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)

There are three different neurological complications of measles infections in the brain: acute postinfectious encephalitis, acute progressive infectious encephalitis, and subacute sclerosing panencephalitis. The diagnosis of measles encephalitis (ME) is established when supported by the clinical picture, mainly of juvenile onset, and confirmed by the presence of cerebrospinal measles antibodies. Although ME is clinically characterized by progressive behavioral and mental deterioration associated with myoclonus, prior reports have suggested that adult-onset may have atypical features. We describe a 28 year-old immunocompetent man, admitted into the hospital due to a rapid motor and cognitive decline after an episode of fever and gastroenteritis. His neurological examination was significant for cognitive impairment, cervical dystonia, spontaneous and action induced myoclonus, choreiform movements, parkinsonism and ataxic gait. He was diagnosed of acute postinfectious ME based on the presence of elevated intrathecal synthesis of measles antibodies in his CSF, and a lymphocytic infiltrate of perivascular distribution without viral inclusions, with PCR negative for measles from brain biopsy. The patient continued to deteriorate to an akinetic mutism state, dying a few weeks later. Adult-onset ME is an entity rarely seen in the Western world. Although myoclonus is the most common movement disorder related to juvenile-onset ME, ataxia and other dyskinesias such as chorea, dystonia, and parkinsonism, can result from this infection when presenting in adult life.
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PMID:[Movement disorders in adult-onset measles encephalitis]. 1259 Mar 79

Subacute sclerosing panencephalitis (SSPE) is a rare entity with an invariably fatal course that progressively affects the central nervous system. It is caused by persistent infection with the wild-type measles virus. While rare in industrial countries, it is not infrequent in developing countries, where there are still areas of endemic measles infection and immunization is not yet generalized. We describe an eight-year-old Spanish girl who presented rhythmic and symmetric myoclonus. She contracted measles at 13 months and required hospitalization. No cognitive deterioration was found. Neuroimaging and the initial electroencephalogram were normal. Oligoclonal bands and high titers of measles antibodies were found in serum and cerebrospinal fluid. She was treated with oral metisoprinol and intraventricular alpha-interferon (IFN-) and showed no further progression of her symptoms. The importance of including SSPE in the differential diagnosis of patients consulting for school failure, neurological deterioration or movement disorders is highlighted. Special attention should be paid to the immigrant population from countries where the incidence of SSPE is greater than in Spain.
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PMID:[Subacute sclerosing panencephalitis: a still existing disease]. 1278 Nov 17

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

This investigation reports the prevalence and clinical profile of subacute sclerosing panencephalitis in two developed cities of southern China. A territory-wide survey was conducted to identify all subacute sclerosing panencephalitis cases diagnosed during 1988-2002 in Hong Kong and Macau. Altogether, 10 cases (male:female = 7:3) were identified of whom six were still alive. The prevalence rate of subacute sclerosing panencephalitis in Hong Kong and Macau in 2002 was 1 per million total population or 5.5 per million children. The mean age of presentation was 9.4 years (range = 4-14 years). Presenting features included myoclonus (60%), deterioration in school performance (30%), and transient visual impairment (10%). The clinical course was highly variable. Most had subacute course, but two deteriorated rapidly and died within 6 months. Seven children had measles infection, and the majority of infection (86%) occurred during the world measles epidemic in 1988. The mean interval between measles infection and onset of subacute sclerosing panencephalitis was 6.5 years (range = 3-11 years). There has been an increasing trend of subacute sclerosing panencephalitis in southern China after the measles outbreak in 1988. Active surveillance of subacute sclerosing panencephalitis for those with measles infection during the 1988 outbreak is necessary to conduct multicenter drug trials for this devastating disease.
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PMID:Subacute sclerosing panencephalitis in children: prevalence in South China. 1524 92

This report describes an atypical case of rapidly progressive subacute sclerosing panencephalitis presenting as transient visual agnosia and myoclonus in a 14-year-old male. There were no typical periodic complexes in serial electroencephalographic monitoring; cerebrospinal fluid measles antibody titer was negative. The diagnosis was made by molecular and histologic examination of open brain biopsy tissue.
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PMID:Acute fulminant subacute sclerosing panencephalitis with absent measles and PCR studies in cerebrospinal fluid. 1535 Oct 25

