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Query: UMLS:C0026838 (spasticity)
 6,471 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

In approximately 10,000 admissions in a 12 months period, at University Teaching Hospital, Lusaka 85 cases of meningitis were recorded. The signs and symptoms in these patients do not greatly differ from other similar studies in Africa. The commonest organism isolated was pneumococcus. There was high mortality rate which was to a large extent due to parents not bringing their children to hospital early enough for medical treatment to be instituted. This is borne out by the fact that 50% of the children with meningitis died within the first 24 hours. The C.S.F. protein and peripheral white blood count may be of prognostic value. Spasticity, cranial nerve palsises hydrocephalus and subdural effusion were the commonest complications.
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PMID:Bacterial meningitis in infancy and childhood in Lusaka (One year prospective sturdy). 0 34

A total of 160 patients suspected of having acquired hydrocephalus were studied either by quantitative isotope ventriculography (QIV) or by lumbar isotope cisternography (LIC). Of these patients, 56 had hydrocephalus. Mental deterioration, gait disturbances, ataxia, spasticity, and incontinence were most frequently present in the hydrocephalic patients, but none of the signs or combinations thereof are pathognomonic of acquired hydrocephalus. These signs are independent of the intracranial pressure (ICP) and the type of hydrocephalus. Surgical shunt procedures were in most cases followed by the disappearance of mental deterioration, gait disturbances, ataxia, and spasticity.
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PMID:Acquired hydrocephalus. I. A clinical analysis of 160 patients studied for hydrocephalus. 31 40

1. Between 1965 and 1974 146 patients with unexplained tetraspasticity were admitted and examined. In 123 cases extended neuroradiologic examination with myelography of the cervical spinal canal was carried out. A space occupying lesion was found in 59 cases: narrow spinal canal, protruding discs. In 64 cases no pathologic processes were seen radiologically. Comparison of both groups: no differences in clinical signs, history or findings. 2. Tetraspasticity alone was the leading sign in 30 cases. The legs were always more severely involved than the upper limbs. In 70% spasticity was more severe on the right. Further clinical analysis depends on additional signs, particularly paresthesiae, pain, disturbed joint-sense. Among the patients with protruding discs heart-and circulatory insufficiency is a little more common, but in the group without protrusion exogenous/endogenous metabolic conditions(intoxication, malabsorption) and neoplasms. -Protein content of CSF is raised equally in both groups, particularly albumin. This is probably due to reduced circulation of CSF. In 6 patients an internal hydrocephalus was found. 3. No single active causative factor could be found nor any familial relationship. In spite of increasingly extended diagnostic techniques no underlying condition could be discovered. Tetraspastic is a "polygenetic" reaction of the central nervous system without a final common path. The cases show that mechanical factors (cervical myelopathy) predispose locally to non-mechanical injuries.
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PMID:[Unexplained tetraspasticity in adults (author's transl)]. 58 67

Although the syndrome of normal pressure hydrocephalus (NPH) was described in the adult as early as 1964, it has only recently been recognized in the child. In this preliminary report, eight myelomeningocele patients with presumed NPH were evaluated before and after ventricular shunting procedures. Cranial computed tomography and serial psychological testing have proved to be particularly valuable both in the pre-operative and post-operative assessment of these patients and have the distinct advantage of being simple, non-invasive diagnostic measures. Continuous intra-ventricular pressure monitoring has shown what promises to be characteristic elevated pressure plateaux imposed on normal baseline cerebrospinal fluid (CSF) pressures in so-called NPH but is a more difficult clinical procedure, necessarily associated with potential complications. Although decreasing response to growth-stimulating hormone can be demonstrated in patients with long-standing hydrocephalus, this endocrine malfunction cannot be considered an early indicator of intracranial pathology. Single IQ scores are inadequate measures of intellectural function in children with NPH and serial examinations should be carried out. Detailed neuropsychological testing will document performance IQ scores well below verbal IQ scores and will generally show failure of psychomotor development to keep pace with chronological ageing. Initial studies indicate that improved performance scores can be expected within 1 1/2 to 3 months following successful ventricular shunting operations, and that any downward trend in pre-operative test scoring can at least be reversed. Statistically significant improvements in full-scale IQ scores have not been seen, however, before the end of the first post-operative year. Clinically, improved attentiveness and sociability, and decreased spasticity (if present prior to surgery) can be expected following shunting. Over-all, ventriculomegaly, normal CSF pressure, stable head size, and non-progressive neurological symptoms cannot be regarded as sufficient criteria for the diagnosis of an arrested state of hydrocephalus, and should suggest NPH, especially in those children who demonstrate a discrepancy between performance and verbal IQ scores and who fail to exhibit continuing psychomotor development with advancing age.
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PMID:Normal pressure hydrocephalus in patients with myelomeningocele. 79 14

