10 cases of nonconvulsive status epilepticus following the termination of generalized convulsive seizures are reported. The stupor status occurred in generalized primary epilepsy (6 cases), in generalized secondary epilepsy (2 cases), and in partial epilepsy (2 cases). Except for generalized secondary epilepsy, the status epilepticus was successfully treated by the intravenous application of anticonvulsants. The therapeutic significance of a differentiation of postconvulsive stupor status from postictal confusional states was stressed.
SUMMARY Four patients meeting the clinical criteria of the rigid spine syndrome are presented; they are one girl with a positive family history and three boys. Clinical and histological findings are discussed in relation to the 14 cases of rigid spine syndrome reported in the literature. The delineations of the syndrome from other benign myopathies with early contractures are discussed suggesting that the rigid spine syndrome probably does not represent a single nosological entity.
EEG and CAT studies were performed in 187 adult chronic epileptics. Acute convulsions, known tumor epilepsies, and seizures due to a rapidly advancing neurologic disease were excluded. Partial seizures of elementary and complex symptoms were nearly equally frequent. In 48 patients the etiology of the seizure disorder remained unknown. Among the verified etiologies residual brain damage were the most significant. In 10 cases the chronic seizure disorder was due to slowly growing brain tumors. CAT findings were normal in 86 patients (46%) and abnormal in 101 (54%). Localizing signs could be demonstrated by neuroradiologic and/or radionuclide tests in 15.7%, by CAT in 28.9%, by neurologic examination in 58.3%, and by EEG in 78.1%. Localized CAT-pathology showed no correlation to the types of partial seizures, whereas a significant correlation could be established with residual brain scars and tumors and with localized neurologic or EEG findings. Epilepsy starting after the age of 15 is more likely to be linked with CAT-pathology than with seizure disorders starting in childhood.
We present two brothers with mental retardation, seizures disorder, generalized muscular hypertonia, kyphoscoliosis, minor anomalies and a prominent midface. GTG-banded chromosome analysis showed a derivative chromosome 14 without clues toward the origin of the rearrangement. Microdissection of the derivative chromosome 14 and subsequent reverse painting demonstrated partial trisomy 7q32-q34 as the unbalanced product of a maternal insertion (14;7). Thus, we identified two cases with pure trisomy 7q32-q34 that allowed further delineation of this aneusomy syndrome.
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