Movement symptoms in European Moyamoya angiopathy - First systematic questionnaire study.

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. PATIENTS AND 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.

Evoked response amplitudes from somatosensory cortices do not determine reaction times to tactile stimuli.

Sensory events cause changes in brain activity, which underlie the perception of and behavioural responses to sensory stimuli. Evoked cortical responses are an important measure of these stimulus-evoked changes in brain activity. However, evidence on the relationship between behavioural responses and evoked responses is inconsistent. Therefore, we used magnetoencephalography to reinvestigate the relationship between evoked responses from somatosensory cortices and behavioural responses to somatosensory stimuli. We characterized modulations of somatosensory-evoked responses exerted by preceding painful and tactile conditioning stimuli (CS), and related these modulations of evoked responses to modulations of reaction times. Our results show that painful CS yield a long-lasting (> 4 s) facilitation of evoked responses, whereas tactile CS result in a shorter lasting (1-2 s) suppression of evoked responses to tactile stimuli. These contrary physiological effects were both associated with a significant shortening of reaction times. These findings indicate that the conditioning effects of painful and tactile stimuli represent essentially different modulatory mechanisms. Moreover, our results show that amplitudes of evoked responses from somatosensory cortices do not determine reaction times to tactile stimuli.