NeuropadiatriePub Date : 1979-05-01DOI: 10.1055/s-0028-1085321
E Del Giudice, J Aicardi
{"title":"Atypical aspects of hypertensive encephalopathy in childhood.","authors":"E Del Giudice, J Aicardi","doi":"10.1055/s-0028-1085321","DOIUrl":"https://doi.org/10.1055/s-0028-1085321","url":null,"abstract":"<p><p>Four patients with hypertensive encephalopathy and misleading focal symptomatology are reported in order to point out the problems of differential diagnosis. The first patient, together with a classical syndrome of hypertensive encephalopathy, had peculiar EEG features consistent with a possible diagnosis of herpes simplex encephalitis. Case 2 presented with a complex clinical syndrome associating bizarre spells suggestive of a psychiatric condition with ocular symptoms pointing to an upper brain stem involvement. The third child was remarkable because of the presence of neuroradiological signs compatible with a space-occupying lesion of the posterior fossa not eventually found after a surgical exploration performed as an emergency procedure. The last patient had central nervous system signs in the context of a Guillain-Barré syndrome: in this case the central symptomatology would not fit the already described pattern of encephalomyelo-radiculo-neuropathy but had to be entirely ascribed to the ill controlled arterial hypertension.</p>","PeriodicalId":76211,"journal":{"name":"Neuropadiatrie","volume":"10 2","pages":"150-7"},"PeriodicalIF":0.0,"publicationDate":"1979-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1055/s-0028-1085321","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"11789357","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 : 1979-05-01DOI: 10.1055/s-0028-1085320
Y Fukuyama, Y Ochiai, T Hayakawa, F Miyagawa
{"title":"Overnight sleep EEG and cerebrospinal fluid monoamines in seizures induced by movement.","authors":"Y Fukuyama, Y Ochiai, T Hayakawa, F Miyagawa","doi":"10.1055/s-0028-1085320","DOIUrl":"https://doi.org/10.1055/s-0028-1085320","url":null,"abstract":"<p><p>Clinical, electrical and biochemical studies in an eight years old boy with movement-induced seizures were reported. The attack was usually triggered by sudden initiation of movement, but rarely occurred without any apparent movement. Repeated jumping provoked attacks constantly, which was recorded cinematographically. No abnormality was found either in ictal or interictal EEGs. Haloperidol aggravated the condition, but 1-DOPA had no effect, while DPH (100 mg/day) controlled attacks perfectly with the serum DPH concentration of just 2.0 ug/dl. In overnight sleep analysis, sleep rhythms and characters of REM sleep were not differed significantly from the standard. After DPH therapy, stabilization of sleep in general was noticed; that is, total sleep time prolonged, number of sleep stages decreased and interrupting awakening disappeared. Probenecid loading test revealed that 5-HIAA was normal, HVA high, and large amount of octopamine was detected in CSF.</p>","PeriodicalId":76211,"journal":{"name":"Neuropadiatrie","volume":"10 2","pages":"138-49"},"PeriodicalIF":0.0,"publicationDate":"1979-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1055/s-0028-1085320","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"11441658","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 : 1979-05-01DOI: 10.1055/s-0028-1085319
J H Ehrich, L Stoeppler, G Offner, J Brodehl
{"title":"Evidence for cerebral involvement in nephropathic cystinosis.","authors":"J H Ehrich, L Stoeppler, G Offner, J Brodehl","doi":"10.1055/s-0028-1085319","DOIUrl":"https://doi.org/10.1055/s-0028-1085319","url":null,"abstract":"<p><p>Cranial computerized tomography (CCT) of 3 children with nephropathic cystinosis and chronic renal failure (CRF) revealed a hydrocephalus internus and externus. In two boys the findings consisted of bilateral dilatation of the ventricular system and of the subarachnoid space; in one boy the alterations were mainly unilateral. The children had repeated convolsions which could not be explained by deterioration of renal function. Their neurological condition was otherwise normal. In six non-cystinotic patients with chronic renal failure, CCT showed normal anatomical structures. The possibility is discussed that the hitherto unknown pathogenetic mechanism of cystinosis leads to diffuse cerebral atrophy, resulting in internal and external hydrocephalus.</p>","PeriodicalId":76211,"journal":{"name":"Neuropadiatrie","volume":"10 2","pages":"128-37"},"PeriodicalIF":0.0,"publicationDate":"1979-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1055/s-0028-1085319","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"11789356","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 : 1979-05-01DOI: 10.1055/s-0028-1085324