A new family is reported of a Bickers-Adams-Edwards syndrome. This family has been studied up to three generations. Two female carriers are known. Among the six male children who are affected, four are severely mentally retarded, have spasticity of the legs, and survived with a mild macrocephaly, and two show a more severe and rapid progression of head enlargement. A partial aqueductal stenosis, with remarkable ventricular dilatation, has been demonstrated by pneumoence-phalography in three boys. A deformity of the thumbs links these six children together. One of them has been treated by a ventriculoperitoneal shunt, when 18 months old, without any improvement in the neurological condition. The mental deficiency is much more severe than could be expected from the degree of hydrocephalus, at least as estimated clinically by the macrocephaly. Hydrocephalus is precocious, and the ventricular dilatation very advanced when seen by PEG studies. Recognition of the female carriers is not possible.
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PMID:X-linked hydrocephalus, with aqueductal stenosis, mental retardation, and adduction-flexion deformity of the thumbs. Report of a family. 99 65

Five cases of myelodysplasia with progressive paraparesis are presented. Three of the five patients developed spasticity, but dissociated sensory loss and loss of sphincter control was not a prominent feature. All were found to have compensated hydrocephalus and extensive communicating hydromyelia. The use of myelography and ventriculography in the diagnosis of hydromyelia is discussed. Ventricular drainage led to clinical improvement in two cases and radiological improvement in one. The relationship of compensated hydrocephalus, meningomyelocele, and progressive hydromyelia postnatally may support the hydrodynamic hypothesis of myelodysplasia.
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PMID:Meningomyelocele and progressive hydromyelia. Progressive paresis in myelodysplasia. 109 75

A Danish family with seven males with hydrocephalus in three generations is described. Frequent findings in this x-linked recessive form of the disease are irregular cranium, asymmetrical face, thumb held across palm, spasticity of the legs and paradoxical plantar responses. The literature on this mode of inheritance is reviewed.
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PMID:Sex-linked hydrocephalus. 118 43

X-linked hydrocephalus-stenosis of the aqueduct of Sylvius sequence (H-SAS, MIM number 30007) is a rare genetic disorder characterized by hydrocephalus, macrocephaly, adducted thumbs, spasticity, agenesis of corpus callosum and mental retardation. We confirm here the localisation of the mutant gene on Xq (Xq 2.8) by linkage analysis in a 5-generation pedigree (maximum lod score of Z = 4.57 at theta = 0.04 with probe St14 at locus DXS52) and emphasise the phenotypic variability of the disease. Ventricular dilatation in affected males was either severe and diagnosed antenatally or moderate and consistent with a long survival with little or no macrocephaly. Since other X-linked syndromes of mental retardation with spasticity and flexion deformities of the thumbs have previously been shown to map to the Xq 2.8 region as well (e.g. MASA syndrome and spastic paraplegia), the present results raise the question of whether H-SAS syndrome, MASA syndrome and spastic paraplegia with mental retardation might represent different phenotypic expression of various mutations at the same locus.
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PMID:X-linked hydrocephalus: clinical heterogeneity at a single gene locus. 139 13

This report describes a new syndrome of dysgenesis of corpus callosum with other anomalies, presenting as microcephaly, mental retardation, spasticity, and unusual facial appearance in 2 Chinese brothers and their maternal cousins. To date, there has not been any case reported in the Chinese population of this syndrome. All 4 patients in this report present with the same unusual face. Hydrocephalus and/or interhemispheric cyst were found among them. This syndrome is transmitted as an X-linked trait. The nosology is reviewed and discussed.
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PMID:X-linked recessive inheritance of dysgenesis of corpus callosum in a Chinese family. 148 21

Alexander's disease is reported in a 6-month-old infant. The clinical course was characterized by hydrocephalus, irritability and psychomotor retardation, with subsequent seizures and spasticity. Findings on ultrasound examination, computerized tomography, magnetic resonance imaging and brain biopsy are presented. The glial nature of the Rosenthal fibers was confirmed by immuno-gold staining. Clinical classification and differential diagnosis are discussed.
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PMID:Alexander's disease: a case report with brain biopsy, ultrasound, CT scan and MRI findings. 165 Mar 1


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