Hypothalamic hamartomas and gelastic epilepsy: a spectroscopic study.
BACKGROUND
Patients with hypothalamic hamartomas present with epileptic attacks of laughter and later experience multiple seizure types and cognitive decline, suggestive of secondary generalized epilepsy. It has been suggested in the past that gelastic seizures originate in the temporal lobes rather than in the hamartoma, but temporal resections have been ineffective. Recent electrophysiologic evidence suggests that the epileptogenic discharges may originate in the hamartoma itself.


METHODS
We used proton magnetic resonance spectroscopic imaging to quantify the amount of neuronal damage in the temporal lobes and hamartomas of patients with hypothalamic hamartomas and gelastic seizures. Five patients were studied and the relative intensity of N-acetylaspartate to creatine (NAA/Cr) was determined for both temporal lobes as well as for the hamartoma. These values were compared with signals from the temporal lobes and hypothalami of normal control subjects.


RESULTS
NAA/Cr was not significantly different from normal control subjects for either temporal lobe, nor was there a significant asymmetry between the two temporal lobes for any of the patients. NAA resonance signals were present in the hamartomas, and the ratio of NAA to Cr was decreased in the hamartomas compared with the hypothalami of normal control subjects (t = 4.5, p = 0.005).


CONCLUSIONS
We found no detectable neuronal damage in the temporal lobes of patients with hypothalamic hamartomas and gelastic epilepsy. This is further evidence that gelastic seizures do not originate in the temporal lobes of these patients.
Department of Neurology and Neurosurgery, McGill University, Montreal Neurological Hospital and Institute, Quebec, Canada.
E
E
Tasch
F
F
Cendes
L M
LM
Li
F
F
Dubeau
J
J
Montes
B
B
Rosenblatt
F
F
Andermann
D
D
Arnold
D000293:Adolescent; D000328:Adult; D002648:Child; D004569:Electroencephalography; D004827:Epilepsy / Q000000981:diagnostic imaging* / Q000209:etiology / Q000503:physiopathology; D005260:Female; D006222:Hamartoma / Q000150:complications / Q000000981:diagnostic imaging* / Q000503:physiopathology; D006801:Humans; D007027:Hypothalamic Diseases / Q000150:complications / Q000000981:diagnostic imaging* / Q000503:physiopathology; D007031:Hypothalamus; D007845:Laughter / Q000502:physiology*; D008279:Magnetic Resonance Imaging / Q000379:methods; D008297:Male; D015899:Tomography, Emission-Computed, Single-Photon