Summary: The loss of muscular tone or muscular inhibition associated with sharp waves observed during abnormal tonic posture in a patient with infantile spasms of early onset is described. The latency between the beginning of a sharp wave and the muscular inhibition varied from 30 to 700 msec, and the duration of the inhibition ranged from 100 to 400 msec. These brief muscular inhibitions may sometimes precede tonic seizures, and these phenomena might be noticeable only in the state of steady tonic muscular contraction. RÉSUMÉ Les auteurs décrivent une perte du tonus musculaire ou une inhibition musculaire qui accompagne des paroxysmes EEG de pointes lentes chez des enfants qui présentent des spasmes infantiles très précoces. La latence entre le début de la pointe lente et l'inhibi‐tion musculaire varie de 30 à 700 msec, et la durée de I'inhibition se situe entre 100 et 400 msec. Ces brèves inhibitions musculaires peuvent précéder une composante tonique et elles peuvent n'être appréciables que lorsque le sujet est en état de contraction musculaire tonique. RESUMEN En un enfermo con espasmos infantiles de comienzo precoz se describe la pérdida de tono muscular o de inhibición muscular asociadas a ondas agudas que se observan durante la postura tónica anormal. La latencia entre el comienzo de la onda aguda y la inhibición muscular varió de 30 a 700 milisegundos y la duración de la inhibición osciló entre 100 y 400 milisegundos. Esta breve inhibición muscular puede preceder ataques tónicos y estos fenómenos pueden apreciarse solamente en el estado de contracción muscular tónica permanente. ZUSAMMENFASSUNG Beschreibung eines Patienten, bei dem BNS‐Krämpfe frühzeitig aufgetrétén sind. Er zeigte während einer abnormen tonischen Haltung den Verlust des Muskeltonus (muskulare Inhibition), die von sharp waves begleitet wurde. Die Latenz zwischen dem Beginn der sharp waves und der muskulären Inhibition variierte zwischen 30 und 700 ms, ihre Dauer zwischen 100 und 400 ms. Diese kurze muskuläre Inhibition kann gelegentlich tonischen Anfällen vorhergehen und diese Phänomene könnten nur zu bemerken sein im Stadium der stetigen muskulären tonischen Kontraktion.

Tonic (physiology)

10.1111/j.1528-1157.1981.tb04111.x

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Citations (8)

Xeroderma pigmentosum: Neurological, Neurophysiological and Morphological Studies

European Neurology (1982)

Susumu Hakamada Kazuyoshi Watanabe Gen Sobue Kimiko Hara Shiuji Miyazaki

In3 cases of xeroderma pigmentosum showing signs of peripheral neuropathy, electrophysiological studies were made. In one of them, a sural nerve biopsy was performed. MCV obtained from the lower limbs were moderately reduced in all cases, and SCV could be obtained in only 1 case from the median nerve. Auditory brain stem responses were not obtainable in all cases. Sural nerve biopsy revealed a marked decrease of myelinated fibers and also suggested severe damage in both myelinated and unmyelinated fibers. Teased fiber study showed myelin ovoids and indicated axonal degeneration. Sensory nerves including the acoustic nerve may suffer damage earlier than the motor nerve. In all cases CT scans revealed brain atrophy and thickening of the skull.

Sural nerve

Axonal Degeneration

Cutaneous nerve

10.1159/000115457

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Citations (23)

Book Review / Announcement

European Neurology (1982)

Susumu Hakamada Kazuyoshi Watanabe Gen Sobue Kimiko Hara Shiuji Miyazaki

10.1159/000115470

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Neurophysiological Study of Newborns with Hypocalcemia

Neuropediatrics (1982)

Kazuyoshi Watanabe Kazukuni Hara Shiuji Miyazaki Susumu Hakamada

The EEG, sleep cycle, visual and auditory evoked potentials (VEP and AEP) were studied in 38 newborns who presented with seizures and had serum calcium levels below 7 mg/dl. Nineteen infants displayed increased theta and alpha, and decreased delta waves with variable but mild degrees of background EEG depression during sleep, which may reflect neuronal hyperexcitability induced by hypocalcemia. Hypocalcemic infants spent less time in quite sleep than control infants, but the time spent in active sleep did not change significantly. Seventy-seven percent of seizures occurred in active sleep. 17% in indeterminate sleep and 1% during wakefulness. Most infants demonstrated normal VEP and AEP, although a few displayed prolonged latency. No correlation was found between peak latency and serum calcium levels. Two infants with complicated hypocalcemia who showed moderately depressed background EEG and other two with loss of sleep cycles developed abnormally, while the other infants showed normal development.

Sleep

Depression

10.1055/s-2008-1059592

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Citations (14)