The Clinical Spectrum of Epilepsy in Children and Adults with Hypothalamic Hamartoma
Version of Record online: 22 SEP 2003
Volume 44, Issue 10, pages 1310–1319, October 2003
How to Cite
Mullatti, N., Selway, R., Nashef, L., Elwes, R., Honavar, M., Chandler, C., Morris, R., Jarosz, J., Buchanan, C. and Polkey, C. (2003), The Clinical Spectrum of Epilepsy in Children and Adults with Hypothalamic Hamartoma. Epilepsia, 44: 1310–1319. doi: 10.1046/j.1528-1157.2003.04103.x
- Issue online: 22 SEP 2003
- Version of Record online: 22 SEP 2003
- Accepted May 4, 2003.
- Hypothalamus hamartoma;
- Gelastic seizures;
- Precocious puberty;
- Stereotactic thermocoagulation
Summary: Purpose: Hamartomas of the hypothalamus (HH) cause an uncommon and unusual epilepsy syndrome. The condition is recognized to affect children, but the presentation in adults is not well understood. We present 19 children and adult patients with HH, including three patients whose epilepsy began in adult life. The patterns of clinical presentation, evolution of the epilepsy from childhood to adult life, and electroclinical diagnostic features are presented.
Methods: Nineteen patients, both children and adults with HH and epilepsy, were evaluated clinically, with EEG, video-EEG, and magnetic resonance imaging (MRI) scanning. Seven patients underwent surgical resection of the hamartoma. Stereotactic thermocoagulation of the hamartoma was performed in two patients.
Results: Gelastic seizures occurred at onset of epilepsy in 15 of 16 early-onset cases. Subsequently, multiple seizures types occurred, which then evolved to mainly partial epilepsy with tonic or complex partial seizures (five of eight adults), or became entrenched symptomatic generalized epilepsy with atypical absences, drop attacks, and secondarily generalized seizures, and cognitive impairment (three of eight adults). In the adult-onset patients, gelastic seizures were not prominent, the epilepsy was milder, and they functioned normally. Stereotactic thermocoagulation of the hamartoma resulted in improvement in seizure control in two patients.
Conclusions: Gelastic seizures are not a prominent feature of epilepsy in adult patients with HH. The epilepsy associated with HH, although severe at onset, can evolve into a milder syndrome in later life. For less severely affected patients, minimally invasive alternatives to the traditionally difficult open surgical treatment should be considered.