Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0026827 (hypotonia)
 5,860 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The authors report the case of an 8 year-old boy who, when he was 2 1/2 years old, suffered from spasmodic mouth twitches. At the age of four, various other symptoms appeared: psychomotor backwardness, frequent fails and a photomyoclonic response on electroencephalogram. At the age of 5 1/2, noticeable difficulties appeared in walking with a broad-based gait, hypotonia, and intentional trembling associated with hypokinesia and dysarthria. When he was six, the first convulsive seizure appeared, then myoclonies which became continuous. The child gradually became bedridden. The family history tends to show these disorders can be linked with a Huntington chorea affecting six generations. This case is very similar to that previously described by the authors, in an 8 year-old girl where an anatomic examination revealed the existence of lesions characteristic of Huntington's disease associated with lesions of the cerebellum. The authors, on the basis of the data provided by the literature, discuss the myoclonic and cerebellous aspect of this infantile form. Lacking anatomic evidence, they stress the interest of the biochemical disturbances affecting the cerebral monoamines noted in this observation.
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PMID:[Myoclonic type of Huntington's chorea (author's transl)]. 14 49

In 77,9 +/- 3,5% of the children with an active phase of rheumatic fever, developing with a prevalent lesion of the heart and joints, symptoms of animal and vegetative nervous system lesions were found. The neurological symptoms correlated with changes of the brain bioelectrical activity. The authors point to some dynamics of symptoms under the influence of anti-rheumatic treatment. Normalization of the brain bioelectrical activity, a regress of symptoms of the vegetative nervous system and muscular hypotonia are somewhat behind the clinical recovery. The existence of pathological activity on the EEG in children after an arrest of clinical symptoms of rheumatic fever may indicate to the possible subsequent development of chorea, the diencephalic syndrome, cerebrasthenic syndrome in these children.
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PMID:[Diagnosis of formes frustes of a rheumatic lesion of the nervous system in children]. 30 54

Abnormal head movements have been studied in a variety of diseases using objective recording techniques and the data analysed with respect to the frequency content of the movement. Flopping, nodding, tic, chorea, myoclonic jerks, and most head tremors involve frequencies of approximately 2 and 4 Hz which correspond to the natural fundamental and second harmonic resonances of the head as determined by the mechanical properties of the head/neck system. These findings provide a basis for classification of abnormal head movements as well as an explanation of the characteristics of those arising from hypotonia of the neck muscles. The similarities between tremor frequencies and natural resonances suggest that in the case of the head, tremor arises from disorders of neural mechanisms normally responsible for the fine control of voluntary head movement and for stabilisation of the head during disturbance of posture. Head movements in cases of congenital nystagmus were found to be of two types. Some were of bizarre waveform, in no way assisted vision, and were taken to be of primarily pathological origin and classified as tremors. Others were learned adaptive responses which assisted vision either by interrupting the nystagmus, as in the case of spasmus nutans, or by compensating for the nystagmus with an inverse waveform and were called nodding. A prerequisite for true compensatory nodding is modified vestibulo-ocular reflex.
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PMID:Abnormal head movements. 49 Jan 76

Two cases of Morvan's chorea are reported. One of the patients presented the characteristic of having had two attacks, the first after organic mercury preparations, and the second after gold salts for inflammatory rheumatism. The second case had facial fibrillations only, and this was followed by a regressive polyradiculoneuritis one month later. This latter case raises certain diagnostic problems. The existence of a particular type of immuno-allergic tendency could be validly related to a triggering effect of various etiological agents (metals such as mercury or gold salts, or infective agents). The absence of hypotonia, and a regressive course appear to be the characteristics that distinguish fibrillary chorea from the continuous activity syndrome of the muscle fibers described by Isaacs.
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PMID:[Morvan's fibrillary chorea]. 55 22

EMG of involuntary muscle contractions and their correlation with clinical pictures in Huntington's disease disclosed a series of motor disorder from chorea to parkinsonism. Irregular brief contractions appearing reciprocally in flexor or extensor muscles were observed in typical chorea with hypotonia. Tonic nonreciprocal contractions appeared in the rigid form. In athetoid movement or dystonic postures, more slowly nonreciprocal fluctuating contractions appeared. In some cases phasic contractions changed from a reciprocal to nonreciprocal pattern by psychic stress. In contrast to the activation of motoneurons in choreatic movements, involuntary brief suppression of voluntary contraction appears in typical chorea. Difference in involuntary movements and muscle tone may result from quantitative differences in involvement of striatal neurons which are the origin of parallel pathways proposed by DeLong and colleagues.
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PMID:The spectrum of motor disorders in Huntington's disease. 132 May 6

