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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Anatomie der hypothalamichen form von pubertas praecox. Precocious puberty and hypothalamic hamartoma. The tumour was completely resected in one case without any serious complications.

Vascular structures in brain tumors. About Blog Go ad-free. Many other types of seizures may appear in these patients, either at the beginning or during the course of the disease. By using this site, you agree to the Terms of Use and Privacy Policy. The lesion shows no significant post-contrast enhancement.

Central precocious puberty is also frequently encountered in these children, typically in patients with sessile tumours. Neurology, 60pp. Log in Sign up. Childs Nerv Syst, 25pp. Cited ruber Lin, at el. Am J Hum Genet, 82pp. Edit article Share article View revision history.

The surface electroencephalogram EEG has a limited ability to show epileptiform activity in this pathology, given the deep location of this lesion and the complex connections of the hamartoma. Our study is tuner retrospective review of 10 paediatric patients diagnosed with hypothalamic hamartoma cjnereum the last 20 years in a paediatric tertiary referral centre.


The electroencephalographic findings recorded were highly variable. On the other hand, sessile or intrahypothalamic hamartomas surround and displace structures in the hypothalamus and the third ventricular wall.

Of the series total, 2 patients did not experience any seizures during the course of the disease. Epilepsy associated with hypothalamic hamartoma is typically refractory to AEDs. The relationship between magnetic resonance imaging findings and clinical manifestations of hypotalamic hamartoma.

Tuber cinereum hamartoma

Patients with hypothalamic hamartoma may suffer other kinds of epileptic manifestations, such as complex partial and generalised seizures. Medical therapy of true precocious puberty due to hamartoma of the tuber cinereum.

The clinical spectrum of epilepsy in children and adults with hypothalamic hamartoma. Results Epidemiological and perinatal data Table 1 Of the 10 patients hamsrtoma our series, 6 were male.

Zur klinik under pathologischen. Lesions can cause gelastic seizures, visual problems, early onset of puberty and behavioral problems 3.

All patients required at least 2 different drugs for seizure control. Age at onset months. Hamartome des tubereinereum bei pubertas praecox: Hypothalamic tuber cinereum and left mammillary body mass lesion; tuber cinereum hamartoma.

Hamartomas of the tuber cinereum and precocious puberty. Age at onset of seizures ranged between a few days and 4 years, with a mean age of 10 months. Lack of enhancement is an important imaging characteristic to help distinguish the tumor from similar masses that can occur in this region. Gonadotropin-releasing hormone and Thyrotropin-releasing hormone distribution and effects in the central nervous system.


Laughing seizures and precocious puberty case report and review of the literature. Hormonal suppressive therapy with luteinizing hormone receptor agonists like leuprorelin can be used to treat the seizure component, and are effective in most patients. Currently, only 2 patients have attained acceptable seizure control as a result of pharmacotherapy.

Mentioned in Royal Pains Season five; an adult patient is diagnosed with it after describing what he calls “church giggles” and early puberty.

Tuber cinereum hamartoma | Radiology Case |

Nevertheless, our series haamrtoma one patient of short stature and another with panhypopituitarism. Light microscopic studies using immunoperoxidase technique Endocrinology, Inclusion in quiz mode: Gelastic epilepsy; onset in neonatal period. We ruled out surgery in 2 patients, since their seizures were controlled adequately by AEDs.

Gamma knife surgery for hypothalamic hamartomas accompanied by medically intractable epilepsy and precocious puberty: Long-term outcome after emergency resection of hypothalamic hamartomas for status gelasticus. Two patients needed to be hospitalised upon birth. Please cite this article as: Fits of laughter gelastic epilepsy with a tumor of the floor of the third ventricle. Etude Clinique, radiologique, histologique. Case 4 Case 4.

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