Departments of Neurology (I.R., S.V., E.A.K., P.C.d.K., E.S.v.E., G.M.T., M.J.H.W.), Leiden University Medical Center, the Netherlands.
Biomedical Data Sciences (E.v.Z.), Leiden University Medical Center, the Netherlands.
Stroke. 2021 May;52(5):1851-1855. doi: 10.1161/STROKEAHA.120.033286. Epub 2021 Apr 5.
Cortical calcifications have been reported in patients with cerebral amyloid angiopathy (CAA), although their prevalence and pathophysiology are unknown. We investigated the frequency of calcifications on computed tomography, their association with intracerebral hemorrhage (ICH) and their coexistence with a striped pattern of the occipital cortex reflecting microcalcifications on ultra-high-field 7T-magnetic resonance imaging in Dutch-type hereditary CAA (D-CAA) and sporadic CAA.
We included D-CAA mutation carriers with a proven APP (amyloid precursor protein) mutation or ≥1 lobar ICH and ≥1 first-degree relative with D-CAA and sporadic CAA patients with probable CAA according to the modified Boston criteria. D-CAA carriers were regarded symptomatic when they had a history of symptomatic ICH. We assessed the presence, location, and progression of calcifications and their association with ICH and the striped occipital cortex.
We found cortical calcifications in 15/81 (19% [95% CI, 11–29]) D-CAA mutation carriers (15/69 symptomatic and 0/12 presymptomatic) and in 1/59 (2% [95% CI, 0–9]) sporadic CAA patients. Calcifications were all bilateral located in the occipital lobes. In 3/15 (20%) of the symptomatic D-CAA patients the calcifications progressed over a period up to 10 years. There was evidence of an association between cortical calcifications and new ICH development (hazard ratio, 7.1 [95% CI, 0.9–54.9], log-rank P=0.03). In 7/25 D-CAA symptomatic carriers in whom a 7T-magnetic resonance imaging was performed, a striped pattern of the occipital cortex was present; in 3/3 (100%) of those with calcifications on computed tomography and 4/22 (18%) of those without calcifications.
Occipital cortical calcifications are frequent in D-CAA but seem to be rare in sporadic CAA. Their absence in presymptomatic carriers and their association with ICH might suggest that they are a marker for advanced CAA. Cortical calcifications on computed tomography seem to be associated with the striped occipital cortex on 7T-magnetic resonance imaging which may possibly represent an early stage of calcification.
脑淀粉样血管病（CAA）患者中已有皮质钙化的报道，但其发生率和发病机制尚不清楚。我们研究了 CT 上钙化的频率，其与脑出血（ICH）的关系，以及在荷兰型遗传性 CAA（D-CAA）和散发性 CAA 中超高场 7T 磁共振成像（MRI）上条纹状枕叶皮层（反映微钙化）的共存情况。
我们纳入了 D-CAA 突变携带者，其具有已证实的 APP（淀粉样前体蛋白）突变或≥1 个脑叶 ICH，以及≥1 个一级亲属患有 D-CAA 和散发性 CAA，根据改良的波士顿标准，这些患者被认为患有可能的 CAA。当 D-CAA 携带者有症状性 ICH 病史时，我们将其视为有症状。我们评估了钙化的存在、位置和进展情况，以及它们与 ICH 和条纹状枕叶皮层的关系。
我们发现 15/81（19%[95%可信区间，11%29%]）名 D-CAA 突变携带者（15/69 名有症状和 0/12 名无症状）和 1/59（2%[95%可信区间，0%9%]）名散发性 CAA 患者存在皮质钙化。钙化均为双侧位于枕叶。在 15 名有症状的 D-CAA 患者中，有 3 名（20%）的钙化在 10 年的时间内进展。皮质钙化与新 ICH 发生之间存在关联（风险比，7.1[95%可信区间，0.9~54.9]，对数秩检验 P=0.03）。在进行了 7T-MRI 检查的 25 名有症状的 D-CAA 携带者中，有 7 名（28%）出现了枕叶皮层的条纹状模式；在 CT 上有钙化的 3 名（100%）和没有钙化的 22 名（18%）中均存在这种情况。
D-CAA 中枕叶皮质钙化很常见，但在散发性 CAA 中似乎很少见。无症状携带者中钙化的缺失以及与 ICH 的关联表明，它们可能是 CAA 进展的标志。CT 上的皮质钙化似乎与 7T-MRI 上的条纹状枕叶皮层有关，这可能代表了钙化的早期阶段。
