Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0026838 (spasticity)
 6,471 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The follow-up results of intensive care for 68 infants with birth weights less than 801 g treated at Stanford University Hospital were reviewed. The overall survival rate for these infants was 35%, but was 50% for those infants who had been successfully resuscitated in the delivery room and were admitted to the Intensive Care Nursery. Infants under 601 g in weight or less than 25 weeks gestation were more likely to die in the delivery room, but survival among those admitted to the Intensive Care Nursery did not depend on birth weight or gestational age. One-minute and 5-minute Apgar scores less than 5 and interstitial emphysema were associated with increased risk of neonatal death. Only two of 22 survivors (9%) were severely handicapped and another eight (36%) had remediable disabilities at 2 years of age. No infant developed hydrocephalus and only one infant had spasticity. We suggest that the low incidence of major handicaps among survivors encourages the vigorous resuscitation of infants weighing less than 801 g at birth, yet strategies must be developed that will minimize both prolonged dying and the cost of intensive care for nonviable infants.
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PMID:Outcome of neonates with birth weights of less than 801 grams. 246 42

Distinct chronic posttraumatic syndromes, ascribed to neurological deficits of patients suffering severe head injuries and being in prolonged coma, are much less frequently encountered in the literature than acute traumatic syndromes. The major components of the posttraumatic midbrain syndrome, resulting from compressive necrosis or vascular infarction at the midbrain level, are ipsilateral cerebellar signs (the predominant one being intention tremor), contralateral pyramidal signs (the predominant one being a spastic-dystonic hemiparesis), dysarthria, and mild to moderate intellectual impairment. Significant bilateral cerebellar dysfunction following head injury, without pyramidal, extrapyramidal, or pseudobulbar signs, constitutes a posttraumatic cerebellar syndrome. Its most disabling component, namely posttraumatic intention tremor, may be alleviated by thalamotomy. Following severe closed head injury, an infrequently encountered posttraumatic entity of dystonic hemiplegia or hemiparesis, which may be alleviated by thalamotomy, can occur, but does not have a specific neuroanatomical basis. Intention tremors following severe head injuries, rarely associated with hydrocephalus and without other significant cerebellar findings, can develop as a dysfunction of the cerebellofugal outflow system. While chronic posttraumatic syndromes can be complex and difficult to treat, cerebellar stimulation has been utilized ipsilaterally to modulate limb spasticity, and bilateral ventrolateral cryothalamectomies staged 4-6 months apart have been successful in alleviating severe (intractable) intention tremors.
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PMID:Rehabilitative neurosurgery: posttraumatic syndromes. 262 1

A case of neonatal Herpes simplex infection is being described, which was diagnosed clinically as well as serologically. It concerns a child, which was born after 35 weeks of gestation. Two days after the delivery the mother showed typically efflorescences of Herpes simplex infection in the abdominal region. On the fifth day after birth the child showed a vesico-bullous exanthema beginning on the head and spreading out on breast and back. On day 14th a serious sepsis-like pattern of the disease with respiratory insufficiency and encephalitic symptoms could be seen. Treatment with Vidarabinphosphat and Acyclovir-Natrium was without definite success. At the age of five months the child showed a pseudobulbar-paralysis with tetra-spasticity. The cranial computer-tomography demonstrated a distinct hydrocephalus e vacuo and the electroencephalography registered only sporadic brain activity.
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PMID:[Herpes simplex infection in the newborn infant]. 298 61

