Hypothalamic hamartomas causing gelastic epilepsy: Two cases and a review of the literature

Georgakoulias, Nikolaos; Vize, C. E.; Jenkins, Alistair; Singounas, E. G.

doi:10.1016/s1059-1311(98)80075-7

Cited by 20 publications

(12 citation statements)

References 10 publications

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“…1 The lesions occur as round or ovoid masses that may be sessile, where they are embedded within or attached to the hypothalamus along a large interface, or pedunculated, where they are attached to the hypothalamus via a stalk. 2,3 The sessile type tends to be associated with medically refractory epilepsy, whereas the pedunculated type tends to be associated with precocious puberty responsive to gonadotropin releasing hormone (GnRH) agonist treatment. The lesions consist of mature neurons, resembling those of the normal tuber cinereum, intermingled with glial cells and occasionally dysplastic neurons.…”

Section: Discussionmentioning

confidence: 99%

“…7,8 Gelastic seizures secondary to HH are difficult to control with antiepileptic drugs alone, as are other HHrelated seizure types, including focal, tonic, and atonic seizures. 2 The fact that better postoperative seizure control correlates with shorter preoperative duration of epilepsy, 9 highlights the importance and timeliness of surgical treatment in well-selected patients. Surgery is also indicated for severe behavioral problems, notably rage attacks that can occur with HH.…”

Section: Discussionmentioning

confidence: 99%

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Laser interstitial thermal therapy: A first line treatment for seizures due to hypothalamic hamartoma?

Gandhi

Rekate

et al. 2017

Epilepsia

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Successful treatment of hypothalamic hamartoma (HH) can result in the resolution of its sequelae including epilepsy and rage attacks. Risks and morbidity of open surgical management of this lesion have motivated the development of laser interstitial thermal therapy (LITT) as a less invasive treatment approach to the disease. Although overall morbidity and risk would appear to be lower, complications related to LITT therapy have been reported, and the longer-term follow-up that is now possible after initial experience helps address the question of whether LITT provides equivalent efficacy compared to other treatment options. We conducted a retrospective analysis of clinical outcomes in eight patients undergoing LITT for HH at our center using the Visualase/Medtronic device. Five patients had refractory epilepsy, one had rage attacks, and two had both. We also compared the published seizure-free outcomes over time and the complication rates for different interventional approaches to the treatment of epilepsy due to HH including open craniotomy, neuroendoscopic, radiosurgical, and radiofrequency approaches. With a mean follow-up of 19.1 months in our series of eight patients, six of seven epilepsy patients achieved seizure freedom, whereas the one patient with rage attacks only did not have improvement of his symptoms. A length of hospital stay of 2.6 days reflects low morbidity and rapid postoperative recuperation with LITT. Considering other reported series and case reports, the overall published seizure freedom rate of 21 of 25 patients is superior to published outcomes of HH cases treated by stereotactic radiosurgery (SRS), craniotomy, or neuroendoscopy, and comparable to radiofrequency ablation. The cumulative experience of our center with other published series supports relatively lower operative morbidity than more invasive approaches and efficacy that is as good or better than open craniotomy procedures and SRS. Although morbidity appears to be lower than other open approaches, complications related to LITT and their avoidance should be considered carefully.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Laser interstitial thermal therapy: A first line treatment for seizures due to hypothalamic hamartoma?

Gandhi

Rekate

et al. 2017

Epilepsia

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“…We could not find any patients with frank GS in the context of frontal or temporal lobe epilepsy, without associated HH. HH is usually sporadic, but in 5% of cases can be associated with the Pallister-Hall syndrome (Georgakoulias, Vize, Jenkins, & Singounas, 1998;Scheffer et al, 2016). The initial presentation in the sporadic cases usually occurs in the first months of life as GS.…”

Section: Discussionmentioning

confidence: 99%

Hypothalamic hamartomas in adulthood: Clinical spectrum and treatment outcome—A unicenter experience

et al. 2019

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IntroductionClinical manifestations of the hypothalamic hamartoma‐epilepsy syndrome (HH‐ES) in adulthood are variable. Efficacy of therapeutic options and outcome are diverse.MethodsRetrospective study of adult patients diagnosed with a HH in magnetic resonance imaging and epilepsy who attended our tertiary Epilepsy Unit between 2003 and 2018. We report the clinical and electroencephalographic features of a series of adult patients with HH and related epilepsy seen in our center together with the treatments and seizure outcome.ResultsWe describe a series of eight patients. Five males (62.5%), median age at evaluation was 28.5 years (IQR: 15.5). Clinical manifestations included focal with preserved and impaired awareness emotional seizures (gelastic seizures [GS]) in six patients (75%), focal tonic, atonic with impaired awareness and focal to bilateral tonic–clonic seizures. Mild GS were the only symptom in one patient. Three patients (37.5%) had endocrinological disturbances such as obesity and hypothyroidism. Fifty percent of the patients showed psychiatric comorbidity such as anxiety disorder and aggressiveness, and two patients had psychomotor delay. Seven patients (87.7%) had drug‐resistant seizures and three of them were treated with radiosurgery. Out of the treated group, only one (33.3%) became seizure‐free 2 years after surgery but developed psychiatric problems. The other two patients had an Engel IV outcome and received a vagal nerve stimulation (VNS) implant. VNS did not lead to changes either in intensity nor in seizure frequency.ConclusionsHypothalamic hamartoma‐epilepsy syndrome clinical manifestations in adult patients are as variable as at pediatric age. Outcome of therapeutic options such as radiosurgery or VNS may be poorer at this stage.

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“…The “laughing tic” may, therefore, be regarded as a form of PL, since it satisfies this set of criteria; however, it is clinically distinct from other neuropathological conditions, discussed below, from which PL classically derives. PL is a clinical phenomenon presenting amidst a range of neuropsychiatric conditions,18 including epilepsy (gelastic seizures),19–21 cerebrovascular disease ( fou rire prodromique ),22–25 brain tumour,26–29 demyelinating disease,30, 31 and OCD 32, 33…”

Section: Discussionmentioning

confidence: 99%

Pathological laughter in Gilles de la Tourette syndrome: An unusual phonic tic

et al. 2010

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Patients with Gilles de la Tourette syndrome (GTS) can display socially inappropriate behaviors as part of their multiform tic phenomenology. Pathological laughter (PL), defined as the presence of episodic and contextually inappropriate outbursts of laughter, has been detailed as a symptom of various psychiatric and neurological conditions. We present a case series of eight subjects diagnosed with GTS who reported PL as part of their tic repertoire. All subjects experienced PL as a simple phonic tic, accompanied by characteristic premonitory urges and significant impairment in social interactions. In addition, all patients presented with multiple tic-related symptoms (mainly self-injurious behaviors and echolalia, n = 7; palilalia, n = 6; coprolalia/mental coprolalia, n = 5), and six patients had comorbid conditions (in particular obsessive-compulsive disorder/behaviors, n = 7; attention-deficit hyperactivity disorder, n = 4). We suggest that the pathophysiological mechanisms underlying the expression of PL as a tic could involve a dissociation between frontostriatal and limbic networks.

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Hypothalamic hamartomas causing gelastic epilepsy: Two cases and a review of the literature

Cited by 20 publications

References 10 publications

Laser interstitial thermal therapy: A first line treatment for seizures due to hypothalamic hamartoma?

Laser interstitial thermal therapy: A first line treatment for seizures due to hypothalamic hamartoma?

Hypothalamic hamartomas in adulthood: Clinical spectrum and treatment outcome—A unicenter experience

Pathological laughter in Gilles de la Tourette syndrome: An unusual phonic tic

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