September 27, 2020

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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Two patients needed to be hospitalised upon birth. Lesions can cause gelastic seizures, visual problems, early onset of puberty and behavioral problems 3. As expected it appears as a nodule of soft tissue iso-attenuating to grey matter, without calcification or contrast enhancement. Neoplasms producing endocrine disturbances in childhood. Dacrystic seizures also appeared in 2 patients; the initial critical episode in 1 of these patients was dacrystic.

Epidemiology Clinical presentation Pathology Radiographic features Treatment and prognosis History and etymology Differential diagnosis References Images: Many causes of the epilepsy have been theorized, with EEG often finding the hamartoma itself as the source of electrical activity, or epileptogenic focus. Gonadal-hypothalamic interaction in prepubertal and pubertal man: To describe the epidemiological and clinical-electroencephalographic characteristics, and associated morbidity of patients with hypothalamic hamartoma, as well as the treatment followed and outcomes.


As they are composed of grey matter, they have imaging appearances similar to the normal cortex. Full screen case with hidden diagnosis.

Tuber cinereum hamartoma – Wikipedia

All epileptic patients in our series experienced gelastic seizures at some point in the course of the disease. Gelastic seizures, precocious puberty and hypothalamic hamartoma. Patients and methods We have retrospectively reviewed the medical histories of 10 patients diagnosed with hypothalamic hamartoma by magnetic resonance imaging MRI over the last 20 years.

More recent epidemiologic studies have found these associations to be less consistent, with gelastic epilepsy predominant in the majority of patients regardless of morphology.

It results from over-secretion of gonadotropin-releasing hormone GnRH which in turn results in over production of luteinizing hormone LH and follicle-stimulating hormone FSH 5.

You can also scroll through stacks with your mouse wheel or the keyboard arrow keys. Le syndrome de puberte precoce, adiposite, polydactylie, oligophrenie lors d’une malformation localisee dans 1’hypothalamus. No patients were lost to follow-up.

Hypothalamic hamartoma | Radiology Reference Article |

The table lists the type of seizures recorded for each patient. J Neuropathol Exp Neurol, 66pp. Published by Wolters Kluwer – Medknow. The histopathology of hypothalamic hamartomas: Paediatric epilepsy syndromes and their surgical treatment, pp. At times it may be difficult to differentiate between gelastic and partial seizures, as they have similar characteristics decreased level of consciousness and orofacial automatisms and both types of seizures may occur concomitantly.

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Our study is a retrospective review of 10 paediatric patients diagnosed with hypothalamic hamartoma in the last 20 years in a cinerejm tertiary referral centre. Coronal section, T2-weighted sequence, showing a tuber cinereum hamartoma. SRJ is a prestige metric based on the idea that not all citations are the same.

Tuber cinereum hamartoma

However, they present more difficulties for seizure control, whether by pharmacological or surgical means. Gelastic, quiritarian and cursive epilepsy: Simple partial seizures were the most common, followed by complex partial seizures and generalised seizures in some cases. Semin Pediatr Neurol, 14pp.

At present, this technique is used as a second option when tumours persist after the initial conventional surgery. Hypothalamic tuber cinereum and left mammillary body mass lesion; tuber cinereum hamartoma. Horm Res, 56pp. Summary of complementary tests and findings. This item has received.