Histopathology of Moyamoya angiopathy in a European patient.

BACKGROUND AND PURPOSE: Moyamoya angiopathy (MMA) is rare outside Asia. Little is known about pathophysiology in European patients. This study aims to elucidate the histopathology of non-Asian MMA and its similarities and differences to those cases from Asia. METHODS: Here, we present a 57-year-old European woman with MMA and describe the post-mortem examination results of the brain and cerebral arteries. RESULTS: Histopathological findings in cerebral blood vessels were identical to those found in Asians. This included thickening and undulation of the internal elastic lamina as well as fibrocellular thickening and proliferation of smooth muscle cells of the intima in the distal segments of the internal carotid arteries and in proximal and middle segments of the anterior and middle cerebral arteries. Collateral vessels showed fragmented internal elastic lamina and thinning of the media with isolated microaneurysms. CONCLUSIONS: Histopathological changes found in this European patient are identical with those described in Asians. Despite suspected different genetic triggers and unknown pathophysiological cascades, MMA in Europeans seems to result in a common final pathway compared with the disease in Asians.

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

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.

Moyamoya angiopathy: early postoperative course within 3 months after STA-MCA-bypass surgery in Europe-a retrospective analysis of 64 procedures.

BACKGROUND: Despite the consensus on the necessity of revascularizing surgery in Moyamoya angiopathy in Asia, the indication in Caucasian Moyamoya patients is discussed controversially. OBJECTIVE: The safety of revascularizing surgery in Europe should be clarified. METHODS: This study retrospectively analyzed the rate of complications as well as clinical symptoms within the first 3 months after bypass surgery between superficial temporal artery and middle cerebral artery (STA-MCA). RESULTS: 64 direct bypass procedures in 45 patients (95.5% Caucasians) were analyzed. The magnetic resonance imaging at day 6 showed subdural hematoma in 60.3%. The mean diameter of these hematomas on magnetic resonance imaging was 5.1 mm (SD 3.4 mm) and increased in 25% at follow-up. No difference was found between those patients with early (day 1) or late (day 7) restarts of antiplatelet therapy. Magnetic resonance imaging at day 6 revealed hyperperfusion syndrome after six of 64 procedures (9.3%). Three of these six had clinical symptoms; two-thirds were transient within seconds. Magnetic resonance imaging depicted stroke after seven procedures (10.9%). Five of these seven patients had no new symptoms. Altogether, after ten procedures (15%), patients complained about clinical symptoms. These were all transient. No new transient ischemic attacks occurred during the 3 month follow-up and no new lesions were detected in magnetic resonance imaging. Only two patients underwent surgery for asymptomatic subdural hematoma. All other subdural hematomas resolved spontaneously. CONCLUSION: Revasculating surgery is a safe procedure in Caucasian patients with Moyamoya angiopathy. The observed complications have a good prognosis.

Efficacy of STA-MCA bypass surgery in moyamoya angiopathy: long-term follow-up of the Caucasian Krupp Hospital cohort with 81 procedures.

BACKGROUND: Despite the consensus on the efficacy of revascularizing surgery in moyamoya angiopathy (MA) in Asia, the indication in Caucasian moyamoya patients is controversially discussed. OBJECTIVE: The efficacy of revascularizing surgery in adult European patients with MA should be clarified. METHODS: This study retrospectively analyzed the rate of further strokes and hemorrhages as well as MRI and Duplex ultrasound features during long-term follow up after STA-MCA bypass. RESULTS: Eighty-one STA-MCA bypass procedures in 54 patients with MA operated in one single German institution were analyzed. All 54 patients (100%) were Caucasians. After two diffusion restricted spots in MRI perioperatively (2.5%) and short-lasting symptoms directly after surgery, no patient experienced further new symptoms related to stroke or hemorrhages nor no new gliotic scars or microbleeds on MRI for 38.2 months. Duplex ultrasound 3 months after surgery documented bypass patency in 100% and sonographic sign for good relevance of the bypass in 96.2%. In addition, the diameter of the donor vessel had increased in 89.9% as an indicator for the relevance of the bypass. Semi-quantitative analysis of perfusion changes in the operated hemispheres demonstrated an increase in perfusion in the MCA territory in 56 of 74 (75.7%) hemispheres 36.7 months after surgery. In MRA images, a reduction of typical moyamoya collaterals was found in 65 of 79 hemispheres (82.3%) after a mean of 37.2 months. CONCLUSION: Direct STA-MCA bypass is an effective therapy in Caucasian patients with hemodynamically compromised MA.

Differential diagnosis between cerebral ischemia, focal seizures and limb shaking TIAs in moyamoya disease.

We describe the clinical course of a young female Caucasian patient with bilateral moyamoya disease in whom we could diagnose the simultaneous occurrence of cerebral ischemia, TIAs, limb shaking TIAs and focal Jacksonian seizures. It is the second clinical communication in the literature elaborating limb shaking TIAs in moyamoya disease.