Clinical characteristics in the core and peripheral types of severe myoclonic epilepsy in infancy.
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In evaluating severe myoclonic epilepsy in infancy (SME), high voltage slow wavegrand mal syndrome (HVSW-GM), and the peripheral group of the former two syndromes, it seems important to take the viewpoint that the grand mal seizure is the common and principal clinical symptom of all three clinical entities.<BR>To investigate general characteristics including pathophysiological factors of these clinical entities, the following clinical criteria common to both SME and HVSW-GM were established: grand mal with onset before the age of one year as the principal seizure type; difficulty in determining the type of epilepsy whether partial or generalized; some degree of mental and motor dysfunction appearing after the seizure onset; difficult detection of epileptic discharges in EEG in the initial stage; and, therapeutic resistance at the commencement of therapy.<BR>Of twenty-two patients meeting all the clinical criteria, twelve patients had been diagnosed as having SME, six as having HVSW-GM, and four as having symptoms peripheral to these syndromes (P).<BR>The percentage of cases with status epilepticus for the SME group was markedly high and that of the obtunded state showed a similar tendency, but neither HVSWGM nor P showed such tendencies. Adding that, each percentae of cases with severe mental dysfunction, abnormal brain CT findings, and poor prognosis for the three groups seemed to increase in the order of HVSW-GM, P and SME.<BR>According to these results, it seems reasonable to regard cases with P as intermediate between cases with SME and HVSW-GM. It also seems that grand mal in combination with other seizure types complicates pathophysiological conditions, and, especially in combination with myoclonic seizures, increases the severity of epilepsy. It is suggested that the three clinical entities, SME, HVSW-GM and P, share certain characteristics and may in fact be more productively conceptualized as representing portions of a continuum.<BR>In conclusion, it is suggested that there moy be a common pathophysiological basis for all three clinical entities and therefore, they may belong to the same clinical entity, "infantile refractory grand mal syndrome".
- 一般社団法人 日本てんかん学会の論文
一般社団法人 日本てんかん学会 | 論文
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