Severe epilepsy with multiple independent spike foci.
Severe epilepsy with multiple independent spike foci is an electroclinical entity with the following characteristics: (1) EEG showing multiple independent spike foci (three or more foci in both hemisphere, i.e., at least one in each hemisphere) and diffuse slowing of the background activity, (2) very frequent multiple types of seizures but mainly generalized minor seizures, (3) frequent association with mental retardation and neurologic abnormalities, (4) underlying causes of various nonspecific prenatal, perinatal, and postnatal cerebral conditions, and (5) poor prognoses for seizures and psychomotor development. It represents a diffuse encephalopathy with mutual transition between other age-dependent epileptic encephalopathies. This electroclinical condition may be appropriately named Markand-Blume-Ohtahara syndrome, after Markand and Blume, who initially described in detail the EEG pattern of multifocal spike discharges with its major clinical correlates, and Ohtahara, who extensively studied the group of patients with both clinical and EEG characteristics and proposed the concept of severe epilepsy with multiple independent spike foci, classifying it as one type of generalized epilepsy.
Yamatogi
Yasuko
Y
Faculty of Health and Welfare Science, Okayama Prefectural University, 111 Kuboki, Soja-shi, Okayama Prefecture, Japan 719-1197. yyamatog@fhw.oka-pu.ac.jp
Ohtahara
Shunsuke
S
D000367:Age Factors; D001921:Brain / Q000503:physiopathology; D004569:Electroencephalography; D004827:Epilepsy / Q000503:physiopathology* / Q000517:prevention & control; D005071:Evoked Potentials; D006801:Humans; D009069:Movement Disorders / Q000209:etiology / Q000503:physiopathology; D015995:Prevalence; D011379:Prognosis; D013577:Syndrome; D013812:Therapeutics