scholarly journals Association of Morphological Characteristics with Precocious Puberty and/or Gelastic Seizures in Hypothalamic Hamartoma

2003 ◽  
Vol 88 (10) ◽  
pp. 4590-4595 ◽  
Author(s):  
H. Jung ◽  
E. Neumaier Probst ◽  
B. P. Hauffa ◽  
C.-J. Partsch ◽  
O. Dammann
Keyword(s):  
Precocious Puberty ◽  
Clinical Symptoms ◽  
Third Ventricle ◽  
Morphological Characteristics ◽  
Central Precocious Puberty ◽  
Male Gender ◽  
Hypothalamic Hamartoma ◽  
Sexually Dimorphic ◽  
Gelastic Seizures ◽  
The Third

Abstract The pathogenesis of central precocious puberty (PP) and/or gelastic seizures due to a hypothalamic hamartoma (HH) is still under debate. We evaluated the association of clinical symptoms with morphology and localization of the HH in 34 patients. The majority (86.4%) of HHs in patients with isolated PP (n = 22; 68.2% females) revealed a parahypothalamic position without affecting the third ventricle (91%). Half of them were pedunculated, and 40.9% showed a diameter less than 10 mm. In contrast, 11 of 12 patients with seizures, eight of whom were male, presented with a sessile intrahypothalamic hamartoma, 10 of which distorted the third ventricle. Logistic regression analysis revealed an increased relative risk (RR) for epilepsy in males (RR, 4.3; 95% confidence interval, 0.96–19). However, combination of the risk factor gender with intrahypothalamic position (RR, 19; 1.3–285) and distortion of the third ventricle (RR, 10; 0.6–164) reduced the risk associated with male gender to 1.1. The position of a HH and involvement of the third ventricle are likely to be more predictive for clinical characteristics than size and shape. Male gender was associated with an intrahypothalamic HH and epilepsy, suggesting a sexually dimorphic developmental pattern of this heterotopic mass.

The relationship between magnetic resonance imaging findings and clinical manifestations of hypothalamic hamartoma

1999 ◽  
Vol 91 (2) ◽  
pp. 212-220 ◽  
Author(s):  
Kazunori Arita ◽  
Fusao Ikawa ◽  
Kaoru Kurisu ◽  
Masayuki Sumida ◽  
Kunyu Harada ◽  
...  
Keyword(s):  
Mental Retardation ◽  
Magnetic Resonance ◽  
Precocious Puberty ◽  
Third Ventricle ◽  
Clinical Manifestations ◽  
Hypothalamic Hamartoma ◽  
Content Type ◽  
The Third ◽  
The Relationship ◽  
Fine Print

Object. Hypothalamic hamartoma is generally diagnosed based on its magnetic resonance (MR) imaging characteristics and the patient's clinical symptoms, but the relationship between the neuroradiological findings and clinical presentation has never been fully investigated. In this retrospective study the authors sought to determine this relationship.Methods. The authors classified 11 cases of hypothalamic hamartoma into two categories based on the MR findings. Seven cases were the “parahypothalamic type,” in which the hamartoma is only attached to the floor of the third ventricle or suspended from the floor by a peduncle. Four cases were the “intrahypothalamic type,” in which the hamartoma involved or was enveloped by the hypothalamus and the tumor distorted the third ventricle.Six patients with the parahypothalamic type exhibited precocious puberty, which was controlled by a luteinizing hormone—releasing hormone analog, and one patient was asymptomatic. No seizures or mental retardation were observed in this group. All patients with the intrahypothalamic type had medically intractable seizures, and precocious puberty was seen in one. Severe mental retardation and behavioral disorders including aggressiveness were seen in two patients. The seizures were controlled in only one patient, in whom stereotactically targeted irradiation of the lesion was performed.This topology/symptom relationship was reconfirmed in a review of 61 reported cases of hamartoma, in which the MR findings were clearly described. The parahypothalamic type is generally associated with precocious puberty but is unaccompanied by seizures or developmental delay, whereas the intrahypothalamic type is generally associated with seizures. Two thirds of patients with the latter experience developmental delays, and half also exhibit precocious puberty.Conclusions. Classification of hypothalamic hamartomas into these two categories based on MR findings resulted in a clear correlation between symptoms and the subsequent clinical course.

scholarly journals Management of Central Precocious Puberty in Children with Hypothalamic Hamartoma

Children ◽  
2021 ◽  
Vol 8 (8) ◽  
pp. 711
Author(s):  
Junghwan Suh ◽  
Youngha Choi ◽  
Jun Suk Oh ◽  
Kyungchul Song ◽  
Han Saem Choi ◽  
...  
Keyword(s):  
Developmental Delay ◽  
Precocious Puberty ◽  
Gnrh Agonist ◽  
Clinical Symptoms ◽  
Benign Lesion ◽  
Standard Deviation Score ◽  
Central Precocious Puberty ◽  
Bone Age ◽  
Hypothalamic Hamartoma ◽  
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%, p = 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, p = 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.

