Markus Kraemer1,2, Jan Claudius Schwitalla3,4, Frank Diesner5, Orhan Aktas6, Hans-Peter Hartung6, Peter Berlit3. 1. Department of Neurology, Alfried Krupp Hospital, Alfried-Krupp-Strasse 21, 45130, Essen, Germany. markus.kraemer@krupp-krankenhaus.de. 2. Department of Neurology, Medical Faculty, Heinrich Heine University, Moorenstrasse 5, 40225, Düsseldorf, Germany. markus.kraemer@krupp-krankenhaus.de. 3. Department of Neurology, Alfried Krupp Hospital, Alfried-Krupp-Strasse 21, 45130, Essen, Germany. 4. Department of General Zoology and Neurobiology, Ruhr-University Bochum, Universitätsstrasse 150, 44780, Bochum, Germany. 5. Department of Neurosurgery, Alfried Krupp Hospital, Alfried-Krupp-Strasse 21, 45130, Essen, Germany. 6. Department of Neurology, Medical Faculty, Heinrich Heine University, Moorenstrasse 5, 40225, Düsseldorf, Germany.
Abstract
INTRODUCTION: 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. METHODS: 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. RESULTS: 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%. CONCLUSIONS: 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.
INTRODUCTION: 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. METHODS: 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. RESULTS: 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%. CONCLUSIONS: 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.
Authors: Markus Kraemer; Rusen Karakaya; Toshinori Matsushige; Jonas Graf; Philipp Albrecht; Hans-Peter Hartung; Peter Berlit; Rudolf Laumer; Frank Diesner Journal: J Neurol Date: 2018-08-28 Impact factor: 4.849
Authors: Wanyang Liu; S T M L D Senevirathna; Toshiaki Hitomi; Hatasu Kobayashi; Constantin Roder; Roman Herzig; Markus Kraemer; Maurits H J Voormolen; Pavlína Cahová; Boris Krischek; Akio Koizumi Journal: J Genet Date: 2013-12 Impact factor: 1.166
Authors: Markus Kraemer; Leonidas Trakolis; Jens Platzen; Jan Claudius Schwitalla; Anna Bersano; Philipp Albrecht; Marc Schlamann; Peter Berlit Journal: Clin Neurol Neurosurg Date: 2016-11-23 Impact factor: 1.876
Authors: Markus Kraemer; Sie-In Lee; Ilya Ayzenberg; Jan Claudius Schwitalla; Rolf R Diehl; Peter Berlit; Bert Bosche; Zaza Katsarava; Mark Obermann Journal: Cephalalgia Date: 2016-04-25 Impact factor: 6.292