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At least 235 records · Page 13Linked to original sources
PubMed1993

Callosotomy for generalized seizures associated with hypothalamic hamartoma.

A case is reported of intractable epilepsy associated with a hypothalamic hamartoma in an 18 year old man. The patient underwent a two-third anterior callsotomy and, subsequently, removal of the hamartoma. Callosotomy did not affect the generalized seizure pattern. The authors believe this to be the first documented case of hypothalamic hamartoma in which callosotomy for seizure control was attempted. The poor response to callosotomy suggests the extracallosal diffusion of the generalized seizures from hypothalamic hamartomas.

Adolescent↗
PubMed1997

Avascular necrosis secondary to postoperative steroid therapy.

Hypothalamic and pituitary tumours may present with vague symptoms owing to excess or lack of hormone production, including diabetes insipidus. Corticosteroids are commonly employed to limit cerebral oedema and at much lower doses to treat secondary hypocorticalism. Continuation of steroids at inappropriately high doses predisposes to the development of avascular necrosis as in the case we describe in a young woman of 34 years. This is a potentially preventable crippling disorder. When prescribing steroids the lowest effective dose should be used.

Adult↗
PubMed1999

Resection of a Langerhans cell histiocytosis granuloma of the hypothalamus: case report.

The natural course and optimal treatment for isolated hypothalamic Langerhans cell histiocytosis (LCH) are unknown. We describe an adult female in whom total resection of a hypothalamic LCH granuloma was performed 12 years after transphenoidal resection of a pituitary adenoma. A retrospective review of the histological specimen of the first operation revealed CD1a positive cells characteristic of LCH along with a plurihormonal adenoma 12 years earlier. No other manifestations of LCH were found and MRI of the brain at the last follow-up 4 years after surgery did not show any recurrent or additional lesion. The diagnosis of isolated hypothalamic LCH is only possible by biopsy and our case demonstrates the feasability of a gross total resection in certain cases.

Female↗
PubMed2000

Endocrinology teaching rounds: primary amenorrhea in a 17-year-old woman.

This endocrinology teaching rounds documents the evaluation of a 17.7-year-old woman with primary amenorrhea. The normal timing of developmental milestones for young women from adrenarche and pubarche through pubertal maturation and growth is reviewed, as are etiologies for amenorrhea. The differential diagnosis of the problem is developed from the patient's initial visit to diagnosis and treatment. The highlights of the management of this young woman after a diagnosis of craniopharyngioma are also discussed. This clinical case emphasizes the importance of understanding normal developmental landmarks and of detecting aberrant physiology-associated amenorrhea even when screening laboratory tests appear normal so that defects in normal development can be addressed earlier in life.

Adolescent↗
PubMed1986

Intraperitoneal bleomycin for ventriculoperitoneal spread of a hypothalamic astrocytoma.

A pediatric patient is presented in whom malignant ascites developed after ventriculoperitoneal shunt placement for a suprasellar astrocytoma. Paracentesis followed by intraperitoneal bleomycin resulted in decreased fluid re-accumulation with minimal side effects. A review of the literature shows that intracavitary chemotherapy, including bleomycin, can result in safe, effective relief of malignant effusion associated with adult tumors. We have demonstrated that such treatment is also applicable to the pediatric population. In this case, the effectiveness of intracavitary bleomycin may be related to its direct action against brain glioma cells. The need for effective treatment of malignant effusions in pediatric patients is growing because of their increased survival time with tumor. We propose that intracavitary bleomycin may provide relief from this potential complication of childhood solid tumors.

Astrocytoma↗
PubMed1993

Mirth, laughter and gelastic seizures.

Little is known about what pathways subserve mirth and its expression laughter. We present three patients with gelastic seizures and laughter elicited by electrical stimulation of the cortex who provide some insight into the mechanisms of laughter and its emotional concomitants. The first patient had seizures manifested by laughter without a subjective feeling of mirth. Magnetic resonance imaging showed a cavernous haemangioma in the left superior mesial frontal region. Ictal subdural electrode recording showed the seizure onset to be in the left anterior cingulate gyrus. Removal of the lesion and of the seizure focus rendered the patient virtually seizure free over 16 months of follow-up. The other two patients had complex partial seizures of temporal lobe origin. Electrical stimulation of the fusiform gyrus and parahippocampal gyrus produced bursts of laughter accompanied by a feeling of mirth. These cases reveal a high likelihood of cingulate and basal temporal cortex contribution to laughter and mirth in humans, and suggest the possibility that the anterior cingulate region is involved in the motor act of laughter, while the basal temporal cortex is involved in processing of laughter's emotional content in man.

