NeuropadiatriePub Date : 1980-02-01DOI: 10.1055/s-2008-1071376
W K Baier
{"title":"The \"startle disease\" in brain-damaged patients: report of a case.","authors":"W K Baier","doi":"10.1055/s-2008-1071376","DOIUrl":"https://doi.org/10.1055/s-2008-1071376","url":null,"abstract":"<p><p>It is reported about one case of so-called startle disease in brain-damaged patients in a 7 year-old girl. Furthermore, two related, non-epileptic paroxysmal startle syndromes, the \"essential startle disease\" and the \"hereditary startle syndrome\" are described and contrasted with \"epileptic seizures induced by unexpected sensory stimuli\" or \"startle epilepsy\". The overt participation of conditioning factors in the present case is understood as a hint at a possible therapeutic access.</p>","PeriodicalId":76211,"journal":{"name":"Neuropadiatrie","volume":"11 1","pages":"72-5"},"PeriodicalIF":0.0,"publicationDate":"1980-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1055/s-2008-1071376","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"17944823","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
NeuropadiatriePub Date : 1980-02-01DOI: 10.1055/s-2008-1071377
G Holmgren, H K Blomquist, G Samuelson
{"title":"Positive effect of a late introduced modified diet in an 8-year-old pku child.","authors":"G Holmgren, H K Blomquist, G Samuelson","doi":"10.1055/s-2008-1071377","DOIUrl":"https://doi.org/10.1055/s-2008-1071377","url":null,"abstract":"<p><p>A modified low phenylalanine diet was instituted in an 8-year-old PKU boy earlier untreated and mentally retarded. The boy was treated during a ten year observation period. A marked progress in mental capacity and in IQ scoring was seen. The difficulties in objective evaluation of the benefit of late introduced treatment with a diet low in phenylalanine is discussed.</p>","PeriodicalId":76211,"journal":{"name":"Neuropadiatrie","volume":"11 1","pages":"76-9"},"PeriodicalIF":0.0,"publicationDate":"1980-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1055/s-2008-1071377","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"17944824","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
NeuropadiatriePub Date : 1980-02-01DOI: 10.1055/s-2008-1071370
F Hanefeld, H Riehm
{"title":"Therapy of acute lymphoblastic leukaemia in childhood: effects on the nervous system.","authors":"F Hanefeld, H Riehm","doi":"10.1055/s-2008-1071370","DOIUrl":"https://doi.org/10.1055/s-2008-1071370","url":null,"abstract":"<p><p>A number of neurological disturbances occur during the treatment of childhood malignancies with cytotoxic drugs like vincristine, methotrexate, cytosine-arabinoside, cyclophosphamide, asparaginase and others. Neurological complications range from peripheral neuropathy, myopathy, myelopathy to encephalopathy with methotrexate induced encephalopathy leading to permanent brain damage or death in most cases. Irradiation of the brain can produce transient or permanent brain damage by a direct effect on nervous tissue and by altering the blood brain barrier or the blood circulation. The many conflicting reports concerning the quality of life of long-term survivors of childhood leukaemia do not give sufficient data for final conclusions. The experiences we have encountered over the past ten years seem to confirm the optimistic echo from others. There is, however, an urgent need for a prospective study to answer the all important question: will a child suffering from leukemia emerge physically and mentally unharmed after long and aggressive treatment?</p>","PeriodicalId":76211,"journal":{"name":"Neuropadiatrie","volume":"11 1","pages":"3-16"},"PeriodicalIF":0.0,"publicationDate":"1980-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1055/s-2008-1071370","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"17321961","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
NeuropadiatriePub Date : 1980-02-01DOI: 10.1055/s-2008-1071373
Y A Shapira, M H Jones, S P Sherman
{"title":"Abnormal eye movements in hyperkinetic children with learning disability.","authors":"Y A Shapira, M H Jones, S P Sherman","doi":"10.1055/s-2008-1071373","DOIUrl":"https://doi.org/10.1055/s-2008-1071373","url":null,"abstract":"<p><p>Eye movements were studied in 29 school children aged 6--12 years diagnosed as having Hyperactivity (HA) with Learning Disabilities (LD) and compared to an age matched control group of 32 children. The children had to track a moving target on a metronome and a canopy, to maintain eye fixation on a stationary target and to read a standard reading material. Accuracy, nystagmoid, reversal and overshoot movements were observed, as well as head movements and blinks. Persistence and deviations were monitored during fixation; duration, accuracy, number of fixations and reversals were monitored during reading. The tracking accuracy and fixation persistence were significantly inferior in the HA-LD children as compared to the control group in most items. This correlated well with the inferior performance on the reading task. This study supports the hypothesis that children with MBD and learning disabilities have primary defects in the motor control of eye movements which contribute significantly to their reading difficulties.</p>","PeriodicalId":76211,"journal":{"name":"Neuropadiatrie","volume":"11 1","pages":"36-44"},"PeriodicalIF":0.0,"publicationDate":"1980-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1055/s-2008-1071373","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"17944821","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
