Hypothalamic hamartoma

Common Name(s)

Hypothalamic hamartoma

Hypothalamic hamartomas (HH) are rare, non-cancerous (benign) tumors that develop in part of the brain called the hypothalamus. Hypothalamic hamartomas occur when the baby’s brain is forming (fetal development) and are present at birth. These tumors typically grow as the child’s brain grows. Hypothalamic hamartomas can cause early puberty (known as central precocious puberty), seizures with developmental delays (known as epilepsy and related neurobehavioral symptoms), or a combination of the two.

Children with central precocious puberty start to present with typical physical puberty features as early as 2 years of age. Children with epilepsy and related neurobehavioral symptoms often present with a specific type of seizure, known as gelastic (laughing) seizures, during infancy. Then, between the ages of 4 and 10 years of age, other types of seizures may develop. In addition, behavioral problems, tantrums, rage attacks and social isolation may occur. 40% of children who have seizures caused by hypothalamic hamartomas also have precocious puberty.

Babies and children who present with seizures or precocious puberty at a young age will likely have brain imaging performed. Hypothalamic hamartomas can be diagnosed with this imaging, which is typically a brain MRI (magnetic resonance imaging). Children who have precocious puberty caused by hypothalamic hamartomas can be treated with medications that correct hormone levels. Seizures caused by hypothalamic hamartomas do not respond well to seizure medications. If symptoms are severe enough, surgical removal of the hypothalamic hamartoma may be necessary. If your child has been diagnosed with a hypothalamic hamartoma, talk with your doctor to discuss the most current treatment options. Support groups can provide additional information and connect you with other families.

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Condition Specific Organizations

Following organizations serve the condition "Hypothalamic hamartoma" for support, advocacy or research.

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Scientific Literature

Articles from the PubMed Database

Research articles describe the outcome of a single study. They are the published results of original research.
The terms "Hypothalamic hamartoma" returned 43 free, full-text research articles on human participants. First 3 results:

A case of central precocious puberty due to concomitant hypothalamic hamartoma and juvenile pilocytic astrocytoma.
 

Author(s): Gönül Çatlı, Ayhan Abacı, Ahmet Anık, Handan Güleryüz, Erdener Özer, Irfan Öcal, Nurullah Yüceer, Kamer Mutafoğlu

Journal: J Clin Res Pediatr Endocrinol. 2014 Sep;6(3):183-6.

 

Central precocious puberty (CPP) is caused by premature activation of the hypothalamo-pituitary-gonadal axis. More than 50% of boys with CPP have an identifiable etiology. Hypothalamic hamartoma (HH), hydrocephalus, tumors, infections, congenital defects, ischemia, radiation, or injury ...

Last Updated: 22 Sep 2014

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Hypothalamic hamartoma in an unusual case with delayed puberty.
 

Author(s): Maryam Nakhaeimoghadam, Parastoo Rostami, Ameneh Zare-Shahabadi, Mehrzad Mehdizadeh, Ali Rabbani, Nima Rezaei

Journal: Acta Med Iran. 2013 ;51(11):819-21.

 

Hypothalamic hamartoma (HH) is a rare intracranial lesion that usually presents with classic triad of central precocious puberty, gelastic epilepsy, and developmental delay. Herein, a 14-year old boy is presented in whom the diagnosis of HH was made by magnetic resonance imaging. ...

Last Updated: 6 Jan 2014

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Hypothalamic hamartoma: is the epileptogenic zone always hypothalamic? Arguments for independent (third stage) secondary epileptogenesis.
 

Author(s): Julia Scholly, Maria-Paola Valenti, Anke M Staack, Karl Strobl, Thomas Bast, Pierre Kehrli, Bernhard J Steinhoff, Edouard Hirsch

Journal: Epilepsia. 2013 Dec;54 Suppl 9():123-8.

 

Gelastic seizures associated with hypothalamic hamartomas (HHs) are a clinicoradiologic syndrome presenting with a variety of symptoms, including pharmacoresistant epilepsy with multiple seizure types, electroencephalography (EEG) abnormalities, precocious puberty, behavioral disturbances, ...

Last Updated: 16 Dec 2013

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Reviews from the PubMed Database

Review articles summarize what is currently known about a disease. They discuss research previously published by others.
The terms "Hypothalamic hamartoma" returned 4 free, full-text review articles on human participants. First 3 results:

Mechanisms of intrinsic epileptogenesis in human gelastic seizures with hypothalamic hamartoma.
 

Author(s): Jie Wu, Ming Gao, Jian-Xin Shen, Shen-Feng Qiu, John F Kerrigan

Journal: CNS Neurosci Ther. 2015 Feb;21(2):104-11.

 

Human hypothalamic hamartoma (HH) is a rare developmental malformation often characterized by gelastic seizures, which are refractory to medical therapy. Ictal EEG recordings from the HH have demonstrated that the epileptic source of gelastic seizures lies within the HH lesion itself. ...

Last Updated: 21 Jan 2015

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[Hypothalamic hamartoma in paediatric patients: clinical characteristics, outcomes and review of the literature].
 

Author(s): C Castaño De La Mota, F Martín Del Valle, A Pérez Villena, M L Calleja Gero, R Losada Del Pozo, M L Ruiz-Falcó Rojas

Journal: Neurologia. 2012 Jun;27(5):268-76.

 

To describe the epidemiological and clinical-electroencephalographic characteristics, and associated morbidity of patients with hypothalamic hamartoma, as well as the treatment followed and outcomes

Last Updated: 28 May 2012

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Hypothalamic hamartoma.
 

Author(s): Kazunori Arita, Kaoru Kurisu, Yoshihiro Kiura, Koji Iida, Hiroshi Otsubo

Journal: Neurol. Med. Chir. (Tokyo). 2005 May;45(5):221-31.

 

The incidence of hypothalamic hamartomas (HHs) has increased since the introduction of magnetic resonance (MR) imaging. The etiology of this anomaly and the pathogenesis of its peculiar symptoms remain unclear, but recent electrophysiological, neuroimaging, and clinical studies have ...

Last Updated: 25 May 2005

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Symptoms, Diagnosis, and Treatment

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