Aicardi syndrome: epilepsy surgery as a palliative treatment option for selected patients and pathological findings

Podkorytova, Irina; Gupta, Ajay; Moosa, Ahsan N.V.; Bingaman, William; Prayson, Richard A.; Pestana-Knight, Elia M.

doi:10.1684/epd.2016.0872

Cited by 5 publications

(3 citation statements)

References 14 publications

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“…There have been cases of introducing a ketogenic diet [22,23]. The benefits of introducing this diet have been demonstrated in randomized clinical trials, as well as in retrospective and prospective studies [24,25,26]. Vagus nerve stimulation (VNS) is also one of the therapeutic methods, which improved seizure control among a group of patients [23].…”

Section: Discussionmentioning

confidence: 99%

Aicardi syndrome – case report and literature review

Ochman-Pasierbek,

Olczyk,

Paprocka

et al. 2024

Ann. Acad. Med. Siles.

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Zespół Aicardiego (<i>Aicardi syndrome</i> – AS) to rzadkie zaburzenie neurorozwojowe, w zdecydowanej większości przypadków występujące u dziewcząt. Zazwyczaj charakteryzuje się klasyczną triadą objawów: napadami zgięciowymi, agenezją ciała modzelowatego i chorioretinalnymi ubytkami w siatkówce. Należy również podkreślić, że padaczka lekooporna jest często głównym objawem klinicznym AS. Ponadto u pacjentów stwierdza się niepełnosprawność intelektualną, zmiany w obrębie narządu wzroku, twarzoczaszki, a także inne zaburzenia neurorozwojowe. W pracy przedstawiono przypadek pacjentki z AS z lekooporną padaczką. Pacjentka jest leczona od 3 miesiąca życia, jednak kolejne modyfikacje farmakoterapii nie doprowadziły do ustąpienia napadów. Istnieje potrzeba prowadzenia dalszych badań, aby właściwie odnieść się do kwestii skutecznego leczenia AS.

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Section: Discussionmentioning

confidence: 99%

Aicardi syndrome – case report and literature review

Ochman-Pasierbek,

Olczyk,

Paprocka

et al. 2024

Ann. Acad. Med. Siles.

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“…In this study, we applied subtotal hemispherotomy to an AS patient with intractable ES. Subtotal hemispherectomy is effective in patients with ES with absent or mild hemiparesis ( 4 , 5 ). In one study, ten of seventeen non-AS patients who underwent subtotal hemispherectomy became seizure-free ( 4 ).…”

Section: Discussionmentioning

confidence: 99%

Case Report: Subtotal Hemispherotomy Modulates the Epileptic Spasms in Aicardi Syndrome

et al. 2021

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The mechanism of epileptic spasms (ES) in Aicardi syndrome (AS) remains obscure. We compared intraoperative high-frequency oscillations (HFOs) and phase-amplitude coupling (PAC) before and after subtotal hemispherotomy in a 3-month-old girl with drug-resistant ES secondary to AS. Fetal ultrasonography showing corpus callosum agenesis, bilateral ventricular dilatation, and a large choroid plexus cyst confirmed AS diagnosis. Her ES started when she was 1 month old and had ten series of clustered ES per day despite phenobarbital and vitamin B6 treatment. After subtotal hemispherotomy, her ES dramatically improved. We analyzed two intraoperative electrocorticography modalities: (1), occurrence rate (OR) of HFOs; (2), PAC of HFOs and slow wave bands in the frontal, central, and parietal areas. We hypothesized that HFOs and PAC could be the biomarkers for efficacy of subtotal hemispherotomy in AS with ES. PAC in all three areas and OR of HFOs in the frontal and parietal areas significantly decreased, while OR of HFOs in the central area remained unchanged after subtotal hemispherotomy. We have demonstrated the usefulness of evaluating intraoperative HFOs and PAC to assess subtotal hemispherotomy effectiveness in AS patients with ES. Disconnecting the thalamocortical and subcortical pathways in the epileptic network plays a role in controlling ES generation.

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“…The traditionally reported treatments for epilepsy associated with Aicardi syndrome are antiseizure drugs, but occasionally palliative surgery is attempted with mixed success. 2 -5…”

mentioning

confidence: 99%

Ketogenic Diet Therapy for Epilepsy Associated With Aicardi Syndrome

et al. 2021

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Introduction: Aicardi syndrome is a rare neurodevelopmental disorder associated with epilepsy in females. Ketogenic diet therapy represents a possible nonpharmacologic treatment in Aicardi syndrome patients. Methods: All patients with Aicardi syndrome seen at Johns Hopkins Hospital (Baltimore, MD) and Johns Hopkins All Children’s Hospital (St Petersburg, FL) treated with ketogenic diet therapy since 1994 were evaluated retrospectively. Results: Fifteen patients, ages 4 months to 34 years, were identified. Ten (67%) patients experienced a ≥50% seizure reduction after 3 months, with 3 (20%) having a ≥90% reduction. Only 1 patient was seizure-free for a short period of time. The number of drugs tried prior to ketogenic diet therapy initiation was correlated with ≥50% seizure reduction at 3 months, 5.8 vs 2.6 in responders versus nonresponders ( P = .01). In addition, the mean number of drugs actively received also correlated, 3.0 vs 1.2, P = .005. Ketogenic diet therapy was slightly more successful in those without infantile spasms, 78% vs 50%, P = .33. Conclusion: Ketogenic diet therapy was helpful in Aicardi syndrome, although seizure freedom was rare. It was especially helpful for those who were more drug-resistant and did not have infantile spasms at ketogenic diet therapy onset.

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Aicardi syndrome: epilepsy surgery as a palliative treatment option for selected patients and pathological findings

Cited by 5 publications

References 14 publications

Aicardi syndrome – case report and literature review

Aicardi syndrome – case report and literature review

Case Report: Subtotal Hemispherotomy Modulates the Epileptic Spasms in Aicardi Syndrome

Ketogenic Diet Therapy for Epilepsy Associated With Aicardi Syndrome

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