NeuropadiatriePub Date : 1980-02-01DOI: 10.1055/s-2008-1071375
G Lyon, C Griscelli, E Fernandez-Alvarez, J Prats-Vinas, P Lebon
{"title":"Chronic progressive encephalitis in children with x-linked hypogammaglobulinemia.","authors":"G Lyon, C Griscelli, E Fernandez-Alvarez, J Prats-Vinas, P Lebon","doi":"10.1055/s-2008-1071375","DOIUrl":"https://doi.org/10.1055/s-2008-1071375","url":null,"abstract":"<p><p>This report is on six cases of a chronic relentlessly progressive encephalitis occurring in boys with congenital hypogammaglobulinemia presumably of the x-linked type, which are thought to represent a separate neurological entity. Intellectual deterioration, dysarthria, spasticity, ataxia, optic atrophy and an increase of lymphocytes in the cerebrospinal fluid, were the main clinical signs. The pathological picture was that of a viral encephalitis, but all virological investigations on brain biopsies and CSF were negative. The significance of intra-cisternal tubuloreticular inclusions in brain endothelial cells, similarities with chronic rubella encephalitis, and the role of the immunological deficiency are discussed. Sofar, the cause of this new type of encephalitis remains obscure.</p>","PeriodicalId":76211,"journal":{"name":"Neuropadiatrie","volume":"11 1","pages":"57-71"},"PeriodicalIF":0.0,"publicationDate":"1980-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1055/s-2008-1071375","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"17321962","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
NeuropadiatriePub Date : 1980-02-01DOI: 10.1055/s-2008-1071371
P G Barth, F C Stam, R F Oosterkamp, P D Bezemer, P A Koopman
{"title":"On the relationship between germinal layer haemorrhage and telencephalic leucoencephalopathy in the preterm infant.","authors":"P G Barth, F C Stam, R F Oosterkamp, P D Bezemer, P A Koopman","doi":"10.1055/s-2008-1071371","DOIUrl":"https://doi.org/10.1055/s-2008-1071371","url":null,"abstract":"The brains of 54 deceased preterm infants were retrospectively studied for signs of germinal layer haemorrhage (GLH) and telencephalic leucoencephalopathy (TLE). On the basis of presence or absence of iron laden macrophages (siderophages) the cases with germinal layer haemorrhage were sub-classified as either \"old\" or \"fresh\" haemorrhage. Cases without GLH (non-GLH) were used as a control group. The distribution of ages at death in the non-GLH group was such that comparison to both fresh GLH cases and old GLH cases was possible. The distribution of pregnancy duration in GLH and non-GLH cases was similar. Statistical evaluation showed that old GLH cases were more often associated with TLE as either fresh GLH or non-GLH cases. By comparison of the three groups it is shown that differences in age at death do not explain the higher incidence of TLE in the old GLH group compared to the fresh GLH group. Hydrocephalus was found in the majority of old GLH cases. From a neuropathological view this complication is unlikely to account for the excess of TLE-lesions in that group. A logic explanation of the findings in this study is that the association between GLH and TLE is due to a common origin: asphyxia. The relatively low incidence of TLE in fresh GLH cases may be due to early death precluding detection of TLE. It is advocated that studies on GLH or TLE be comprehensively based on both phenomena to further the understanding on how they contribute to death or handicap.","PeriodicalId":76211,"journal":{"name":"Neuropadiatrie","volume":"11 1","pages":"17-26"},"PeriodicalIF":0.0,"publicationDate":"1980-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1055/s-2008-1071371","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"17943294","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
NeuropadiatriePub Date : 1980-02-01DOI: 10.1055/s-2008-1071380
E Auff, H Schuster
{"title":"[Giant connatal glioma (author's transl)].","authors":"E Auff, H Schuster","doi":"10.1055/s-2008-1071380","DOIUrl":"https://doi.org/10.1055/s-2008-1071380","url":null,"abstract":"<p><p>A clinico-neuropathological report is given on a four months old girl with head enlargement first recognized at the age of four weeks. A giant intracranial tumor was found at autopsy, which was histologically an isomorphous, largely pilocytic astrocytoma without morphological features suggesting rapid growth. This connatal astrocytoma seems unique in size, suggesting tumor formation already during very early embryonal development.</p>","PeriodicalId":76211,"journal":{"name":"Neuropadiatrie","volume":"11 1","pages":"91-5"},"PeriodicalIF":0.0,"publicationDate":"1980-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1055/s-2008-1071380","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"17944826","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
NeuropadiatriePub Date : 1980-02-01DOI: 10.1055/s-2008-1071378
K Maekawa, H Ohta, I Tamai
