Moyamoya angiopathy – Is there a Western phenotype?

Hever, Pennylouise; Alamri, Alexander; Tolias, Christos M.

doi:10.3109/02688697.2015.1096902

Cited by 30 publications

(35 citation statements)

References 62 publications

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“…There are not many systematic, long-term follow-up studies of MMA patients, making it difficult to reliably inform the patient and their families about prognosis, develop prognostic scores, estimate the best preventive approaches, or suggest more aggressive treatments in patients who are expected to have a poor outcome. Furthermore, the characteristics of MMA disease seem to be different in various patient populations with different ethnical backgrounds [11]. The major studies reporting long-term outcomes of MMA patients have included 1146 [12], 104 [13], and 101 [14] patients with 5.2–9 years, 29–46 months, and 26.5 months of follow-up time reported.…”

Section: Discussionmentioning

confidence: 99%

Moyamoya angiopathy: long-term follow-up study in a Finnish population

et al. 2018

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Background and purpose Moyamoya angiopathy (MMA) is a chronic cerebrovascular disorder predominantly starting in childhood or early adulthood and thus affects the whole lifetime. Little is known on MMAs long-term outcomes in European patients. We report long-term follow-up data on Finnish MMA patients. Methods We included patients from our Helsinki University Hospital MMA database and arranged long-term follow-up visits for all the patients. This follow-up included a review of the medical records accumulated in due time, detailed neurological and neuropsychological evaluation, and outcome measures modified Rankin Scale (mRS) and Barthel Index (BI). Results There were 61 MMA patients with a mean follow-up period of 9.5 years (SD 6.7 years; range 1.3–35.4 years; 581 patient-years). Only two patients had died and two-thirds ( n = 40, 65.6%) had no new events during the follow-up period. Eight patients (13.1%) had an ischemic and five patients (8.2%) a hemorrhagic stroke during the follow-up. There were no differences between operated ( n = 26) and conservatively ( n = 35) treated groups regarding recurrent events or the outcome measured with mRS or BI. Finnish MMA patients reported significantly poorer physical and psychological health aspects of QOL when compared to the general Finnish population. Symptoms of low mood were found in 27 (56%) patients. Conclusions Finnish MMA patients have a benign and stable course with a ~3.5 % annual stroke risk. We found no differences in the clinical outcomes between the operated and conservative groups, however, the psychosocial well-being requires more attention in MMA patients.

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Section: Discussionmentioning

confidence: 99%

Moyamoya angiopathy: long-term follow-up study in a Finnish population

et al. 2018

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“…The later onset of disease as well as the greater female predominance, postulated to be typical of the Western phenotype, are in line with our results. 10…”

Section: Discussionmentioning

confidence: 99%

Moyamoya vasculopathy – Patient demographics and characteristics in the Finnish population

Saarela

Mustanoja

Pekkola

et al. 2016

International Journal of Stroke

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Background and purpose Moyamoya vasculopathy, a rare steno-occlusive progressive cerebrovascular disorder, has not been thoroughly studied in Caucasian populations. We established a registry of Finnish patients treated at the Helsinki University Hospital, to collect and report demographic and clinical data. Methods We collected data both retrospectively and prospectively from all the patients with a moyamoya vasculopathy referred to our hospital between January 1987 and December 2014. All patients underwent a neurological outpatient clinic visit. Results We diagnosed 61 patients (50 females, 10 children) with moyamoya vasculopathy. The mean age at the disease-onset was 31.5 ± 17.9 years. The two most common presenting symptoms were ischemic stroke (n = 31) and hemorrhage (n = 8). Forty-four percent underwent revascularization surgery, and 70% were prescribed antithrombotic treatment. Conclusions The results support in part the Western phenotype of the disease considering the later presentation and larger female predominance compared to the Asian moyamoya vasculopathy reports. However, the proportion of ischemic strokes and hemorrhagic strokes is closer to Japanese population than German population. The absence of familial cases points to a different genetic profile in the Finnish patients.

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“…The clinical presentations of MMD include transient ischemic attacks (TIAs), ischemic stroke, hemorrhagic stroke, seizures, headache, and cognitive impairment [11]. Few published studies support the theory of a distinct western phenotype in MMD, with female preponderance, lower rates of hemorrhages in adults, and lower evidence of familial occurrence [12]. TIAs in MMD are of paroxysmal onset and typically resolve within 24 hours whereas MS exacerbations tend mainly to evolve subacutely over days and to resolve over days-to-weeks [13].…”

Section: Discussionmentioning

confidence: 99%

Moyamoya Disease May Mimic Multiple Sclerosis?

Spanou

Evangelopoulos

Velonakis

et al. 2019

Case Reports in Neurological Medicine

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Introduction. A wide range of medical conditions may mimic multiple sclerosis. Among them, cerebrovascular diseases, including moyamoya disease, need to be excluded since they share common clinical features and radiographic findings with multiple sclerosis. Case Report. A 44-year-old woman experienced transient numbness of her right sided face and arm and was referred to our unit due to small brain lesions in magnetic resonance imaging, with a possible diagnosis of multiple sclerosis. Neurological examination was unremarkable except for plantar reflexes and jerky deep tendon reflexes. Brain magnetic resonance angiography revealed findings typically seen in moyamoya disease, confirmed with digital subtraction angiography. Antiplatelet therapy started, but few days later, she developed suddenly global aphasia and right hemiparesis (National Institutes of Health Stroke Scale/NIHSS 6). Brain magnetic resonance imaging revealed acute infarct in the distribution of the left middle cerebral artery. At her discharge, she was significantly improved (NIHSS 3). Conclusion. Diagnosis of multiple sclerosis is often challenging. In particular, in young patients with transient neurological symptoms and atypical white matter lesions in magnetic resonance imaging, cerebrovascular disorders such as moyamoya disease should be considered in the differential diagnosis. Detailed clinical and neuroimaging evaluation are mandatory for the correct diagnosis.

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Moyamoya angiopathy – Is there a Western phenotype?

Cited by 30 publications

References 62 publications

Moyamoya angiopathy: long-term follow-up study in a Finnish population

Moyamoya angiopathy: long-term follow-up study in a Finnish population

Moyamoya vasculopathy – Patient demographics and characteristics in the Finnish population

Moyamoya Disease May Mimic Multiple Sclerosis?

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