Adult↗
PubMed1993

Pulsatile administration of gonadotrophin releasing hormone as a diagnostic tool to distinguish hypothalamic from pituitary hypogonadism following neurosurgery.

Nine patients suffering from severe amenorrhoea following neurosurgical or radiotherapy for pituitary tumour or craniopharyngeoma were treated with pulsatile gonadotrophin releasing hormone (GnRH) administration. Seven patients did exhibit ovulatory cycles when GnRH was administered i.v. at a dose of 20 micrograms/pulse, but not at lower doses or when GnRH was administered s.c. Pulsatile GnRH administration may be used to assess the functional integrity of pituitary gonadotrophs and to distinguish pituitary from hypothalamic site of lesion resulting in hypogonadotrophic hypogonadism. It may also be used successfully for treatment of infertility in such patients.

Adolescent↗
PubMed1980

Surgical syndromes of the hypothalamus.

The clinical syndromes described with lesions of the hypothalamus are summarized in Table 9.5-9.7. The anterior hypothalamic syndrome consists of insomnia and loss of thirst regulatory mechanisms. In occasional larger lesions which interrupt the output from the supraoptic and paraventricular nuclei, diabetes insipidus has been noticed. In the tuberal region of the hypothalamus the most prominent findings are those that are caused by the disruption of the final common pathway to the pituitary. This results in endocrinopathy, most often the syndrome originally reported by Frohlich, with failure of sexual maturation and obesity. In the tuberal region, differences between lesions of the medial and lateral portions are quite marked. Medial lesions result in obesity while bilateral lesions result in anorexia and emaciation. The diencephalic syndrome of infancy with it's severe emaciation in young years and obesity in later years clearly indicates a different organizational pattern in the neonatal hypothalamus. Emotional disorders may be seen with lesions either in the medial or lateral hypothalamus at the tuberal level. Finally, in the posterior hypothalamic region, which includes the greatest effector apparatus, hypersomnia, apathy, and poikilothermia have been reported. Emotional disturbances and the Wernicke-Korsakoff syndrome also seemed to be associated with lesions in this area. The hypothalamus remains the single most important integrator of vegetative and endocrinologic regulation of the body. Cushing said of the hypothalamus, "here in this hidden spot, almost to be covered with a thumb nail, lies the very main spring of primitive existence: vegetative, emotional and reproductive".

Adenoma↗
PubMed1985

Diabetes insipidus from metastatic oat cell carcinoma: recovery after brain irradiation.

A hypothalamic metastasis was demonstrated by computed tomography in a 71-year-old patient, with previously unknown small cell lung cancer, who presented with diabetes insipidus and biological signs of hypothyroidism and hypogonadism. Brain irradiation resulted in resolution of polyuria, elevation of thyroid hormones, improvement of pituitary responsiveness to hypothalamic releasing hormones, and complete disappearance of contrast-enhanced suprasellar metastasis.

Aged↗
PubMed2004

Hypopituitarism and diabetes insipidus with localized hypertrophic pachymeningitis (Tolosa-Hunt syndrome) associated with Hashimoto thyroiditis.

We report a 69-year-old woman with intracranial pachymeningitis showing hypopituitarism, diabetes insipidus, and Tolosa-Hunt syndrome associated with Hashimoto thyroiditis confirmed by autopsy. A large tumorous lesion of the hypothalamo-pituitary gland was revealed on magnetic resonance imaging, after the patient complained of gait and visual field disturbance. These symptoms subsided after thyroid hormone supplementation. Hypopituitarism and diabetes insipidus were diagnosed after cessation of the treatment by the patient herself. Multiple cranial nerve palsies and orbito-frontalgia appeared. Methylprednisolone pulse therapy improved the symptoms, but they recurred when the dose of glucocorticoid was decreased. The patient died of brain thrombosis. Autopsy revealed typical findings of Hashimoto thyroiditis and intracranial pachymeningitis involving the cranial base and pituitary gland. The high titer of rheumatoid factor and Hashimoto thyroiditis in this patient suggest an immunological role in the pathogenesis of pachymeningitis.