{"title":"Transient brain shrinkage in infantile spasms after ACTH treatment. Report of two cases.","authors":"K Maekawa, H Ohta, I Tamai","doi":"10.1055/s-2008-1071378","DOIUrl":"https://doi.org/10.1055/s-2008-1071378","url":null,"abstract":"<p><p>This is the report of two cases of infantile spasms, manifesting transient brain shrinkage in computerized tomography (CT) after ACTH treatment. ACTH was given for 8 weeks to a 8-months-old Japanese girl with infantile spasms. First CT performed at 2 weeks after the final ACTH injection, displayed moderate brain shrinkage. Second CT at 4 months showed marked diminution of the shrinkage. ACTH was also given for 8 weeks to a 14 months old Japanese boy with infantile spasms. First CT, just before ACTH treatment, showed mild cortical atrophy, the second at 7 days after the final ACTH injection revealed marked brain shrinkage and moderate ventricular dilatation, and the third at 2 months, disclosed mild improvement of the shrinkage. ACTH or corticoateroid has widespread effects on the developing nervous system. In animal experiments, ACTH or steroids interfere with brain growth of young rats. CT findings of transient brain shrinkage in a child with infantile spasms might suggest that intensive treatment with ACTH or steroids in infancy interferes with brain growth as seen in the results of animal experiments.</p>","PeriodicalId":76211,"journal":{"name":"Neuropadiatrie","volume":"11 1","pages":"80-4"},"PeriodicalIF":0.0,"publicationDate":"1980-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1055/s-2008-1071378","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"17321963","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
NeuropadiatriePub Date : 1980-02-01DOI: 10.1055/s-2008-1071374
J Heijbel, S Blom, P G Bergfors
{"title":"Simple febrile convulsions. A prospective incidence study and an evaluation of investigations initially needed.","authors":"J Heijbel, S Blom, P G Bergfors","doi":"10.1055/s-2008-1071374","DOIUrl":"https://doi.org/10.1055/s-2008-1071374","url":null,"abstract":"<p><p>A prospective epidemiological study of the incidence of simple febrile convulsions (FC) was performed in a northern Swedish county. The yearly incidence was 700/100 000. The children with FC were re-examined three years later. The pre- and perinatal complications were significantly more common in the group of children with FC than in a randomly collected group of controls. The value of an extensive investigation program is discussed. Between six months and five years of age the children with FC can be separated from those with other cerebral diseases causing epileptic seizures by recording a thorough history followed by a somatic and neurological physical examination and a determination of acute blood glucose. In children less than six months of age and in children in which meningitis or encephalitis cannot be ruled out, a lumbar puncture and a blood culture should be performed. If the onset of the convulsion has been after five years of age, if the seizures are partial or have a duration of more than 30 minutes, or if signs of cerebral disease are found, the examination should be supplemented with EEG and relevant investigations. There is no need for an extensive routine program of investigations in the care of children with FC.</p>","PeriodicalId":76211,"journal":{"name":"Neuropadiatrie","volume":"11 1","pages":"45-56"},"PeriodicalIF":0.0,"publicationDate":"1980-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1055/s-2008-1071374","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"17944822","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
NeuropadiatriePub Date : 1980-02-01DOI: 10.1055/s-2008-1071379
T Deonna, L S Prod'hom
{"title":"Temporal lobe epilepsy and hemianopsia in childhood of perinatal origin. An overlooked and potentially treatable disease? Report of two cases, one with a demonstrable etiology.","authors":"T Deonna, L S Prod'hom","doi":"10.1055/s-2008-1071379","DOIUrl":"https://doi.org/10.1055/s-2008-1071379","url":null,"abstract":"<p><p>Two children with a history of traumatic delivery developed severe temporal lobe epilepsy starting at age 4 and 10 respectively. The main neurological finding was total homonymous hemianopsia. The CAT Scan showed a large localised hypodensity in the occipital region on one side consistent with an old infarction in that region. A similar clinical syndrome was described in 1974 by Remillard as temporal lobe epilepsy and perinatal occlusion of the posterior cerebral artery (PCA). In one of our cases, the clinical course suggested that vascular occlusion in the PCA territory resulted from temporal lobe herniation related to a subdural hematoma at birth. It is proposed that this mechanism may apply to some instances of that condition. This clinical syndrome is now increasingly diagnosed as a large number of epileptics are now submitted to CAT. The diagnosis can be delayed until a seizure disorder develops because of the frequent absence of obvious neuromotor signs or visual complaints in the first years of life. Early recognition of this particular type of neonatal cerebral injury could possibly lead to prevention or early treatment.</p>","PeriodicalId":76211,"journal":{"name":"Neuropadiatrie","volume":"11 1","pages":"85-90"},"PeriodicalIF":0.0,"publicationDate":"1980-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1055/s-2008-1071379","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"17944825","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}