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  • 1
    ISSN: 1432-1076
    Keywords: Acute lymphoblastic leukemia ; Cognitive functions ; Psychomotor speed ; School achievements
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Several studies have reported a decline in intelligence and cognitive functions in survivors of childhood acute lymphoblastic leukemia (ALL). Other investigators, however, have found no intellectual impairment in these children. Fifty-one long-term survivors of ALL, having been treated according to the protocols of the BFM Study Group from 1970 to 1979, were assessed retrospectively using neurophysical methods. The results were compared with those obtained from 30 patients with other malignancies, who had received neither radiation therapy to the central nervous system (CRT) nor any methotrexate during chemotherapy. Additionally, neurological examinations and cranial computed tomography (CCT) were performed. Neuropsychological examinations included verbal functions, intelligence (performance), psychomotor speed, motor skills and sensory integration. The results of verbal tests and the IQs, tested by nonspeed-related measures, were within normal limits in both groups. About one-third of all patients showed mild disturbances of psychomotor speed and motor skills. Children with leukemia had lower scores than those with solid tumors for nearly all tasks, but only tests for sensory integration revealed significant differences between former ALL patients and tumor patients. Furthermore, the following results were obtained related to different therapeutic modalities: (1) The higher total radiation doses had been during CRT (maximum 24 GY), the more neuropsychological functions were impaired, particularly motor accuracy and sensory integration. (2) These disturbances improved with the length of survival. Widening of subarachnoidal space was found in 33% of the CCT obtained. There was no correlation between the intellectual functions of the survivors and the CCT abnormalities. Neurological findings mainly consisted of slight fine motor disturbances. The results of the study suggest that psychomotor functions are more likely to be affected by antineoplastic therapy than are higher cognitive functions.
    Type of Medium: Electronic Resource
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  • 2
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 137 (1981), S. 49-57 
    ISSN: 1432-1076
    Keywords: Cerebrospinal fluid ; Protein electrophoresis ; Acute cerebellar ataxia ; Medulloblastoma ; Astrocytoma ; Pontine glioma
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract CSF-protein profiles of 25 children with acute cerebellar ataxia and of 39 children with intracranial midline tumours at diagnosis were examined by quantitative zone electrophoresis in agarose gel. The profiles were compared with those obtained from a control group of 86 cases, and those from 61 patients with aseptic meningitis and 40 children with bacterial meningitis. The data from the latter groups demonstrated the CSF-protein pattern of moderate or severe disturbance of the blood-CSF barrier (B-CSF-B), respectively. The children with acute cerebellar ataxia showed minor signs of a B-CSF-B impairment and no increase of γ-globulin. These findings point to a slight acute vascular lesion. CSF changes indicative of a moderate-to-severe dysfunction of the B-CSF-B occurred in the majority of the patients with cerebellar astrocytomas, pontine gliomas, tumours around the 3rd ventricle, and medulloblastomas. Therefore acute cerebellar ataxia can be differentiated from intracranial midline tumours in most cases by means of CSF-protein electrophoresis. A striking finding was that 12 out of 14 children with medulloblastomas revealed a marked increase of γ-globulin. Since in 5 of these cases oligoclonal γ-globulin could be detected, this finding means local immunoglobulin synthesis within the CNS. The marked increase of γ-globulin which almost exclusively occurred in association with medullo-blastomas allows their seperation from acute cerebellar ataxia and the other tumour groups. Quantitative agarose gel electrophoresis can be a complementary diagnostic test in children with acute ataxia and suspected of having a CNS infection, or in cases with a negative CT brain scan in which intracranial midline tumour is a likely possibility.
    Type of Medium: Electronic Resource
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  • 3
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 141 (1983), S. 122-126 
    ISSN: 1432-1076
    Keywords: Adducted thumb syndrome ; Congenital myopathy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We report on a female newborn baby with a “myopathic” stiff face, open mouth, high-arched palate, microgenia, generalized muscular hypotonia, limited extension of elbows, wrists and knees, flexed adducted thumbs, velopharyngeal insufficiency, and hypertrichosis. Death occurred at 3 months due to respiratory insufficiency. Muscular biopsy revealed myopathic abnormalities.
    Type of Medium: Electronic Resource
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