Aged↗
PubMed2004

Primary hypothalamic lymphoma with panhypopituitarism presenting as stiff-man syndrome.

We report an unusual case of primary hypothalamic lymphoma with hypopituitarism presenting as Stiff-man syndrome (SMS). A 64-year-old man was hospitalized due to a 3-week history of general weakness, anorexia, vomiting, weight loss, and muscle pain and spasms precipitated by motion and tactile stimuli resulting in muscle stiffness and difficulty in mobility. Physical examination revealed normal sensorimotor function and reflexes, except for bitemporal visual field defect. Routine laboratory and gastrointestinal examinations provided no remarkable clues. Endocrine assessment revealed low levels of morning cortisol, thyroxine, and anterior pituitary hormones but an increase in prolactin level. The patient's muscle pain and stiffness improved dramatically within 2 days after hydrocortisone therapy and thyroxine replacement. Magnetic resonance imaging (MRI) of the brain confirmed an 18-mm enhancing hypothalamic tumor with optic chiasm involvement, which proved to be a B-cell lymphoma. The results of the extensive studies for systemic lymphoma were negative, suggesting a primary hypothalamic lymphoma. The tumor regressed completely and was invisible on MRI scan after adjuvant radiotherapy. The patient's condition was satisfactory and there was no recurrence of SMS during the 2-year follow-up period. This case demonstrated that primary hypothalamic lymphoma complicated with adrenal insufficiency may manifest as SMS. Early diagnosis and prompt intervention can lead to a favorable outcome and reduce morbidity.

Adrenal Insufficiency↗
PubMed1981

Hypothalamic hamartoma.

Hypothalamic hamartoma is a rare tumor with onset of symptoms in infancy or early childhood. Clinical presentation includes precocious puberty, laughing spells, and seizures. Computed tomography of two cases of hypothalamic hamartomas revealed a mass lesion in the suprasellar--interpeduncular cisterns (with the density of) the surrounding normal brain. The mass was not enhanced by injection of contrast material.

Child↗
PubMedDate not supplied

Neuroimaging findings in a suprasellar granular cell tumor.

A 27-year-old woman presented with a history of amenorrhea, visual disturbance, and diabetes insipidus. Magnetic resonance imaging showed a large enhancing suprasellar mass with associated edema involving the left striatum. The lesion was hypometabolic on fluorodeoxyglucose-positron emission tomography. At surgery, a subtotal resection of a vascular tumor that appeared to arise from the posterior pituitary and hypothalamus was carried out. Pathologic examination revealed a granular cell tumor. We report the preoperative neuroimaging findings in this rare posterior pituitary stalk tumor.

Adult↗
PubMed1985

Identification of gonadotropin-releasing hormone in neurons of a hypothalamic hamartoma in a boy with precocious puberty.

We have studied a 3 1/12-year-old boy who presented with a hypothalamic mass and precocious puberty. His history suggested a course of isosexual precocity progressing from birth. Gelastic seizures also began at an early age. Endocrine evaluation revealed normal thyroid-stimulating hormone and growth hormone secretion, elevated basal and stimulated prolactin concentrations, and luteinizing hormone responses to sequential intravenous injections of gonadotropin-releasing hormone (GnRH) that were pubertal in pattern and magnitude. A needle biopsy of the mass recovered tissue that contained neurons histologically similar to those found in the normal hypothalamus, and the mass was characterized as a hypothalamic hamartoma. Immunohistochemical staining of this tissue with anti-GnRH antiserum demonstrated positive staining for GnRH immunoreactivity in neurons. This suggests a neurosecretory pathogenesis for the precocious puberty found in patients with hamartomas in the hypothalamic region.

Biopsy, Needle↗