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Query: UMLS:C0004134 (ataxia)
 15,886 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We describe 3 children with a progressive encephalopathy that was characterised by irritability, convulsions, cranial nerve palsies, ataxia, nystagmus, walking difficulties, delayed psychomotor development, hemiplegia/tetraplegia, visual disturbance, vomiting, neck stiffness, and non-specific signs of raised intracranial pressure. A final diagnosis was made in all 3 patients from necropsy material. The clinical features were ascribed to multiple inflammatory, predominantly lymphocytic, reactions and raised intracranial pressure. This condition is an atypical form of haemophagocytic lymphohistiocytosis, which normally presents with fever, hepatosplenomegaly, and cytopenias. By contrast, the disease pattern in our 3 children was dominated by cerebromeningeal involvement, which can precede the typical systemic symptoms of haemophagocytic lymphohistiocytosis. An awareness of this condition is important because treatments are available.
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PMID:Cerebromeningeal haemophagocytic lymphohistiocytosis. 134 23

The case notes of 29 children ranging in age between 6 months and 17 years were reviewed for the period 1978-88. This study was done to determine if there were any differences when compared with other series in the developed countries. In addition, the distribution, tumour type, clinical presentations, and outcome were reviewed. Of the 29 tumours, 15 tumours were confined to the posterior fossa (52 per cent), and tumours were located supratentorially (48 per cent). Of the 15 tumours in the posterior fossa, five were medulloblastomas, four brain stem astrocytomas, and six cerebellar astrocytomas. The supra-tentorial tumours included, five astrocytomas, two pituitary tumours, two cranio-pharyngiomas, one hypothalamic glioma, and four intraventricular tumours. Clinical presentation was confined to symptoms and signs related to raised intracranial pressure, ataxia, seizures, and cranial nerve involvement. All the tumours were resected surgically except for the brain stem tumours, which were treated in most cases with radiation therapy. The overall mortality was 48 per cent (14 patients died). We were unable to comment on long-term survival except for a 100 per cent survival in patients with pituitary and cerebral tumours, and 67 per cent in patients with cerebellar astrocytomas.
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PMID:Primary pediatric brain tumours in Barbados: 10-year analysis (1978-1988). 202 66

Disproportionate enlargement or isolation of the fourth ventricle are rare complications of ventriculo-peritoneal shunt for post-haemorrhagic hydrocephalus. Obvious features such as ataxia may indicate raised intracranial pressure in the posterior fossa. The child may, however, present with less specific signs of vomiting, anorexia, and lethargy and these symptoms may be misinterpreted as secondary to dilation of the lateral ventricular system due to malfunction of the ventriculo-peritoneal shunt. Two children with disproportionate enlargement of the fourth ventricle and two children with isolation of the fourth ventricle are described to illustrate the wide variations in clinical presentation. These rare complications can be diagnosed by real time ultrasound examination of the brain or computed tomography of the head. They should be considered in the differential diagnosis of a child who deteriorates after lateral ventriculo-peritoneal shunting for post-haemorrhagic hydrocephalus, and dealt with specifically to avoid the risk of upward herniation of the enlarged fourth ventricle.
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PMID:Specific enlargement of the fourth ventricle after ventriculo-peritoneal shunt for post-haemorrhagic hydrocephalus. 349 66

Four cases of gliomatosis cerebri are reported that demonstrate the variability of the clinical course. A review of these and 32 cases from the literature revealed that the most striking finding was change in personality and mental status. Hemiparesis, ataxia, papilledema, headache, seizure, and brainstem signs were the next most common clinical findings. Laboratory and radiographic tests, including angiography and pneumoencephalography, often showed only minor and nonspecific changes in the face of profound mental deterioration. Increased intracranial pressure usually occurred late but was probably an important factor of the terminal course in most patients. The pathologic changes were typical, with diffuse infiltration of astrocytoma cells through brain stem, subcortical white matter, and, to a lesser extent, cerebral cortex.
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PMID:Gliomatosis cerebri. Report of four cases and review of the literature. 449 66