Arachnoidal Cyst of the Third Ventricle with Precocious Puberty

1971 ◽  
Vol 64 (9) ◽  
pp. 1139-1142 ◽  
Author(s):  
AMIN A. FARIS ◽  
GEORGE F. BALE ◽  
BLAND CANNON
Keyword(s):  
Precocious Puberty ◽  
Third Ventricle ◽  
The Third ◽  
Arachnoidal Cyst

scholarly journals Central precocious puberty due to hypothalamic hamartoma in neurofibromatosis type 1

HORMONES ◽  
2016 ◽  
Vol 15 (1) ◽  
pp. 144-146 ◽  
Author(s):  
Emanuele Bartolini ◽  
Stefano Stagi ◽  
Perla Scalini ◽  
Andrea Bianchi ◽  
Antonio Ciccarone ◽  
...  
Keyword(s):  
Precocious Puberty ◽  
Neurofibromatosis Type 1 ◽  
Central Precocious Puberty ◽  
Neurofibromatosis Type ◽  
Hypothalamic Hamartoma

Central precocious puberty due to hypothalamic hamartoma in a 7-month-old infant girl

2007 ◽  
Vol 167 (5) ◽  
pp. 583-585 ◽  
Author(s):  
I. H. Rousso ◽  
M. Kourti ◽  
D. Papandreou ◽  
A. Tragiannidis ◽  
F. Athanasiadou
Keyword(s):  
Precocious Puberty ◽  
Central Precocious Puberty ◽  
Hypothalamic Hamartoma ◽  
Infant Girl

Hypothalamic Hamartoma in a Dog

1977 ◽  
Vol 14 (2) ◽  
pp. 138-145 ◽  
Author(s):  
R. W. Cook
Keyword(s):  
Cerebrospinal Fluid ◽  
Third Ventricle ◽  
Fluid Pressure ◽  
Hypothalamic Hamartoma ◽  
Tuber Cinereum ◽  
Mammillary Bodies ◽  
Spongiform Change ◽  
The Third ◽  
The Brain ◽  
Rostral Part

A 10-month-old female, Wire-haired Pointing Griffon dog had a hamartoma of the hypothalamus. Episodes of sudden flaccid collapse had increased in frequency and duration for 7 months. Cerebrospinal fluid pressure was normal. A flat, pedunculated mass, 2.5×3.0×0.9 cm, covered the brain stem between the pituitary gland and pons. Its 1.2-cm-diameter connection to the hypothalamus obliterated the mammillary bodies and extended to the tuber cinereum, distorting the hypothalamus and displacing the third ventricle which also divided the rostral part of the mass. The tissue of the hamartoma resembled gray matter with bullous cytoplasmic vacuolation of many neurons, spongiform change, gemistocytosis and microscopic foci of calcification.

Hypothalamic hamartoma simulating a suprasellar arachnoid cyst: resolution of precocious puberty following microsurgical lesion resection

2014 ◽  
Vol 14 (1) ◽  
pp. 101-107 ◽  
Author(s):  
Sunil Manjila ◽  
Timothy W. Vogel ◽  
Yunwei Chen ◽  
Mark S. Rodgers ◽  
Alan R. Cohen
Keyword(s):  
Arachnoid Cyst ◽  
Precocious Puberty ◽  
Central Precocious Puberty ◽  
Hypothalamic Hamartoma ◽  
Suprasellar Region ◽  
Cystic Changes ◽  
Long Term Follow Up ◽  
Suprasellar Arachnoid Cyst

Hypothalamic hamartomas (HHs) are rare developmental lesions arising from the inferior hypothalamus that may cause gelastic seizures and central precocious puberty. Cystic changes in HHs are rare, usually occurring in giant lesions. The authors describe an unusual case of cystic HH masquerading as a suprasellar arachnoid cyst in an 18-month-old girl presenting with precocious puberty. Microsurgical removal of the lesion led to complete resolution of the precocious puberty on long-term follow-up. This case is the first reported HH with pathological demonstration of corticotropin-releasing hormone immunostaining in the solid tumor and glial cells in the cyst wall of the lesion. The clinical and radiological characteristics of HHs are reviewed, along with the unique surgical strategies used to manage cystic lesions in the suprasellar region.

Giant solid-cystic hypothalamic hamartoma

2011 ◽  
Vol 30 (2) ◽  
pp. E7 ◽  
Author(s):  
Christian Dorfer ◽  
Gregor Kasprian ◽  
Angelika Mühlebner ◽  
Thomas Czech
Keyword(s):  
Precocious Puberty ◽  
Hypothalamic Hamartoma ◽  
Gelastic Seizures ◽  
Classification Schemes ◽  
Hypothalamic Hamartomas ◽  
Cystic Hamartoma

Hypothalamic hamartomas are rare lesions for which different classification schemes have been proposed. The authors report on an exceptionally large solid-cystic hamartoma that led to hydrocephalus, precocious puberty, and intractable gelastic seizures. They discuss potential mechanisms of the development of hypothalamic hamartomas.

scholarly journals Central precocious puberty due to hypothalamic hamartoma in neurofibromatosis type 1

HORMONES ◽  
2015 ◽  
Author(s):  
Emanuele Bartolini ◽  
Stefano Stagi ◽  
Perla Scalini ◽  
Andrea Bianchi ◽  
Antonio Ciccarone ◽  
...  
Keyword(s):  
Precocious Puberty ◽  
Neurofibromatosis Type 1 ◽  
Central Precocious Puberty ◽  
Neurofibromatosis Type ◽  
Hypothalamic Hamartoma
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