This report describes a case of metrizamide encephalopathy with persistent disturbance of consciousness and extrapyramidal symptoms. These two conditions have rarely been reported among the various adverse effects of metrizamide. An 11-year-old girl had been in almost good health until she was ten years old, at which time she received a ventriculo-peritoneal shunt operation, suffering from hydrocephalus of unknown etiology. At the age of eleven, she was admitted to our hospital due to hydrocephalus recurrence. She was examined by metrizamide shunt-gram (1200 mg iodide/4 ml). On the next day, she became drowsy. The CT scan disclosed the periventricular penetration of metrizamide into the medial part of the thalamus and the caudate nucleus. Thirteen days later, disturbance of consciousness continued, and extrapyramidal symptoms, that is, rigo-spasticity and postural tremor, were observed. Oral administration of L-threo-DOPS, the direct precursor of noradrenaline, was effective against the persistent disturbance of consciousness and L-DOPA was effective against the extrapyramidal symptoms. She soon recovered almost to normal and no neurological deficit remained. We thus conclude that the CT scan findings and effects of L-threo-DOPS and L-DOPA suggest that metrizamide encephalopathy in this case were respectively due to its periventricular penetration into the medial part of the thalamus and the caudate nucleus, and the resultant deficiency of the ascending noradrenergic reticular activating system and the nigrostriatal dopaminergic system.
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PMID:[Metrizamide encephalopathy in a child with hydrocephalus--effects of L-threo-DOPS on persistent disturbance of consciousness and L-dopa on extrapyramidal symptoms]. 314 37

Dementia--a syndrome of acquired intellectual deterioration--is an etiologically non-specific condition which is permanent, progressive, or reversible. In the evaluation of demented patients, a careful exposure history will determine the possible role of drugs, metals, or toxins. The physical examination may reveal focal deficits in cases of intracranial mass lesions and spasticity or ataxia of the lower limbs if hydrocephalus is present. Coexistance of dementia and peripheral neuropathy usually indicates a toxic or metabolic disorder. Asterixis, myoclonus, and postural tremor are common in toxic-metabolic dementias, while resting tremor, choreoathetosis, and rigidity occur in progressive extrapyramidal disorders. EEG is focally abnormal in cases of cerebral mass lesions and exhibits generalized slowing in toxic-metabolic encephalopathies. CT will aid in the identification of hydrocephalus, subdural hematomas, and intracranial mass lesions. A thorough laboratory evaluation including complete blood count, erythrocyte sedimentation rate, electrolytes, blood urea nitrogen and blood sugar, liver and thyroid tests, calcium and phosphorus levels, B12 and folate levels, serum copper and ceruloplasmin, VDRL, chest X-ray, electrocardiogram, and lumbar puncture may demonstrate treatable disorders that are adversely affecting intellectual function. Elderly individuals are particularly susceptible to the effects of toxic or metabolic disorders, and a mild dementia might be exaggerated by relatively minor fluctuations in metabolic status. Treatable causes of dementia should be considered in all demented patients.
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PMID:[Treatable dementia syndromes]. 358 48

Of 99 children in the Royal Blind School, Edinburgh (which serves Scotland and part of N E England), 15 had optic atrophy (hydrocephalus 4, intracranial haemorrhage 2, prematurity 2, fetal distress 2, birth asphyxia 2, cerebral atrophy 1, cardiac arrest during hernia operation 1, and leukaemia 1). Fourteen had congenital cataract, 12 congenital retinal aplasia (Leber's congenital amaurosis) and 11 retinopathy of prematurity. There were small numbers in many other diagnostic categories, including three with non-accidental head injury. Mental retardation, spasticity, and nystagmus were frequent other correlates in all diagnostic categories. 'Very probably hereditary' was a conservative attribution in 36, while 'probable' seemed appropriate for 12-that is, almost 48% were hereditary. Only about 11 cases might have been prevented through genetic counselling, which testifies to the frequency of autosomal recessive hereditary disease, although no parents were consanguineous.
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PMID:Blindness in schoolchildren: importance of heredity, congenital cataract, and prematurity. 365 73

The neurosurgical role in rehabilitation rehabilitation was studied. Over a 5-year period, 850 individuals were referred to a rehabilitation center after initial acute care. Surgery was indicated for 66 patients. There were 28 quadriplegic or paraplegic individuals with intractable spasticity. Percutaneous radiofrequency foramenal rhizotomies were found to be 98% effective in relieving posttraumatic spasticity. In 14 patients with cognitive impairment, intellectual improvement had reached a plateau level. These persons underwent computed tomography scanning and cisternography, revealing significant communicating hydrocephalus. After surgical shunt therapy, cognitive improvement was noted in 86%. Nineteen individuals were sent for rehabilitation following spine fracture or progressive quadriplegia; 17 were found to have persistent spinal instability requiring surgical stabilization by fusion. This was successful in all cases without complications. Two persons required decompressive spinal operations, resulting in neurological stabilization or improvement. Five patients developed pain, spasticity, ascending neurological deficit, or autonomic dysreflexia due to posttraumatic syrinx. These symptoms were stabilized or improved following syringosubarachnoid shunting. The authors submit that comprehensive neurosurgical reevaluation is desirable in patients received for rehabilitation. Periodic neurosurgical follow-up is recommended. The neurosurgeon's role is not limited to the acute process.
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PMID:Rehabilitative neurosurgery. 370 90

