Atypical “Benign” Partial Epilepsy or Pseudo-Lennox Syndrome. Part I: Symptomatology and Long-Term Prognosis

Purpose: Atypical benign partial epilepsy (ABPE) or pseudo-Lennox syndrome (PLS) is characterised by generalised minor seizures and focal sharp slow waves and spikes (SHW) as observed in Rolandic epilepsy (RE), but with exceptional pronounced activation during sleep. The aim of this study was to describe the full spectrum of ABPE in the hitherto largest group of patients.

Methods: We retrospectively analysed the clinical and EEG data of 43 children who fulfilled the following criteria: occurrence of generalised minor seizures as described for ABPE (i.e., atonic-astatic seizures, myoclonic seizures, atypical absences) and focal SHW identical to those observed in RE, but with generalisation during sleep.

Results: Language development prior to onset of epilepsy was retarded in 26 % of patients. In 74 %, age at onset of epilepsy ranged from 2 to 6 years. Manifestation occurred earlier in boys than in girls. Generalised minor seizures constituted the predominating seizure type in 67 % of patients. Twenty-eight percent of patients suffered from simple partial seizures of the oro-facial region or generalised tonic-clonic seizures originating from the oro-facial region. Additionally, generalised tonic-clonic (44 %), unilateral (21 %), partial motor (44 %), versive (12 %), focal atonic (9 %), and complex-partial seizures (2 %) were observed. A bioelectrical status was recorded in 56 % of patients during sleep. No tonic seizures and no fast spike series (bursts of 10 - 20 Hz rhythms) were observed. At last follow-up, 84 % of patients were in clinical remission. All subjects older than age 15 were seizure-free. However, 56 % of patients attended a school for mentally handicapped children.

Conclusions: ABPE or PLS broadly overlaps with RE, electrical status epilepticus during sleep, and Landau-Kleffner syndrome. Regarding the epilepsy, the prognosis is excellent, mental deficit, however, seems to be frequent. The differentiation from Lennox-Gastaut syndrome and myoclonic astatic epilepsy is essential. Instead of ABPE, the term pseudo-Lennox syndrome is proposed.

Key words

Atypical benign partial epilepsy - Pseudo-Lennox syndrome - Rolandic epilepsy - Landau-Kleffner syndrome - Electrical status epilepticus during slow sleep

