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)

In case 1, 41-year-old male, developed progressive demetia, paretic gait disturbance and pyramidal signs with the duration of three years. The neuropathological study revealed systemic atrophy as type Pick-disease i.e., lobal atrophy in the frontal and the parieto-occipital regions, degenerative changes in the basal ganglia and in the thalamus, nerve cell loss in the substantia nigra and myelin pallor in the pyramidal tract. Lafora-like inclusions were found in the cerebral cortex and in the cochleal nucleus. In case 2, 45-year-old male, showed character change, cerebellar symptomes and mental deteriotation, and ulcers on the oral mucosa during about 15 years long period. Neuropathological examination showed chronic encephalitis in the brain stem, vacuolar change in the neuron in the olivary nucleus and Lafora-like inclusions in the cochlear nucleus. Though neither generalized conversion nor myoclonus were clinicaly observed in these cases, the inclusions showed histochemically strong similarity with that of the Lafora-disease. These Lafora-like inclusions were compared with those in the literatur, which were reported on various disease of CNS. Finally in respect of predilection of the inclusions, it is likely that the inclusions result from same metabolic disturbance in the cochlear neurons in the Lafora-disease as well as in the present cases.
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PMID:[Clinical and histopathological studies of cases of lafora-like inclusion bodies]. 19 69

A progressive neurological illness characterized by myoclonus, motor and sensory deficits, and lethargy occurred in a patient with Hodgkin's disease and was fatal within two months. A focal inclusion cell encephalitis was demonstrated by immunohistological means to be due to measles virus. Measles encephalitis must be considered a potential opportunistic agent in the immune-compromised host.
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PMID:Subacute measles encephalitis complicating Hodgkin's disease in an adult. 21 19

A 10 1/2 years old boy fell brutally ill with a fit followed by confusion, and then by deep coma, with 40 degrees C fever and morbilliform rash. Consciousnesse came back within ten days, with transient Parkinson-like tremor. Myoclonus persisted for about six months. Complete recovery was followed up for six years. A diagnosis of encephalitis was considered on early EEG evidence (stereotyped repetitive sharp wave bursts) and was confirmed by isolation of ECHO 5 virus from brain specimen, and ultrastructural observation of characteristic cytopathic effect (disappearance of organelles, proliferation of smooth membranes) and of probable viral particules in astrocytes, without any inflammatory process.
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PMID:[Curable ECHO 5 virus encephalitis. Clinical, electroencephalographic, virologic and ultrastructural study]. 88 30

A patent, who suffered from nonprogressive athetotic-myoclonic hyperkinesia of the left arm and spastic paresis of the underdeveloped left leg since early childhood, experienced a febrile episode at the age of 23, after which a weakness and ataxia of the right limbs with frequent falling persisted; at the age of 28, he developed a right-sided rigidity, tremor at rest and mask-like face as sequelae of encephalitis disseminata exacerbations. The violent intention myoclonus of the left side of the body could be abolished by stereotactic coagulation in the V.o.p and V.o.a and zona incerta until death 11 days later. The athetotic myoclonic hyperkinesia is the consequence of a left-sided severe status marmoratus of the right putamen with extensive loss of nerve cells and shrinkage. The additional nerve cell loss in the right substantia nigra due to demyelinating encephalitic foci did not produce Parkinson symptoms, because these require a normal striatum. This loss did, however, exaggerate the original hyperkinesia to a violent intention myoclonus, which was abolished by interruption of denatatothalamic afferents to the V.o.p nucleus and of pallidothalamic afferents to the V.o.a nucleus. The Parkinson syndrome of the right side is due to demyelinating foci of different ages. The Parkinson symptoms were manifest on the right side, because the left status marmoratus did not severely reduce the striatal nerve cells. In this case, there is no indication that the introduction of the stereotactic electrode has precipitated new demyelinating foci.
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PMID:Stereotactic treatment of action myoclonus in a case of combined status marmoratus and multiple sclerosis. A contribution to the pathophysiology of basal ganglia with multiple lesions in both the striatum and the substantia nigra. 109 75

