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ENeurologicalSci Sep 2021•A patient exhibited IgG4-related hypothalamo-hypophysitis.•Prominent high-signal areas of swelling were observed in the hypothalamus, tuber cinereum, infundibulum,...
•A patient exhibited IgG4-related hypothalamo-hypophysitis.•Prominent high-signal areas of swelling were observed in the hypothalamus, tuber cinereum, infundibulum, and bilateral optic nerve systems.•MRI T1WI with contrast media demonstrated enhanced neurohypophysis and cystic swelling, and compressed anterior pituitary.•MRI findings improved rapidly after 4 days of steroid therapy.
PubMed: 34466672
DOI: 10.1016/j.ensci.2021.100362 -
Radiology Case Reports Jun 2024Ewing sarcoma is the second most frequent primary bone tumour of childhood and adolescence. The aim of this report is to describe the imaging, pathology, clinical...
Ewing sarcoma is the second most frequent primary bone tumour of childhood and adolescence. The aim of this report is to describe the imaging, pathology, clinical findings, and treatment of a primary intradural extramedullary Ewing sarcoma with a unique intracranial metastatic component in a pediatric patient. A 14-year-old girl with a history of mood disorders presented to the emergency department with a 3-week history of neck torticollis, cervical pain, paresis, and paresthesia of the upper and lower extremities on the left side. Initially, non-organic causes such as somatization or conversion disorder were suspected. She returned 3 months later when her symptoms worsened. MRI of the head and spine was performed, and demonstrated the presence of a suprasellar, retro-chiasmatic mass lesion. There was also diffuse leptomeningeal enhancement, another well-defined intradural extramedullary lesion the sacral region and several multifocal cauda equina soft tissue nodules. The patient first underwent surgery. The patient was also treated with a combination of chemotherapy (vincristine, doxorubicin and cyclophosphamide alternating with ifosfamide and etoposide (VDC/IE)) and radiation as per the Children's Oncology Group AEWS1221 protocol. Most recent imaging conducted 22 months after the initial mass discovery revealed improvement of the suprasellar mass lesion with residual stable appearance of the prominence and enhancement of the pituitary stalk and tuber cinereum. There was interval improvement of the spinal lesions with no convincing residual. Clinically, at almost three years since initial imaging findings, and 25 months since completing treatment, she is stable from an oncology perspective.
PubMed: 38572274
DOI: 10.1016/j.radcr.2024.02.101 -
AJNR. American Journal of Neuroradiology 1989Hamartoma of the tuber cinereum is a well-recognized cause of central precocious puberty. We report three patients with an isodense, nonenhancing mass within the... (Comparative Study)
Comparative Study
Hamartoma of the tuber cinereum is a well-recognized cause of central precocious puberty. We report three patients with an isodense, nonenhancing mass within the interpeduncular cistern identified by CT. In a fourth patient, the CT scan was normal. MR imaging was obtained in all cases and demonstrated a sessile or pedunculated mass of the posterior hypothalamus arising from the region of the tuber cinereum. The smallest mass was 2 mm in diameter and was found in the patient in whom the CT scan was normal. The signal intensity of the masses was generally homogeneous and isointense relative to gray matter on T1- and intermediate-weighted images, and hyper-intense on T2-weighted images. MR imaging accurately diagnoses hypothalamic hamartomas, identifies small hamartomas of the tuber cinereum more sensitively than CT does, and provides optimal imaging for serial evaluation while the patient is being treated medically.
Topics: Child; Child, Preschool; Female; Hamartoma; Humans; Hypothalamic Neoplasms; Hypothalamus; Magnetic Resonance Imaging; Male; Puberty, Precocious; Tomography, X-Ray Computed; Tuber Cinereum
PubMed: 2501981
DOI: No ID Found -
Journal of Postgraduate Medicine Jan 1987
Review
Topics: Female; Hamartoma; Humans; Hypothalamic Neoplasms; Hypothalamus; Male; Tuber Cinereum
PubMed: 3302225
DOI: No ID Found -
Frontiers in Neurology 2022The intrahypothalamic phenotype of hypothalamic hamartomas (HH) is associated with epilepsy, and the parahypothalamic phenotype usually leads to central precocious...
BACKGROUND
The intrahypothalamic phenotype of hypothalamic hamartomas (HH) is associated with epilepsy, and the parahypothalamic phenotype usually leads to central precocious puberty but not neurological comorbidities or seizures. No study has confirmed the pathological role of parahypothalamic hamartomas in epileptogenesis, and the underlying mechanism is yet to be elucidated.
OBJECTIVE
We aimed to investigate whether parahypothalamic hamartomas are intrinsically epileptogenic and elucidate the underlying pathway of epileptogenesis.
METHODS
We reviewed 92 patients with HH-related epilepsy, categorized them by the classification system of Delalande and Fohlen, and further classified Type I (corresponding to parahypothalamic HH) into the following three groups based on the relationship between the lesion and mammillary bodies (MB): entirely invaded (Group 1), partially connected (Group 2), and not connected at all (Group 3). We examined different anatomical features with their relationship to clinical manifestations. Stereoelectroencephalography (SEEG) was implanted in both HH and extra-HH cortices in different groups to identify the epileptogenic zone. Corticocortical evoked potentials (CCEPs) were also used to determine the pathological correlation among different regions to determine the related epileptogenic network.
