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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 three patients suffering since infancy from transient attacks of paresis. The flaccid pareses most frequently affect the extremities in a hemiplegic fashion, but occasionally there is monoparesis or quadriparesis. The laterality and degree of the paresis are variable. Conciousness is always preserved, and in two cases attacks were preceded by ocular motor disturbances (skew deviation, nystagmoid jerks and conjugate deviations). Exceptionally, the transient hemiparesis may be preceded by a grand mal epileptic fit, though they are more likely to appear sporadically and independently of the paretic changes. In the interparoxysmal periods the children showed pronounced hypotonia, hyperkinetic extrapyramidal features and oligophrenia. Neuroradiological procedures have excluded brain anomalies of vascular or other aetiology and simple biochemical analyses were negative. EMG during paretic periods have revealed central motor neuron lesions, while EEG demonstrated non-specific paroxysmal features. A brain-stem dysfunction in the aetiology is postulated.
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PMID:Paroxysmal hemipareses in childhood. 52 Jul 18

Dorsal column lesions in the high cervical region of the monkey result in severe defects of movements projected into space and contactual orienting reactions of the forelimbs. The hindlimbs are less affected provided a pathway through the lateral columns, Morin's tract, remains intact. Interruption of this pathway results in a defect of hindlimb function similar to that of the forelimbs. Cerebellar ablations in monkeys result in postural and movement disorders, including hypotonia of limb extensor muscles. An important mechanism underlying the hypotonia is a depression of the responses to muscle extension of spindle primary afferents owing to a decrease of fusimotor activity. In the decerebellate animal abnormalities of limb trajectory during active movements projected into space (cerebellar "dysmetria") appear to result principally from dysfunction of systems separate from the peripheral fusimotor efferent-spindle afferent reflex arc. Precentral cortical ablation results initially in a contralateral hypotonic hemiparesis, later in a hypertonic hemiparesis. A depression of the responses of muscle spindle afferents occurs during the hypotonic phase, but during the hypertonic phase spindle function returns to normal levels. Accordingly a depression of fusimotor function appears to be important in the hypotonic phase of hemiplegia; however, there is no evidence that an enhancement of fusimotor function underlies the hypertonic phase. Bilateral section of the medullary pyramids results in an enduring hypotonic paresis. Abnormalities of contactual orienting responses of limbs are similar to those following dorsal column lesions. Responses of spindle primary afferents are depressed during the initial stages after acute pyramidotomy, then approach but do not reach normal levels. It is concluded that the dorsal columns constitute an afferent, and the pyramidal tracts an efferent, pathway important in oriented contactual reactions of the limbs. The hypotonia resulting from cerebellar lesions, precentral ablation, and pyramidal tract section stems, at least in part, from a depression of the fusimotor innervation of muscle spindle afferent activity.
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PMID:Primate models of postural disorders. 105 89

Four children had progressive degeneration of the cerebral cortex, with hepatic cirrhosis. They and four previously described ones, are representative of a distinct form of hepatocerebral degeneration. Onset of the neurological disorder is between ages 1 and 3 years, at times with mild developmental delay. Explosive onset of intractable convulsions, leaving the child in a stuporous and demented state, is characteristic. Generalized hypotonia or hemiparesis were observed in several affected children. Clinical evidences of hepatic disease, including ascites and jaundice, occurred late, if at all. The illness ended fatally within ten months of onset of convulsions. Pathological findings in the brain are neuronal loss and gliosis, in a pattern that is indistinguishable from that in degeneration of the cerebral gray matter in infancy (Alpers disease). The hepatic lesions consist of cirrhosis or of subacute hepatitis, with superimposed fatty infiltration of hepatocytes. The disorder is genetically determined, with recessive inheritance.
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PMID:Infantile diffuse cerebral degeneration with hepatic cirrhosis. 125 62

The purpose of this investigation was to compare three methods of assessing the excitability of lower motoneurones--TA-reflex, H-reflex and F-wave--in 120 patients with spastic hemiparesis following a stroke. The H-reflex was recorded from the soleus muscle after submaximal electrostimulation of the tibial nerve. The T-Achilles (TA) reflex was recorded from the soleus muscle after percussion of the Achilles tendon. The F-wave was recorded in the distal limb muscles after supramaximal electrostimulation of the median, ulnar, fibular and tibial nerves. The patient's healthy side was used as a control. The TA-reflex, H-reflex and F-wave showed increased amplitudes on the spastic side. All amplitude ratios: TA/M, H/M, Fmax/M and Fmean/M were increased. The H-reflex thresholds were decreased. The F-wave duration, persistence and number of phases were also increased on the spastic side. Despite clinically decreased muscle tone, there were no changes in TA or H-reflex parameters after treatment. On the other hand, F-wave parameters tended to normalize after treatment in all groups. In conclusion, the F-wave is a more sensitive method than the TA and H-reflexes in assessing the excitability of the lower motoneurone.
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PMID:A comparison of methods to assess the excitability of lower motoneurones. 156 9

A 9-month-old Turkish boy was diagnosed as having Griscelli disease (Chediak-Higashi-like syndrome). Clinical signs consisted of silver-grey hair and a relatively light skin colour, recurrent episodes of fever, with or without detectable infections, increasing hepatosplenomegaly, hypotonia and motor retardation. Laboratory studies showed pancytopenia of varying degree but neither inclusion bodies nor vacuoles were seen in his leucocytes. Serum immunoglobulin levels were normal except for a IgG2 deficiency. In the mixed lymphocyte reaction the stimulation capacity of the leucocytes was decreased. Microscopic examination of his hair and electron-microscopic examination of a skin biopsy further confirmed the diagnosis. Shortly before the diagnosis was made, the child developed cerebral symptoms with hemiparesis and convulsions. A CT scan suggested cell infiltration of the brain. A few weeks later the boy died of an infection.
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PMID:Griscelli disease with cerebral involvement. 182 34

