CLINICAL AND ELECTROPHYSIOLOGICAL FINDINGS IN 17 PATIENTS WITH HYPOTHALAMIC HAMARTOMAS
Abstract number :
2.077
Submission category :
Year :
2002
Submission ID :
1520
Source :
www.aesnet.org
Presentation date :
12/7/2002 12:00:00 AM
Published date :
Dec 1, 2002, 06:00 AM
Authors :
Joerg J. Schmitt, Friedrich G. Woermann, Ingrid Tuxhorn, Bernd Kruse, Reinhard Schulz, Alois Ebner. Presurgical Evaluation, Klinik Mara 1, Bielefeld, Germany
RATIONALE: This study was designed to analyze the clinical spectrum of epilepsy in a large number of patients with hypothalamic hamartomas.
METHODS: Data from 17 patients (aged 5 to 45 years) with a hypothalamic lesion found in MRI were analyzed. The clinical course of epilepsy, seizure semiology and electrophysiological findings were correlated with size and site of lesion.
RESULTS: Laughing as a prominent symptom occurred in 14 of 17 patients, usually as part of a seizure with psychomotor or tonic semiology. The remaining 3 patients had an ictal grin or an aura consisting of an urge to laugh or smile. All patients had focal seizures (tonic 8, psychomotor 11, generalized tonic-clonic 4, with an aura in 7 patients). Only one patient had a normal interictal EEG, of the remaining patients, 6 had generalized and regional slowing over one or more cerebral regions, 7 had regional slowing, which was unilateral in 3. In these 3 patients, unilateral slowing was always ipsilateral to the side of lesion, in our sample always over the right hemisphere.
15 of 17 patients had regional epileptiform potentials (temporal only: 1, extratemporal only: 4) or seizure patterns, occurring in two patients exclusively unilateral, in 7 patients bilateral regional and in 6 patients additionally generalized. Two patients with generalized epileptiform potentials also had a photoparoxysmal response in EEG.
Mild to severe mental impairment was present in 8, pubertas praecox in 2 of 17 patients. Neurological examination revealed no focal abnormality except a facial nerve palsy in one patient. No patient became seizure-free through medication.
CONCLUSIONS: This study demonstrates that the clinical spectrum of this disease ranges from isolated laughing attacks without any further impairment to severely handicapped patients with additional significant mental impairment and endocrine dysfunction.
Despite the characteristic symptomatology, diagnosis was delayed for years in several of our patients for various reasons, indicating that the incidence of this condition is probably underestimated.