Two young males from Central Province were referred to Port Moresby General Hospital with chronic headaches. In each case the initial impression of tuberculous meningitis needed revision in the light of strongly positive cryptococcal serology. Cryptococcal meningitis occurs with sufficient frequency in Papua New Guinea that it should be considered in cases of raised intracranial pressure, ataxia, cranial neuropathy and visual disturbances. Delay in treatment may be disastrous. In contrast to the developed world where fungal meningitides show a predilection for the immunosuppressed, the experience to date in Papua New Guinea is that the majority of cases in both adults and children occur in ostensibly immunocompetent individuals. The epidemiology, clinical course and current treatment of this potentially curable disease are discussed.
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PMID:Cryptococcal meningitis in children in Papua New Guinea: a reminder. 766 53

We describe a case of congenital intra-fourth ventricular arachnoid cyst and review the literature. A one-and-half-year-old boy had presented with delayed milestones, intermittent raised intracranial pressure and ataxia. Excision of the arachnoid cyst has cured him.
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PMID:Arachnoid cyst within the fourth ventricle--a case report. 856 42

Alexander disease is usually classified according to the age of onset, e.g. an infantile form with onset during the first two years of life, a juvenile form with onset in childhood, mainly school age. It has been recognized, however, that the clinical course can be very variable within these groups. Thus, this clinical classification is not a useful predictor of severity and progression of the disease. This is demonstrated here on the basis of the history of seven own patients and a literature review. Only an onset in very early infancy, during the neonatal period, seemed to be associated with a rather uniform pattern of disease course, often leading to early death. This neonatal form showed very stereotyped symptoms, in part different from later onset: Early, often intractable, generalized seizures; hydrocephalus with raised intracranial pressure due to aqueductal stenosis because of pathological astroglia proliferation; lack of developmental progression but without prominent spasticity or ataxia; elevated CSF protein content. This was associated with the well-established neuroradiological findings, e.g. severe white matter affection with fronto-temporal predominance, involvement of basal ganglia and periventricular enhancement as an obligatory symptom. The identification of this early onset form is especially important as seizures and signs of raised intracranial pressure may mislead the diagnosis.
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PMID:Alexander disease--classification revisited and isolation of a neonatal form. 1083 83

Primary malignant melanoma of the leptomeninges is a rare and aggressive tumor in children and accounts for less than 1% of all pediatric malignancies. Usually its symptoms include raised intracraneal pressure resulting from hydrocephalus secondary to tumoral obliteration of basal cisterns, but the passage of time from the initial symptomatology to diagnosis is frequently delayed. A 7-year-old male with primary leptomeningeal melanoma is reported. At the beginning, he presented ataxia and dysarthria followed by symptoms of raised intracranial pressure, complex partial seizures, progressive loss of consciousness, and coma. Cerebrospinal fluid analysis demonstrated raised opening pressure, normal glucose, and increased protein concentration, but malignant melanoma cells were not found. Magnetic resonance imaging scans depicted bright signals in the subarachnoid spaces on T(1) images and gadolinium-enhanced focal lesions. Cerebral biopsy was proposed, but it was not authorized. Definitive diagnosis was thus made by pathologic postmortem examination.
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PMID:Primary leptomeningeal melanoma in a child. 1151 18

A 6-year-old girl, previously diagnosed with neurofibromatosis type 1 (NF-1) presented with ataxia and symptoms of raised intracranial pressure. Diagnostic work up disclosed a posterior fossa tumor. Histopathological study of the excised neoplasm showed a cerebellar medulloblastoma. We review the current literature and suggest that the association of medulloblastoma with NF-1 is not a chance occurrence, and that it might be pathogenically related. We propose that medulloblastoma should be added to the list of malignancies that are apt to occur in NF-1.
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PMID:Medulloblastomas in neurofibromatosis type 1. Case report and literature review. 1205 4

An extremely unusual case demonstrating rare behaviour of an ependymoma is presented. The tumour spread in an en-plaque fashion and involved the ependymal lining of the entire ventricular system. The patient, a 56-year-old male, presented with symptoms of raised intracranial pressure and truncal ataxia. The management issues are analyzed on the basis of a brief literature survey.
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PMID:En-plaque ventricular ependymoma. 1263 69


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