG complexes and periodic movements spontaneously occur when cortical excitability reaches a certain critical level, but both phenomena are elicited even before if the sensory stimuli as an imperative signal requiring motor execution are presented. This finding most likely implies involvement of the sensorimotor integration mechanism in these periodic phenomena.
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PMID:Implication of sensorimotor integration in the generation of periodic dystonic myoclonus in subacute sclerosing panencephalitis (SSPE). 1110 2

Subacute sclerosing panencephalitis (SSPE) is a progressive neurological disorder of childhood and early adolescence. It is caused by persistent defective measles virus. Brain biopsies or postmortem histopathological examination show evidence of astrogliosis, neuronal loss, degeneration of dendrites, demyelination, neurofibrillary tangles, and infiltration of inflammatory cells. Patients usually have behavioral changes, myoclonus, dementia, visual disturbances, and pyramidal and extrapyramidal signs. The disease has a gradual progressive course leading to death within 1-3 years. The diagnosis is based upon characteristic clinical manifestations, the presence of characteristic periodic EEG discharges, and demonstration of raised antibody titre against measles in the plasma and cerebrospinal fluid. Treatment for SSPE is still undetermined. A combination of oral isoprinosine (Inosiplex) and intraventricular interferon alfa appears to be the best effective treatment. Patients responding to treatment need to receive it life long. Effective immunisation against measles is the only solution presently available to the problem of this dreaded disease.
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PMID:Subacute sclerosing panencephalitis. 1180 85

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


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