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Gelastic epilepsy--a case report with SPECT studies
S Khadilkar1, K Menezes, V Lele
1Department of Neurology, Grant Medical College and Sir JJ Group of Hospitals, Mumbai.
Summary
This study reports a case of a young male with lifelong uncontrollable laughter and incontinence. Findings suggest the right cingulate gyrus, not the hypothalamic hamartoma, may be involved in seizure pathway.
Area of Science:
- Neurology
- Neuroimaging
- Epileptology
Background:
- Epilepsy is a chronic neurological disorder characterized by recurrent seizures.
- Hypothalamic hamartomas are rare congenital tumors associated with gelastic seizures and other epilepsy types.
Observation:
- A 24-year-old male presented with daily gelastic seizures (uncontrollable laughter) and urinary incontinence since age nine.
- Seizures sometimes progressed to generalized tonic-clonic seizures.
- Standard neurological exams and video electroencephalography (EEG) were normal.
Findings:
- Magnetic resonance imaging (MRI) revealed a hypothalamic hamartoma.
- Interictal single-photon emission computed tomography (SPECT) showed normal perfusion in the hamartoma.
- Ictal SPECT demonstrated increased perfusion in the right cingulate gyrus during seizures, but not in the hamartoma.
Implications:
- This case highlights the diagnostic challenges in certain epilepsy presentations.
- Findings suggest the right cingulate gyrus plays a role in the seizure origin or propagation pathway, distinct from the hypothalamic hamartoma.
- Further research is needed to elucidate the complex relationship between hypothalamic hamartomas and cingulate gyrus involvement in gelastic seizures.