TY - JOUR
T1 - Ictal and nonictal paroxysmal events in infantile neuroaxonal dystrophy
T2 - Polygraphic study of a case
AU - Santucci, Margherita
AU - Ambrosetto, Giovanni
AU - Cristina Scaduto, M.
AU - Morbin, Michela
AU - Vittoria Tzolas, E.
AU - Rossi, Paola Giovanardi
PY - 2001
Y1 - 2001
N2 - 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.
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. 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.
KW - Electroencephalography
KW - Epileptic and nonepileptic paroxysmal events
KW - Infantile neuroaxonal dystrophy
KW - Neuropathology
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U2 - 10.1046/j.1528-1157.2001.0420081074.x
DO - 10.1046/j.1528-1157.2001.0420081074.x
M3 - Article
C2 - 11554895
AN - SCOPUS:0034870385
VL - 42
SP - 1074
EP - 1077
JO - Epilepsia
JF - Epilepsia
SN - 0013-9580
IS - 8
ER -