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

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

The effects of rapid intracarotid injection of 20 to 100 mg of sodium amobarbital were studied in three patients with bilateral myoclonic status epilepticus due to Jakob-Creutzfeldt disease, sequelae to anoxic encephalopathy, and hepatic coma, respectively. In each instance, the drug produced prompt abatement of clonic jerks contralaterally and attenuation of electroencephalographic epileptiform discharges ipsilaterally. These results suggest that the cerebral cortex actively participates in the elaboration of certain types of bilateral myoclonus in human beings.
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PMID:Electrographic and clinical effects of intracarotid sodium amobarbital on bilateral myoclonic status epilepticus. 32 84

Serial EEG studies and full neuropathological investigations (optic and electronic microscopy of biopsy and necropsy material) were carried out on two patients: 1. A 68-year-old man: development in two and a half months of Creutzfeldt-Jakob's disease signalled by early clinical and EEG changes and confirmed by associated spongiosis of the triad characteristic of Alzheimer's disease, which was unexpected in this case. 2. A 43-year-old man: first phase of four years of progressive deterioration, followed by an encephalopathic syndrome with myoclonus developing in twelve months. The serial EEG studies showed discontinuous periodic paroxystic activity from the start of the second phase of the disease, although the first biopsy still showed nothing but the signs of Alzheimer's disease. A fortnight later, a second biopsy revealed ultrastructural microspongiosis. Examination of necropsy material confirmed the extensive association of the characteristic images of the two processes. On the basis of these two case studies and some similar cases published in the literature, the authors discuss the possible etiological links between these two diseases and stress the importance of the EEG and cerebral biopsy for the purposes of differential diagnosis. (Acta neurol. belg., 1977, 77, 202-212).
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PMID:[Association of Alzheimer's disease and Creutzfeldt-Jakob's disease (author's transl)]. 33 54

A 51-year-old man who died of Creutzfeldt-Jakob disease (CJD), had transient dyskinesias with intention myoclonus and exaggerated startle reaction in early life. This may suggest a link between myoclonic encephalopathy of infants and CJD, and an incubation period of more than 40 years of the transmissible agent of CJD.
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PMID:Creutzfeldt-Jakob disease. Infection of infancy or childhood? 37 67

The clinico-pathological features of 4 cases of Creutzfeldt-Jakob disease, a transmissible virus dementia, are reported. The onset of the disease varied between 54 and 81 years of age: the course is dramatic and the demise occurs 1-4 months after the onset of symptoms. The clinical picture includes mental deterioration with amnesia, aphasia, apraxia and neurologic symptoms, mainly consisting in progressive rigidity and myoclonus. All cases show the typical 1/sec pseudorithmic spikes on EEG. Histological changes include severe astrocytosis of the cerebral cortex, status spongiosus and mild neuronal loss. The changes are more marked in the parieto-occipital lobes. Criteria of clinical and pathological diagnosis are reviewed. The precautions to be taken in handling biological as well as bioptic and autoptic specimens are emphasized, in order to avoid accidental transmission of the disease. It seems essential to institute a register of cases of Creutzfeldt-Jakob disease in Italy to assess the incidence and diffusion of the disease in our country.
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PMID:[Four cases of Creutzfeldt-Jakob's disease (author's transl)]. 38 1

We present two patients aged 66 and 69, with a rapidly progressive disease (10 and 15 months' duration) in which the presenting symptom was instability of gait. Later dementia was also a prominent feature. One case had myoclonus. Repeated EEGs showed symmetrical slowing in one case and periodic generalised bursts of triphasic waves at 1 cps superimposed upon a slow (3-4 cps) background activity in the other. The pathological findings consisted of classical Creutzfeld-Jakob disease (CJD), Kuru plaques (KP) were disseminated in the brain, but were more numerous in the cerebellum, putamen and thalamus. Neurons with large vacuoles in the cytoplasm were numerous in the putamen, thalamus and anterior horns. Stress is laid upon the common findings in both CJD and Kuru (K) (clinical features, pathological data, lack of antibody response, transmissibility, change in pattern on transmission). The possibility of a common origin of the two diseases is discussed.
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PMID:Kuru plaques in the brain of two cases with Creutzfeldt-Jakob disease. A common origin for the two diseases? 39 Jan 1

One hundred twenty-four consecutive cases of Creutzfeldt-Jakob disease (CJD) in France, verified by biopsy or autopsy between 1968 and 1977, were analyzed with respect to their clinical characteristics. The series comprised equal numbers of men and women, with the most frequent age at onset being 60 to 64 years and the most frequent duration, two to three months. A prodromal illness was observed in more than one-third of the patients. Clinical presentations and symptom frequencies are tabulated, and a multifactor analysis has been performed to obtain those combinations of symptoms and signs which occurred at least as often as the triad of dementia, myoclonus, and a positive electroencephalogram. Two groups of atypical cases are also emphasized: one with sudden, strokelike presentation and rapidly evolving illness of less than 2 months' duration, and the other with a long clinical course of between 2 and 10 years.
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PMID:Creutzfeldt-Jakob disease in France: II. Clinical characteristics of 124 consecutive verified cases during the decade 1968--1977. 39 Nov 41

A few autopsy proved cases of Alzheimer's disease with myoclonus have been hitherto reported. We think that myoclonus is a frequent clinical feature in advanced cases of Alzheimer's disease. Our second case is such an example with a chronic evolution. In cases like our first one, with a short history, myoclonus, and atypical, diphasic, periodic complexes in the EEG, clinical differential diagnosis with Creutzfeldt-Jakob disease can be very difficult. Alzheimer disease has been considered an unitary clinico-pathological entity. However, transmission to the non human primates has been successfully achieved only in familiar cases but no in the sporadic ones. On the other hand some of his neuropathological features have been found in two cases of Creutzfeldt-Jakob disease, one of them successfully transmitted to the chimpanzee. All these points could eventually modify our present unitary concept on Alzheimer's disease.
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PMID:[Myoclonus in Alzheimer's disease. 2 anatomoclinical cases]. 39 58

Myoclonus in association with dementia of later adult life has been considered almost pathognomonic of Creutzfeldt-Jakob disease. However, myoclonus may also be seen with Alzheimer disease, and when the myoclonus occurs as an early manifestation of Alzheimer disease, distinction from Creutzfeldt-Jakob disease may prove difficult.
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PMID:Myoclonus in Alzheimer disease. A confusing sign. 76 61

A patient with progressive neurological deterioration characterized by cognitive impairment, myoclonus, Parkinson's syndrome, an abnormal electroencephalogram and fasciculations was considered for brain biopsy for suspected Creutzfeldt-Jakob disease. Complete clinical recovery followed discontinuation of lithium and nortriptyline. Awareness of this unusual drug-induced Creutzfeldt-Jakob like syndrome can avoid costly, invasive and unnecessary investigative procedures.
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PMID:Drug-induced Creutzfeldt-Jakob like syndrome. 139 Jun 20


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