1/43. hamartoma of the suprasellar cistern in a 5-year-old girl.A 5-year-old girl with precocious puberty secondary to a suprasellar hamartoma is presented. magnetic resonance imaging (MRI) revealed a lesion without attachment to the tuber cinereum or the mamillary bodies. Total resection of the tumour was performed.- - - - - - - - - - ranking = 1keywords = resonance (Clic here for more details about this article) |
2/43. Hypothalamic Langerhans cell histiocytosis with no eosinophils.A 26-year-old man presented with diabetes insipidus, sexual dysfunction and memory impairment. Nuclear magnetic resonance imaging and pathologic examination revealed Langerhans cell histiocytosis of the hypothalamus, which contained no eosinophils. The lesional cells were positive for S-100 protein and CD1a antigen, and negative for CD68 antigen. The possible pathogenesis, absence of eosinophils and prognosis are discussed.- - - - - - - - - - ranking = 5.0149270841558keywords = magnetic resonance, resonance (Clic here for more details about this article) |
3/43. Gamma knife radiosurgery for hypothalamic hamartomas in patients with medically intractable epilepsy and precocious puberty. Report of two cases.hamartoma of the hypothalamus represents a well-known but rare cause of central precocious puberty and gelastic epilepsy. Due to the delicate site in which the tumor is located, surgery is often unsuccessful and associated with considerable risks. In the two cases presented, gamma knife radiosurgery was applied as a safe and noninvasive alternative to obtain seizure control. Two patients, a 13-year-old boy and a 6-year-old girl, presented with medically intractable gelastic epilepsy and increasing episodes of secondary generalized seizures. Abnormal behavior and precocious puberty were also evident. Magnetic resonance (MR) imaging revealed hypothalamic hamartomas measuring 13 and 11 mm, respectively. After general anesthesia had been induced in the patients, radiosurgical treatment was performed with margin doses of 12 Gy to 90% and 60% of isodose areas, covering volumes of 700 and 500 mm3, respectively. After follow-up periods of 54 months in the boy and 36 months in the girl, progressive decrease in both seizure frequency and intensity was noted (Engel outcome scores IIa and IIIa, respectively). Both patients are currently able to attend public school. Follow-up MR imaging has not revealed significant changes in the sizes of the lesions. Gamma knife radiosurgery can be an effective and safe treatment modality for achieving good seizure control in patients with hypothalamic hamartomas.- - - - - - - - - - ranking = 1keywords = resonance (Clic here for more details about this article) |
4/43. Langerhans cell histiocytosis limited to the pituitary-hypothalamic axis--two case reports.Langerhans cell histiocytosis rarely presents as a solitary lesion in the pituitary-hypothalamic region, and is indistinguishable from germinoma, which occurs much more frequently, especially in Japanese. A 14-year-old girl and a 9-year-old girl presented with polydipsia and polyuria as the initial symptoms. Magnetic resonance (MR) imaging demonstrated a round mass at the pituitary stalk appearing as isointense on T1-weighted imaging and hyperintense on T2-weighted imaging. Endocrinological examination revealed mild hypopituitarism with central diabetes insipidus. Both patients underwent open craniotomy. Histological examination revealed granulomatous tissue with eosinophil infiltration and frequent Langerhans histiocyte clustering, compatible with the diagnosis of Langerhans cell histiocytosis. Low-dose local irradiation of 20 Gy was administered. First patient was followed up for 8 years, and her hypopituitarism gradually improved to a minimal level with only amenorrhea as the residual symptom. Recent MR imaging showed no residual mass at the region. Second patient was followed up for 15 months, and her diabetes insipidus is stable. MR imaging performed 5 months after the treatment showed marked reduction of the mass. These cases reemphasize the importance of histological diagnosis for lesions with similar neuroimaging appearances. biopsy and low-dose irradiation are an effective treatment for this rare and essentially benign lesion, as opposed to attempting total removal of the mass.- - - - - - - - - - ranking = 1keywords = resonance (Clic here for more details about this article) |
