Epileptic Syndromes Related to the Hypothalamic Hamartomas

Febrile Infection-Related Epilepsy Syndrome (FIRES) is a unique catastrophic epilepsy syndrome, and the development of drug-resistant epilepsy (DRE) is inevitable. Recently, anakinra, an interleukin-1 receptor antagonist (IL-1RA), has been increasingly used to treat DRE due to its potent anticonvulsant activity. We here summarized its effects in 38 patients (32 patients with FIRES and six with DRE). Of the 22 patients with FIRES, 16 (73%) had at least short-term seizure control 1 week after starting anakinra, while the remaining six suspected anakinra-refractory cases were male and had poor prognoses. Due to the small sample size, an explanation for anakinra refractoriness was not evident. In all DRE patients, seizures disappeared or improved, and cognitive function improved in five of the six patients following treatment. Patients showed no serious side effects, although drug reactions with eosinophilia and systemic symptoms, cytopenia, and infections were observed. Thus, anakinra has led to a marked improvement in some cases, and functional deficiency of IL-1RA was indicated, supporting a direct mechanism for its therapeutic effect. This review first discusses the effectiveness of anakinra for intractable epileptic syndromes. Anakinra could become a new tool for intractable epilepsy treatment. However, it does not currently have a solid evidence base.

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Hypothalamic Hamartomas: A Comprehensive Review of Literature – Part 3: Updates on Radiotherapy Management

Clinical Neurology and Neurosurgery ◽

10.1016/j.clineuro.2020.106077 ◽

2020 ◽

Vol 197 ◽

pp. 106077

Author(s):

Safwan O. Alomari ◽

Mohammed N. El Houshiemy ◽

Shadi Bsat ◽

Charbel K. Moussalem ◽

Mohammed Allouh ◽

...

Keyword(s):

Review Of Literature ◽

Comprehensive Review ◽

Hypothalamic Hamartomas

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Drop Attacks and Epileptic Syndromes

Psychiatry and Clinical Neurosciences ◽

10.1111/j.1440-1819.1993.tb02053.x ◽

1993 ◽

Vol 47 (2) ◽

pp. 211-216 ◽

Cited By ~ 1

Author(s):

Katsuyuki Fukushima ◽

Tateki Fujiwara ◽

Kazuichi Yagi ◽

Masakazu Seino

Keyword(s):

Drop Attacks ◽

Epileptic Syndromes

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Optimized SEEG-guided radiofrequency thermocoagulation in the treatment of pediatric hypothalamic hamartomas

Seizure ◽

10.1016/j.seizure.2021.01.023 ◽

2021 ◽

Vol 86 ◽

pp. 102-108

Author(s):

Min Wang ◽

Yi Zhang ◽

Ping Xue ◽

Yuanfeng Zhou ◽

Wei Shi ◽

...

Keyword(s):

Hypothalamic Hamartomas ◽

Radiofrequency Thermocoagulation

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Autonomic and hormonal ictal changes in gelastic seizures from hypothalamic hamartomas

Electroencephalography and Clinical Neurophysiology ◽

10.1016/s0013-4694(98)00074-1 ◽

1998 ◽

Vol 107 (5) ◽

pp. 317-322 ◽

Cited By ~ 25

Author(s):

A Cerullo ◽

P Tinuper ◽

F Provini ◽

M Contin ◽

A Rosati ◽

...

Keyword(s):

Gelastic Seizures ◽

Hypothalamic Hamartomas

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Surgical management of hypothalamic hamartomas in patients with gelastic epilepsy

Neurosurgical FOCUS ◽

10.3171/foc/2008/25/9/e8 ◽

2008 ◽

Vol 25 (3) ◽

pp. E8 ◽

Cited By ~ 16

Author(s):

Bassam Addas ◽

Elisabeth M. S. Sherman ◽

Walter J. Hader

Keyword(s):

Quality Of Life ◽

Epileptic Encephalopathy ◽

Clinical Syndrome ◽

Seizure Freedom ◽

Interstitial Radiotherapy ◽

Hypothalamic Hamartomas ◽

Surgical Options ◽

Gelastic Epilepsy ◽

Refractory Seizures

Gelastic epilepsy (GE) associated with hypothalamic hamartomas (HHs) is now a well-characterized clinical syndrome consisting of gelastic seizures starting in infancy, medically refractory seizures with or without the development of multiple seizure types, and behavioral and cognitive decline. It has been postulated that the development of the HH-GE syndrome is a result of a progressive epileptic encephalopathy or secondary epileptogenesis, which is potentially reversible with treatment of the HH. A variety of surgical options for the treatment of HHs exist, including open and endoscopic procedures, radiosurgery, interstitial radiotherapy, and stereotactic radiofrequency thermocoagulation. Surgical treatment can result in seizure freedom in up to 50% of patients and can be accompanied by significant improvements in behavior, cognition, and quality of life. Partial treatment of HHs may be sufficient to reduce seizure frequency and improve behavior and quality of life with less risk. A component of reversible cognitive dysfunction may be present in some patients with an HH-GE syndrome.

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Practice parameter: Temporal lobe and localized neocortical resections for epilepsy

Neurology ◽

10.1212/01.wnl.0000055086.35806.2d ◽

2003 ◽

Vol 60 (4) ◽

pp. 538-547 ◽

Cited By ~ 513

Author(s):

J. Engel ◽

S. Wiebe ◽

J. French ◽

M. Sperling ◽

P. Williamson ◽

...

Keyword(s):

Temporal Lobe ◽

Controlled Trial ◽

Mesial Temporal Lobe Epilepsy ◽

Class I ◽

Partial Seizures ◽

Complex Partial Seizures ◽

Single Class ◽

Intention To Treat ◽

Epileptic Syndromes ◽

Class Iv

Objectives/Methods: To examine evidence for effectiveness of anteromesial temporal lobe and localized neocortical resections for disabling complex partial seizures by systematic review and analysis of the literature since 1990.Results: One intention-to-treat Class I randomized, controlled trial of surgery for mesial temporal lobe epilepsy found that 58% of patients randomized to be evaluated for surgical therapy (64% of those who received surgery) were free of disabling seizures and 10 to 15% were unimproved at the end of 1 year, compared with 8% free of disabling seizures in the group randomized to continued medical therapy. There was a significant improvement in quantitative quality-of-life scores and a trend toward better social function at the end of 1 year for patients in the surgical group, no surgical mortality, and infrequent morbidity. Twenty-four Class IV series of temporal lobe resections yielded essentially identical results. There are similar Class IV results for localized neocortical resections; no Class I or II studies are available.Conclusions: A single Class I study and 24 Class IV studies indicate that the benefits of anteromesial temporal lobe resection for disabling complex partial seizures is greater than continued treatment with antiepileptic drugs, and the risks are at least comparable. For patients who are compromised by such seizures, referral to an epilepsy surgery center should be strongly considered. Further studies are needed to determine if neocortical seizures benefit from surgery, and whether early surgical intervention should be the treatment of choice for certain surgically remediable epileptic syndromes.

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