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Query: UMLS:C0027066 (myoclonus)
 4,275 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

5 patients with cerebral anoxia of various etiology had postanoxic coma with the syndrome of periodic synchronous and sterotyped myoclonus first mentioned by Lance and Adams. These authors observed action myoclonus as a sequel to this syndrome which is rarely survived. Most reports have dealt mainly with its bad prognostic significance. Although the localization of action myoclonus has much been discussed, and is still controversial, little attention has been paid to the possible localizing significance of its acute precursor syndrome. Most patients with this type of myoclonus (all in our series) present a lower pontine/upper oblongata syndrome. The distribution patterns of the jerks are constant, do not represent topographic but functional relationships showing innervation patterns which are presumably integrated in the vestibular system and upper oblongata reticular formation. Interval histograms show that the jerks, unlike those in subacute sclerosing panencephalitis, are not repeated at regular intervals. Several rhythms might be intermingled, or a rhythmic (physiological?) pacemaker could be modified by proprioception of the jerks. The latter possibility is supported by the observation that EEG discharges in one patient became rhythmic when the jerks ceased. The only other instance of rhythmicity was in a very special observation of continuous seesaw alternations of slow jerks of m. levator palpebrae sup., and tonic contractions of m. corrugator glabellae. Rhythmicity in this case had a 1:2 relation to heart rate indicating the nucleus tractus solitarii region as a possible site of origin of the impulses. Our observations indicate an upper oblongata origin for this type of myoclonus. Autopsies in 3 of our cases revealed widespread severe anoxic damage but nothing to add to the pathological localization.
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PMID:Periodic synchronous and stereotyped myoclonus with postanoxic coma. 6 98

Myoclonus is a phenomenon which cuts through a considerable number of neurological conditions. It occurs in a variety of epileptic conditions (Primary generalized epilepsy, hypsarrhythmia, Lennox-Gastaut syndrome, also known as "petit mal variant"), in inborn errors of metabolism (Tay-Sachs disease, forms of ceroid lipofuscinosis), in neurobiochemically still poorly understood forms of degenerative processes such as Essential hereditary myoclonus epilepsy (Lafora-Unverricht-Lundborg), in benign heredo-degenerative disorders (Hartung's syndrome), in CNS infections (SSPE, Jakob-Creutzfeldt disease), in metabolic encephalopathies (renal failure, hypoglycemia), in CNS poisoning, in acute cerebral anoxia and in post-anoxic states. The EEG plays a crucial role in the differential diagnosis of these conditions by the demonstration of a) presence or absence of typical inter-ictal abnormalities, and b) various correlates of the myoclonic ictal event.
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PMID:Myoclonus and the electroencephalogram, a review. 11 May 3

This article gives an overview of electroencephalographic results obtained in the case of subacute sclerosing panencephalitis. Severe disturbances of background activity have been observed in all patients. Repetitive complexes usually occur transitorily, so that they cannot always be detected in individual cases. Repetitive complexes must be regarded as being typical of the disease, and consideration must be given in this connection to their particular characteristics (repetition frequency, constancy of form, relations to disorders characterized by myoclonus) and the clinical picture.
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PMID:[The EEG in subacute sclerosing panencephalitis (SSPE)]. 101 72

Progressive rubella panencephalitis is a very rare slow virus disease of the nervous system. The authors present a case, concerning a young man, aged 20 years, died 11 months after the onset of the disease. The following peculiarities of the case are emphasized: 1) the clinical symptomatology and the evolution (myoclonus, lack of cerebellar impairment) could suggest the diagnosis of SSPE; 2) the EEG recordings showed epileptiform abnormalities, long latency diffuse periodic complexes and--during interferon therapy and simultaneously with a temporary clinical improvement--the appearance of short latency anterior periodic complexes.
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PMID:Progressive rubella panencephalitis. Follow-up EEG study of a case. 129 90

A case of subacute sclerosing panencephalitis (SSPE) was reported. The patient was a 16-year-old boy and he initially developed visual disturbance. His neurologic symptoms were myoclonus, dementia, and visual disturbance which was rare as an initial symptom in SSPE. Fundoscopy revealed bilateral pole chorioretinitis and macular degeneration. Serial MRI study demonstrated the lesions in the brain and right eye ball. The distribution varied as time went on. T2-weighted MR images showed the lesions more clearly than T1-weighted MR images. In this case dementia was marked but the lesions in the cerebrum on the MR images were considerably smaller than expected. Although MR image is useful to show the lesions, the discrepancy between clinical signs and MR images may be present in the early stage in SSPE.
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PMID:[A case of SSPE presenting visual disturbance and serial MRI study]. 139 26

