This article has been cited by other articles in PMC. They experienced several types of seizure gelastic, tonic, partial, atonic, generalized tonic-clonic, dacrystic, infantile spasm, mental retardation, and behavioral and endocrinological abnormalities. Forty-nine interventions were carried out. Every patient, with the exception of the first, underwent hamartoma disconnection pterional approach, six patients; endoscopy, 15 patients; both, 11 patients. The endoscopic approach was carried out with a frameless stereotactic system to enhance feasibility and efficacy of the disconnecting procedure.

Author:Mir Dull
Language:English (Spanish)
Genre:Personal Growth
Published (Last):2 June 2014
PDF File Size:10.16 Mb
ePub File Size:6.77 Mb
Price:Free* [*Free Regsitration Required]

Cinco pacientes presentaron problemas cognitivos. Solo en 3 de los 8 pacientes se ha conseguido aceptable control de su epilepsia. Cinco pacientes de la serie desarrollaron pubertad precoz. Objective: To describe the epidemiological and clinical-electroencephalographic characteristics,and associated morbidity of patients with hypothalamic hamartoma, as well as the treatment followed and outcome Patients and methods: We have retrospectively reviewed the medical histories of 10 patients diagnosed with hypothalamic hamartoma by magnetic resonance imaging over the last 20 years.

Results: The age of onset of epilepsy in patients with hypothalamic hamartoma in our series was between the first days of life and 2 years.

Of the 10 total patients, 8 had epileptic seizures during its progress. All of them had gelastic seizures, in addition to other types of seizures,with the most common being partial simple seizures. The electroencephalographic findings recorded were highly variable. One of the patients developed epileptic encephalopathy.

Five patients had some kind of conduct disorder. Five patients had cognitive problems. At least 2different antiepileptic drugs were measured in 8 of the patients who had seizures, and in 6 of these some type of non-pharmacological treatment had been used with the objective of seizure control.

Only in 3 of 8 patients has been achieved Acceptable control of epilepsy had only beenachieved in 3 out the 8 patients. Five patients of the series developed precocious puberty. Theaverage time of follow-up of the series was approximately 6 years. Conclusions: Epilepsy is the most frequent manifestation of hypothalamic hamartomas.

Mostcases were drug-resistant, which led to difficulties in the management of these patients,requiring surgery for their control on many occasions. Psychiatric comorbidity and cognitiveim pairment is common.


Tumor hipotalámico

Symptoms often begin in early infancy and are progressive, often with general cognitive and functional disability. Clinical presentation Lesions can cause gelastic seizures, visual problems, early onset of puberty and behavioral problems 3. Gelastic seizures are typically of short duration seconds and characterized by uncontrollable laughter, without impairment of consciousness, which contrasts with frontotemporal gelastic seizures which usually are longer lasting and can result in loss of consciousness 4. Central precocious puberty is also frequently encountered in these children, typically in patients with sessile tumors.


Hamartoma Hipotalâmico

Torr To report a case of retinal astrocytic hamartoma with vitreous hemorrhage and a hair pin-like vessel adhering to a posterior vitreous membrane. Conclusions Radiological imaging methods are important for accurate diagnosis of this very rare condition that can be confused with a malignancy. Gamma knife surgery for hypothalamic hamartomas hamargoma refractory epilepsy: Ten fibrolipomatous hamartomas were identified: Both patients became seizure-free only after the temporal lobectomy. Fat is easier to recognize using MRI, particularly with the aid of fat-suppression techniques.

Related Articles