Abstract

Sir, Hypothalamic hamartoma (HH) is a congenital malformation of the hypothalamus that presents with precocious puberty or seizures. The typical seizure type associated with HH is gelastic seizure. Gelastic seizures which are characterized by frequent attacks of inappropriate laughter, often begin early in life, sometimes in the newborn period [1]. Rarely, seizures associated with crying, known as dacrystic seizures, may occur [2]. A 2-year-old boy presented with history of abnormal crying spells since 4 months of age. He had unexplained crying spells with facial grimacing and weeping lasting 5– 10 min. Initially these episodes occurred 2–3 times per wk, but by 6 months of age, these started occurring multiple times every day. The episodes were stereotyped. His developmental milestones were age appropriate. There was no history of vomiting, visual disturbances, hearing impairment and weakness of any part of the body. Examination revealed an alert child with normal facies and no evidence of neurocutaneous stigmata or precocious puberty. The neurological and systemic examination were unremarkable. MRI of the brain (Fig. 1) showed suprasellar mass lesion involving the hypothalamus, which was isointense to the gray matter, suggestive of hypothalamic hamartoma. The interictal EEG was normal. Apart from gelastic seizures, other seizure types in hypothalamic hamartoma include complex partial seizures, generalized tonic seizures, drop attacks and infantile spasms [3]. Dacrystic seizures, which are characterized by spontaneous paroxysmal attacks of weeping with lacrimation associated with contorted facies, may also occur, as in the present patient [2]. There may be a combination of gelastic and dacrystic seizures in the same patient and these are generally considered to be variations of the same seizure manifestation [3, 4]. When the sole presentation is with dacrystic seizures, the episodes may be mistaken for infantile colic, breath holding spells or cyanotic spells. Diagnostic difficulty is compounded by the fact that the interictal and ictal EEG are often normal in such children [4]. The stereotyped and recurrent nature of dacrystic, nonprovoked episodes in the present patient strongly suggested an epileptic nature. The present case highlights the need for awareness of the unusual and subtle seizure types in young children, so that they may be appropriately diagnosed and managed. K. Mondal : S. Sharma (*) : S. Aneja Department of Pediatrics, Lady Hardinge Medical College and associated Kalawati Saran Children’s Hospital, New Delhi, India e-mail: sharma.suvasini@gmail.com

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