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Episodes of prolonged “trance-like state” in an infant with hypothalamic hamartoma

Hypothalamic hamartoma (HH) is one of the most common causes of central precocious puberty (CPP) in first few years of life. It can present with either seizures or CPP, although both manifestations coexist in the majority of the children. Gelastic seizures (GS), or laughing spells, are usually the f...

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Autores principales: Kumar, Rakesh, Yadav, Jaivinder, Sahu, Jitendra Kumar, Tripathi, Manjul, Ahuja, Chirag, Dayal, Devi
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Korean Society of Pediatric Endocrinology 2019
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6449620/
http://dx.doi.org/10.6065/apem.2019.24.1.55
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author Kumar, Rakesh
Yadav, Jaivinder
Sahu, Jitendra Kumar
Tripathi, Manjul
Ahuja, Chirag
Dayal, Devi
author_facet Kumar, Rakesh
Yadav, Jaivinder
Sahu, Jitendra Kumar
Tripathi, Manjul
Ahuja, Chirag
Dayal, Devi
author_sort Kumar, Rakesh
collection PubMed
description Hypothalamic hamartoma (HH) is one of the most common causes of central precocious puberty (CPP) in first few years of life. It can present with either seizures or CPP, although both manifestations coexist in the majority of the children. Gelastic seizures (GS), or laughing spells, are usually the first type of seizures seen in patients with HH. Although a wide variety of seizure types are known to occur in children with HH, GS are most common and consistent seizure type. The clinical presentation of HH may vary with the size and position of the mass, although large tumours typically present with both CPP and seizures. Although CPP can be managed with medical therapy, seizures can be very difficult to treat, even with multiple antiepileptic drugs. Noninvasive gamma knife surgery has been used with some success for the treatment of refractory epilepsy. We present a case of HH with very early onset seizures and CPP. The patient had an atypical form of seizures described by the parents as a "trance-like state" in which the patient had prolonged episodes of unresponsiveness lasting for hours with normal feedings during the episodes. GS occurred late in the course and were refractory to various combinations of antiepileptic drugs. A brain magnetic resonance imaging showed a large sessile HH (>20 mm). Later in the course of the disease, the patient experienced cognitive and behavioural problems. The patient underwent gamma knife surgery at nearly 5 years of age and experienced a modest response in seizure frequency. This case highlights the presentation of HH as a previously unreported seizure morphology described as a prolonged "trance-like state."
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spelling pubmed-64496202019-04-10 Episodes of prolonged “trance-like state” in an infant with hypothalamic hamartoma Kumar, Rakesh Yadav, Jaivinder Sahu, Jitendra Kumar Tripathi, Manjul Ahuja, Chirag Dayal, Devi Ann Pediatr Endocrinol Metab Case Report Hypothalamic hamartoma (HH) is one of the most common causes of central precocious puberty (CPP) in first few years of life. It can present with either seizures or CPP, although both manifestations coexist in the majority of the children. Gelastic seizures (GS), or laughing spells, are usually the first type of seizures seen in patients with HH. Although a wide variety of seizure types are known to occur in children with HH, GS are most common and consistent seizure type. The clinical presentation of HH may vary with the size and position of the mass, although large tumours typically present with both CPP and seizures. Although CPP can be managed with medical therapy, seizures can be very difficult to treat, even with multiple antiepileptic drugs. Noninvasive gamma knife surgery has been used with some success for the treatment of refractory epilepsy. We present a case of HH with very early onset seizures and CPP. The patient had an atypical form of seizures described by the parents as a "trance-like state" in which the patient had prolonged episodes of unresponsiveness lasting for hours with normal feedings during the episodes. GS occurred late in the course and were refractory to various combinations of antiepileptic drugs. A brain magnetic resonance imaging showed a large sessile HH (>20 mm). Later in the course of the disease, the patient experienced cognitive and behavioural problems. The patient underwent gamma knife surgery at nearly 5 years of age and experienced a modest response in seizure frequency. This case highlights the presentation of HH as a previously unreported seizure morphology described as a prolonged "trance-like state." Korean Society of Pediatric Endocrinology 2019-03 2019-03-31 /pmc/articles/PMC6449620/ http://dx.doi.org/10.6065/apem.2019.24.1.55 Text en © 2019 Annals of Pediatric Endocrinology & Metabolism This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Case Report
Kumar, Rakesh
Yadav, Jaivinder
Sahu, Jitendra Kumar
Tripathi, Manjul
Ahuja, Chirag
Dayal, Devi
Episodes of prolonged “trance-like state” in an infant with hypothalamic hamartoma
title Episodes of prolonged “trance-like state” in an infant with hypothalamic hamartoma
title_full Episodes of prolonged “trance-like state” in an infant with hypothalamic hamartoma
title_fullStr Episodes of prolonged “trance-like state” in an infant with hypothalamic hamartoma
title_full_unstemmed Episodes of prolonged “trance-like state” in an infant with hypothalamic hamartoma
title_short Episodes of prolonged “trance-like state” in an infant with hypothalamic hamartoma
title_sort episodes of prolonged “trance-like state” in an infant with hypothalamic hamartoma
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6449620/
http://dx.doi.org/10.6065/apem.2019.24.1.55
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