J Wiesse, G Ulrich, W Scheuler
{"title":"[A contribution to the psychophysiology of infantile dysmegalopsy (author's transl)].","authors":"J Wiesse, G Ulrich, W Scheuler","doi":"10.1055/s-0028-1085324","DOIUrl":"https://doi.org/10.1055/s-0028-1085324","url":null,"abstract":"<p><p>A 9 year old boy was able to evoke microptic experiences voluntarily. Clinical and historical signs of minimal brain dysfunction were present. An epileptic etiology could neither be proven nor ruled out. Synchronized EEG- and Video recording allowed us to analyze the EEG and the corresponding behavior by using sequential spectral analysis of the EEG data. Coinciding with a progressive increase in rhythmic 6-7 cps. activity we observed a behavior which appeared to correspond to a state of narrowed attention. He signalized the onset of a micropsy a few seconds after the transition from this pattern into one which showed a relative voltage decrease and the reappearance of some alpha-activity. The end was signalized by him coinciding with the recurrence of normal waking activity. Our findings--like those of others--suggest that the gradual development of hypnagogic phenomena is bound to a state of lowered vigilance. The reappearance of alpha activity and the voltage decrease seem to represent an unstable intermediary state with partial arousal preceding the restitution of normal wakefulness. Since the onset of the micropsy was signalized only after manifestation of the intermediary EEG-state, we conclude that partial arousal is a prerequisite for giving an indication of hypnagogic experiences in a quasi retrospective manner.</p>","PeriodicalId":76211,"journal":{"name":"Neuropadiatrie","volume":"10 2","pages":"183-94"},"PeriodicalIF":0.0,"publicationDate":"1979-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1055/s-0028-1085324","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"11789359","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 : 1979-02-01DOI: 10.1055/s-0028-1085313
H M Chawla, V Virmani
{"title":"A case of myoclonic epilepsy with unusual presentation.","authors":"H M Chawla, V Virmani","doi":"10.1055/s-0028-1085313","DOIUrl":"https://doi.org/10.1055/s-0028-1085313","url":null,"abstract":"<p><p>A case of stimulus-precipitated myoclonic jerks is presented. Besides spontaneous jerks, auditory, stimuli striking the palms, abdominal pressure or distension, evoked the jerks. Intermittent stroboscopic stimulation did not precipitate the attacks. The child had spontaneous recovery which is maintained, clinically and EEG wise for the last one year.</p>","PeriodicalId":76211,"journal":{"name":"Neuropadiatrie","volume":"10 1","pages":"51-5"},"PeriodicalIF":0.0,"publicationDate":"1979-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1055/s-0028-1085313","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"11327310","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 : 1979-02-01DOI: 10.1055/s-0028-1085316
A Larbrisseau, G Jasmin, C Hausser, P Brochu, G Geoffroy
{"title":"Generalized giant axonal neuropathy-a case with features of Fazio-Londe disease.","authors":"A Larbrisseau, G Jasmin, C Hausser, P Brochu, G Geoffroy","doi":"10.1055/s-0028-1085316","DOIUrl":"https://doi.org/10.1055/s-0028-1085316","url":null,"abstract":"<p><p>We present the 8th case so far published of generalized giant axonal neuropathy. Many features of the neurological picture of this 8 year 8 month old boy were suggestive of progressive bulbar paralysis of childhood. Muscle and sural nerve biopsies disclosed typical nodular axonal swellings measuring between 10--29 nm. Ultrastructure of the inflated portions of the axon consisted almost exclusively of 10 nm filaments. Normally appearing constituents were found in the non-dilated portions of the axons with this particularity that glycogen and mitochondria were aggregated. Previous clinical and pathological evidence indicates that this rare disorder not only affects the peripheral nerves but also the whole CNS. Our case differs from the others reported in literature by its rapidly deteriorating course and by the prominent involvement of some brain-stem motor functions; we offer the theory that a link might be implicated between this entity and Fazio-Londe disease.</p>","PeriodicalId":76211,"journal":{"name":"Neuropadiatrie","volume":"10 1","pages":"76-86"},"PeriodicalIF":0.0,"publicationDate":"1979-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1055/s-0028-1085316","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"11785474","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 : 1979-02-01DOI: 10.1055/s-0028-1085309
