Ictal and nonictal paroxysmal events in infantile neuroaxonal dystrophy: Polygraphic study of a case

Margherita Santucci, Giovanni Ambrosetto, M. Cristina Scaduto, Michela Morbin, E. Vittoria Tzolas, Paola Giovanardi Rossi

Research output: Contribution to journalArticle

Abstract

A 7.5-year-old girl, with infantile neuroaxonal dystrophy (INAD), showed a gradual deterioration from 16 months; at age 5 years she was bedridden, with severe tetraplegia, strabismus, nystagmus and optic atrophy, and dementia. From age 5.5 years, she had paroxysmal tonic events. Video-polygraphic recordings disclosed two different kinds of motor events: (a) epileptic tonic seizures, in wakefulness and sleep, associated with autonomic changes and ictal EEG discharges; and (b) nonepileptic prolonged clusters of brief tonic spasms, without ictal modifications of the EEG. Both motor events were characterized by a minimal and clinically similar tonic contraction of the upper extremities. Video-polygraphic studies are mandatory for a correct paroxysmal event classification and treatment in INAD patients.

Original languageEnglish
Pages (from-to)1074-1077
Number of pages4
JournalEpilepsia
Volume42
Issue number8
DOIs
Publication statusPublished - 2001

Keywords

  • Electroencephalography
  • Epileptic and nonepileptic paroxysmal events
  • Infantile neuroaxonal dystrophy
  • Neuropathology

ASJC Scopus subject areas

  • Clinical Neurology
  • Neuroscience(all)

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    Santucci, M., Ambrosetto, G., Cristina Scaduto, M., Morbin, M., Vittoria Tzolas, E., & Rossi, P. G. (2001). Ictal and nonictal paroxysmal events in infantile neuroaxonal dystrophy: Polygraphic study of a case. Epilepsia, 42(8), 1074-1077. https://doi.org/10.1046/j.1528-1157.2001.0420081074.x