5/43. Hypothalamic hamartoma associated with an arachnoid cyst.A hypothalamic hamartoma associated with an arachnoid cyst in an 8-year-old boy is reported herein. He presented with precocious puberty, and neuroimaging studies demonstrated a solid mass in the prepontine cistern and a huge arachnoid cyst in the left cranial fossa. The mass appeared isointense to the surrounding cerebral cortex on T1-weighted magnetic resonance images, hyperintense on T2-weighted images, and was not enhanced after administration of Gd-DTPA. The patient underwent a left frontotemporal craniotomy and a cyst-peritoneal shunt was inserted. Histological features of the cyst wall and the mass were characteristic of an arachnoid cyst and hamartoma, respectively. While a hypothalamic hamartoma associated with an arachnoid cyst is rare, such a case may help clarify the geneses of both anomalous lesions.- - - - - - - - - - ranking = 5.0149270841558keywords = magnetic resonance, resonance (Clic here for more details about this article) |
6/43. The use of stereotactic radiosurgery to treat intractable childhood partial epilepsy.PURPOSE: Although conventional surgery is presently used to treat seizures of temporolimbic and neocortical origin, deep-seated lesions are often associated with morbidity. Stereotactic radiosurgery is a noninvasive procedure that effectively treats patients with vascular malformations and brain tumors, but its efficacy for epileptogenic foci is limited, especially in children. methods: Between 1995 and 1999, four candidates who had medically uncontrolled seizures and localized seizure foci were selected for stereotactic radiosurgery, with a mean age of 9.75 years at the time of surgery (range, 4-17 years). Seizure foci were identified on the basis of ictal and interictal video-EEG. Magnetic resonance (MR) images were obtained before and after surgery. Ictal single-photon emission computed tomography (SPECT) was performed by using stabilized hexamethyl-propyleneamine oxime (HMPAO; 300 microcuries/kg) with early injection after electrographic ictal onset. The clinical features of the patients are given. All radiosurgical procedures were performed with the gamma knife unit with the Leksell stereotactic frame, stereotactic MRI imaging, and the Gamma Plan workstation. Seizure outcome was scored according to Engel's classification. RESULTS: Two patients had hypothalamic hamartoma (HH), and two had neocortical epilepsy. At mean follow-up of 39.2 months (range, 26-69 months), two patients were seizure free, one with a HH and one with a suggestive developmental tumor in the insular cortex by MRI findings. The other patient with HH had 90% reduction of seizures. One patient with a widespread seizure focus that involved the motor strip was unimproved. The two patients with HH also exhibited markedly improved neurobehavioral status after surgery. There were no significant complications of radiosurgical therapy. CONCLUSIONS: Our findings suggest that gamma knife surgery is a potentially valuable treatment modality for children with medically intractable epilepsy due to a well-localized seizure focus that is difficult to excise by conventional techniques or for whom they are deemed unsuitable. More widespread application in childhood epilepsy should be investigated in larger series.- - - - - - - - - - ranking = 1keywords = resonance (Clic here for more details about this article) |
7/43. Gelastic seizure with hypothalamic hamartoma: proton magnetic resonance spectrometry and ictal electroencephalographic findings in a 4-year-old girl.Gelastic seizure is a rare symptom often associated with hypothalamic hamartoma. We present here a 4-year-old girl with gelastic epilepsy caused by hypothalamic hamartoma and report the magnetic resonance spectrometry and electroencephalographic (EEG) findings. At the age of 2 1/2 years, she developed brief, repetitive laughing attacks or mixed attacks with laughing and crying, which were refractory to carbamazepine. An interictal EEG showed intermittent slow waves in the left frontocentral region and sporadic positive sharp waves in the left centroparietal area. Ictal EEG demonstrated dysrhythmic theta activity in the left central area 3 seconds after the onset of laughing. brain magnetic resonance imaging demonstrated a large sessile mass, isointense to gray matter, in the region of the hypothalamus, suggesting hypothalamic hamartoma. Proton magnetic resonance spectrometry of the hypothalamic hamartoma revealed a significant reduction of the N-acetylaspartate/serum creatinine ratio. The altered chemical shift imaging with magnetic resonance spectrometry in our patient suggests a biochemical abnormality in the tissue of the hypothalamic hamartoma. Moreover, this abnormal function of the hamartoma tissue might be closely related to epileptogenesis because the time difference between the ictal laughter and the subsequent EEG changes in the ictal EEG does not support the idea that the activated cortex is the epileptogenic focus.- - - - - - - - - - ranking = 40.119416673246keywords = magnetic resonance, resonance (Clic here for more details about this article) |
