Hypothalamic hamartomas. Part 1. Clinical, neuroimaging, and neurophysiological characteristics

Mittal, Sandeep; Mittal, Monika; Montes, José L.; Farmer, Jean Pierre; Andermann, Frédérick

doi:10.3171/2013.3.focus1355

Cited by 61 publications

(64 citation statements)

References 100 publications

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“…The clinical, electrophysiological, and neuroimaging characteristics of HHs have been outlined in Part I of this 2-part review. 41 Hypothalamic hamartomas. Part 2.…”

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confidence: 99%

Hypothalamic hamartomas. Part 2. Surgical considerations and outcome

Mittal

Montes

et al. 2013

FOC

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Hypothalamic hamartomas are uncommon developmental heterotopic masses composed of an intermixed array of neurons, glia, and myelinated fibers closely associated with the mammillary bodies. Gelastic seizures, the hallmark feature of hypothalamic hamartomas, commonly present in early childhood. However, patients usually also display a disabling clinical syndrome, which may include various other types of refractory seizures with secondary generalization together with progressive cognitive, behavioral, and psychiatric dysfunction. The hamartoma itself has been unequivocally shown to be intrinsically epileptogenic. Over the past 2 decades there has been considerable effort to develop neurosurgical techniques to treat the epileptic syndrome effectively as well as to improve the neurocognitive and behavioral outcome.

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“…The clinical, electrophysiological, and neuroimaging characteristics of HHs have been outlined in Part I of this 2-part review. 41 Hypothalamic hamartomas. Part 2.…”

mentioning

confidence: 99%

Hypothalamic hamartomas. Part 2. Surgical considerations and outcome

Mittal

Montes

et al. 2013

FOC

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“…6,13 They have long been associated with gelastic and other seizure types. 13 There are many methods of surgical treatment for these tumors.…”

Section: Discussionmentioning

confidence: 99%

“…13 Multiple surgical options are available if the seizures are refractory to medical management, 14 though damage to the hypothalamus and seizure recurrence rates are significant concerns with open surgery 17 or stereotactic radiosurgery (SRS). 20 Less invasive means of ablation or disconnection, including SRS or radiofrequency thermal coagulation, offer less potential damage to the hypothalamus but suffer from the disadvantages of a prolonged time to treatment effect and lack of direct feedback during ablation, respectively.…”

Section: Neurosurg Focus 41 (4):e8 2016mentioning

confidence: 99%

Magnetic resonance imaging–guided laser interstitial thermal therapy for previously treated hypothalamic hamartomas

Burrows¹,

Marsh²,

Worrell

et al. 2016

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OBJECTIVE Hypothalamic hamartomas (HHs) are associated with gelastic seizures and the development of medically refractory epilepsy. Magnetic resonance imaging–guided laser interstitial thermal therapy (MRg-LITT) is a minimally invasive ablative treatment that may have applicability for these deep-seated lesions. Here, the authors describe 3 patients with refractory HHs who they treated with MRg-LITT. METHODS An institutional review board–approved prospective database of patients undergoing Visualase MRg-LITT was retrospectively reviewed. Demographic and historical medical data, including seizure and medication histories, previous surgeries, procedural details, and surgical complications, along with radiological interpretation of the HHs, were recorded. The primary outcome was seizure freedom, and secondary outcomes included medication reduction, seizure frequency, operative morbidity, and clinical outcome at the latest follow-up. RESULTS All 3 patients in the multi-institutional database had developed gelastic seizures related to HH at the ages of 7, 7, and 9 years. They presented for further treatment at 25, 28, and 48 years of age, after previous treatments with stereotactic radiosurgery in all cases and partial hamartoma resection in one case. One ablation was complicated by a small tract hemorrhage, which was stable on postoperative imaging. One patient developed hyponatremia and experienced weight gain, which were respectively managed with fluid restriction and counseling. At the most recent follow-up at a mean of 21 months (range 1–32 months), one patient was seizure free while another had meaningful seizure reduction. Medication was reduced in one case. CONCLUSIONS Adults with gelastic seizures despite previous treatments can undergo MRg-LITT with reasonable safety and efficacy. This novel therapy may provide a minimally invasive alternative for primary and recurrent HH as the technique is refined.

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“…127,128 They are classically associated with gelastic seizures, precocious puberty, and developmental delay, although there is considerable variability in their presentation. 129 Hamartomas can develop along the floor of the third ventricle or within the hypothalamus proper or be pedunculated, often with attachments to the mammillary bodies or tuber cinereum. 129,130 On MRI, they do not strictly follow gray matter signal intensity, and most are mildly hyperintense on T2 (probably due to glial tissue) and iso-to hypointense on T1-weighted sequences ( Figure 21).…”

Section: Hypothalamic Hamartomamentioning

confidence: 99%

“…129 Hamartomas can develop along the floor of the third ventricle or within the hypothalamus proper or be pedunculated, often with attachments to the mammillary bodies or tuber cinereum. 129,130 On MRI, they do not strictly follow gray matter signal intensity, and most are mildly hyperintense on T2 (probably due to glial tissue) and iso-to hypointense on T1-weighted sequences ( Figure 21). [128][129][130] Like normal brain parenchyma, hamartomas have a functional blood-brain barrier and should not show contrast enhancement.…”

Section: Hypothalamic Hamartomamentioning

confidence: 99%

Sellar and Parasellar Imaging

Zamora

Castillo

2016

NEUROSURGERY

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The skull base is a complex anatomical region that harbors many important neurovascular structures in a relatively confined space. The pathology that can develop at this site is varied, and many disease processes may present with similar clinical and neuroimaging findings. While computed tomography maintains a role in the evaluation of many entities and can, for instance, delineate osseous erosion with great detail and characterize calcified tumor matrices, magnetic resonance imaging (MRI) is the mainstay in the neuroimaging assessment of most pathology occurring at the skull base. Various MRI sequences have proven to be robust tools for tissue characterization and can provide information on the presence of lipids, paramagnetic and diamagnetic elements, and tumor cellularity, among others. In addition, currently available MRI techniques are able to generate high spatial resolution images that allow visualization of cranial nerves and their involvement by adjacent pathology. The information obtained from such examinations may aid in the distinction of these disease processes and in the accurate delineation of their extent prior to biopsy or treatment planning.

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Hypothalamic hamartomas. Part 1. Clinical, neuroimaging, and neurophysiological characteristics

Cited by 61 publications

References 100 publications

Hypothalamic hamartomas. Part 2. Surgical considerations and outcome

Hypothalamic hamartomas. Part 2. Surgical considerations and outcome

Magnetic resonance imaging–guided laser interstitial thermal therapy for previously treated hypothalamic hamartomas

Sellar and Parasellar Imaging

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