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)

Motor disorders reported in the present paper do not result from cortical ablations stricto sensu since some white matter was excised in every patient. However they appear to suggest that, as suggested by Walshe (1935), the central region and premotor area are a functional entity, i.e. they work as a whole. The extensive lesions of the premotor area, leaving untouched the motor region, have the same motor and tonic consequences as lesions limited to the central region. This point which appears specific for man does not imply that the premotor region subserves activities similar to those subserved by the central region. Rather it may suggest a deafferentiation of the central region, the consequences of which would be more important than is generally assumed. Extensive central or premotor lesions determine various tonic disorders: a well known spasticity, with exaggeration of the stretch reflex, associated with an increase in passive swinging of segments of limbs and in extensibility of joints. These two latter phenomena are usually defined as hypotonia. With premotor and precentral lesions the hypotonia disappears and a hemiplegic posture is observed. This hemiplegic posture is a dystonia which apparently does not result directly from the exaggeration of the stretch reflex. Anatomically it appears to result from lesions of both central and premotor regions. This is in agreement with Denny-Brown's (1966) contention that an extrapyramidal region lies rostral to the prerolandic sulcus. As suggested by Evarts (1973) motor regions appear to control automatic as well as voluntary movements. They probably play a role in the trophic function of muscle, since, despite rehabilitation, amyotrophy was present in every case reported in the present paper.
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PMID:Motor consequences of motor area ablations in man. 83 9

Clonazepam or 5-(2-chlorphenyl)-1, 3-dihydro-7-nitro-2H-1,4benzodiazepin-2-one, is a close structural and pharmacological relative of nitrazepam. It has a broad spectrum of activity against the various types of epilepsy, and is effective in many patients whose condition has proved resistant to other antiepileptic drugs. Its chief uses are in status epilepticus, in which intravenous clonazepam may replace diazepam as the drug of first choice, and in the minor motor seizures of childhood, particularly petit mal absences, the Lennox-Gastaut syndrome and infantile spasms. Clonazepam is also at least as effective as current treatment in psychomotor and myoclonic epilepsies, but seems unlikely to replace phenytoin and the barbiturates in the treatment of grand mal or focal motor seizures except in patients resistant to standard therapy. Initial success with clonazepam can be followed by loss of effect, but benefit can often be restored, at least initially, by temporary interruption and re-institution of treatment. Side-effects are common with clonazepam. Most patients experience drowsiness and fatigue, which are frequent causes of withdrawal, together with lesser incidences of ataxia, dystonia, hypotonia, and hyperactivity. These effects usually disappear with continued therapy, and are minimised by gradual introduction of the drug over 2-4 weeks. Hypersalivation and excessive bronchial secretion may be a problem in children and infants.
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PMID:Clonazepam: a review of its pharmacological properties and therapeutic efficacy in epilepsy. 97 34

On four occasions since 1978, this 53 year-old woman presented with a right hemicorporal hypotonia, symptomatic of a hemispheric cerebellar syndrome. In 1981, she experienced the progressive development of a cervical dystonia. CT scan and RM scan showed a cavernous angioma in the right cerebellar hemisphere. The 18F-2-fluoro-2-deoxy-glucose PET scan revealed a right cerebellar and a contralateral cortical and striatal hypometabolism. This crossed cerebello-cortical diaschisis can be interpreted as a functional interruption of the cerebello-cerebral pathways. This case raises the question of the role played by a cerebellar lesion in the development of a focal dystonia.
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PMID:[Cerebellar cavernous angioma, cervical dystonia and crossed cortical diaschisis]. 196 70

A 2-year-old boy suffered aphasia, hypotonia, dystonia, and loss of activity and spontaneous speech during an active stage of juvenile rheumatoid arthritis with pericarditis, fever, anemia, and a high antinuclear antibody titer. These neurologic signs slowly improved with corticosteroid treatment but fluctuated over 1 year. The neuroimaging studies revealed irregular mass lesions in the basal ganglia bilaterally mainly involving the globus pallidus. They gradually decreased in size and almost disappeared after 1 year. A stereotactic brain biopsy revealed a slight proliferation of astrocytes. Chorioretinitis was also observed during the clinical course. A chronic inflammatory process involving cerebral vessels was suspected, although angiography did not demonstrate cerebral vasculitis. The possibility of central nervous system lymphoma could not be eliminated. The type of aphasia and the relation to the lesion sites are discussed.
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PMID:Basal ganglia mass lesions in juvenile rheumatoid arthritis. 205 56

A girl of first cousin parents presented in the 1st year of life with a progressive neurological disease with muscle weakness and hypotonia, accompanied later by dystonia. Investigations, including gas chromatography of urine, showed no abnormality. Autopsy showed marked neuronal loss and gliosis in the putamen and globus pallidus. The activity of glutaryl-CoA dehydrogenase in cultured fibroblasts was normal, but the activity of electron transfer flavoprotein was markedly diminished. Retrospective study of urine by capillary gas chromatography/mass spectrometry showed small amounts of glutaric and other organic acids. This is the first report of striatal degeneration in association with glutaric acidaemia type II. The neuropathological changes were milder than those in glutaric acidaemia type I.
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PMID:Striatal degeneration in glutaric acidaemia type II. 271 49

