Hamartomas hipotalámicos: distintas formas de debut. Casos clínicos / Hypothalamyc hamartomas: different ways of clinical debut. Cases report

Los hamartomas hipotalámicos son malformaciones no neoplásicas de sustancia gris compuestas por neuronas hiperplásicas. Suelen ser lesiones pequenas localizadas en la base del cerebro, en el piso del tercer ventrículo y, generalmente, asintomáticas. Sin embargo, pueden ocurrir con alteraciones conductuales-cognitivas, crisis epilépticas y/o signos de pubertad precoz central en función de la localización en la que se encuentren. Se presentan dos pacientes de 2 años 8 meses y 7 años, con presencia de hamartomas hipotalámicos diagnosticados tras el estudio de pubertad precoz central. La paciente de menor edad presenta, además, crisis gelásticas, típicamente asociadas a hamartomas hipotalámicos. Tras los hallazgos clínicos y radiológicos, se trataron con análogos de gonadotropinas, y se observó una regresión de los signos puberales y una no progresión del tamano de los hamartomas.

Hypothalamic hamartomas are benign tumors of gray substance composed by hyperplasic neurons. They are usually asymptomatic small masses with extensions into the third ventricular cavity. In some instances they can cause cognitive behavioral alterations, seizures and/or central precocious puberty depending on the location. Here we present two cases of central precocious puberty due to hypothalamic hamartomas at 2(8/12) and 7 years of age. The younger patient also presents gelastic seizures, typically associated with hypothalamic hamartomas. After the clinical and radiological findings, they started treatment with GnRH analogues and a regression of the puberty signs without progression in the hamartomas size was observed.

Suprasellar ganglioglioma with unusual diffuse involvement of the entire optico-chiasmal hypothalamic pathway.

Gangliogliomas (GG) are mixed glioneuronal tumors of the central nervous system (CNS), occurring mostly in the pediatric population, with common sites being temporal lobes and less commonly in the frontal and parietal lobes. We report a case of a 7-year-old child who presented with bilateral visual defects for 6 months. Magnetic resonance imaging (MRI) of the brain revealed an intensely enhancing mass lesion with calcification in the sellar and suprasellar region involving the optic chiasm and the left optic nerve. The mass showed almost bilaterally symmetrical diffuse spread along the optic tracts posteriorly and hypothalamus, temporal lobes, thalami and the basal ganglia. The lesion was radiologically indistinguishable from chiasmatic astrocytoma or a germ cell tumor but histopathological features were of a ganglioglioma. While a few optic apparatus gangliogliomas have been reported in the literature, such widespread diffuse involvement of the entire optico-chiasmal hypothalamic pathway is unusual.

Interstitial radiosurgery for the treatment of hypothalamic hamartomas

Patients presenting with hypothalamic hamartomas often are not only compromised by pharmacoresistant epileptic seizures, typically [gelastic epilepsy,] but also by behavioural problems and cognitive dysfunction. We report here on the effect of treatment using stereotactic interstitial radiosurgery with placement of [125] l-seeds into hypothalamic hamartomas in a pilot series of 9 patients. Outcome in terms of seizure reduction was complete seizure freedom or simple partial seizures only in 4 patients, significant reduction in seizure frequency in another 3 patients. Two patients did not profit from seed implantation. In patients rendered seizure free or suffering only from auras, improvement of behaviour was reported by parents and colleagues or schoolteachers. Parents' rating according to the Child Behaviour Checklist showed improvements with regard to social problems and attention. Self-rating of quality of life by adult patients showed improvements in activities, working situation, and self perception. There were no major side effects

Localización del daño ocasionado por la diabetes mellitus en el eje hipotálamo-hipofisiario-gonadal en ratas con diabetes inducida / Localization damage caused for the diabetes mellitus in the hypotalamous - pituitary - gonad in rats diabetes induced

Debido a la controversia que existe con la relación a la zona específica del eje Hipotálamo-Hipófisis-Gónada donde la diabetes produce la alteración, al presente estudio se realizó con el propósito de localizar el daño ocasionado por esta patología en el mencionado eje, en ratas con diabetes inducida, mediante la determinación de las concentraciones séricas de tetosterona, posterior a la administración de hormona liberadora de gonadotropinas, (LHRH), hormona folículo estimulante (FSH) y hormona luteinizante (LH). Para ello, se estudiaron 10 ratas a las cuales se les indujo la diabetes con estreptozotocina (ETZ), como grupo control se estudiaron 5 ratas sin diabetes inducida. El grupo de ratas diabéticas se dividió en dos grupos: A y B después de las determinaciones basales de tetosterona (electroluminiscencia), glucosa (método enzimático) y el peso corporal de los animales. Al primero se le administró LHRH y al segundo FSH-LH. En las ratas con diabetes inducida se encontraron disminuidos el peso corporal y el volumen testicular, así como los niveles de testosteronas, este último patrámetro aumentó de manera significativa después de administrar el tratamiento hormonal. Estos resultados sugieren que la lesión ocasionada por la diabetes mellitus es a nivel hipotálamico

effect of a hypothalamic lesion on blood cholestrol in the lizard, uromastix hardwickii