We report here a case of a patient with subacute sclerosing panencephalitis (SSPE) and we have analyzed periodic events using dipole tracing methods to clarify the origin of periodic synchronous discharges and myoclonus. Both source generators were located in the subcortical part of the cerebrum, an area adjacent to the thalamus. Although the pathophysiology of periodic events in SSPE has been controversial, dipole tracing methods may contribute to clarify the origin of periodic events in SSPE.
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PMID:The origin of myoclonus and periodic synchronous discharges in subacute sclerosing panencephalitis. 150 77

A retrospective study of 36 confirmed cases of subacute sclerosing panencephalitis (SSPE) was carried out to detect any variation in the clinical and EEG patterns previously described. There were 31 males and 5 females, aged 4 to 24 years. Onset of SSPE at or after 15 years of age was observed in 22.2% of cases. Rapid progression of the disease was observed in 52%. The first symptom was myoclonus in 61.6%, mental regression in 22.2% and generalised seizures in 11.1% of cases. Low positive measles antibody titres in the CSF were found in 36.1% of cases. EEG analysis revealed slow background in 69.2% and periodic complexes in 94.4% of cases. Atypical periodic complexes, focal abnormalities and paroxysms of bisynchronus sharp activity were also observed.
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PMID:Subacute sclerosing panencephalitis. 181 21

We reported a 15-year-old male patient of subacute sclerosing panencephalitis (SSPE), progressed from stage I to stage IV. On his admission, his clinical state was stage I with disturbance of generalized attention, myoclonus of upper limbs and no focal cortical signs. After rapid progression to stage III in a month, his clinical state became fairly stable and gradually advanced to stage IV. We followed him with serial CT and MR images. On imagings we found initial lesion in left parietoocipital lobe and spread in right parietoocipital lobe, anterior lobe and then brain stem, showing low density on plain CT scans and high intensity of T2 weighed MR images. This initial lesion situated in watershed area was hardly distinguishable from watershed infarction. In addition, we found blood flow decrease in vertebral artery system with SPECT and Doppler flowmetry. These findings suggested vascular pathogenesis of SSPE. We showed the imaging processes of SSPE progression from formation of initial lesion to demyelination and gliosis of total brain.
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PMID:[A case of SSPE that showed watershed infarction like image on MRI initially, and progressed to whole brain atrophy]. 193 70

Clinical signs, laboratory data and electroencephalography were studied in 80 cases of subacute sclerosing panencephalitis. Myoclonus with or without intellectual deterioration was the most frequent symptoms (56,2 p. 100). EEG was one of the more specific tests; diazepam injection made it more sensitive. Level of complement fixing antibodies in the CSF was sometimes low, even in advanced cases (29 out of 70; 41 p. 100). CT was normal in 70 p. 100 of cases, showed cerebral atrophy in 17 p. 100 and some other (uncommon) signs in 13 p. 100 of cases.
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PMID:[Symptoms and clinical signs, laboratory data in 80 cases of subacute sclerosing panencephalitis]. 307 Jun 96

A case of SSPE in a 29-year-old man is presented. The clinical picture was typical with progressive dementia associated with periodic myoclonus. The electroencephalogram showed periodic complexes; the serum and cerebrospinal fluid (CSF) measles antibodies obtained by complement fixation were 1:256 and 1:32 respectively; the CSF gamma globulin was raised (32%) with an oligoclonal distribution. The fundoscopic examination demonstrated a retinal change in maculae and the computed tomography showed cysts and calcifications characteristic of Cysticercus cellulosae. The latter findings probably were not implicated with the SSPE because neurocysticercosis is a very common disease in this region of the country and frequently there are asymptomatic cysts and/or calcifications on CT. The essential clinical and epidemiologic picture of SSPE are discussed and probably the cases with an adult onset had a more rapid outcome to death.
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PMID:[Subacute sclerosing panencephalitis in an adult: report of a case]. 409 42


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