Infantile Spasms in One Member of a Family with Benign Familial Neonatal Convulsions

Mori, Kenji; Yano, Ichiro; Hashimoto, Toshiaki

doi:10.1111/j.1528-1157.1993.tb00438.x

Cited by 12 publications

(7 citation statements)

References 22 publications

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“…The infants are neurologically normal, and most have a spontaneous remission; ∼10–15% will have subsequent epilepsy, usually generalized motor seizures. A single case of infantile spasms has been reported in a family with BFNC (34). Baxter and Kandler (35) reported an infant whose seizures presumably began in utero.…”

Section: Etiologymentioning

confidence: 99%

Neonatal Seizures and Syndromes

Tharp

2002

Epilepsia

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Summary: Neonatal seizures frequently accompany neonatal encephalopathies. Seizures occur in ∼1.8-5/1,000 live births in this country and are caused by virtually any condition that affects neonatal brain function. This review provides a simple classification of seizures and emphasizes that many abnormal intermittent behaviors in this age group are not accompanied by ictal EEG patterns. Additionally, Յ50% of neonatal seizures are not associated with abnormal clinical behavior. This is a common phenomenon, particularly after anticonvulsant treatment in which the clinical seizures are suppressed but electrographic seizures continue unabated. Seizures also may be caused by genetic disorders, several of which are benign, familial, and caused by channelopathies involving potassium channels. The review also discusses the epileptic syndromes seen in neonates, including early myoclonic encephalopathy, Ohtahara syndrome, pyridoxine dependency, and glucose transporter type 1 syndrome.

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Section: Etiologymentioning

confidence: 99%

Neonatal Seizures and Syndromes

Tharp

2002

Epilepsia

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“…6 Indeed, several families have been described in whom some individuals show benign neonatal convulsion spontaneously disappearing within a few weeks, while others present with more severe epileptic seizures and unfavorable prognosis. [7][8][9][10] Two genes have been identified to be responsible for BFNC, KCNQ2 on chromosome 20q13.3 11,12 and KCNQ3 on chromosome 8q24. 13 Both genes encode for voltage-gated potassium (K ϩ ) channel subunits belonging to the KCNQ subfamily.…”

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confidence: 99%

A novel mutation in KCNQ2 associated with BFNC, drug resistant epilepsy, and mental retardation

et al. 2004

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“…In contrast, almost all occasional seizures were partial seizures and tonic spasms were not present (Watanabe 1993). BFNC is classified as generalized epilepsy in the current International Classification, but ictal EEGs of their seizures have been well documented in a few cases (Giroud et al 1989, Wakai et al1990, Andrew and Stafstrom 1993, Hirsh et al 1993, Mori et al 1993, Ronen et al 1993, Bye 1994. Twelve of 16 neonates, including our subjects (eight infants), in whom ictal EEGs were recorded, had partial seizures and four had generalized seizures.…”

Section: Discussionmentioning

confidence: 77%

Epilepsies of neonatal onset: seizure type and evolution

Watanabe

Miura

Natsume

et al. 1999

Develop Med Child Neuro

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Most neonatal seizures are occasional seizures and not true epilepsy. This study investigates seizure types of true neonatal epilepsies and their evolution with development. Seventy‐five children with epilepsies of onset within 1 month of life, who were examined between 1970 and 1995, and whose seizure types could be confirmed with ictal EEG recordings, were studied. The patients were followed up for a minimum of 3 years and the evolution of epileptic syndromes was investigated. Sixty‐three (84%) of 75 patients had partial seizures, while nine had generalized seizures, and only three had both generalized and partial seizures. Twenty‐three of 24 neonates with benign familial or non‐familial neonatal convulsions presented with partial seizures; these syndromes should not necessarily be categorized into generalized epilepsy as they are in the present International Classification. Age‐dependent changes were a common feature of symptomatic neonatal epilepsies. Eighteen (41%) of 44 patients with symptomatic epilepsies of neonatal onset developed West syndrome in infancy. Fifteen (83%) of these 18 patients presented with symptomatic localization‐related epilepsy in the neonatal period. In seven of these 15 patients, West syndrome was followed by localization‐related epilepsy. Symptomatic localization‐related epilepsy with transient West syndrome in infancy is another type of age‐dependent epileptic syndrome.

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Infantile Spasms in One Member of a Family with Benign Familial Neonatal Convulsions

Cited by 12 publications

References 22 publications

Neonatal Seizures and Syndromes

Neonatal Seizures and Syndromes

A novel mutation in KCNQ2 associated with BFNC, drug resistant epilepsy, and mental retardation

Epilepsies of neonatal onset: seizure type and evolution

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