Subacute sclerosing panencephalitis can show variations in clinical course, and some ophthalmologic abnormalities can be seen as cortical blindness and optic atrophy. A 4-year-old girl was referred to our hospital with a complaint of diplopia, vomiting, and ataxia. On physical examination, she was found to have stage IV papilledema with retinal hemorrhage. She was diagnosed as having idiopathic intracranial high pressure until magnetic resonance imaging demonstrated T2-weighted hyperintense lesions. After observation of head drop attacks and detection of elevated antimeasles antibodies in cerebrospinal fluid, the diagnosis of subacute sclerosing panencephalitis was established, and isoprinosine and carbamazepine were started for treatment. However, because carbamazepine failed to control the head drop attacks, topiramate was also included, and the attacks were kept under control with topiramate. The case presented in this article is a good example of subacute sclerosing panencephalitis in which, at early stages, some of the signs and symptoms can lead to an erroneous diagnosis. In addition, we have demonstrated that topiramate might be a good choice for treatment for the persistent myoclonus seen in this type of patient.
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PMID:Unusual manifestation of subacute sclerosing panencephalitis: case with intracranial high-pressure symptoms. 1552 63

Visual-spatial agnosis, praxis deficits and hallucinations can be the features of subacute sclerosing panencephalitis (SSPE) in the early period. This study describes a 15-year-old boy with SSPE presenting with visual agnosia, prosopagnosia, simultanagnosia, optic ataxia, and oculomotor apraxia which are compatible with Balint syndrome. MRI revealed heterogenous and abnormal signal changes in the bilateral parieto-occipital areas. The signals were more prominent on the left. The case discussed herein is important because he was diagnosed as Balint's syndrome by means of clinical and neuroradiological findings before the onset of known symptomatology as dementia/myoclonus of SSPE. The case is also illustrative of the need to emphasize the prompt evaluation of a patient's cortical features before clinical progression becomes apparent. This evaluation should be performed in the early period if correct diagnosis is to be reached.
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PMID:Subacute sclerosing panencephalitis presenting with Balint's syndrome. 1637 13

Clinical and laboratory characteristics of 39 patients with adult onset subacute sclerosing panencephalitis (SSPE) are described and compared to those of juvenile onset patients regarding preceding measles, age at onset, gender, interval between onset and diagnosis, clinical profile, and course during follow up. Diagnosis was based on clinical and electroencephalographic findings and raised anti-measles antibody titres in cerebrospinal fluid. Mean age at SSPE symptom onset was 20.9+/-4.9 years and mean interval from onset to diagnosis was 6.3+/-9.6 months. Referral diagnosis was accurate in only 12 patients. Presenting symptoms included myoclonus, behavioural changes, seizures, and cognitive, visual, and extrapyramidal disturbance. All patients received symptomatic therapy; 19 also received disease modifying agents. Five of seven pregnant women had successful deliveries. The follow-up period varied widely (maximum 60 months, median 9 months). The profile of adult onset SSPE did not differ from the rest of the cohort, except for a longer interval between measles infection and symptom onset (p<0.0001). SSPE in adults poses diagnostic challenges for clinicians. A high index of suspicion and appropriate investigations are necessary for early diagnosis and counselling.
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PMID:Adult onset subacute sclerosing panencephalitis: clinical profile of 39 patients from a tertiary care centre. 1646 98

A 20-year-old woman with subacute sclerosing panencephalitis, bedridden because of dystonic posturing and very frequent myoclonic involuntary movements, improved dramatically with carbamazepine. The favorable effect of the carbamazepine on myoclonus was demonstrated by discontinuing and reintroducing carbamazepine, and videoing the consequent reappearance and disappearance of the myoclonus. [Published with video sequences].
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PMID:Myoclonus relieved by carbamazepine in subacute sclerosing panencephalitis. 1656 32

The clinical features of subacute sclerosing panencephalitis (SSPE) include the following : stage I, behavioral changes; stage II, myoclonus and motor deterioration; stage III, mental and motor deterioration to coma; stage IV, a vegetative state. Anti-measles titers of serum and cerebrospinal fluid (CSF) are elevated in the patients with SSPE. CSF IgG levels and IgG index are also elevated in SSPE. The characteristic electroencephalography in stage II of SSPE shows periodic high voltage slow wave bursts. In the early stage, psychiatric diseases and epilepsy should be ruled out. Neurodegenerative diseases and other encephalitis/encephalopathy should be also excluded.
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PMID:[Clinical course and findings in and differential diagnosis of subacute sclerosing panencephalitis]. 1769 87


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