A hypersomnolent girl with decreased CSF hypocretin level after removal of a hypothalamic tumor.
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Adolescent↗
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Results for “Hypothalamic Neoplasms”
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Russell's diencephalic syndrome.
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Astrocytoma↗
Resection of the lesion in patients with hypothalamic hamartomas and catastrophic epilepsy.
BACKGROUND: Patients with hypothalamic hamartomas (HH) often have severe refractory epilepsy, incapacitating behavioral abnormalities, and cognitive decline. Attempts to control the seizure disorder by resection of apparently epileptogenic mesial temporal or other cortical structures have failed consistently. OBJECTIVE: To report a series of 13 patients in whom the hamartoma itself was resected. METHODS: All patients underwent preoperative evaluation between ages 2 and 33 years and had subtotal or complete resection of the hamartoma. Follow-up ranged from 1 to 5.5 years (mean: 2.8 y). RESULTS: Preoperatively, all patients had variable combinations of gelastic, complex partial, and generalized seizures. Eight had drop attacks. In addition, all had marked behavior abnormalities and cognitive impairment. Postoperatively, two patients are completely seizure-free and 11 are either seizure-free or have achieved a greater than 90% reduction of drop attacks and generalized tonic-clonic seizures. However, minor gelastic, complex partial, and atypical absence seizures have persisted in 11 patients, although at significantly reduced rates. In addition, there has been a dramatic improvement in behavior and cognition. Three patients had an anterior thalamic and one a capsular infarct, which left only minimal long-term deficits. Exact location of the lesion in relation to the interpeduncular fossa and the walls of the third ventricle correlated with extent of excision, seizure control, and complication rate. CONCLUSION: Resection can alleviate both the seizures and the behavioral and cognitive abnormalities of hypothalamic hamartomas, but complications are frequent.
Adolescent↗
Hypothalamic hamartoma: report of two cases.
Two histologically confirmed hypothalamic hamartomas, one in a 7-year-old boy and another in a 10-year-old boy, are reported. One patient had precocious puberty, epileptic laughter, and abnormal behavior; the other had cerebral seizures. Partial removal of the tumors had no effect on precocious puberty; however, behavior improved in the first patient, and seizure control improved in the second patient.
Child↗
Asymptomatic large hypothalamic hamartoma associated with polydactyly in an adult.
A hypothalamic hamartoma is a congenital tumor-like neural malformation. It is usually seen in children and is associated with neuroendocrinological symptoms, seizures, or psychological impairments. An asymptomatic hypothalamic hamartoma in an adult is extremely rare. This report describes an asymptomatic adult with a large hypothalamic hamartoma associated with polydactyly in his feet. Both polydactyly and hamartoma are rare lesions; therefore, this may not be a coincidental presentation. It is thought to have occurred in the embryonic period presumably between 37 and 40 gestational days.
Adult↗
Hypothalamic hamartomas: with special reference to gelastic epilepsy and surgery.
This study presents six patients with hypothalamic hamartomas diagnosed on the basis of magnetic resonance imaging. Histological confirmation was performed in three patients who underwent surgery. Immunohistological assays were used to determine the neurosecretory pattern. Four patients presented with epilepsy, including gelastic seizures. Other symptoms included behavioral abnormalities in four patients and precocious puberty and visual impairment in two patients. One patient presented associated developmental defects. Good results without morbidity were achieved with surgical resectioning in two patients with large hamartomas associated with behavioral abnormalities and gelastic epilepsy that was unresponsive to conventional medical treatment and in one patient with visual impairment. We propose a classification of the hypothalamic hamartomas based on topographical and clinical data obtained from 36 selected cases in the literature and six of our own cases. This classification should help to classify the various treatment methods and the surgical risks into four subgroups (Types la, lb, lla, and llb). We conclude that the surgical approach is a realistic alternative in certain cases, including large and broad-based Type llb hamartomas associated with gelastic epilepsy and behavioral disorders.
Adult↗
Endoscopic resection of hypothalamic hamartoma using a novel variable aspiration tissue resector.