RESULTS
A total of 13 patients presented with parahypothalamic HH and 10 (76.9%) presented with non-GS only, with late-onset age and normal cognitive development, which is different from classical clinical features. SEEG showed that HH is intrinsically epileptogenic in MB-involved parahypothalamic groups. No statistical difference was found in onset age ( = 0.213), and lesions horizontally oriented from the tuber cinereum without connection to MB were not involved in seizure genesis. CCEP indicated a pathological connection among HH, middle cingulate cortex, and insular cortex.
CONCLUSION
The parahypothalamic HH can also cause epilepsy and is different from classic HH-related seizures, by non-GS only with the late-onset age and normal cognitive development. MB is proven to be related to non-GS by the mamillo-cingulate-cortex pathway.
PubMed: 36172032
DOI: 10.3389/fneur.2022.981488 -
Cureus Jun 2023Hypothalamic hamartomas are benign tumors composed of ectopic neural and glial tissue. They have a low prevalence and are usually associated with central precocious...
Hypothalamic hamartomas are benign tumors composed of ectopic neural and glial tissue. They have a low prevalence and are usually associated with central precocious puberty or epilepsy with gelastic seizures. The presentation beyond childhood is rare, and the symptoms are not the same as in childhood. Here, we report the case of a woman who presented with secondary amenorrhea and headaches revealing a giant hamartoma of the tuber cinereum (TC). The hormonal assessment showed moderate hyperprolactinemia. Synacthen testing was normal. Magnetic resonance imaging revealed a suprasellar hamartoma on the TC measuring 20 mm with sellar extension. The optic chiasma and cavernous sinuses were clear. Hyperprolactinemia was explained by mechanical compression of the pituitary stalk. The patient started cabergoline orally (1 mg per week)with an improvement of the prolactin levels and had a natural pregnancy six months later without incident. Surgery was not indicated due to the difficult transsphenoidal access and the absence of major clinical symptoms.
PubMed: 37461751
DOI: 10.7759/cureus.40532 -
AJNR. American Journal of Neuroradiology 1991The neuroimaging studies, clinical evaluations, and surgical and pathologic findings in five children with biopsy-proved hamartomas of the tuber cinereum were reviewed....
The neuroimaging studies, clinical evaluations, and surgical and pathologic findings in five children with biopsy-proved hamartomas of the tuber cinereum were reviewed. Surgical and/or MR findings showed that patients with precocious puberty had pedunculated lesions while those with seizures had tumors that were sessile with respect to the hypothalamus. The radiologic studies included six MR examinations in four patients and CT studies in all five patients. Three children presented with precocious puberty and two with seizures, one of which was a gelastic (spasmodic or hysteric laughter) type of epilepsy. MR studies were obtained both before and after surgery in two patients, only preoperatively in a third patient, and only postoperatively in the fourth child. MR was superior to CT in displaying the exact size and anatomic location of the hamartomas in all cases. The mass was isointense with gray matter on sagittal and coronal T1-weighted images, which best displayed the relationship of the hamartoma to the third ventricle, infundibulum, and mammillary bodies. Intermediate- or T2-weighted images showed signal characteristics of the hamartoma to be isointense (one case) or hyperintense (two cases) relative to gray matter. The difference in T2 signal intensity did not correlate with any obvious differences in histopathology. CT showed attenuation isodense with gray matter, and no calcium. There was no enhancement on CT. There was no enhancement on MR in the one case in which contrast medium was administered. Preservation of the posterior pituitary bright spot was noted on all pre- and postoperative T1-weighted MR scans.(ABSTRACT TRUNCATED AT 250 WORDS)
Topics: Child; Child, Preschool; Female; Hamartoma; Humans; Hypothalamic Neoplasms; Magnetic Resonance Imaging; Male; Puberty, Precocious; Retrospective Studies; Seizures; Tomography, X-Ray Computed; Tuber Cinereum
PubMed: 1902033
DOI: No ID Found -
Children (Basel, Switzerland) Aug 2021Hypothalamic hamartoma (HH) is a rare, congenital, and benign lesion of the tuber cinereum, typically presenting with central precocious puberty (CPP), gelastic seizure,...
Hypothalamic hamartoma (HH) is a rare, congenital, and benign lesion of the tuber cinereum, typically presenting with central precocious puberty (CPP), gelastic seizure, and developmental delay. This study aimed to investigate CPP in HH patients and compare clinical features between before and after gonadotropin-releasing hormone (GnRH) agonist treatment. A total of 30 HH patients under 18 years of age who visited Severance Children's Hospital between January 2005 and May 2020 were retrospectively reviewed. Fourteen patients were male (46.7%) and sixteen (53.3%) were female, with a mean age at diagnosis was4.2 ± 2.9 years. During follow-up, 24 patients (80.0%) were diagnosed with CPP, 15 patients (50.0%) had gelastic seizure, and 13 patients (43.3%) had developmental delay. The gelastic seizure was significantly associated with sessile type HH rather than pedunculated type HH (85.7% vs. 18.8%, = 0.001). After GnRH agonist treatment, discrepancies between bone age and chronological age decreased (3.3 ± 1.3 years to 2.0 ± 1.7 years, = 0.002). Additionally, height standard deviation score for bone age was increased, and predicted adult height increased significantly in females, while males showed an increasing trend. Clinical symptoms of HH were closely associated with the location of HH, and GnRH agonist treatment was safe and effective in the management of CPP caused by HH.
PubMed: 34438602
DOI: 10.3390/children8080711