Alternating hemiplegia in childhood (Verret, 1971) is a disorder presenting with frequent episodes of alternating hemiplegia from early infancy. We report a patient with this disorder, along with a pathophysiological study and a discussion about the available therapies for this disorder. The patient, an 11-year-old boy, visited our hospital with episodes of alternating hemiplegia from early infancy. His family history showed that many members suffered from migraine. He was born with asphyxia. Mental and motor developmental delays were seen from early infancy. The hemiplegic episodes with ipsilateral facial palsy had occurred frequently from early infancy. The episodes were frequently induced by emotional stress. The duration of hemiplegia varied from 10 minutes to 3 days. From the age of 11 years, he had begun to have migrainous attacks with or without hemiplegic episodes. Neurological examination revealed slight muscle hypotonia, choreoathetosis and dystonic movements induced by locomotion, positive Myerson sign, increased deep tendon reflexes and Babinski reflex. CAG, VAG and CAT revealed normal findings. EEG revealed diffuse generalized slowing during hemiplegic episodes. Measurement of regional cerebral blood flow (CBF) by 133Xe inhalation method revealed a slight decrease of bilateral CBF during a quadriplegic episode. Positron emission tomography using C15O2 revealed a slight decrease of CBF at the insula, putamen and claustrum of the left side during a right sided episode. Increased excretion of urinary 5-HIAA was seen during one episode. From our clinical and laboratory findings, we think this disorder may be a special type of migraine. Therapeutic trials of diazepam and flunarizine were both effective, but the initial effectiveness was decreased after 5 months.
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PMID:[A patient with alternating hemiplegia in childhood]. 273 28

The diagnosis of postinfectious encephalomyelitis with symmetric lesions in the basal ganglia was confirmed by magnetic resonance imaging in 2 patients. A 7-year-old patient experienced severe dystonia and hyperreflexia; magnetic resonance imaging demonstrated bilateral lesions in the putamina and basis pontes. The other patient, a 2-year-old female, manifested hypotonia, facial grimacing, and athetosis. Symmetric lesions in the globus pallidus and substantia nigra were demonstrated by imaging studies. The nature and monophasic course of illness in these 2 patients, as well as the symmetric involvement of specific regions of the basal ganglia, may result from an immune-mediated postinfectious demyelinating process.
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PMID:Postinfectious encephalomyelitis with localized basal ganglia involvement. 280 90

The development of nine infants with enlarged subarachnoid spaces identified by specific CT-scan criteria was prospectively followed to two to three years of age. Infants with intracranial hemorrhage, CNS anomaly, microcephaly or other factors of potentially major negative impact on their development were excluded. All study infants had normal or only minimally increased ventricular size and none developed hydrocephalus. Head circumference was greater than or equal to 90th percentile in all cases, and six fathers also had a head circumference greater than or equal to 90th percentile. Six infants had gross motor delay and mild hypotonia in the first year. One, born at 30 weeks gestational age, had transient dystonia. At follow-up all the infants were developing normally, apart from four with minor concerns. Infants with macrocephaly or rapid head-growth, CT-scan findings of enlarged subarachnoid spaces and normal to minimally increased ventricular size, and who have a parent with macrocephaly, have a good developmental prognosis and a characteristic pattern of neuromotor development in the first year.
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PMID:Developmental prognosis for infants with benign enlargement of the subarachnoid spaces. 358 87

A prospective study was undertaken of the outcome at 1 year in 129 preterm infants of less than 34 weeks gestation (range 27 to 34 weeks) who underwent detailed neurologic assessment and ultrasound scanning in the neonatal period and again at 40 weeks postmenstrual age, and an independent neurodevelopmental assessment at 12 months chronologic age. Of the 129 infants, 37 (29%) had ultrasound evidence of periventricular hemorrhage. At 40 weeks postmenstrual age the infants were classified as neurologically normal, abnormal, or borderline on the basis of the neurologic examination. Of the 62 infants considered normal at 40 weeks, 57 (91%) were assessed as normal at one year, compared to only 14 (35%) of the 39 infants considered abnormal (P less than 0.001). Ten (85%) of the 12 normal infants with associated periventricular hemorrhage were normal at 1 year, compared to 47 (94%) of the 50 normal infants without periventricular hemorrhage, whereas 5 (25%) of 20 abnormal infants with associated periventricular hemorrhage and 9 (47%) of the 19 without periventricular hemorrhage were normal at 1 year. There was no direct correlation in individual cases between the severity of neurologic deficit and the presence or severity of periventricular hemorrhage. Infants with a cluster of abnormal signs were more likely to have later dystonia or cerebral palsy than those with marked hypotonia but no other abnormality.
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PMID:Correlation of neurologic assessment in the preterm newborn infant with outcome at 1 year. 647 Aug 69

A 45-year-old woman with a history of probable perinatal craniocerebral trauma resulting in mild asymptomatic right hemiatrophy developed right leg weakness and hypotonia alternating with dystonia only after prolonged exertion at age 12. At age 27, she developed right-sided parkinsonism. Exertional paresis and dystonia and parkinsonism responded completely to levodopa; however, she developed a progressive reduction in the duration of action of levodopa over the first 4 years of treatment. Investigations including computed tomography, magnetic resonance imaging, [18F]fluorodopa, and [18F]fluorodeoxyglucose positron emission tomography scans suggested a static lesion involving the left substantia nigra. This unusual exertion-induced weakness and hypotonia alternating with hypertonia and dystonia has not been reported previously. The role of dopamine deficiency in dystonia and the role of levodopa in the development of fluctuations in Parkinson's disease are discussed. Review of the literature, including this patient, emphasizes the heterogeneity of the syndrome of hemiparkinsonism-hemiatrophy.
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PMID:Hemiatrophy, juvenile-onset exertional alternating leg paresis, hypotonia, and hemidystonia and adult-onset hemiparkinsonism: the spectrum of hemiparkinsonism-hemiatrophy syndrome. 756 31


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