A further case of opsoclonus with myoclonus is described. When this syndrome occurs in childhood an associated neuroblastoma should be excluded. In the majority of cases at all ages no underlying disease will be found, although a preceding history of minor upper respiratory or gastrointestinal infection may be elicited, suggesting that a possible encephalitis affecting brain stem mechanisms may be the cause. The prognosis is, as a rule, excellent although full recovery may not occur for many months. Corticosteroids and nitrazepam may have a place in the treatment of severely affected patients with distressing symptoms.
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PMID:Opsoclonus with myoclonus. 121 86

Three cases (case 1, female, aged 30; case 2, male, aged 32; case 3, male, aged 34) of benign brainstem encephalopathy with truncal ataxia were reported. Two patients had prodromal symptoms Neurological examination revealed truncal ataxia in all cases. As additional neurological signs, anisocoria, mydriasis, nystagmus, ptosis, transient opsoclonus, and facial palsy were seen. There was neither drowsiness nor myoclonus in the three cases. On laboratory examinations, cold agglutination test revealed significant elevation in two cases. The examination of cerebrospinal fluid showed a moderate rise of proteins in one case, but did not revealed pleocytosis in any of the cases. Magnetic resonance imaging of one patient revealed an area of high intensity in the left pontine tegmentum by T2-weighed imaging. The prognosis for all these cases was good, and the reappearance of neurological signs was not present until now. Our cases were different from brainstem encephalitis (Bickerstaff's encephalitis) because of an absence of disturbed consciousness and no pleocytosis in the cerebrospinal fluid. Our cases were also different from "myoclonus-opsoclonus syndrome" because of an absence of myoclonus. We discussed a possibility of a new clinical syndrome which we call "benign brainstem encephalopathy with truncal ataxia".
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PMID:[Benign brainstem encephalopathy with truncal ataxia--a clinical study of 3 cases]. 128 89

We report two cases of rhythmic palatal myoclonus (RPM) first presenting with symptoms unrelated to myoclonus and a possible etiology of encephalitis and the second presenting with ear click and palatal myoclonus. The response to treatment was unsatisfactory in both.
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PMID:Rhythmic palatal myoclonus. 130 43

Two cases are reported who developed myoclonus as the only manifestation of a post-infectious syndrome without evidence of encephalitis or the opsoclonus-myoclonus syndrome. Case 1 had generalised myoclonus following an influenza-like illness, while case 2 had right upper limb segmental myoclonus following uncomplicated chicken pox. Neither had any localising neurological signs or abnormality on investigation. Both recovered completely within six months of the onset. Similar cases are reviewed from the literature and it is suggested that such cases be called "isolated" post-infectious myoclonus.
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PMID:"Isolated" postinfectious myoclonus. 146 9

Segmental myoclonus arising in the spinal cord occurs with several viral infections, including herpes zoster radiculitis. Usually, abnormal movements follow the rash and require drug treatment to suppress. We report a patient with AIDS in whom arm and shoulder myoclonus preceded herpes zoster involving the same segments contralaterally on two occasions. Myoclonus remitted promptly with antiviral treatment. Unlike in other immunosuppressed patients, encephalitis did not occur after the second episode.
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PMID:Segmental myoclonus preceding herpes zoster radiculitis. 152 47

A case of sporadic shigellosis is reported. A 30 year old woman, presented neurologic dysfunction, mild watery diarrhea and fever which had started one week before her admission. Abdominal tenderness, confusion, delirium, impairment of orientation and of recent memory were found. Abnormal motor activity with rigidity, tremors, myoclonus around the mouth also existed. There were neither focal signs nor signs of meningeal irritation. Computed tomography and nuclear magnetic resonance of the brain were normal. Laboratory analyses did not explain the anomalies. Several deep ulcers were disclosed by colonoscopy and a S. flexneri was isolated from the material extracted by endoscopy. Nonspecific colitis was informed by pathology. The patient was treated with 400 mg ciprofloxacin for 14 days, with improvement of the clinical picture. The most prominent aspect in this case was the clinical presentation with predominant neurologic symptoms creating the impression of a primary nervous system disease. Admission diagnosis was viral encephalitis. We believe that all of the anomalies present in our patient, the diarrhea and encephalopathy, were produced by the same etiologic agent, S. flexneri and by its toxins.
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PMID:[Encephalopathy in an adult with Shigella]. 192 94


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