8/43. adult-onset idiopathic hypogonadotropic hypogonadism presented with erectile and ejaculatory disorder.A 37-year-old man who had fathered a child five years previously presented with erectile and ejaculatory disorder. Endocrinological examinations revealed isolated luteinizing hormone-releasing hormone (LHRH) deficiency of hypothalamus, resulting in hypogonadotropic hypogonadism, and no causative abnormality was detected in imaging studies, including magnetic resonance imaging (MRI). Having a diagnosis of adult-onset hypogonadotropic hypogonadism, the patient received pulsatile subcutaneous administration of gonadotropin-releasing hormone (GnRH). Sperm analysis and serum level of testosterone improved to normal in a few months. His wife became pregnant using artificial insemination with her husband's semen 15 months after the beginning of the treatment.- - - - - - - - - - ranking = 5.0149270841558keywords = magnetic resonance, resonance (Clic here for more details about this article) |
9/43. Diencephalic syndrome of emaciation in an adult associated with a third ventricle intrinsic craniopharyngioma: case report.OBJECTIVE AND IMPORTANCE: Diencephalic syndrome of emaciation (DS) is seen almost exclusively in infants and young children, and only two cases of DS in adults have been reported previously. We describe a case of DS associated with a third ventricle intrinsic craniopharyngioma in an adult patient. CLINICAL PRESENTATION: A 54-year-old man presented with profound emaciation, disorientation, memory loss, and psychological disorders. Computed tomographic scanning and magnetic resonance imaging of his brain disclosed a tumor within the third ventricle. The preoperative endocrinological examination indicated an elevated growth hormone level and a decreased somatomedin C level. INTERVENTION: The patient underwent partial resection of the tumor, which was adherent to the floor of the third ventricle but not to the ventricle wall, by an interhemispheric-transcallosal approach. The histological examination revealed a squamous papillary-type craniopharyngioma. The patient received 50 Gy of radiotherapy, which resulted in 90 to 95% reduction of the tumor size within 6 months, as indicated by the magnetic resonance images presented. The psychological disorders, memory disturbance, and severe emaciation improved gradually thereafter. CONCLUSION: Although extremely rare, DS can occur in an adult harboring a tumor in the anterior hypothalamus.- - - - - - - - - - ranking = 10.029854168312keywords = magnetic resonance, resonance (Clic here for more details about this article) |
10/43. Histologic diagnosis and management of hypothalamic tumors in children by the use of newly developed flexible neuroendoscopes.Hypothalamic tumors are difficult to treat surgically, and chemotherapy and/or radiation are given based on the histology and the neuraxis staging of the tumors. We have developed flexible neuroendoscopes (Yamadori-type 8 and 9) which have excellent image quality and maneuverability as well as capabilities for biopsy and electrocoagulative debulking of the cystic tumors. We report the successful application of the neuroendoscopes to 10 children with hypothalamic tumors diagnosed with computed tomography or magnetic resonance imaging. Histologic diagnosis was obtained from all the patients. Cystic tumors were evacuated, hydrocephalus managed with endoscopic operations, and some benign tumors were removed totally. The relatively non-invasive approach reported here would represent a significant technical advance in the diagnosis and management of hypothalamic and other ventricular tumors.- - - - - - - - - - ranking = 5.0149270841558keywords = magnetic resonance, resonance (Clic here for more details about this article) |
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