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Hypothalamic hamartoma

Report of two cases

Shunji Nishio, Shigeru Fujiwara, Yasutaka Aiko, Iwao Takeshita, and Masashi Fukui

H amartoma has been defined as a benign nodular or tumor-like mass composed of a mixture of differentiated tissues normally present in the organ in which it occurs, but in an abnormal location. 1, 15, 23 In the central nervous system (CNS), this lesion is a relatively rare occurrence and is usually encountered in proximity to the hypothalamus. 3, 11, 16, 23 Clinically, most of the published cases of hypothalamic hamartoma were associated with precocious puberty and/or epilepsy. 17, 29, 32 Although surgical treatment for these conditions has been considered

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Pantaleo Romanelli, Alexander Muacevic, and Salvatore Striano

body inferiorly ( Figs. 1 and 2 ). The hypothalamic attachment is often asymmetrical; it has been noted that even small unilateral HHs attached to one side of the hypothalamus are epileptogenic. 1 , 2 , 15 , 49 , 50 Both T2-weighted fast spin echo and proton density MR imaging studies are the best modalities for delineating the relation of the HH (typically hyperintense) to the postcommissural fornix and the mammillothalamic tracts, which are markedly hypointense compared with the HH ( Fig. 1 ). Hypothalamic hamartomas growing into the interpeduncular fossa assume

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Christian Dorfer, Gregor Kasprian, Angelika Mühlebner, and Thomas Czech

it for submission: all authors. Administrative/technical/material support: Dorfer. Acknowledgments The authors thank Drs.Walter Pobegen and Barbara Jauk (Klinikum Klagenfurt-Wörthersee) for clinical and follow-up data, and Dr. Johann Gasser (Klinikum Klagenfurt-Wörthersee) for providing the MR images. References 1 Arita K , Ikawa F , Kurisu K , Sumida M , Harada K , Uozumi T , : The relationship between magnetic resonance imaging findings and clinical manifestations of hypothalamic hamartoma . J Neurosurg 91 : 212 – 220 , 1999 2

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Scott D. Wait, Adib A. Abla, Brendan D. Killory, Peter Nakaji, and Harold L. Rekate

the child grows. 21 , 23 Furthermore, children with these lesions may develop problems with social interaction, including frequent rage attacks. In contrast, Delalande Type I HH can cause central precocious puberty and may be treated medically with excellent results. 4 This type of HH seldom manifests with epilepsy or behavioral problems, and microsurgical excision tends to be curative. 5 , 14 The Barrow Hypothalamic Hamartoma Program was established late in 2003. Thus far, we have treated many patients with HH. 1 , 3 , 5–10 , 13 , 15–21 , 23 , 25 The goal of

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Yoshikazu Kyuma, Ei Kato, Kenichi Sekido, and Takeo Kuwabara

P recocious puberty of cerebral origin is induced by premature secretion of the hypothalamic gonadotropin-releasing factor in organic brain disease. This disease has been reported much less frequently than idiopathic precocious puberty or pseudoprecocious puberty associated with tumors or gland diseases. Hypothalamic hamartoma is a rare condition. It is a tumor-like neural malformation attached to the base of the brain in the region of the tuber cinereum and mamillary bodies. It extends into the interpeduncular cistern with a very short pedicle. Twenty

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Jeffrey V. Rosenfeld

itself and rapidly spreading to the cortex. 5 , 13 , 16 There are multiple possible routes by which seizures spread from the HH into adjacent and remote parts of the brain. One pathway is that the seizures spread directly through the mammillary body to the thalamus and beyond via the mammillothalamic tracts, 13 so that disconnection of the HH from the mammillary body has become one of the key goals of the disconnection surgery. Sessile HHs are attached to one or both mammillary bodies. 12 Hypothalamic hamartoma is a model for human subcortical epilepsy, secondary

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Bassam Addas, Elisabeth M. S. Sherman, and Walter J. Hader

importantly, their association with gelastic seizures has led to the belief that not only the seizures themselves but the accompanying encephalopathy may be reversible with surgical treatment of the HH. The treatment of HHs through a variety of surgical approaches 1 , 3 , 9 , 13 , 19 , 21 , 22 , 27 , 31 , 35 , 36 , 38 , 41 , 49 , 50 , 54 has demonstrated that seizures can cease and improvement in behavior and cognitive dysfunction is possible, supporting the concept that HHs may be associated with an element of reversible encephalopathy. Hypothalamic hamartomas are a

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Joong-Uhn Choi, Kook-Hee Yang, Tae-Gon Kim, Jong Hee Chang, Jin Woo Chang, Byung-In Lee, and Dong-Seok Kim

endoscopic procedures. In three cases we observed postoperative disconnection-like syndrome including mental dullness, verbal anosmia, unilateral tactile anomia, unilateral constructional apraxia, and lack of somesthetic transfer. This disconnection-like syndrome continued 3 to 7 days after endoscopic disconnection but spontaneously disappeared without neurological deficits. Their families reported a dramatic improvement in behavior in the 2nd postoperative month in all patients. Discussion Hypothalamic hamartomas are rare nonneoplastic malformations that

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Lucy O'Connor, Thomas Curl-Roper, Nicola Reeves, Andras A. Kemeny, and Vivek A. Josan

Hypothalamic hamartomas are intrinsically epileptogenic; they classically present with gelastic seizures that begin in infancy, which often prove refractory to medical management. 6 , 8 , 10 , 14 , 17 Progression of these high-frequency seizures is invariably severe; they spread rapidly through the cortex, generating multiple seizure types and, eventually, secondary epileptogenesis. If untreated, this “relentlessly deteriorating clinical course” results in an epileptic encephalopathy that causes cognitive decline, mental impairment, and psychiatric morbidity. 8 , 10 , 17

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Yoshua Esquenazi, David I. Sandberg, and Harold L. Rekate

H ypothalamic hamartomas are congenital, nonneoplastic lesions formed by heterotopic gray matter, neurons, glial cells, and fiber bundles. These lesions are found in the hypothalamus attached to the tuber cinereum or more commonly to one or both mammillary bodies. Hypothalamic hamartoma is a rare condition, with a prevalence estimated at 1 in 50,000–100,000. 28 Children with HHs may present with central precocious puberty, gelastic seizures, a combination of these, or may be asymptomatic. Hypothalamic hamartoma is the only etiology of central precocious