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)

This is a case of Ramsay Hunt syndrome with mental disorder. The patient had action myoclonus, grand mal seizure and severe cerebellar ataxia. Schizophrenia-like symptoms including delusion of persecution and self-reference, auditory hallucination and incoherence were characteristically observed before the neurological disturbance became manifest. Subsequently, euphoria, disinhibition, moria and mild dementia appeared with neurological symptoms. The possibility of Ramsay Hunt syndrome to accompany organic mental syndromes and the relationship between cerebellar dysfunction and psychiatric symptoms are discussed.
Jpn J Psychiatry Neurol 1991 Dec
PMID:Ramsay Hunt syndrome with mental disorder. 181 81

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.
J Assoc Physicians India 1991 Dec
PMID:Subacute sclerosing panencephalitis. 181 21

Muscle pain occurs in various neuromuscular disorders with characteristic physiological or biochemical abnormalities. There is, however, a group of patients in whom there is no clear physiological or structural basis for their pains. This syndrome has been called fibrositis or fibromyalgia. Sleep abnormalities have been reported in some of these patients, but have not been confirmed by others. We studied 8 patients with this disorder and found sleep abnormalities that were characterized by nocturnal myoclonus, alpha-delta sleep, and abnormalities compatible with depression. Polysomnography was, therefore, instrumental in helping direct the treatment of these patients. Therapeutic approaches aimed to correct the specific disorders were effective in improving the pain symptoms.
Arq Neuropsiquiatr 1991 Dec
PMID:Polysomnography in idiopathic muscle pain syndrome (fibrositis). 184 93

Two patients with hitherto unrecognized spells of involuntary movements in legs are described. They occurred on dropping off to sleep, were preceded by a burning sensation and resembled myoclonus. Subcutaneous injection of epinephrine reproduced the spells. Neurogenic bladder of uninhibited type was noted on cystometry. Epidural or sympathetic nerve block of the lumbar region relieved all the symptoms including the neurogenic bladder. A few minutes before the spells, a rise in blood pressure and pulse rate was observed in one of the cases. Pathophysiological resemblance to painful legs and moving toes was pointed out, but more widespread involvement of the spinal cord was suspected.
J Neurol Sci 1990 Dec
PMID:Myoclonus with burning sensation in legs that remits with sympathetic blockade. 196 4

2'-Deoxycoformycin (DCF) is an experimental drug with specific lymphocytotoxic activity which has proved effective in the treatment of some lymphoproliferative syndromes (LPS). This paper deals with the results achieved in 6 patients with LPS, five of them with T-cell and one with B-cell malignancies, who received DCF as initial (2 cases) or salvage (4 cases) therapy. One patient with adult T-cell leukaemia-lymphoma achieved complete remission maintained for 36 months of follow-up; partial remission was attained in three others and the remaining two patients failed to respond to the treatment. Kidney toxicity was seen in three cases, and in two of them it was necessary to stop the treatment. Kidney toxicity was seen in three cases, and in two of them it was necessary to stop the treatment. Other untoward effects included nausea and vomiting (2 patients), myoclonus, dysaesthesia of the limbs and conjunctivitis arida (one case each). No haematologic or infectious complications were present in association with DCF treatment.
Sangre (Barc) 1990 Dec
PMID:[Treatment of various lymphoproliferative syndromes with deoxycoformycin: results in 6 patients]. 208 59

We report 11 adults who exhibited myoclonic status epilepticus (MSE) after cardiac arrest. Based on pathologic, electroencephalographic, and clinical evidence, we conclude that our patients died from the initial anoxic-ischemic insult rather than as a result of MSE. We suggest that the seizures in these nonsurvivors were self-limited events arising from lethal damage to neurons. Thus, in patients with bilaterally synchronous facial myoclonus, bilateral loss of pupillary or oculovestibular reflexes, and suppression and burst-suppression on EEG, it is not warranted to use anesthetic barbiturates to treat MSE.
Neurology 1990 Dec
PMID:The significance of myoclonic status epilepticus in postanoxic coma. 212 7