Computerized analysis of gait was performed in six patients with hydrocephalus (mean age, 75.7 years), for comparison with other elderly patients with gait problems and age-matched controls. A decrease in velocity and stride, an increase in sway, and the proportion of time spent in double-limb stance were nonspecific features correlated with declining performance. Certain features appeared to characterize hydrocephalic gait when compared among groups; cadence was diminished, and there was a reduction of step height and a decreased counterrotation of the shoulders relative to the pelvis. An abnormal tendency toward cocontraction in antagonist muscle groups was observed in electromyographic data from the leg muscles, suggesting that the normal phased activation of muscle groups is disturbed. Although data are not conclusive, we believe that the gait disorder in normal-pressure hydrocephalus reflects a subcortical motor control disorder rather than a phenomenon of spasticity or apraxia.
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PMID:Gait disorder in late-life hydrocephalus. 382 77

Dementia, a syndrome of acquired intellectual deterioration, is an etiologically nonspecific condition that can be permanent or reversible. When evaluating demented patients, a careful exposure history will determine the possible role of drugs, metals, or toxins. Physical examination may reveal focal deficits in cases of intracranial mass lesions and spasticity or ataxia of the lower limbs if hydrocephalus is present. Coexistence of dementia and a peripheral neuropathy usually indicates the existence of a toxic or metabolic disorder. Depressed mood, sleep disturbance, anorexia, impotence, constipation, and psychomotor retardation indicate the presence of a depressive syndrome. Asterixis, myoclonus, and postural tremor are common in toxic-metabolic dementias, whereas resting tremor, choreoathetosis, or rigidity occur in progressive extrapyramidal disorder. EEG is focally abnormal in cases of cerebral mass lesions and shows generalized slowing in toxic-metabolic encephalopathies. CT will aid in the identification of hydrocephalus, subdural hematomas, and intracranial mass lesions. A thorough laboratory evaluation including complete blood count, erythrocyte sedimentation rate, electrolytes, blood urea nitrogen and blood sugar, liver and thyroid function tests, serum calcium and phosphorus levels, B12 and folate levels, serum copper and ceruloplasmin, VDRL, chest X-ray, electrocardiogram, and lumbar puncture may demonstrate treatable disorders that are adversely affecting intellectual function. Elderly individuals are particularly susceptible to the effects of toxic or metabolic disorders, and a mild dementia may be exaggerated by relatively minor fluctuations in metabolic status. Treatable causes of dementia should be sought in all demented patients.
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PMID:Treatable dementias. 635 58

Stenosis of the aqueduct of Sylvius accounts for about one third of cases of congenital hydrocephalus. At least 32 families have been reported in which the aqueduct stenosis is inherited in an X linked fashion. In half of these families, flexed adducted thumbs were noted in some affected family members. Occasionally other male members were mentally retarded, suggesting limited expression of the gene. The problem of giving genetic advice to an isolated, clinically unremarkable, case of aqueduct stenosis remains, so a family study was undertaken based on 24 such cases seen at The Hospital for Sick Children over a 19-year period. There were 15 male and nine female index patients. The diagnosis was confirmed in all cases by air encephalogram. One boy had a radial club hand and another developed clasped thumbs secondary to spasticity. No cases had hyaloidoretinal dysplasia. The 15 boys had 18 brothers and 19 sisters, of whom one sister was similarly affected. The nine girls had 12 sibs, none of whom was affected. This study, combined with a similar study in the USA, suggests that the empirical risk of recurrence of a sporadic case of aqueduct stenosis is about 4.5%.
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PMID:A family study of hydrocephalus resulting from aqueduct stenosis. 727 16


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