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Open Access 30 Oct 2025Original Article
EnglishSpanish
Epilepsy in Patients With Moyamoya Angiopathy
Mario Bautista-Lacambra 1,2,3,*, Vanesa Garayoa-Irigoyen 1,2,4, Luisa-Fernanda Tique-Rojas 1, María Seral-Moral 5, Jesús Moles-Herbera 6, Amparo López-Lafuente 7, Rosario Barrena-Caballo 8, Carlos Tejero-Juste 9, Marta Palacín-Larroy 1,2,3,10, Herbert Tejada-Meza 1,2,3,8,10
Affiliations
Article Info

1 Servicio de Neurología, Hospital Universitario Miguel Servet, 50009 Zaragoza, España

2 Sección de Neurovascular, Servicio de Neurología, Hospital Universitario Miguel Servet, 50009 Zaragoza, España

3 Grupo de Investigación en Neurociencias, Instituto de Investigación Sanitaria (IIS) Aragón, 50009 Zaragoza, España

4 Departamento de Medicina, Dermatología y Psiquiatría de la Universidad de Zaragoza, 50009 Zaragoza, España

5 Unidad de Epilepsia Fármaco-Resistente, Hospital Universitario Miguel Servet, 50009 Zaragoza, España

6 Servicio de Neurología, Hospital Universitario San Jorge, 22004 Huesca, España

7 Servicio de Neurocirugía, Hospital Universitario Miguel Servet, 50009 Zaragoza, España

8 Sección de Neuropediatría y Neurometabolismo, Servicio de Pediatría del Hospital Universitario Miguel Servet, 50009 Zaragoza, España

9 Unidad de Neurorradiología Intervencionista del Servicio de Radiología del Hospital Universitario Miguel Servet, 50009 Zaragoza, España

10 Servicio De Neurología, Hospital Clínico Universitario Lozano Blesa, 50009 Zaragoza, España

*Correspondence: mariobautistalacambra@gmail.com (Mario Bautista-Lacambra)

Abstract

Introduction:

Moyamoya angiopathy is a cerebrovascular disease characterized by progressive stenosis of the intracranial internal carotid arteries. There is limited literature addressing epilepsy in this condition, especially in Western countries.

Methodology:

This was a retrospective study conducted in the public hospitals of Aragón, analyzing data from all patients diagnosed with moyamoya angiopathy between 1981 and 2024. Epidemiological aspects of the disease were studied, as well as the presence of epilepsy and its management in this group of patients.
Results:

A total of 26 patients were included, with an estimated prevalence in Aragón of 1.71 cases per 100,000 inhabitants. The mean age at diagnosis was 36.64 years, with an equal sex distribution. Half of the patients presented with moyamoya syndrome. Fifty percent of the patients experienced a seizure and 42.31% of the total met diagnostic criteria for epilepsy. Most seizures were focal (81.8%), with a predominance of frontal lobe semiology. Levetiracetam was the most commonly used treatment. Up to four of the twelve patients with epilepsy met diagnostic criteria for drug-resistant epilepsy.
Conclusions:

Although the prevalence of moyamoya in our series was lower than in Asian populations, the prevalence of epilepsy was significantly higher (50% of patients with seizures vs 0.9–18.9% in Asian series). In our cohort, epilepsy in moyamoya angiopathy was associated with the occurrence of syncope, cognitive impairment, affective disorders, and an earlier age at diagnosis. There are no other studies available addressing the percentage of drug-resistant epilepsy in these patients.

Keywords

  • moyamoya disease
  • moyamoya syndrome
  • moyamoya angiopathy
  • epilepsy
  • seizures
  • drugresistant epilepsy
  • treatment

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