منابع مشابه
Progressive familial myoclonus epilepsy.
Seven cases of progressive familial myoclonus epilepsy occurring in three families are presented. The patients were in different stages of the illness. The EEG was abnormal in all. It is suggested that these cases belong clinically to the Lafora bodies group. Nystagmus and optic atrophy, seen in one patient, have not been described previously. Myoclonic jerks did not respond to treatment with d...
متن کاملProgressive myoclonus epilepsy without Lafora bodies.
Many attempts have been made to define consistent clinical and pathological entities within the syndrome of progressive myoclonus epilepsy. The existence of a specific metabolic defect underlying one form of the disease is suggested by the presence of characteristic cerebral inclusion bodies (Lafora and Glueck, 1911) and of material with similar staining properties in liver and muscle (Harriman...
متن کاملSome observations on electroencephalograms of myoclonus epilepsy.
Electroencephalographic observations of four cases of myoclonus epilepsy are reported. A feature in EEG findings was that spikes became more prominent in the later stage of illness. Photic stimulation had a distinct activating effect on producing paroxysms, and an increase in number of paroxysms depended not on the frequency of stimulus but on the intensity. A sleep EEG showed ill-formed humps ...
متن کاملLafora disease: a progressive myoclonus epilepsy.
Lafora disease is a rare inborn error of metabolism resulting in storage of a polyglucosan in tissues including the brain, skin and liver. Four children are described with progressive myoclonus epilepsy and intellectual deterioration in whom this diagnosis was made. In two the diagnosis was confirmed by the presence of periodic acid schiff (PAS) positive, diastase resistant, colloidal iron stai...
متن کاملA case of atypical benign partial epilepsy with action myoclonus
We describe a boy, 3 years and 6 months old, who experienced a rolandic seizure accompanied by a cluster of atypical absence seizures, the EEGs for which corresponded to those of atypical benign partial epilepsy (ABPE). Of note, this patient suffered from developmental delay beginning in infancy and exhibited giant middle-latency somatosensory evoked potentials with action myoclonus. With the e...
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ژورنال
عنوان ژورنال: Proceedings of the Royal Society of Medicine
سال: 1928
ISSN: 0035-9157
DOI: 10.1177/003591572802100415