PMID- 11554895 OWN - NLM STAT- MEDLINE DCOM- 20011204 LR - 20190813 IS - 0013-9580 (Print) IS - 0013-9580 (Linking) VI - 42 IP - 8 DP - 2001 Aug TI - Ictal and nonictal paroxysmal events in infantile neuroaxonal dystrophy: polygraphic study of a case. PG - 1074-7 AB - 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. Videopolygraphic 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. FAU - Santucci, M AU - Santucci M AD - Department of Child Neurology and Psychiatry, Neurological Institute, University of Bologna, Italy. santucci@neuro.unibo.it FAU - Ambrosetto, G AU - Ambrosetto G FAU - Scaduto, M C AU - Scaduto MC FAU - Morbin, M AU - Morbin M FAU - Tzolas, E V AU - Tzolas EV FAU - Rossi, P G AU - Rossi PG LA - eng PT - Case Reports PT - Journal Article PL - United States TA - Epilepsia JT - Epilepsia JID - 2983306R SB - IM MH - Arm/physiopathology MH - Child MH - Electroencephalography/*statistics & numerical data MH - Electromyography/statistics & numerical data MH - Epilepsy/*diagnosis/epidemiology/physiopathology MH - Female MH - Humans MH - Monitoring, Physiologic/*statistics & numerical data MH - Muscle Contraction/physiology MH - Muscle, Skeletal/physiopathology MH - Neuroaxonal Dystrophies/*diagnosis/epidemiology/physiopathology MH - Polysomnography/statistics & numerical data MH - Sleep/physiology MH - Videotape Recording MH - Wakefulness/physiology EDAT- 2001/09/14 10:00 MHDA- 2002/01/05 10:01 CRDT- 2001/09/14 10:00 PHST- 2001/09/14 10:00 [pubmed] PHST- 2002/01/05 10:01 [medline] PHST- 2001/09/14 10:00 [entrez] AID - epi09001 [pii] AID - 10.1046/j.1528-1157.2001.0420081074.x [doi] PST - ppublish SO - Epilepsia. 2001 Aug;42(8):1074-7. doi: 10.1046/j.1528-1157.2001.0420081074.x.