Gyratory seizures (GS) are a rare seizure type characterized by body rotation of ≥180° around its vertical axis. While GS have been documented in various epileptic syndromes, their occurrence in association with hypothalamic hamartomas (HH) has not been reported previously. This case report introduces the first documented instance of GS in a patient with a HH, a non-neoplastic tumor originating from the tuber cinereum. The patient, a 25-year-old female, with a history of recurrent seizures since childhood, initially presented with gelastic seizures, marked by inappropriate laughter, and subsequent evolution of symptoms including right oculocephalic version and gyratory seizures to the right side. Despite multiple antiepileptic medications, seizures persisted. Neuroimaging revealed a HH in the right hypothalamic region. The presence of polydactyly prompted consideration of Pallister Hall syndrome (PHS). PHS is an autosomal dominant condition linked to GLI3 gene mutations. While some features of PHS were absent in this case, the presence of both gelastic and gyratory seizures indicated the hypothalamus as the lesion site, despite inconclusive electroencephalogram findings. This report underscores the novel association of GS with HH and highlights the importance of considering PHS in patients with HH and polydactyly presenting with gelastic and gyratory seizures. Understanding GS in HH may offer insights into broader hypothalamic lesion-related epileptic phenomena.
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http://dx.doi.org/10.14581/jer.24008 | DOI Listing |
Cureus
November 2024
Department of Neurology, Hiroshima Citizens Hospital, Hiroshima, JPN.
Punctate hippocampal hyperintensity (PHH) on diffusion-weighted imaging (DWI) is a well-known observation in patients with transient global amnesia (TGA), which is characterized by acute self-limiting episodes of anterograde and retrograde amnesia. These lesions occur unilaterally or bilaterally in the CA1 regions of the hippocampus, which are crucial for memory processes. PHH on DWI is well-documented in TGA but rare in other conditions.
View Article and Find Full Text PDFEpileptic Disord
October 2024
Division of Child and Adolescent Neurology, Department of Neurology, Mayo Clinic, Rochester, Minnesota, USA.
J Epilepsy Res
June 2024
Department of Neurology, Meitra Hospital, Calicut, India.
Indian J Pediatr
June 2023
Comprehensive Neuro-Intensive Care Program, Department of Neurology, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Thiruvananthapuram, Kerala, India.
Seizure
December 2020
Department of Neurology, Seth GS Medical College and KEM Hospital Mumbai, India.
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