The study evaluates TBC [Total Blood Cholesterol] during complete starvation following a hypothalamic lesion. For this purpose 240 lizards were divided into three groups. Animals of groups A and B were kept in captivity. Group A represented only starved animals. While group B represented starved lesioned Group. Group C consisted of 10 fresh animals purchased very day to represent a fed group. The cholesterol estimation of A, B and C groups was done from day 1 to day 7 Sachet's method. Results showed presence of hypocholestrolemia in group A animals during starvation. The decline being great from day 5 to 7. In response to the hypothalamic lesion in group B animals cholesterol increased on day 1, steeply declined on day 2 and then gradually increased till days 7

monitoring of potential long-term complications in treated adult cancer patients

A wide variety of long-term complications may be noted following conventional cancer treatment. These include overwhelming postsplenectomy sepsis syndrome, central and peripheral nervous system toxicity, cardiovascular complications, chronic liver damage, and secondary malignancy. Follow-up monitoring usually incorporates both physical examination and the result of radiological and laboratory investigations. Primary care physicians will often be responsible for the majority of the follow-up monitoring involving cured cancer patients and need to be aware of possible long-term therapy-related problems, especially those that present years after successful treatment of the original malignancy

Cellular localization of atrial natriuretic peptide and tissue kallikrein in the human hypothalamus

In the kidney, renal atrial natriuretic peptide (ANP) is considered to play an important role wter and salt homeostasis. Immunoreactive ANP in the brain of lower invertebrates, such as the rat, has been shown to be localizaed in the hypothalamus and septum. Several studies have investigated the possibility of a regulatory system in the brain similar to that of the kidney. Since neuronal function is acutely sensitive to disturbances of the intracranial water and salt balance we have attempted to immunolocalize ANP-containing cells in the normal human hypothalamus, using a polyclonal antiserum specific to ANP. Also, we have observed tissue kallikrein (TK), using a polylonal antiserum specific to TK, in the same areas as ANP. A regulatory role for TK on prolactin has been suggested as the rationale for the co-localization of these two hormones in human prolactinomas. Therefore, it could be suggested that TK plays a similar role in the processing of precursor ANP in the brain. It is contemplated to examine the status of these peptides in patients with cerebral oeodema

Hypothalamic hamartomas and precocious puberty.

Ten children, five boys and five girls with true precocious puberty at an early age were found to have hypothalamic hamartomas on brain imaging. Very early onset of puberty, varying from a few weeks to three years of age, and rapid progression were characteristic. Accelerated growth velocity and markedly advanced bone age were evident in all. Gonadotropin and gonadal hormone levels were elevated above the prepubertal range. Six children had associated developmental delay or hyperactivity.

Quiste epidermoide hipotalámico e hiperprolactinemia secundaria / Hypotalamic epidermoid cyst and secundary hyprprolactinemia

La hiperprolactinemia secudaria a tumor hipotalámo es una patología poco frecuente de gran interés clínico. Se presenta el caso de una mujer de 32 añosde edad con padecimiento de siete meses de evolución manifestado por cefalea intermitente, disminución de la agudeza visual, amenorrea y galactorrea. Posteriormente inicia poliuria, polidipsia, hiperfagia, aumento ponderal y trastornos del sueño. a la exploración física se encontró defecto campimétrico visual, alteración de los pares craneales I, II, III y VII y lesión piramidal. Los estudios hormonales fueron compatibles con diabetes insípida y síndrome hiperprolactinémico. La tomografía computada evidenció la presencia de tumoración hipotalámica hiperémica con componenete quístico. La paciente fue intervenida quirúrgicamente, encontrándose tumor quístico de la línea media de la región hipotalámica, el cual no pudo ser extirpado en su totalidad, reportándose como diagnóstico anatomopatológico definitivo quiste epidermoide de la línea media. La evolución postquirúrgica de la paciente fue mala, fallecido 48 horas después de la cirugía.