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.

Author: Junos Kagazilkree
Country: Singapore
Language: English (Spanish)
Genre: Personal Growth
Published (Last): 7 July 2013
Pages: 300
PDF File Size: 15.73 Mb
ePub File Size: 8.11 Mb
ISBN: 701-3-55217-463-7
Downloads: 26209
Price: Free* [*Free Regsitration Required]
Uploader: Balkis

Epilepsy associated with hypothalamic hamartoma is typically refractory to AEDs.

Verschiedene Gruppen der hypothalamischen pubertas praecox. Hamartoma of the hypothalamus. It can take years before signs of compression appear.

It measures about 1. Type of registered seizures. The intelligence quotient was within the normal range in 2 patients, with borderline intellectual functioning in 1 patient, mild mental retardation in finereum patient and moderate mental retardation in the last patient. Our study is a retrospective review of 10 paediatric patients diagnosed with hypothalamic hamartoma in the last 20 years in a paediatric tertiary referral centre.

Symptoms often begin in early infancy and are progressive, often with general cognitive and functional disability.

Emergency transcallosal resection of hypothalamic hamartoma for status gelasticus. Uber die pathologic des Hypothalamus, Schweizer. Therefore, where gelastic seizures are present, we should be aware that hypothalamic hamartoma is not the only possible aetiology.


The initial technique cinereu, in 6 of the 7 patients who underwent surgery was resection.

We used this procedure as a second treatment approach for one of the cases with a subtotal surgical resection. Multiple patients presented 2 or more of these problems. Nervous system neoplasia Puberty Hypothalamus disorders Rare diseases. Pubertas praecox bei einer hyperplastischen missbildung des hypothalamus. Neurology, 60conereum. Endokri-nologie und diagnostik in vivo. Rev Neurorad, 12pp. Intractable epilepsy can respond to surgery resection or disconnection, depending on the morphology of the tumor and this can be performed via cindreum open craniotomy or more recently via an endoscope 5.

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

Hypothalamic hamartoma | Radiology Reference Article |

As shown by several series of patients with epilepsy associated with hypothalamic hamartoma, gelastic seizures are resistant to AEDs and exert an effect on the cognitive and behavioural disorders present in these cases. J Neuropathol Exp Neurol, 67pp. About Blog Go ad-free. Patients and methods We have retrospectively reviewed the medical histories of 10 patients dell with hypothalamic hamartoma by magnetic resonance imaging MRI over the last 20 years.

Any nodularity within should raise the suspicion of tuber cinereum hypothalamic hamartoma. The remainder hamartomx the brain is unremarkable. Surgery and type of surgery. Type I Type II.

There was a problem providing the content you requested

Five tubed developed precocious puberty during the course of the disease. Childs Nerv Syst, 25pp. Hypothalamic hamartoma Tuber cinereum. Utility of early single photon emission computed tomography SPECT in neonatal gelastic epilepsy associated with hypothalamic hamartoma.


Five patients had some kind of conduct disorder. However, a delay of approximately 4 years in diagnosing the lesion may explain the size of the largest hamartoma. Surgery is offered if there is failure of medical therapy or rapid growth of lesion, with specific options including stereotactic thermocoagulation, gamma knife radiosurgeryand physical resection by transsphenoidal microsurgery.

Lack of enhancement is an important imaging characteristic to help distinguish the tumor from similar hmartoma that can occur in this region. Six examples of precocious sexual development. These lesions may grow slowly in the interpeduncular cistern without displacing adjacent structures. Results Epidemiological and perinatal data Table 1 Of the 10 patients in our series, 6 were male.

Tuber cinereum hamartoma – Wikipedia

Many other types of seizures may appear in these patients, either at the beginning or during the course of the disease. June Pages Posterior hypothalamus hamartomas and gangliogliomas causing precocious puberty. Pubertas praecox bei Hamartie des medio-basalen Hypotlialamus mit heterotoper Retinaan-lage.