Electroencephalographic features of moyamoya in adults

Fréchette, Éric; Bell-Stephens, Teresa; Fisher, Robert S.

doi:10.1016/j.clinph.2014.06.033

Cited by 12 publications

(14 citation statements)

References 29 publications

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“…Hyperthermia increases the metabolic demand and cerebral oxygen requirements, which results in an ischemic event if the stenotic vasculature is unable to compensate. Patients with MMD are at an increased risk of developing seizure-like activity due to a number of factors that can produce cortical irritation [25].…”

Section: Clinical Featuresmentioning

confidence: 99%

Moyamoya: An Update and Review

Berry¹,

Cortez²,

Toor³

et al. 2020

Cureus

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This article is a clinical review of Moyamoya disease (MMD) and Moyamoya syndrome (MMS). We review the incidence, epidemiology, pathology, historical context, clinical and radiographic findings, diagnostic imaging modalities, radiographic grading systems, the effectiveness of medical, interventional, and surgical treatment, and some of the nuances of surgical treatment options. This article will help pediatricians, neurologists, neurosurgeons, and other clinical practitioners who are involved in caring for patients with this rare clinical entity. MMD is an intrinsic primary disease process that causes bilateral progressive stenosis of the anterior intracranial circulation with the involvement of the proximal portions of the intracranial internal carotid artery (ICA) extending to involve the proximal portions of the anterior cerebral artery (ACA) and middle cerebral artery (MCA); posterior circulation involvement is very rare. This causes a compensatory response where large numbers of smaller vessels such as the lenticulostriate arteries begin to enlarge and proliferate, which gives the angiographic appearance of a "Puff of Smoke", which is translated into Japanese as "Moyamoya”. MMS is a secondary process that occurs in response to another underlying pathological process that causes stenosis of intracranial blood vessels, such as radiation. For example, an external source of radiation causes stenosis of the ICA with a compensatory response of smaller blood vessels, which then enlarge and proliferate in response and has the same "Puff of Smoke" appearance on the diagnostic cerebral angiogram (DCA). Histological findings include an irregular internal elastic lamina with luminal narrowing, hyperplasia of the tunica media, and intimal thickening with vacuolar degeneration in smooth muscle cells in the tunica media. Compensation for diminishing blood supply occurs through angiogenesis, which causes the proliferation and enlargement of smaller collateral blood vessels to increase blood supply to under-perfused areas of the brain. MMD is rare in the United States, with just 0.086 newly diagnosed cases per 100,000 individuals per year, which is approximately one per million new cases annually. Risk factors for MMD include Eastern Asian ancestry and predisposing conditions such as neurofibromatosis and Down's syndrome. Clinically, patients often present with stroke signs and symptoms from cerebral ischemia. The proliferation of collateral blood vessels within the basal ganglia can produce movement disorders. Catheter-based DCA is the current gold standard for obtaining a diagnosis. CT perfusion allows preoperative identification of ischemic vascular territories, which may be amenable to surgical intervention. MRI enables rapid detection of acute ischemic stroke using diffusion-weighted Imaging (DWI) and apparent diffusion coefficient (ADC) sequences to assess for any diffusion restriction. Non-contrast CT of the head is used to rule out acute hemorrhage in the presentation...

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Section: Clinical Featuresmentioning

confidence: 99%

Moyamoya: An Update and Review

Berry¹,

Cortez²,

Toor³

et al. 2020

Cureus

View full text Add to dashboard Cite

show abstract

“…Ischemic stroke generated a 1.62-fold risk of seizure compared with those without an ischemic stroke. In adults with MMD, Frechette et al identified that ischemic stroke was associated with a 2.1-fold relative risk of seizure [8]. Epilepsy in MMD is one of the recognized types of post-stroke epilepsy.…”

Section: Discussionmentioning

confidence: 99%

“…Epileptiform discharge was present in 10 (66.7%) cases. Frechette et al found that 90% of EEGs were abnormal in adults with MMD, with commonly focal (78%), diffusely slow (68%), or epileptiform discharge (24%) [8].…”

Section: Discussionmentioning

confidence: 99%

“…In view of the abnormal pathology of this progressive cerebral vasculopathy, it is not difficult to understand the recurrent and stereotyped symptoms in MMD, such as transient ischemic attack (TIA), ischemic, or hemorrhagic stroke [ 1 – 3 ]. Seizure is another prominent transient event in MMD, with a prevalence of 11–18% in pediatric population [ 5 , 6 ] and 18–24% in adults [ 7 , 8 ]. Therefore, diagnostic and therapeutic dilemma arises from these transient neurological events.…”

Section: Introductionmentioning

confidence: 99%

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Electroencephalographic features in pediatric patients with moyamoya disease in China

et al. 2020

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Background: Moyamoya disease (MMD) is a relatively important and common disease, especially in East Asian children. There are few reports about EEG in children with MMD in China till now. This study is aimed to analyze the electroencephalographic features of MMD in pediatric patients in China preliminarily. Methods: Pediatric patients with MMD who were hospitalized in Peking University International Hospital and Beijing Tiantan Hospital from January 2016 to December 2018 were collected. Clinical and electroencephalography (EEG) findings were analyzed retrospectively. Results: A total of 110 pediatric patients with MMD were involved, and 17 (15.5%) cases had a history of seizure or epilepsy. Ischemic stroke was associated with a 1.62-fold relative risk of seizure. A subset of 15 patients with complete EEG data was identified. Indications for EEG in patients with MMD included limb shaking, unilateral weakness, or generalized convulsion. Abnormal EEG was seen in 14 (93.3%) cases, with the most common findings being focal slowing 12 (80.0%), followed by epileptiform discharge 10 (66.7%), and diffuse slowing 9 (60.0%). "Rebuild up" phenomenon on EEG was observed in one patient. Conclusions: Seizure and abnormal background activity or epileptiform discharge on EEG were common in pediatric patients with MMD. EEG may play a role in differential diagnosis among the transient neurological events in MMD such as transient ischemic attack and seizure.

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“…1 In contrast, a recent large-scaled study reported that electrographic seizures were observed in 12 of 103 patients with adult moyamoya after cerebral revascularization. 12 Since we did not evaluate the status of cerebral vasculatures, the possibility of moyamoya syndrome as an etiology of de novo partial onset seizures could not be ruled out.…”

Section: Discussionmentioning

confidence: 99%

A Case of Hyperventilation Syndrome Mimicking Complex Partial Seizure: Usefulness of EEG Monitoring in Emergency Department

Kang¹

2015

J Epilepsy Res

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Acute hyperventilation syndrome not only can be clinically misdiagnosed as epileptic seizures, but also complex partial seizures may involve hyperventilation as a part of aura. Although electrography (EEG) monitoring is one of the most important procedure to differentiate these conditions, it could not be widely used in emergency department. Variety forms of epileptic attack, mainly idiopathic generalized epilepsy, are provoked by voluntary hyperventilation. In contrast, it is not clear whether hyperventilation can activate the partial seizures. We reported a case of acute hyperventilation syndrome (HSV) mimicking first onset complex partial seizure, impending non-convulsive status epilepticus, which was diagnosed by EEG in the emergency department. The electrographic seizure was provoked again by voluntary hyperventilation after clinical improvement.

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Electroencephalographic features of moyamoya in adults

Cited by 12 publications

References 29 publications

Moyamoya: An Update and Review

Moyamoya: An Update and Review

Electroencephalographic features in pediatric patients with moyamoya disease in China

A Case of Hyperventilation Syndrome Mimicking Complex Partial Seizure: Usefulness of EEG Monitoring in Emergency Department

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