MR Imaging and Spectroscopy of a Tuber Cinereum Hamartoma in a Patient with Growth Hormone Deficiency and Hypogonadotropic. Hamartoma of the hypothalamus and tuber cinereum may be regarded as a midline .. Brower, B. and Brummelcamp, R.: Le syndrome de puberte precoce. La edad de debut de la epilepsia en los pacientes con hamartoma . (MB). Coronal section, T2-weighted sequence, showing a tuber cinereum hamartoma.

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Lin, Shu-Ren, Brysen, M. Spironolactone and endocrine dysfunction. Patients with hypothalamic hamartoma may suffer other kinds of epileptic manifestations, such as complex partial and generalised seizures. Hmartoma the series total, 2 patients did not experience any seizures during the course of the disease. Psychiatric aspects of patients with hypothalamic hamartoma and epilepsy.

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Currently, only 2 patients have attained acceptable seizure control as a result of pharmacotherapy. This may be accompanied by orofacial automatisms.

All epileptic patients in our series experienced gelastic seizures at some point in the course of the disease. Tumor of the tuber cinereum with precocious puberty: Mean follow-up time in the series was 6 years. Surgery for arachnoid cyst. Long-term outcome after emergency resection of hypothalamic hamartomas for status gelasticus.

Thank you for updating your details. Epilepsia, 44pp. Cursive epilepsy und gelastic epilepsy. Patients having these seizures may exhibit groaning and flushing, followed soon after by crying.


Oliver and Boyd, Edinburgh, Londonpp. It has been shown that they produce successful outcomes with regard to seizure control and improvement of related disorders, and involve fewer risks than conventional surgery. In this series, 6 patients had some type of developmental delay or learning disorder. Pubertas praecox bei hyper-plastischen Fehlbildung des tuber cinereuiu. These lesions may grow slowly in the interpeduncular cistern without dinereum adjacent structures.

Tuber cinereum hamartoma

The age of onset of epilepsy in patients with hypothalamic hamartoma in our series was between the first days of life and 2 years. Treatment modality for intractable epilepsy in hypothalamic hamartomatous lesions.

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With chronic seizures, cognitive decline can develop, which can manifest as poor school performance, decreased nervous stimulus IQor limited socialization. They are thought to arise from anomalous neural migration between 35 and 40 days in utero time of hypothalamic formation.

Hypothalamic hamartoma in paediatric patients: It is a congenital malformation, included on the spectrum of gray matter heterotopias.

All patients required at least 2 tubeer drugs for seizure control. Summary of the characteristics of patients cibereum hypothalamic hamartoma in our series. Childs Nerv Syst, 22pp.

There are two types of hypothalamic hamartomas, depending on their radiological imaging classification: Le syndrome de puberte precoce, adiposite, polydactylie, oligophrenie lors d’une malformation localisee dans 1’hypothalamus. Continuing navigation will be considered as acceptance of this use. Size of the hamartoma via MRI cm.

These seizures have also been described in the literature. Epilepsia, 51pp. Neurotrophic keratitis secondary to cerebral Surgical resection of hypothalamic hamartomas for severe behavioral symptoms.


Neuropsychiatr Dis Treat, 4pp. Histologically, hypothalamic hamartomas resemble normal hypothalamic neurones, although some dysplastic neurones and glial cells have also been described 3.

Gelastic seizures are the most common seizures in patients with hypothalamic hamartoma, especially in childhood, and almost always constitute the first epileptic manifestation. J Clin Endocrinol Metab,pp.

Tuber cinereum hamartoma | Radiology Case |

Precocious puberty due to hypothalamic tumor hamartoma in a negroid boy. The mean time between onset of seizures and surgery was 5 years. Precocious puberty and hypothalamic hamartoma.

Pubertas praecox bei einer hyperplastischen missbildung des hypothalamus. Epilepsy is the most frequent manifestation of hypothalamic hamartomas. Phakomatosis Q85 Different electroclinical manifestations of the epilepsy associated with hamartomas connecting to the middle or posterior hypothalamus.

Type I Type II. Nevertheless, emerging techniques, such as stereotaxic radiosurgery especially gamma knife surgeryare being promoted as the first line of treatment of the near future.


Several surgical approaches have been proposed for resecting hamartomas microsurgical resection or disconnection, endoscopic resection. Type of registered seizures. The most commonly used classification is morphologic, dividing hypothalamic hamartomas into either sessile or pedunculated morphology 3, Ann Neurol, 58pp.