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Atypical "benign" partial epilepsy or pseudo-Lennox syndrome. Part I: symptomatology and long-term prognosis.
Neuropediatrics. 2001 Feb; 32(1):1-8.N

Abstract

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., atonicastatic 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.

Authors+Show Affiliations

Neuropaediatric Department, University of Kiel, Germany. AHAHN@pedneuro.uni-kiel.deNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article

Language

eng

PubMed ID

11315196

Citation

Hahn, A, et al. "Atypical "benign" Partial Epilepsy or pseudo-Lennox Syndrome. Part I: Symptomatology and Long-term Prognosis." Neuropediatrics, vol. 32, no. 1, 2001, pp. 1-8.
Hahn A, Pistohl J, Neubauer BA, et al. Atypical "benign" partial epilepsy or pseudo-Lennox syndrome. Part I: symptomatology and long-term prognosis. Neuropediatrics. 2001;32(1):1-8.
Hahn, A., Pistohl, J., Neubauer, B. A., & Stephani, U. (2001). Atypical "benign" partial epilepsy or pseudo-Lennox syndrome. Part I: symptomatology and long-term prognosis. Neuropediatrics, 32(1), 1-8.
Hahn A, et al. Atypical "benign" Partial Epilepsy or pseudo-Lennox Syndrome. Part I: Symptomatology and Long-term Prognosis. Neuropediatrics. 2001;32(1):1-8. PubMed PMID: 11315196.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Atypical "benign" partial epilepsy or pseudo-Lennox syndrome. Part I: symptomatology and long-term prognosis. AU - Hahn,A, AU - Pistohl,J, AU - Neubauer,B A, AU - Stephani,U, PY - 2001/4/21/pubmed PY - 2001/9/21/medline PY - 2001/4/21/entrez SP - 1 EP - 8 JF - Neuropediatrics JO - Neuropediatrics VL - 32 IS - 1 N2 - 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., atonicastatic 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. SN - 0174-304X UR - https://www.unboundmedicine.com/medline/citation/11315196/Atypical_"benign"_partial_epilepsy_or_pseudo_Lennox_syndrome__Part_I:_symptomatology_and_long_term_prognosis_ L2 - http://www.thieme-connect.com/DOI/DOI?10.1055/s-2001-12216 DB - PRIME DP - Unbound Medicine ER -