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Benign familial and non-familial infantile seizures Fukuyama-Watanabe-Vigevano syndrome (F-W- V Syndrome) - two cases report

Purpose: Benign familial and non-familial infantile convulsions, represent a benign, age- related idiopathic epileptic syndrome of infancy, manifesting in partial seizures with or without secondary generalization, mostly clusters with an onset between the 3rd and 20th month of life. Psychomotor development is normal. Watanabe described non- familial and Vigevano familial form of the disease with an autosomal dominant mode of inheritance with mutations of genes on 19q, 16p, and 2q. The aim of this study was to show the clinical and electroencephalographic features and good clinical course of two children with seizures characteristic of F-W-V syndrome. Method and results: The prenatal and perinatal histories of both our unrelated patients were normal. Family history of the boy revealed that his father and uncle suffered recurrent seizures in infancy period. The boy´s first seizures began when he was 5 months old. The attacks were occured after one and three months following onset of the disease, in clusters lasting from two to four days. The girl suffered her first seizures at age 7 months. Antiepileptic therapy started after a recurrence of seizures. Full clinical control of seizures was achieved in both patients in their 9th month. Treatment was performed throughtout around two years in both patients. Repeated interictal waking and sleeping EEGs, as well as images of brain MRI in both children were normal. Rare metabolic diseases with epileptic seizures were excluded. A two-year clinical follow-up after discontinuing treatment showed normal psychomotor development. Conclusion: The clinical diagnosis of F-W-V syndrome is based on the clinical form and age of seizures onset, normal results of interictal electrocortical activity, cessation of seizures until the end of infant period and normal psychoneuromotor development. Analysis of the boy´s family pedigrees suggests the possibility of familial forms of benign infantile convulsiones and indicates genetic tests.To our knowledge, this is the first report on F-W-C syndrome in our population.

benign infantile seizures ; Fukuyama-Watanabe-Vigevano syndrome

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