References

• 1 Aicardi J. Atypical partial benign epilepsy of childhood. Aicardi J Epilepsy in Children. 2nd ed. New York; Raven Press 1994: 146-151
• 2 Aicardi J, Chevrie J J. Atypical benign partial epilepsy of childhood.  Dev Med Child Neurol. 1982;  24 281-292
• 3 Aicardi J, Levy Gomes A. Clinical and electroencephalographic symptomatology of the “genuine” Lennox-Gastaut syndrome and its differentiation from other forms of epilepsy of early childhood. Degen R, Dreyfuss FE Localised and Generalised Epilepsies of Early Childhood. Amsterdam; Elsevier 1992 (Epilepsy Res Suppl 6): 185-193
• 4 Beaumanoir A. The Landau-Kleffner syndrome. Roger J, Bureau M, Dravet Ch, Dryfuss FE, Perret A, Wolf P Epileptic Syndromes in Infancy, Childhood and Adolescence. 2nd ed. London, Paris, Rome; John Libbey 1992: 231-243
• 5 Beaumanoir A, Nahory A. Les épilepsies bénignes partielles: 11 cas d'épilepsie partielle frontale à évolution favorable.  Rev EEG Neurophysiol. 1983;  13 207-211
• 6 Beaumanoir A, Dravet C. The Lennox-Gastaut syndrome. Roger J, Bureau M, Dravet Ch, Dryfuss FE, Perret A, Wolf P Epileptic Syndromes in Infancy, Childhood and Adolescence. 2nd ed. London, Paris, Rome; John Libbey 1992: 115-132
• 7 Beaussart M. Benign epilepsy of children with Rolandic (centro-temporal) paroxysmal foci - a clinical entity. Study of 221 cases.  Epilepsia. 1972;  13 795-811
• 8 Bray P F, Wiser W C. Evidence for a genetic etiology of temporal-central abnormalities in focal epilepsy.  New Engl J Med. 1964;  271 926-933
• 9 Commission on classification and terminology of the international league against epilepsy . Proposal for revised classification of epilepsies and epileptic syndromes.  Epilepsia. 1989;  30 389-399
• 10 Caraballo R, Cersósimo R, Fejerman N. A particular type of epilepsy in children with congenital hemiparesis associated with unilateral polymycrogyria.  Epilepsia. 1999;  40 865-871
• 11 Deonna Th, Ziegler A-L, Despland P-A. Combined myoclonicastatic and benign focal epilepsy of childhood (atypical “benign” partial epilepsy of childhood). A separate syndrome?.  Neuropediatrics. 1986;  17 144-151
• 12 Doose H. Symptomatology in children with focal sharp waves of genetic origin.  Eur J Ped. 1989;  149 210-215
• 13 Doose H. Myoclonic astatic epilepsy of early childhood. Roger J, Bureau M, Dravet Ch, Dryfuss FE, Perret A, Wolf P Epileptic Syndromes in Infancy, Childhood and Adolescence. 2nd ed. London, Paris, Rome; John Libbey 1992: 103-114
• 14 Doose H. Genetic EEG traits in the pathogenesis of epilepsy.  J Epilepsy. 1997;  10 97-110
• 15 Doose H, Gerken H, Völzke E. On the genetics of EEG anomalies in childhood. I. Abnormal theta rhythms.  Neuropädiatrie. 1972;  3 386-401
• 16 Doose H, Baier W K. Theta rhythms in the EEG: a genetic trait in childhood epilepsy.  Brain Dev. 1988;  10 347-351
• 17 Doose H, Baier W K. Benign partial epilepsies and related conditions: multifactorial pathogenesis with hereditary impairment of brain maturation.  Eur J Pediatr. 1989;  149 152-158
• 18 Doose H, Ernst J P, Castiglione E, Diebold U. Pseudo-Lennox-Syndrom und verwandte Krankheitsbilder - frontale Epilepsien?. Köhler B Aktuelle Neuropädiatrie. Berlin, Heidelberg, New York; Springer 1992: 96-101
• 19 Doose H, Brigger-Heuer B, Neubauer B A. Children with focal sharp waves: Clinical and genetic aspects.  Epilepsia. 1997;  38 788-796
• 20 Doose H, Tibow I, Castiglione E, Neubauer B A. Febrile convulsions with focal sharp waves - a subgroup of benign partial epilepsies of childhood with multifactorial etiology.  J Epilepsy. 1998;  11 341-354
• 21 Doose H, Hahn A, Neubauer B A, Pistohl J, Stephani U. Atypical “benign” partial epilepsy or pseudo-Lennox syndrome. Part II: Family study.  Neuropediatrics. 2001;  32 9-13
• 22 Drury I, Beydoun A. Benign partial epilepsy of childhood with monomorphic sharp waves in centrotemporal and other locations.  Epilepsia. 1991;  32 662-667
• 23 Eeg-Olofssen O, Petersen I, Sellden U. The development of the electroencephalogram in normal children from age 1 through 15 years.  Neuropediatrics. 1971;  2 375-404
• 24 Engle M, Lüders H, Chutorian A M. The significance of focal EEG spikes in epileptic and non-epileptic children who are otherwise normal.  Ann Neurol. 1977;  2 257
• 25 Feyerman N, Caraballo R, Tenembaum S N. Atypical evolutions of benign localization-related epilepsies in children: are they predictable?.  Epilepsia. 2000;  41 380-390
• 26 Gerken H, Doose H. On the genetics of EEG anomalies in childhood. III. Spikes and waves in resting record and/or during hyperventilation.  Neuropädiatrie. 1973;  4 88-97
• 27 Guerrini R, Dravet C, Genton P, Bureau M, Roger J, Rubboli G, Tassinari A. Epileptic negative myoclonus.  Neurology. 1993;  43 1078-1083
• 28 Guerrini R, Pammegiani M, Bureau M. Localized cortical dysplasia: good seizure outcome after sleep-related electrical status epilepticus. Guerrini R, Canapicchi R, Zifkin B, Anderman F, Roger J, Pfanner P Dysplasias of Cerebral Cortex and Epilepsy. Philadelphia; Lippincott-Raven Publishers 1996: 329-336
• 29 Hejbel J, Blom S, Rasmuson M. Benign epilepsy of childhood with centro-temporal EEG foci. A genetic study.  Epilepsia. 1975;  16 285-293
• 30 Kanazawa O, Kawai I. Status epilepticus characterized by repetitive asymmetrical atonia: Two cases accompanied by partial seizures.  Epilepsia. 1990;  31 536-543
• 31 Lerman P, Kivity S. Benign focal epilepsy of childhood.  Arch Neurol. 1975;  32 261-264
• 32 Lerman P, Kivity-Ephraim S. Focal epileptic EEG discharges in children not suffering from clinical epilepsy: etiology, clinical significance, and management.  Epilepsia. 1981;  22 551-558
• 33 Loiseau P, Pestre M, Dartigues J F, Commenges D, Barberger-Gateau C, Cohadon S. Long-term prognosis of two forms of childhood epilepsy: typical absence seizures and epilepsy with Rolandic (centrotemporal) EEG foci.  Ann Neurol. 1983;  13 642-648
• 34 Lüders H, Lesser R P, Dinner D S, Morris H H. Benign focal epilepsy of childhood. Lüders H, Lesser RP Epilepsy Electroclinical Syndromes. London, Berlin, Heidelberg, New York, Tokyo; Springer 1987: 303-346
• 35 Morikawa T, Mazakazu S, Kazuichi Y. Long-term outcome of four children with continuous spike-waves during sleep. Roger J, Bureau M, Dravet Ch, Dryfuss FE, Perret A, Wolf P Epileptic Syndromes in Infancy, Childhood and Adolescence. 2nd ed. London, Paris, Rome; John Libbey 1992: 257-265
• 36 Nayrac P, Beaussart M. Les pointes-ondes prérolandiques: expression EEG très particulière. Etude électroclinique de 21 cas.  Rev Neurol. 1957;  99 201-206
• 37 Neubauer B A, Fiedler B, Himmelein B, Kämpfer F, Lässker U, Schwabe G. et al . Centrotemporal spikes in families with Rolandic epilepsy. Linkage to chromosome 15 q14.  Neurology. 1998;  51 1608-1612
• 38 Oguni H, Uehara T, Imai K, Osawa M. Atonic epileptic drop attacks associated with generalized spike- and slow wave complexes: video-polygraphic study in two patients.  Epilepsia. 1997;  38 813-818
• 39 Papile L, Burstein J, Burstein R, Koffler H. Incidence and evolution of subependymal and intraventricular hemorrhage: a study of infants with birth weight less than 1500 grams.  J Pediatr. 1978;  116 529-534
• 40 Tassinari C A, Bureau M, Dravet C, Dalla Bernadina B, Roger J. Epilepsy with continuous spikes and waves during slow sleep - otherwise described as ESES (epilepsy with electrical status epilepticus during slow sleep). Roger J, Bureau M, Dravet Ch, Dryfuss FE, Perret A, Wolf P Epileptic Syndromes in Infancy, Childhood and Adolescence. 2nd ed. London, Paris, Rome; John Libbey 1992: 245-256