Cognitive deficits in children with gelastic seizures and hypothalamic hamartoma

Frattali, Carol; Liow, Kore; Craig, G. H.; Korenman, Lisa M.; Makhlouf, F.; Satô, Susumu; Biesecker, LG; Theodore, William H.

doi:10.1212/wnl.57.1.43

Cited by 95 publications

(85 citation statements)

References 15 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…10,23,24,43,70 Tassinari and colleagues 106 published an extensive review of patients with gelastic seizures in which they reported on 60 patients with gelastic seizures without the presence of HH, suggesting that ictal laughter is not necessarily pathognomonic for HH. In fact, gelastic seizures may also be present in frontal and temporal lobe epilepsies.…”

Section: Epileptic Syndromesmentioning

confidence: 99%

“…2 In addition to delinquency and aggressive outbursts, a slowly progressive decline in cognitive functioning has also been frequently observed in patients with HH-related gelastic seizures. 6,10,14,43,78 The decline of behavior and cognitive function typically parallels the exacerbation of the seizure disorder. Quiske et al 91 published the first in-depth report on cognitive performance in adult and juvenile patients with gelastic seizures and HH, in which they found cognitive impairments in more than half of their patients.…”

Section: Behavioral and Cognitive Disordersmentioning

confidence: 99%

See 1 more Smart Citation

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

Mittal

Montes

et al. 2013

FOC

View full text Add to dashboard Cite

Hypothalamic hamartomas are uncommon but well-recognized developmental malformations that are classically associated with gelastic seizures and other refractory seizure types. The clinical course is often progressive and, in addition to the catastrophic epileptic syndrome, patients commonly exhibit debilitating cognitive, behavioral, and psychiatric disturbances. Over the past decade, investigators have gained considerable knowledge into the pathobiological and neurophysiological properties of these rare lesions. In this review, the authors examine the causes and molecular biology of hypothalamic hamartomas as well as the principal clinical features, neuroimaging findings, and electrophysiological characteristics. The diverse surgical modalities and strategies used to manage these difficult lesions are outlined in the second article of this 2-part review.

show abstract

Section: Epileptic Syndromesmentioning

confidence: 99%

Section: Behavioral and Cognitive Disordersmentioning

confidence: 99%

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

Mittal

Montes

et al. 2013

FOC

View full text Add to dashboard Cite

show abstract

“…4,51,55) Developmental delays correlate with the severity, frequency, and variety of the seizure patterns. 8,25,55) Of all reported patients with HH, 49% manifested cognitive disturbance and 31% had behavioral problems; all of them had seizures. 51) The behavioral problems have been described as restlessness, violence including severe rage attacks, emotional instability, obsession, autism, and anti-social behavior.…”

Section: Cognitive Dysfunction and Behavioral Problemsmentioning

confidence: 99%

“…26) Recurrent excitatory damage produced by the spreading of seizure activity from the HH through the mammillothalamic pathway and associated brain area may cause the progressive cognitive deterioration in patients with HH. 25) …”

Section: Underlying Pathophysiology Of Symptoms I Seizuresmentioning

confidence: 99%

Hypothalamic Hamartoma

Arita

Kurisu

Kiura

et al. 2005

Neurol. Med. Chir.(Tokyo)

View full text Add to dashboard Cite

The incidence of hypothalamic hamartomas (HHs) has increased since the introduction of magnetic resonance (MR) imaging. The etiology of this anomaly and the pathogenesis of its peculiar symptoms remain unclear, but recent electrophysiological, neuroimaging, and clinical studies have yielded important data. Categorizing HHs by the degree of hypothalamic involvement has contributed to the accurate prediction of their prognosis and to improved treatment strategies. Rather than undergoing corticectomy, HH patients with medically intractable seizures are now treated with surgery that targets the HH per se, e.g. HH removal, disconnection from the hypothalamus, stereotactic irradiation, and radiofrequency lesioning. Although surgical intervention carries risks, total eradication or disconnection of the lesion leads to cessation or reduction of seizures and improves the cognitive and behavioral status of these patients. Precocious puberty in HH patients is safely controlled by long-acting gonadotropin-releasing hormone agonists. The accumulation of knowledge regarding the pathogenesis of symptoms and the development of safe, effective treatment modalities may lead to earlier intervention in young HH patients and prevent the decline in their cognitive abilities and quality of life. This review of hypothalamic hamartomas presents current classifications, pathophysiologies, and treatment modalities.

show abstract

“…Owing to significant, but partial, efficacy 25 patients (62.5 %) were treated twice. The preoperative cognitive deficits, behavioral disturbances, and investigated relationship of seizure severity and anatomical type to cognitive abilities were characterized [71,72]. Low peripheral doses were used because of the close relationship with optic pathways and hypothalamus (median 17 Gy; range 13-26 Gy).…”

Section: Epilepsymentioning

confidence: 99%

Gamma Knife for Functional Diseases

Régis

2014

Neurotherapeutics

View full text Add to dashboard Cite

Cognitive deficits in children with gelastic seizures and hypothalamic hamartoma

Cited by 95 publications

References 15 publications

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

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

Hypothalamic Hamartoma

Gamma Knife for Functional Diseases

Contact Info

Product

Resources

About