OBJECTIVE: We present a novel variable aspiration tissue resector for use with neuroendoscopy. METHODS: Two patients, 4 and 14 years old, respectively, presented with intractable gelastic seizures refractory to maximal medical therapies. Magnetic resonance imaging showed mass lesions of the third ventricle consistent with hypothalamic hamartoma. RESULTS: The patients underwent magnetic resonance imaging wand-guided endoscopic resection of the tumor with the Suros novel variable aspiration tissue resector. There were no device-associated complications or adverse events. The hamartoma was disconnected in one patient, and gross total resection was achieved in the other. CONCLUSION: Endoscopy for tumor resection is still frustrated by the lack of surgical tools, such as ultrasonic aspirators, comparable with those available for use during open procedures. The variable-aspiration tissue resector reported here can be used to resect tumor tissue safely. These two cases demonstrate that gross total resection of small hypothalamic hamartomas is feasible with minimal morbidity through an endoscopic approach.
Adolescent↗
Magnetic resonance imaging of hypothalamic hamartomas.
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Child, Preschool↗
[Treatment of hypothalamic-pituitary tumors--experiences at Hiroshima University School of Medicine].
Hypothalamic-pituitary tumors are unique tumors which are present with various disorders of the cerebrum, cranial nerve, and endocrine system, and which critically ruin the patients' quality of life. However, during the past two decades, hypothalamic-pituitary tumor treatment has developed dramatically. The curative method of the pituitary adenoma had not yet been introduced in the mid-1970s, but was accomplished several years after and spread rapidly through out the world. The main contributors which led to this development were the introduction of micro-neurosurgery (including transsphenoidal surgery), the development of new imaging modalities such as computed tomography (CT) and magnetic resonance imaging (MRI), and the introduction of newer, more effective medicine. Other kinds of hypothalamic-pituitary tumor are also being cured by function-oriented surgery, drugs, and radiation therapy, which give the long-term useful life to patients. Regrettably, there are some malignant tumors which still resist to any multidisciplinary remedy. Efforts to prolong the useful lives of patients with malignant hypothalamic-pituitary tumors and also to improve the quality of life of patients with benign tumors should be continued. The most important point in managing hypothalamic pituitary tumors is to focus on "patient-oriented treatment" tailoring treatment to individual symptoms and needs.
Adenoma↗
Hypercalcemia in an euthyroid patient with secondary hypoadrenalism and diabetes insipidus due to hypothalamic tumor.
A 20-year-old Japanese man with a hypothalamic tumor (most likely germ-cell tumor) which caused secondary hypoadrenalism, hypogonadism and diabetes insipidus developed hypercalcemia and acute renal failure. The serum levels of intact PTH (iPTH), PTH-related protein (PTH-rP), 1,25-dihydroxy vitamin D (1,25- (OH)2 D), ACTH, cortisol, gonadotropins and testosterone were decreased, but his serum levels of triiodothyronine (T3) and thyroxine (T4) were within the normal range at admission, with depressed TSH and slightly increased thyroglobulin. The hypercalcemia was refractory to extensive hydration and calcitonin, but was ameliorated by pamidronate. After irradiation of the hypothalamic tumor, panhypopituitarism gradually developed. The patient has been normocalcemic for the last 2 years and is doing well under replacement therapy with glucocorticoid, L-thyroxine, methyltestosterone and 1-desamino D arginine vasopressin (dDAVP). As to the mechanism of euthyroidism at admission, transient destructive thyroiditis associated with hypopituitarism or delayed development of hypothyroidism following the hypoadrenalism was suggested. This is the first reported case of hypercalcemia in secondary hypoadrenalism due to hypothalamic tumor. Hypercalcemia was most likely induced by increased bone resorption, which was probably elicited by the combined effects of deficient glucocorticoid and sufficient thyroid hormones in addition to hypovolemia and reduced renal calcium excretion. Furthermore, severe dehydration due to diabetes insipidus and disturbance of thirst sensation caused by the hypothalamic tumor aggravated the hypercalcemia, leading to acute renal failure.
Acute Kidney Injury↗
Pathogenesis of extracellular fluid abnormalities of hypothalamic hypodipsia-hypernatremia syndrome.
