The Clinical Characterizations of Benign Partial Epilepsy in Infancy

Abstract

The aim of this study is to clarify the detailed clinical features of benign partial epilepsy in infancy. The subject of the study was 33 patients with benign partial epilepsy in infancy confirmed by a long-term follow-up beyond 8 years of age. The data were obtained from medical records of the patients in combination with the data obtained from telephone interview. The median age at the first and last seizure was 5 and 8 months, respectively. In 26 patients, seizures disappeared within 3 months after the onset. Family history of benign partial epilepsy in infancy was seen in 17 patients. The median number of seizures was 7. A cluster of seizures was observed in 26 patients. The type of seizures was complex partial seizures alone in 6 patients, secondarily generalized seizures alone in 9, and both types in 18. Decreased responsiveness, lateral eye deviation, and cyanosis were commonly observed. Initial interictal electroencephalograms were normal in all patients. However, paroxysmal discharges were recognized in 2 patients in the second EEG during the first year of life. The main features of benign partial epilepsy in infancy were a high incidence of a cluster of seizures, short persistence of seizures, and normal initial interictal EEGs.

Key words

Benign partial epilepsy - infancy - clinical manifestation - interictal EEG - infantile seizure

References

• 1 Caraballo R, Pavek S, Lemainque A, Gastaldi M, Echenne B, Motte. et al . Linkage of benign familial infantile convulsions to chromosome 16p12-q12 suggests allelism to the infantile convulsions and choreoathetosis syndrome.  Am J Hum Genet. 2001;  68 788-794
• 2 Capovilla G, Giordano L, Tiberti S, Valseriati D, Menegati E. Benign partial epilepsy in infancy with complex partial seizures (Watanabe's syndrome): 12 non-Japanese new cases.  Brain Dev. 1998;  20 105-111
• 3 Capovilla G, Beccaria F, Montagnini A. Benign focal epilepsy in infancy with vertex spikes and waves during sleep. Delineation of the syndrome and recalling as benign infantile focal epilepsy with midline spikes and waves during sleep (BIMSE).  Brain Dev. 2006;  28 85-91
• 4 Dravet C, Catani C, Bureau M, Roger J. Partial epilepsies in infancy: a study of 40 cases.  Epilepsia. 1989;  30 807-812
• 5 Dulac O, Cusmai R, de Oliveira K. Is there a partial benign epilepsy in infancy?.  Epilepsia. 1989;  30 798-801
• 6 Echenne B, Humbertclaude V, Rivier F, Malafosse A, Cheminal R. Benign infantile epilepsy with autosomal dominant Inheritance.  Brain Dev. 1994;  16 108-111
• 7 Engel J. A proposed diagnostic scheme for people with epileptic seizures and with epilepsy: report of the ILAE Task Force on classification and terminology.  Epilepsia. 2001;  42 796-803
• 8 Guipponi M, Rivier F, Vigevano F, Beck C, Crespel A, Echenne B. et al . Linkage mapping of benign familial infantile convulsions (BFIC) to chromosome 19 q.  Hum Mol Genet. 1997;  6 473-477
• 9 Kaleyias J, Khurana D S, Valencia I, Legido A, Kothare S V. Benign partial epilepsy in infancy: myth or reality?.  Epilepsia. 2006;  47 1043-1049
• 10 Lee W L, Low P S, Rajan U. Benign familial infantile epilepsy.  J Pediatr. 1993;  123 588-590
• 11 Nelson G B, Olson D M, Hahn J S. Short duration of benign partial epilepsy in infancy.  J Child Neurol. 2002;  17 440-445
• 12 Okumura A, Watanabe K, Negoro T, Hayakawa F, Kato T, Maruyama K. et al . Long-term follow-up of patients with benign partial epilepsy in infancy.  Epilepsia. 2006;  47 181-185
• 13 Okumura A, Watanabe K, Negoro T. Benign partial epilepsy in infancy long-term outcome and marginal syndromes.  Epilepsy Res. 2006;  70 Suppl 2 - 3 168-173
• 14 Sakai Y, Kira R, Torisu H, Yasumoto S, Saito M, Kusuhara K. et al . Benign convulsion with mild gastroenteritis and benign familial infantile seizure.  Epilepsy Res. 2006;  68 269-271
• 15 Striano P, Bordo L, Lispi M L, Specchio N, Minetti C, Vigevano F. et al . A novel SCN2A mutation in family with benign familial infantile seizures.  Epilepsia. 2006;  47 218-220
• 16 Szepetowski P, Rochette J, Berquin P, Piussan C, Lathrop G M, Monaco A P. Familial infantile convulsions and paroxysmal choreoathetosis: a new neurological syndrome linked to the pericentromeric region of human chromosome 16.  Am J Hum Genet. 1997;  61 889-898
• 17 Uemura N, Okumura A. Benign convulsions with mild gastroenteritis - a worldwide clinical entity.  Brain Dev. 2005;  27 78
• 18 Vigevano F, Fusco L, DiCapua M, Ricci S, Sebastianelli R, Luchcini P. Benign familial infantile convulsions.  Eur J Pediatr. 1992;  151 608-612
• 19 Vigevano F. Benign familial infantile seizures.  Brain Dev. 2005;  27 172-177
• 20 Watanabe K, Yamamoto N, Negoro T, Takaesu E, Aso K, Furune S. et al . Benign complex partial epilepsies in infancy.  Pediatr Neurol. 1987;  3 208-211
• 21 Watanabe K, Yamamoto N, Negoro T, Takahashi I, Aso K, Maehara M. Benign infantile epilepsy with complex partial seizures.  J Clin Neurophysiol. 1990;  7 409-416
• 22 Watanabe K, Negoro T, Aso K. Benign partial epilepsy with secondarily generalized seizures in infancy.  Epilepsia. 1993;  34 635-638
• 23 Watanabe K, Okumura A. Benign partial epilepsies in infancy.  Brain Dev. 2000;  22 296-300
• 24 Weber Y G, Berger A, Bebek N, Maier S, Karafyllakes S, Meyer N. et al . Benign familial infantile convulsions: linkage to chromosome 16p12-q12 in 14 families.  Epilepsia. 2004;  45 601-609
• 25 Zhou X, Ma A, Liu X, Huang C, Zhang Y, Shi R. et al . Infantile seizures and other epileptic phenotypes in a Chinese family with a missense mutation of KCNQ2.  Eur J Pediatr. 2006;  165 691-695