Clinical presentation of Moyamoya angiopathy in Europeans: experiences from Germany with 200 patients

  • Markus KraemerEmail author
  • Jan Claudius Schwitalla
  • Frank Diesner
  • Orhan Aktas
  • Hans-Peter Hartung
  • Peter Berlit
Original Communication



Moyamoya angiopathy (MMA) is a rare vasopathy, especially among European Caucasians. Data about demographics, clinical presentation, comorbid conditions, radiological findings as well as laboratory and cerebral spinal fluid (CSF) data are sparse.


Patients with MMA treated in the Alfried Krupp Hospital, Essen, Germany, between 2010 and 2017 with focus on demographic, clinical, radiological and laboratory as well as CSF data were evaluated retrospectively. Patients with non-Caucasian family background were excluded from this study.


Altogether 200 European Caucasian patients with MMA were identified. There was a female predominance of 3.2:1. The mean age at first presentation was 32.9 years and the mean age of diagnosis was 36.0 years. Eleven of 194 index patients (5.7%) showed a familial presentation. In 11.6% posterior cerebral artery was additionally involved, in 4% additionally cerebral aneurysm and in 2.5% dysgenesis of corpus callosum was found. Most patients suffered from transient ischemic attacks (71.5%) and stroke (82%). Cerebral hemorrhage was found in 9.5%. Livedo racemosa was an associated symptom in 12.8% of patients and thyroid diseases were found in 23.8%.


Compared with Asian data, cerebral hemorrhages are infrequent and female predominance is accentuated among European Caucasians. Some former unknown rare features like associated livedo racemosa, dysgenesis of corpus callosum and associated syncope have been discovered systematically for the first time in this huge European Caucasian cohort.


Moyamoya angiopathy Europeans Clinical presentation Demographics Moyamoya Caucasians Europe 



There was no funding.

Compliance with ethical standards

Conflicts of interest

Markus Kraemer received research grants from Novartis and Merck Serono and travel/accommodation/meeting expenses or lecture honoraria from Bayer Schering, Biogen Idec, Merck Serono, Novartis, Teva and Shire Germany. Jan Claudius Schwitalla has no conflict of interest. Frank Diesner has no conflict of interest. Hans-Peter Hartung received, with approval of the Rector of Heinrich-Heine-University and the CEO of University of Düsseldorf Hospital honoraria for consulting, serving on steering committees and speaking from Biogen, CSL Behring, Geneuro, Genzyme, LFB, Medimmune, Merck, Novartis, Octapharma, Opexa, Receptos/Celgene, Roche, Sanofi, and Teva. Orhan Aktas received, with approval of the Rector of Heinrich-Heine-University, grants from the German Research Foundation (DFG), the German Ministry for Education and Research (BMBF) as part of the German Competence Network Multiple Sclerosis (KKNMS; for NEMOS NationNMO-PAT FKZ 01GI1602B), the Eugène Devic European Network (EU-FP7), honoraria and travel/accommodation/meeting expenses from Almirall, Bayer, Biogen, Medimmune, Merck Serono, Novartis, Roche, Sanofi-Genzyme, and Teva. Peter Berlit has no conflict of interest.

Ethical standards

All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.


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Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2019

Authors and Affiliations

  1. 1.Department of NeurologyAlfried Krupp HospitalEssenGermany
  2. 2.Department of General Zoology and NeurobiologyRuhr-University BochumBochumGermany
  3. 3.Department of NeurosurgeryAlfried Krupp HospitalEssenGermany
  4. 4.Department of Neurology, Medical FacultyHeinrich Heine UniversityDüsseldorfGermany

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