Sydenham's chorea (chorea minor, St. Vitus dance, rheumatic encephalitis), described by Thomas Sydenham in 1686, is considered one of the major manifestations of rheumatic fever (1, 2, 3, 4). Clinically it is characterized by involuntary movements, hypotonia, dysarthria, emotional disorders, and less frequently, by other neurological manifestations such as weakness, headache, seizures and sensory abnormalities (1,4). The motor disorders may be generalized or unilateral, in this case constituting a hemichorea (3). Chorea may present associated to other rheumatic fever manifestations during an acute episode, or in isolated form, characterizing the so-called "pure" chorea (5, 6, 7). Its etiology and pathophysiological mechanisms are still unclear, although its relation with a previous pathophysiological group A Beta-hemolytic streptococcus infection is well established (8). There is also evidence of the participation of immunological mechanisms in its pathogenesis, such as the finding of serum anti-nucleus caudatus and anti-subthalamic antibodies (9) and increase in IgG levels in cerebrospinal fluid of patients with chorea (10). In developed countries due to the reduction in rheumatic fever incidence and decrease in frequency of chorea as its manifestation (3, 11), the latter has become rare. However, in developing countries rheumatic fever remains a public health problem. In Brazil, in the last years an increase in the incidence of chorea has been observed as part of the clinical picture of rheumatic fever (12). The present study reports the clinical and laboratory findings of 187 cases of Sydenham's chorea followed-up during the period of January 1980 to December 1990 in two university centers in the city of Sao Paulo, Brazil.
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PMID:Sydenham chorea: clinical and laboratory findings. Analysis of 187 cases. 134 Oct 4

P, an Indonesian boy, 5 years old, 15 kg of body weight and 110 cm of body length, was admitted to the PTP IX Hospital on March 30, 1987 with cerebral palsy. The patient had fever since 10 days before admission which lasted 7 days; he showed restlessness by involuntary movements when catching, thus when holding something in his hand, it fell off. He had difficulty in walking and talking. On physical examination, were hyperemic the tonsils and pharynx; there was muscular hypotonia, flexed arms and metacarpophalangeal extension. Laboratory findings showed leukocytosis, increased ASTO titer, positive CRP and normal cerebral spinal fluid. Head CT scan showed no abnormality. Electrocardiogram and EEG were in normal limits. Throat swab culture produced Group A Streptococcus beta hemolyticus. The diagnosis of sydenham's chorea was established. Procaine penicillin, phenobarbital and vitamins were administered to the patient. After 5 weeks in the ward the patient was doing well, and now he is working and speaking normally; he is on ambulatory treatment with 1.2 million units/month of long-acting benzathine penicillin, at the Department of Child Health, Tembakau Deli Hospital, Medan.
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PMID:Sydenham's chorea. 207 7

A 15-year-old boy was admitted at age 10 because of muscle weakness, choreatic involuntary movements, and a profound hypotonia. He was diagnosed as having chorea mollis, a rare variant of Sydenham's chorea. He made a full recovery and received prophylaxis with monthly injections of benzathinebenzyl penicillin for the next 5 years. Six months after discontinuation of the prophylaxis he had a recurrence. The choreatic movements were successfully treated with haloperidol. However, a hypotonia and mild psychiatric symptoms still persist one year later. The clinical course, observed in our patient, is in concordance with the results of recent studies, reporting, that a number of patients with Sydenham's chorea may be left with mild to moderate neurologic and psychiatric sequelae.
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PMID:[Chorea mollis, a rare variant of chorea minor]. 237 43

An analysis is presented of 35 cases of chorea as a symptom of polycythaemia. This analysis reveals: (a) whereas polycythaemia occurs predominantly in males (3:2), polycythaemic chorea (PC) occurs predominantly in females (5:2), at a real ratio of female:male = 4:1, the prevalence being 1-2.5% of polycythaemic patients; (b) PC manifests predominantly after the age of 50 (8 cases before, 27 after 50 years), making polycythaemia the first disorder to be considered in cases of so-called 'senile' chorea; (c) PC is generalised, with predominant involvement of faciolingual and brachial muscles, and associated with muscular hypotonia; (d) PC may last from periods of weeks to years, usually responds to haloperidol, venesection or 32P-treatment, but may persist, or recur with treatment, or remit spontaneously, and (e) no relationship exists between the choreatic syndrome and (the rare finding of) a small infarct in the caudate nucleus. The cause of the choreatic syndrome in polycythaemia is presumably to be explained as a neostriatal hyperviscosity syndrome producing venous stasis, reduced brain blood flow and impaired tissular O2/glucose metabolism. The state of dopaminergic hyperactivity is presumably enhanced by relatively increased neostriatal catecholestrogens. The hypothesis of polycythaemic excess of dopamine-laden platelets releasing excess of dopamine in the neostriatum needs to be confirmed by laboratory evidence of platelet counts.
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PMID:Chorea and polycythaemia. 637 Jul

A case of chorea-acanthocytosis (CA) syndrome is described. CA is a rare, inherited syndrome characterized by normolipoproteinemic acanthocytosis and progressive neurological disturbances (orofacial dyskinesia, limb chorea, lip and tongue biting, distal muscle wasting, muscle hypotonia, absent or diminished tendon reflexes) with adult onset. Thus far, 10 independent reports of CA have been published. The present case is the first patient reported in Europe outside Great Britain. Due to obvious clinical similarities between CA and Huntington's chorea, particular attention is drawn to the differential diagnosis between these 2 syndromes. Investigation of the red blood cell morphology should necessarily be performed in the examination of choreic patients, particularly when the disorder is familial.
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PMID:Chorea-acanthocytosis. Neurological disease with acanthocytosis. 661 27


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