Continuos Spike and Wave Discharges during Slow Sleep (CSWS) in Children with Lesions in the Basal Ganglia - Possible Role of the Thalamus and Caudate Nucleus in Epileptogenesis.
Abstract number :
1.184
Submission category :
Year :
2001
Submission ID :
2606
Source :
www.aesnet.org
Presentation date :
12/1/2001 12:00:00 AM
Published date :
Dec 1, 2001, 06:00 AM
Authors :
J. Kröll, M.D., Swiss Epilepsy Centre, Zurich, Switzerland; R.A. Sälke-Kellermann, M.D., Swiss Epilepsy Centre, Zurich, Switzerland; A. Bauerfeind, M.D., Neurophysiology, Swiss Epilepsy Centre, Zurich, Switzerland; E. Martin, Prof. M.D., Neuroradiology an
RATIONALE: The role of basal ganglia, especially of the thalamus, in the generation of epileptic spike and wave (SW) discharges is well known in generalized epilepsies. Only scarce information is available on epilepsies in patients with lesions of the basal ganglia. We report on four children with lesions in the thalamus and caudate nuclei suffering from epilepsy with CSWS.
METHODS: All children had a whole-night sleep EEG recording as well as a video-EEG investigation during wakefulness. Magnetic resonance images (MRI) were performed. The patients had a neurological examination and a neuropsychological assessment.
RESULTS: There were one girl and three boys, mean age 7.11 years. The aetiology was postpartal haemorrhagia in two cases, neonatal encephalitis in one, and unknown in the remaining. Two children showed a subtle hemiplegia. All patients had a typical CSWS. During daytime, one patient had a non-convulsive status epilepticus over several months whereas the others showed abundant generalized SW activity. Clinical seizures (atypical absences, complex focal seizures, or generalized tonic-clonic seizures) only seldomly occurred, but they were therapy-refractory. All children had severe neuropsychological and behavioural problems. MRI findings: atrophy of the right thalamus (patient 1), atrophy of the right thalamus and right caudate nucleus (patient 2), partial destruction of the left thalamus and the left basal ganglia (patient 3), bilateral atrophy of the caudate nuclei (patient 4). Two children showed additional changes of the cerebral white matter. There were no cortical lesions.
CONCLUSIONS: Our hypothesis is that generalized SW activity in these patients may be generated due to a loss of balance between excitatory and inhibitory influences concerning the thalomo-cortical network. Animal studies have shown that disturbed interaction of the cerebral cortex and the thalamus, including the network between the thalamus and the caudate nucleus, can generate generalized SW discharges. There were no detectable lesions of the cerebral cortex in these patients. Seizures were rare, but all children suffered from severe cognitive deficits.