H Doose, E Völzke
{"title":"Petit mal status in early childhood and dementia.","authors":"H Doose, E Völzke","doi":"10.1055/s-0028-1085309","DOIUrl":"https://doi.org/10.1055/s-0028-1085309","url":null,"abstract":"<p><p>The course of 117 children with so-called centrencephalic myoclonic-astatic petit mal (Doose et al. 1970) was evaluated with the question, whether petit mal status can be the cause of dementia frequently seen in these patients. A dementia is found definitely more frequently in patients with petit mal status. With regard to the animal experimental studies of Wasterlain (1978, Lit.) and Meldrum (1978, Lit.) it seems possible that the prlonged generalized hypersynchronous discharges of the petit mal status lead to a serious disturbance of the neuronal energy metabolism and thus to cerebral organic damages or disturbances of the brain development.</p>","PeriodicalId":76211,"journal":{"name":"Neuropadiatrie","volume":"10 1","pages":"10-4"},"PeriodicalIF":0.0,"publicationDate":"1979-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1055/s-0028-1085309","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"11785470","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 : 1979-02-01DOI: 10.1055/s-0028-1085315
E Meyer
{"title":"Neurocutaneous syndrome with excessive macrohydrocephalus. (Sturge-Weber/Klippel-Trenaunay syndrome).","authors":"E Meyer","doi":"10.1055/s-0028-1085315","DOIUrl":"https://doi.org/10.1055/s-0028-1085315","url":null,"abstract":"<p><p>2.5 years of follow-up of a boy with progressive macrohydrocephalus possibly as a consequence of an intracranial vascular malformation. There are prominent features of a Sturge-Weber as well as a Klippel-Trenaunary-syndrome.</p>","PeriodicalId":76211,"journal":{"name":"Neuropadiatrie","volume":"10 1","pages":"67-75"},"PeriodicalIF":0.0,"publicationDate":"1979-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1055/s-0028-1085315","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"11433957","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 : 1979-02-01DOI: 10.1055/s-0028-1085312
A Lundberg
{"title":"Gross and fine motor performance in healthy Swedish children aged fifteen and eighteen months.","authors":"A Lundberg","doi":"10.1055/s-0028-1085312","DOIUrl":"https://doi.org/10.1055/s-0028-1085312","url":null,"abstract":"<p><p>Gross and fine motor performance have been assessed in thirtynine 15-month-old and thirtynine 18-month-old children selected through systematic sampling from a population of healthy newborns. The performance was estimated with corresponding subscales of Denver Developmental Screening Test and Motor age test. In addition fine motor performance was assessed by the \"Drawing test\" for which the expected performance at 15 and 18 months is given. Of the 78 children, 83% had crawled on hand and knees, 9% crept on their tummy and 8% shuffled on their bottom before walking. Median age for walking was 11.8 months, the 97th percentile 16.0 months. Of the 15-month-old children, 90% walked with ability to stop and start, and 90% of the 18-month-old were in addition able to walk backwards, run stiffly, climb up and down a standard staircase and seat themselves in an armchair of suitable size. Of the 15-month-old, 90% were able to build a tower of two cubes or more, and 90% of the 18-month-old were able to build a tower of three cubes or more (size 1 1/2 inch). Almost 90% of the 15-month-old and all of the 18-month-old had the cognitive ability to coordinate a crayon and paper. In drawing attempts of the 15 month-old, the weak unstable combination with a top-tong/tong grasp and 1/2 pronated forearm was the dominating hand-grasp fore-arm position. In the 18 month-old a more mature grasp and forearm combination, mainly palmar grasp with or without stablizing index finger + overpronated forearm, was found.</p>","PeriodicalId":76211,"journal":{"name":"Neuropadiatrie","volume":"10 1","pages":"35-50"},"PeriodicalIF":0.0,"publicationDate":"1979-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1055/s-0028-1085312","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"11785473","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}