Longitudinal clinicoelectrophysiologic study of a case of Lafora disease proven by skin biopsy

Katsuhiro Kobayashi, K. Iyoda, Y. Ohtsuka, S. Ohtahara, M. Yamada

Research output: Contribution to journalArticle

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Abstract

A longitudinal clinicoelectrophysiologic study was undertaken of a 15-year 2-month-old girl with Lafora disease who was diagnosed by skin biopsy and an immunohistochemical method with antisera against Lafora bodies. From age 10 years 5 months, 4 months after onset, EEG disclosed progressive deterioration of background activity and incremental increase in epileptic discharges. Photosensitivity was unique: Occipital spikes and diffuse spike-wave discharges were provoked by low-frequency repetitive photic stimuli but without elicitation of myoclonic seizures. Photosensitivity completely disappeared after age 13 years 10 months. High-voltage somatosensory evoked potentials (SEPs) and high-voltage flash visual evoked potentials (F-VEPs) were seen before age 13. After age 13, progressive prolongation of I-III and I-V interpeak latencies of auditory brainstem responses (ABRs), progressive prolongation of latencies of photo-evoked eyelid microvibrations, delayed latencies of pattern-reversal visual evoked potentials, and a decrease in the V/I amplitude ratio of ABRs and the previously high F-VEP amplitudes were observed.

Original languageEnglish
Pages (from-to)194-201
Number of pages8
JournalEpilepsia
Volume31
Issue number2
Publication statusPublished - 1990

Fingerprint

Lafora Disease
Visual Evoked Potentials
Longitudinal Studies
Brain Stem Auditory Evoked Potentials
Biopsy
Skin
Somatosensory Evoked Potentials
Eyelids
Immune Sera
Electroencephalography
Seizures

Keywords

  • Electroencephalography
  • Evoked potentials
  • Lafora disease
  • Progressive myoclonus epilepsy
  • Skin biopsy

ASJC Scopus subject areas

  • Clinical Neurology
  • Neuroscience(all)

Cite this

Kobayashi, K., Iyoda, K., Ohtsuka, Y., Ohtahara, S., & Yamada, M. (1990). Longitudinal clinicoelectrophysiologic study of a case of Lafora disease proven by skin biopsy. Epilepsia, 31(2), 194-201.

Longitudinal clinicoelectrophysiologic study of a case of Lafora disease proven by skin biopsy. / Kobayashi, Katsuhiro; Iyoda, K.; Ohtsuka, Y.; Ohtahara, S.; Yamada, M.

In: Epilepsia, Vol. 31, No. 2, 1990, p. 194-201.

Research output: Contribution to journalArticle

Kobayashi, K, Iyoda, K, Ohtsuka, Y, Ohtahara, S & Yamada, M 1990, 'Longitudinal clinicoelectrophysiologic study of a case of Lafora disease proven by skin biopsy', Epilepsia, vol. 31, no. 2, pp. 194-201.
Kobayashi, Katsuhiro ; Iyoda, K. ; Ohtsuka, Y. ; Ohtahara, S. ; Yamada, M. / Longitudinal clinicoelectrophysiologic study of a case of Lafora disease proven by skin biopsy. In: Epilepsia. 1990 ; Vol. 31, No. 2. pp. 194-201.
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