A 26-year-old man with hypothalamic hypodipsia-hypernatremia syndrome is reported, who presented with adipsia, hypernatremia, and impaired osmolality-mediated arginine vassopressin (AVP) secretion. A chorionic gonadotropin-secreting tumor was detected in the anterior hypothalamus and treated with external irradiation. After the treatment, hypernatremia persisted and was not corrected by fluid loading, osmolality-mediated AVP secretion remained impaired. Despite the absence of signs of hydropenia, hypovolemia was suggested by low blood pressure and elevated plasma indices of the renin-angiotensin system, and supported by blood volume determination. The plasma aldosterone concentrations were inappropriately low for the renin-angiotensin status. The plasma atrial natriuretic polypeptide (ANP) level was normal in spite of hypovolemia and increased more than double after fluid loading. Hypernatremia, primarily caused by hypodipsia and impaired osmolality-mediated AVP secretion, secondarily sustained ANP secretion and suppressed aldosterone release, which conceivably contributed to the development and perpetuation of hypovolemia in this patient.
Adult↗
Central precocious puberty: clinical and imaging aspects.
We review briefly the definition of central precocious puberty (CPP), and discuss early puberty and very early puberty. The association of hypothalamic hamartoma and empty sella with CPP is described. The contribution of new imaging techniques - CT, MRI and ultrasound in the differential diagnosis of CPP is discussed.
Brain Diseases↗
Evidence for decreased growth hormone in patients with hypothalamic hamartoma due to Pallister-Hall syndrome.
Pallister-Hall syndrome (PHS) is characterized by hypothalamic hamartoma, bifid epiglottis, and central or postaxial polydactyly. Familial transmission is autosomal dominant; isolated cases also occur. To screen for hypothalamic-pituitary dysfunction in PHS, we studied a 12 year-old boy (patient #1), and 14 additional patients (patients #2-14: 7M, 7F; ages 4-72 yr). We performed serial sampling of GH, LH/FSH, TSH, and cortisol from 20.00-08 00 h. At 08.00 h, we measured IGF-I, peak responses of LH and FSH after GnRH, and cortisol after ACTH. We found that 6/7 children, including patient #1, and 6/8 adults had low or absent spontaneous GH secretion and/or low levels of IGF-I. Patient #1 also had accelerated pubertal development, but no other patient had abnormalities of the pituitary-gonadal axis, and none of the 14 patients had an abnormal thyroid or adrenal axis. We conclude that decreased pituitary GH secretion is common in PHS, and may exist in the absence of other forms of endocrine dysfunction.
Abnormalities, Multiple↗
Pallister-Hall syndrome with hypoparathyroidism.
We report on a 5 2/12 year-old boy who presented with convulsions, precocious puberty due to a 1.9 x 1.9 x 1.6 hypothalamic hamartoma of the tuber cinereum, and some dysmorphic features including polysyndactyly of hands and feet, suggesting the diagnosis of Pallister-Hall syndrome. Hypocalcemia due to hypoparathyroidism was demonstrated, and this combination has not been reported previously. Therefore, we can add hypoparathyroidism as another feature of Pallister-Hall syndrome.
Abnormalities, Multiple↗
Prolactin dynamics in patients with non-secretin tumours of the hypothalamic-pituitary region.
Twenty-four patients with non-secreting tumours of the hypothalamic-pituitary region, diagnosed by radiographic procedures and confirmed at surgery, were examined before and after the surgical treatment to establish a correlation between Prl responsiveness to dynamic tests and the location of pathological tissue. Three groups of patients were identified. In 10 patients with an intrasellar tumour (group I), Prl had a positive response to TRH, metoclopramide (MCP), and nomifensine (NOM), both pre- and postoperatively. In 4 patients with tumours located entirely in the hypothalamus (group II), Prl responded to TRH, but remained unresponsive to MCP and NOM. Only a partial normalization of the Prl responses was obtained after surgery. Finally, 10 patients had an intra- and extrasellar tumour (group III). In 8 of them Prl responded to TRH, but the increment was lesser than that observed in hypothalmic tumours. Prl did not respond to MCP and NOM. After surgery, Prl responsiveness reappeared in some patients (6 after TRH, 4 after MCP, 3 after NOM). In 2 patients with extensive pituitary damage, Prl did not respond to all dynamic tests pre- or postoperatively. In conclusion, Prl dynamic tests are a reliable tool for studying the altered control of Prl secretion in these patients, since plasma Prl stems from only normal lactotropes surrounding the tumoural tissue. The location of the tumour has a key role in influencing the pattern of Prl response to direct and indirect dynamic tests.
