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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Horm Res, 57pp. Epileptic laughter with precocious puberty. A review on the management of epilepsy associated with hypothalamic hamartomas. Childs Nerv Syst, 25pp.

A case of pubertas praecox. The histopathology of hypothalamic hamartomas: Report of two cases surviving surgical removal of the tumor.

Tuber cinereum hamartoma | Radiology Case |

In 1 patient, these seizures appeared in the first days of life, and in 2 others, at the age of 2 years; these data coincide with other studies in the literature in which researchers mention this type of seizure in the first days of life. Giant hypothalamic hamartoma and dacrystic seizures. One female patient had a previous diagnosis of neurofibromatosis type Age at symptom onset was highly variable, ranging from the first days of life in 2 patients in the series, to 6 years in the patient who had a hamartoma that was incidentally discovered while using MRI to assess neurofibromatosis type Are you a health professional able to prescribe or dispense drugs?

SRJ is a prestige metric based on the idea that not all citations are the same. What is the most likely diagnosis?


Neurology, 58pp. Epileptic Disord, 9pp. Fits of laughter gelastic epilepsy with a tumor of the floor of the third ventricle. Gamma knife surgery for hypothalamic hamartomas accompanied by medically intractable epilepsy and precocious puberty: Cinfreum literature includes series with varying numbers of patients, but some of these series also include adult patients.

It is associated with endocrine and neurological symptoms.

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Lack of enhancement is an important imaging characteristic to help distinguish the tumor from similar tubwr that can occur in this region.

All these patients showed abnormal results.

They received medical follow-up in our department for 6 years. With chronic seizures, cognitive decline can develop, which can manifest as poor school performance, decreased nervous stimulus IQor limited socialization.

True isosexual precocious puberty of central origin-clinical and endocrinological aspects.

Any nodularity within should raise the suspicion of tuber cinereum hypothalamic hamartoma. Generalized epilepsy in hypothalamic hamartoma: Psychiatric aspects of patients with tuberr hamartoma and epilepsy.

Tuber cinereum hamartoma

Therefore, where gelastic seizures are present, we should be aware that hypothalamic hamartoma is not the only possible aetiology. One of these last 5 patients needed 2 additional reoperations, which produced similar results. Gelastic epilepsy and true precocious puberty due to hypothalamic hamartoma. The initial clinical symptoms of patients with hypothalamic hamartoma were as follows: Computed tomography CT was carried out in only one patient.

Acta Neurol Scand,pp. In any case, we must be mindful of the potential uamartoma of surgery hypothalamic damage, memory loss, polyphagia or diabetes insipidus, and vascular damage 55,57, The intelligence quotient was within the normal range in 2 patients, with borderline intellectual functioning in 1 patient, mild mental retardation in 1 patient and moderate mental retardation in the last patient. They are thought to arise from anomalous neural migration between 35 and 40 days in utero time of hypothalamic formation.


Spironolactone and endocrine dysfunction. Case 3 Case 3. Report of a case hamartooma review of the literature with a pathophysiological explanation of the precocious sexual syndrome.

Coronal section, T2-weighted sequence, showing a tuber cinereum hamartoma. Neoplasms producing endocrine disturbances in childhood. We used this procedure as a second treatment approach for one of the cases with a subtotal surgical resection. The distribution of luteinizing hormone releasing hormone LH-RH in the hypothalamus of the rhesus monkey. Transcallosal resection of hypothalamic hamartomas in patients with intractable epilepsy.

Loading Stack – 0 images remaining. Small, non-enhancing mass lesion contagious with the tuber cinereum is almost always diagnostic of tuber cinereum hamartoma. Neurosurgery, 62cienreum.

Hypothalamic hamartoma | Radiology Reference Article |

The clinical spectrum of epilepsy in children and adults with hypothalamic hamartoma. Only one patient had a family history of epilepsy. The classic presentation is gelastic or laughing epilepsya disorder characterized by spells of involuntary laughter with interval irritability and depressed mood. These seizures have also been described in the literature.

Most other lesions encountered in the region either have markedly different signal intensity or demonstrate enhancement.