An infant developed chronic respiratory failure after aseptic meningoencephalitis at 5 months of age. Neurologic evaluations at 16 and 17 months were normal except for an abnormal pharyngeal stage of swallowing, lower extremity hypotonia, and a mild left hemiparesis. Spontaneous breathing during sleep at 16 months was characterized by alveolar hypoventilation, athetoid truncal movements, and disorganized respiratory muscle activity. At 27 months of age, improvement in sleep-related breathing was accompanied by a change in respiratory pattern characterized by alternating inspiratory and expiratory muscular activation. The findings indicate that disorganized as well as diminished output from the central respiratory pattern generator may result in central alveolar hypoventilation.
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PMID:Respiratory dysrhythmia. A new cause of central alveolar hypoventilation. 375 16

We present the case history of a boy, who died at the age of 3 1/2 years after a rapidly progressive neurologic disorder, characterized by psychomotor retardation, hypotonia, hemiparesis, seizures and myoclonic contractions. Histopathologic studies showed slight lipid storage in liver. Autopsy showed the characteristic features of progressive infantile poliodystrophy (Alpers' disease); ultrastructural examination showed an increased density of mitochondria in cerebral gray matter. Biochemical studies in leukocytes, cultured fibroblasts and liver indicated a deficiency in the citric acid cycle between succinate and fumarate; this deficiency was not present in muscle tissue. This study supports the view that progressive infantile poliodystrophy is associated with abnormalities in pyruvate metabolism and/or in cell mitochondria.
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PMID:Progressive infantile poliodystrophy (Alpers' disease) with a defect in citric acid cycle activity in liver and fibroblasts. 681 59

Lingual motricity was prospectively studied in 32 consecutive stroke patients with hemiparesis or hemiplegia involving the face. We excluded patients with vertebrobasilar infarcts, controlateral stroke, arteriovenous malformations, internal carotid artery dissection, severe aphasia or severe bucco-lingual apraxia. The study population consisted of 18 males and 14 females with a mean age of 64.2 years. Seventeen patients had a lesion located in the right hemisphere and 15 in the left one. The lesion was of ischemic origin in 19 patients and hemorrhagic in 13. Lingual motricity was studied at rest and at protraction. We recorded whether the following abnormalities were present: lingual deviation, limitation of protraction or lateral movements, atrophy, hypotonia and fasciculations. Sixteen patients had a deviation of the tongue at protraction. The other abnormalities were deviation of the tongue at rest, limitation of lateral movements at protraction, unilateral lingual hypotonia and limited protrusion. Of 16 patients with deviation of the tongue at protraction, 10 had no deviation at rest. Of the 6 remainders, 2 had deviation of the tongue towards the opposite side, at rest, and 4 towards the same side. Most patients with deviation of the tongue at protraction, had a lesion of the posterior limb of the internal capsule and of the posterior part of the lenticular nucleus. Most patients without lingual deviation, had a lesion of the capsule-lenticular region and of the superior portion of the internal capsule, just under the corona radiata.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Lingual motility in unilateral hemispheric vascular complications. Study of the cortico-hypoglossal afferences]. 767 21

In a prospective study, we analyzed the intraoperative electroencephalographic (EEG) changes during open heart surgery with deep hypothermia in 66 infants aged 6 months or younger, 70% of whom were neonates. Suppression of amplitude and continuity at the nadir of temperature reduction and following rewarming, and the appearance of periodic paroxysmal activity, was compared with neurologic abnormalities before and following operation, patient characteristics, and operation variables. EEG changes disclosed no relationship to abnormal neurologic findings, age at operation, type of anesthetic, duration of cardiopulmonary bypass (CPB), duration of low-flow CPB or cooling, temperature at circulatory arrest (HCA) or low flow, or nasopharyngeal-venous return temperature differences. EEG suppression following rewarming was associated with the use of thiopentone and duration of HCA. Use of thiopentone was also related to decreased levels of alertness at the end of the first postoperative week. We could not demonstrate any association between operation variables, including duration of HCA, and postoperative neurologic findings which include abnormalities of tone, alertness, seizures, generalized pyramidal signs, choreoathetosis, and hemiparesis. Severe hypotonia before operation was associated with continuing severe hypotonia during the postoperative period. EEG changes during cooling for open heart surgery on infants appear to be physiologic, and these plus EEG suppression following HCA or low-flow CPB are not useful predictors of early neurologic morbidity.
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PMID:EEG changes during open heart surgery on infants aged 6 months or less: relationship to early neurologic morbidity. 802 60

A 3-year-old girl with left hemiparesis suffered from bilateral paresis, motor rigidity, gait disturbance, axial hypotonia, dysarthria, apathy, and incontinence. After steroid therapy, mild improvement occurred, but muscle weakness, gait disturbance, and rigidity remained. Leigh encephalopathy was excluded on the basis of muscle biopsy and laboratory findings. Computed tomography and serial magnetic resonance imaging at an early stage revealed right-sided dominant lesions in the putamen and caudate nucleus and later bilateral striatal lesions, appearing as hyperintense signals on T2-weighted images and mixtures of hypo- and hyperintense signals on T1-weighted images. This is the first demonstration of serial magnetic resonance imaging findings in infantile bilateral striatal necrosis.
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PMID:Serial MRI in infantile bilateral striatal necrosis. 802 66


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