title: Movement symptoms in European Moyamoya angiopathy - First systematic questionnaire study.
abstract: OBJECTIVE
  Movement disorders are a rare manifestation of Moyamoya angiopathy (MMA). Data on prevalence and clinical presentation are warranted. Possible involuntary movements include focal motor seizures, tremor, limb-shaking transient ischemic attacks, choreiform and spastic or dystonic movement disorders.
  
  
  METHODS
  We developed a questionnaire to systematically assess movement disorders in MMA. Patients' history of involuntary movements and their clinical presentation were assessed systematically by interview. Additionally, demographic data were assessed as well as localization of movements, possible trigger factors and the presence of other symptoms.
  
  
  RESULTS
  The questionnaire was administered to 63 European patients with MMA. The response rate was high with 93.6% participating patients. Twenty-eight patients (47.4%) reported involuntary movement disorders including periodic tremor, irregular jerks, involuntary movements with loopy or pranced character, stiffness and muscle cramps. From those patients, 16 (57.1%) individuals had the symptoms prior to the diagnosis of MMA. The most common involuntary movements were irregular jerks witnessed by 17 (60.7%) patients, followed by stiffness and muscle cramps in 10 (35.7%). Eight (28.6%) Patients suffered from unintended loopy and pranced character, while 4 individuals (14.3%) remembered periodic tremor. Of the 28 patients who witnessed movement disorders, 23 had undergone revascularization surgery (82.1%). From the latter subgroup, movement disorders were reversed in 7 out of 12 patients (58.3%) with irregular jerks and 4 out of 7 patients (57.1%) with unintended loopy and pranced character.
  
  
  CONCLUSIONS
  Our study elucidates the high incidence of movement disorders in an unselected consecutively recruited cohort of European MMA patients.
authors.affiliation: Department of Neurology, Alfried-Krupp-von Bohlen und Halbach Hospital, Alfried-Krupp-Straße 21, 45130 Essen, Germany. Electronic address: markus.kraemer1977@gmx.de.
authors.forename: Markus
authors.initials: M
authors.lastname: Kraemer
authors.affiliation: Department of Neurosurgery, Alfried-Krupp-von Bohlen und Halbach Hospital, Alfried-Krupp-Straße 21, 45130 Essen, Germany.
authors.forename: Leonidas
authors.initials: L
authors.lastname: Trakolis
authors.affiliation: Department of Neurology, Alfried-Krupp-von Bohlen und Halbach Hospital, Alfried-Krupp-Straße 21, 45130 Essen, Germany; Department of Neurology, St. Josef-Krankenhaus Essen-Kupferdreh, Heidbergweg 22-24, 45257 Essen, Germany.
authors.forename: Jens
authors.initials: J
authors.lastname: Platzen
authors.affiliation: Department of Neurology, Alfried-Krupp-von Bohlen und Halbach Hospital, Alfried-Krupp-Straße 21, 45130 Essen, Germany.
authors.forename: Jan Claudius
authors.initials: JC
authors.lastname: Schwitalla
authors.affiliation: Cerebrovascular Disease Unit, IRCCS Foundation C. Besta, Neurological Institute, Milan, Italy.
authors.forename: Anna
authors.initials: A
authors.lastname: Bersano
authors.affiliation: Department of Neurology, Medical Faculty, Heinrich Heine University Düsseldorf, Germany.
authors.forename: Philipp
authors.initials: P
authors.lastname: Albrecht
authors.affiliation: Department of Neuroradiology, University Hospital Giessen, 35385 Giessen, Germany.
authors.forename: Marc
authors.initials: M
authors.lastname: Schlamann
authors.affiliation: Department of Neurology, Alfried-Krupp-von Bohlen und Halbach Hospital, Alfried-Krupp-Straße 21, 45130 Essen, Germany.
authors.forename: Peter
authors.initials: P
authors.lastname: Berlit
mesh_terms: D000328:Adult; D016021:Epidemiologic Studies; D005260:Female; D005858:Germany / Q000453:epidemiology; D006801:Humans; D008297:Male; D008875:Middle Aged; D009069:Movement Disorders / Q000175:diagnosis / Q000453:epidemiology / Q000209:etiology*; D009072:Moyamoya Disease / Q000150:complications* / Q000175:diagnosis / Q000453:epidemiology; D011795:Surveys and Questionnaires
keywords: Choreiform and spastic or dystonic movement disorders; Focal motor seizures; Limb-shaking transient ischemic attacks; Movement symptoms; Moyamoya angiopathy; Tremor