Adenoma↗
Growth hormone (GH) responses to the combined administration of GH-releasing hormone plus GH-releasing peptide 6 in adults with GH deficiency.
In recent years the health problems of adults with growth hormone deficiency (GHD) and the benefits of GH replacement therapy have received considerable attention. However, the reliability of conventional GH tests in the assessment of pituitary GH reserve in this group of patients is still controversial. In this study, we assessed GH secretion after the combined administration of GH-releasing hormone (GHRH) (1 microgram/kg iv) and GH-releasing peptide 6 (GHRP-6, 1 microgram/kg iv) in adult patients diagnosed with GHD by conventional GH testing, and correlate this response with insulin-like growth factor I levels. Twenty-one subjects (13 male, 8 female) with long-standing diagnosis of GHD aged 21-54 years were studied. In 13 subjects GH responses to GHRH plus GHRP-6 were markedly reduced (peak GH response < 10 mU/l), whereas in the remaining eight the response was greater (range 11-100 mU/l). In conclusion, our data show that combined administration of GHRH plus GHRP-6 elicited a significant increase in plasma GH levels in about 40% of patients diagnosed with GHD by conventional GH testing.
Adult↗
Evolution of childhood central diabetes insipidus into panhypopituitarism with a large hypothalamic mass: is 'lymphocytic infundibuloneurohypophysitis' in children a different entity?
We report on a 15-year-old girl who had presented with acute onset central diabetes insipidus at the age of 8 years; this was followed by growth failure due to acquired growth hormone deficiency. Initial magnetic resonance imaging showed a uniformly enlarged pituitary stalk and absence of posterior pituitary hyperintensity. Frequent patient examination and magnetic resonance imaging gave unchanged results until after 5 years a large hypothalamic mass and panhypopituitarism were found. Dynamic magnetic resonance imaging documented hypothalamic-pituitary vasculopathy. Histopathological examination revealed perivascular inflammatory lymphoplasmic infiltrates with no granulomatosis or necrosis and negative staining for S-100 protein, suggesting autoimmune inflammatory disease (lymphocytic infundibuloneurohypophysitis?). The response to glucocorticoid pulses (30 mg/kg per day for 3 days i.v.) was favorable. the hypothalamic mass being halved and partial anterior pituitary function recovery maintained for 2 years after the start of treatment. We suggest that long-term surveillance is needed for isolated and chronic thickening of the pituitary stalk and that dynamic magnetic resonance imaging can contribute to the demonstration of hypothalamic-pituitary vascular impairment associated with local vasculitis.
Age of Onset↗
Severe hypersomnolence after pituitary/hypothalamic surgery in adolescents: clinical characteristics and potential mechanisms.
OBJECTIVES: After resection of hypothalamic/pituitary tumors, children are at risk for development of hormonal deficiencies, obesity, and hypersomnolence. However, the prevalence and pathophysiology of these complications are unclear. The purpose of this study was to assess the prevalence and severity of hypersomnolence in children after resection of pituitary tumors and to study the potential factors that contribute to this sleepiness if present. We further hypothesized that decrements in orexin levels may contribute to the sleepiness. METHODS: Six children who underwent hypothalamic/pituitary surgery were identified. Five of these patients and 5 matched control subjects underwent overnight polysomnography followed by a multiple sleep latency test. Children who had a primary sleep disorder (eg, obstructive sleep apnea) underwent treatment and were restudied subsequently (n = 2). Blood levels of pituitary hormones were measured. Blood and cerebrospinal fluid (CSF) were drawn from 4 patients and 3 control subjects to measure orexin levels. RESULTS: Endocrine control was appropriate in all children. Although patients had longer sleep duration but similar sleep efficiency than control subjects, relatively severe daytime somnolence was present (mean sleep latency: 10.3 +/- 5.3 minutes vs 26.2 +/- 1.1 minute in control subjects). Sleepiness did not correlate with body mass index or age. Furthermore, serum and CSF orexin levels did not differ between patients and control subjects. CONCLUSIONS: Severe daytime sleepiness is frequent among children who undergo pituitary/hypothalamic surgery and does not seem to result from inappropriate cortisol or thyroxine replacement, disturbed nocturnal sleep, or low levels of orexin in the serum or CSF. We therefore speculate that other, unidentified neurohormonal mechanisms may mediate the excessive sleepiness of these patients.
Adolescent↗