[On the paper by A. Schinzel: "Fetal hydantoin syndrome in siblings" Schweiz. med. Wschr. 109, 68-72 (1979)].
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Biomedical subjects
Publications and source records attributed to K Karbowski.
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Upper lid and vertical eye movements are usually preserved in patients with the "locked-in" syndrome, a condition manifested by anarthria, spastic quadriparesis, and intact mental function, and caused by lesions in the ventral portion of the pons. We describe a presumed mesencephalic "locked-in" syndrome in a patient who had total bilateral ptosis at the onset of his disease and later on experienced complete bilateral oculomotor nerve paralysis.
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The most important characteristic of the EEG in old age is the frequency decrease of background activity, which can be traced back to a lessening of cerebral oxygen uptake. One third of healthy test persons aged 60 or over also show temporal (usually left sided) slow wave groups. The degree of EEG changes in cerebro-vascular insults depends on the nature and the localization of these disturbances. Temporary clinical and electroencephalographic focal seizure symptoms are observed, in particular, in circulatory disturbances within the temporo-parieto-occipital territory. Recurring epileptic seizures which start only in old age usually have either a vascular or a tumoral cause. During the attack-free intervals EEG spikes or sharp waves may be less frequently recorded than in younger test persons. Thanks to its highly typical EEG pattern, Creutzfeldt-Jakob subacute spongiform encephalopathy can be distinguished from other presenile or senile affections of the brain.
Continuous myoclonus in a localised area of the body was observed in three patients. In two cases the myocloni sometimes developed into motor Jacksonian convulsions. All three patients had neurological signs on the same side as their continuous twitching indicating a lesion of the contralateral hemisphere. The surface EEG did not show changes which could be directly correlated with continuous convulsions in any of the cases. The cause was found to be a malformation of the hemisphere in one case, a recent encephalomalacia in the second and a severe hyperosomolar diabetic electrolyte imbalance in the third. Epilepsia partialis continua Kozevnikov differs from motor Jacksonian epilepsy in the continuous non-attack character and the absence of a "march of convulsions". Pathophysiologically they are both forms of focal cortical status epilepticus.
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In the light of three case histories, other personal observations and the literature, the clinical and electroencephalographical differences between absences in the limited sense and continuous LENNOX petit-mal state are described and the problems of the latter discussed. As a rule, petit-mal state is diagnosed as such in young people or adults, and practically never before the 10th year of life. In about two thirds of cases, its clinical symptomatology consists of a twilight condition lasting some hours to a few days, coupled with inertia and apathy. The remaining third of the patients usually experience milder disturbances, e.g. in the form of concentration difficulties, tiredness, and (more rarely) severe forms including lethargy. The EEG correlate of a petit-mal state is made up of continuous bilaterally synchronous, frontally marked (less frequently with exclusively frontal localization), usually irregular spike waves or poly-spike waves, which frequently occur in only rudimentary forms and register a frequency of 2 1/2-4 c/sec. For the treatment of petit-mal state, benzodiazepines and in particular clonazepam (Rivotril) (1-2 mg i.v.) are recommended. During the interval condition the same therapy as with an absence epilepsy, e.g. succinimides or dipropylacetate (Depakine) is administered. Anti-grand-mal remedies, especially hydantoins, may trigger petit-mal status.
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Polygraphic examinations (EEG, EKG, electrooculogram and recording of respiration) were carried out on 120 full term healthy newborns. EEG recordings taken in wakefulness states showed a low-voltage background activity with a noticeable share of 8 c/sec. waves. The latter occurred in 12.5% of the examined newborn infants in more regular groups, lasting from 3--6 seconds, and reached an amplitude of up to 20--25 muV. The EEG sleep recordings differed from case to case. Certain identical EEG-patterns could be observed during active as well as during quiet sleep. Typical "tracés alternants" occurred in 63.4% of the recordings, as a rule in the quiet sleep phase. In nearly 1/3 of these cases they contained isolated fine spikes or sharp waves of variable localisation. In 70% of the sleep recordings bilateral fronto-central paroxysmal steeper transients and in 6.7% spindly about 14 c/sec. wave groups were observed, mainly during active sleep phases. In nearly 20% of the 52 newborns where the centro-temporal electrodes were connected transversally, single sharp and slow waves were recorded. They were localized resp. more pronounced in the right hemisphere. They did not appear in any of the 68 cases in which the electrodes were connected longitudinally only.
Under the same clinical picture the "absence" may group various forms of epileptic and non-epileptic disease entities, each having a different pathogenesis and a different EEG correlate. The investigative procedures, therapy and prognosis are likewise non-uniform. This suggests the need for a nomenclatorial division for these different nosological units and their classification into typical and atypical absences, absence-like partial (focal) fits, and paroxysmal nonepileptic disturbances of consciousness.
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