Novel insights into symptomatology of moyamoya disease in pediatric patients: survey of symptoms suggestive of orthostatic intolerance

Abstract

<jats:sec><jats:title>OBJECTIVE</jats:title><jats:p>A specific population of young patients with moyamoya disease (MMD) persistently experience physical symptoms not attributable to focal ischemia. These symptoms, highly suggestive of orthostatic intolerance (also termed “orthostatic dysregulation”), were investigated and reported as potential determinants of quality of life in young MMD patients.</jats:p></jats:sec><jats:sec><jats:title>METHODS</jats:title><jats:p>Forty-six patients (6–30 years of age) were selected from a group of 122 patients who were diagnosed with MMD before 18 years of age. The authors administered a structured questionnaire consisting of 11 items based on screening checklists published in the Japanese clinical guidelines for juvenile orthostatic dysregulation in young patients. The results were tabulated, and correlations with clinical data were explored.</jats:p></jats:sec><jats:sec><jats:title>RESULTS</jats:title><jats:p>Thirty-seven (80%) patients (mean age 15.9 years) responded to the questionnaire. Frequent headache, vertigo/dizziness on standing, fatigue, difficulty with getting out of bed, and motion sickness were the top 5 symptoms, resulting in 57% of patients being unable to attend school. Forty-three percent of the patients demonstrated multiple symptoms suggestive of orthostatic intolerance, even as long as 5 years after revascularization surgery. The number of symptoms was inversely associated with the number of years after surgery (p = 0.028). The number of symptoms was not associated with a history of surgery, clinical presentations, vascular involvement, cerebral perfusion, brain lesions, or history of transient ischemic attacks.</jats:p></jats:sec><jats:sec><jats:title>CONCLUSIONS</jats:title><jats:p>The present study provided novel insight into the symptomatology of young patients with MMD. Failure to notice nonfocal physical symptoms can significantly impair quality of life in young patients with MMD even years after successful revascularization surgery. These symptoms may serve as independent clinical markers used to assess disease outcome, although the underlying mechanisms of this disease are, as of yet, unclear.</jats:p></jats:sec>

Neurological vertigo in the emergency room in pediatric and adult age: systematic literature review and proposal for a diagnostic algorithm

“…Moreover vasculitis associated with rheumatologic disorders such as Wegener’s granulomatosis, systemic lupus erythematosus and juvenile rheumatoid arthritis could cause vertigo related to impaired vertebrobasilar circulation [ 61 ]. Indeed Moya-Moya disease, characterized by stenosis of the intracranial carotid arteries and basal collateral arteries, can be associated with vertigo on standing [ 62 ]. In these cases, a full physical examination usually helps to find out other associated neurologic deficits.…”

Assessment of Single-Barrel Superficial Temporal Artery-Middle Cerebral Artery Bypass in Treatment for Adult Patients with Ischemic-Type Moyamoya Disease

“…Previous studies reported that the combination of direct and indirect bypass is the optimal treatment in adult MMD [ 7 , 12 ]. It was reported that the annual risks of symptomatic infarction and hemorrhage were 0.2% and 0.4%, respectively, after analyzing 77 combined revascularization surgeries of 60 adult MMD patients and reported that the combined revascularization surgery resulted in satisfactory long-term improvement in clinical application, hemodynamic states, angiography, and prevention of recurrent stroke or TIA [ 13 , 14 ]. A potential mechanism is that direct revascularization could be effective at the early stage after surgery, maintaining its dominant role in collateralization, and these distal cerebral vessels progressively become partly or entirely occluded [ 15 ].…”

Aspirin use and platelet aggregation in ischemic onset-type pediatric moyamoya patients with intractable headaches (moya-ache)

“…Relevant literature includes reports of improvement of headache following revascularization (direct, indirect anastomosis). In spite of revascularization and CBF improvement, many cases of pediatric moyamoya patients are left with the challenge to improve the persisting headache [2,6,7,9]. Regrettably, a Fig.…”

“…The intractable headache can significantly reduce the quality of life (QOL) [6][7][8]. Its pathophysiological mechanisms remain unclear, although some of them such as stimulation of dural nociceptors by vasodilation of the transdural collaterals and chronic hypoxemia have been suggested [9]. In some cases, headaches are strong and associated with nausea at the time of waking up; however, they seem to be naturally alleviated around noon.…”