HYPOTHALAMIC HAMARTOMA WITH GELASTIC EPILEPSY, PRECOCIOUS PUBERTY ANDPOLYDACTYLY

An entity including gelastic epilepsy, precocious puberty, polydactyly and a hypothalamic hamartoma type IIa is described in a 16-year-old f emale patient. Polydactyly was detected at birth, she developed precoc ious puberty st four years of age, and gelastic epilepsy was diagnosed at age seven. The precocious puberty was successfully treated medical ly and her treatment was discontinued at the age of 10 years, but the gelastic seizures were difficult to control. When the patient was 11 y ears old, MRI revealed a hypothalamic hamartoma. The combination of th ese four features is very rare in the literature.