Clinical and biochemical studies are reported on a 32-year-old man with GM1 gangliosidosis who presented with a slowly progressive dystonia that began when he was aged 7 years and eventually became almost totally incapacitating at the age of 35. There was only mild intellectual deterioration, but myoclonus, seizures and macular cherry-red spots were never observed. Proton-density and T2-weighted MRI scans showed symmetrical hyperintense lesions of both putamina. No increase of GM1 ganglioside was found in plasma or cerebrospinal fluid, and the metabolism of GM1 ganglioside in cultured skin fibroblasts from the patient was also almost normal, although the residual activity of GM1 ganglioside beta-galactosidase activity was only 10% of normal. These findings suggest that impaired GM1 ganglioside metabolism is not present systemically as it is in the infantile and juvenile types of the disorder, but is mainly confined to the central nervous system in chronic GM1 gangliosidosis.
J Neurol 1990 Dec
PMID:A case of chronic GM1 gangliosidosis presenting as dystonia: clinical and biochemical studies. 212 25

Rhythmic palatal myoclonus (RPM) is a rare movement disorder consisting of continuous synchronous jerks of the soft palate, muscles innervated by other cranial nerves and, rarely, trunk and limb muscles. It usually develops secondary to brainstem or cerebellar disease (symptomatic RPM). Some patients, however, fail to show evidence of a structural lesion (essential RPM). A total of 287 cases with RPM from the literature including 210 cases with symptomatic and 77 cases with essential RPM have been reviewed and analysed statistically to look for criteria separating the two conditions. Patients with essential RPM usually have objective earclicks as their typical complaint which is rare in the symptomatic form. Eye and extremity muscles are never involved. The jerk frequency is lower in essential than in symptomatic RPM. Patients with essential RPM are younger and have a balanced sex distribution as compared with a male preponderance in the symptomatic form. The rhythmicity of RPM seems to be more profoundly influenced by sleep, coma and general anaesthesia in essential than in symptomatic RPM. We conclude from these results that essential RPM should be separated as a distinct clinical entity. Symptomatic RPM is a rhythmic movement disorder whose pathogenesis is quite well established. The cells of the hypertrophied inferior olives are believed to represent the oscillator. Among other possibilities, essential RPM may represent its functional analogue, based on transmitter changes only. Such a relationship could be of theoretical interest for the understanding of rhythmic hyperkinesias in general.
Brain 1990 Dec
PMID:Symptomatic and essential rhythmic palatal myoclonus. 227 39

We describe two patients and a previously reported patient who acquired unique pendular vergence oscillations of the eyes and concurrent contractions of the masticatory muscles, i.e., oculomasticatory myorhythmia (OMM). The smooth disjunctive eye movements cycled with a frequency of 0.8 to 1.2 Hz. An analysis of peak velocities (15 to 200 degrees/sec) with respect to peak amplitudes (5 to 25 degrees) revealed dynamics characteristic of normal vergence movements. The pathological alterations resulting in pendular vergence oscillations implicate a separately functioning, physiologically normal vergence system within the brainstem. In addition to paralysis of vertical gaze, each patient also experienced progressive somnolence and intellectual deterioration. An intestinal biopsy in 1 patient established a diagnosis of Whipple's disease, which led to appropriate treatment and amelioration of the OMM. A pathological diagnosis of Whipple's disease of the central nervous system was made in the other 2 patients; results of an intestinal biopsy in one of these patients were normal. No patient had palatal myoclonus, and olivary pseudohypertrophy was not found in two autopsy examinations. Thus, OMM is a distinct movement disorder and has been recognized only in Whipple's disease. We conclude that patients with OMM should be treated presumptively for Whipple's disease of the central nervous system, even if a jejunal biopsy is normal.
Ann Neurol 1986 Dec
PMID:Oculomasticatory myorhythmia: a unique movement disorder occurring in Whipple's disease. 243 19

A 70-year-old man developed a syndrome of progressive nondominant parietal and occipital dysfunction including palinopsia and a visual field defect. Despite the marked focality of his clinical findings, radiologic studies were normal. Myoclonus and ataxia began 6 weeks after onset of his illness at which time brain biopsy confirmed Creutzfeldt-Jakob disease (CJD). This is the first reported case of palinopsia due to CJD.
J Clin Neuroophthalmol 1989 Dec
PMID:Palinopsia as a presenting manifestation of Creutzfeldt-Jakob disease. 253 Nov 61


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