Brunereau L, Levy C, Laberge S, Houtteville J, Labauge P
Service de Radiologie-Adultes (Pr Rouleau), CHU Bretonneau, Tours, France.
Surg Neurol. 2000 May;53(5):475-82; discussion 482-3. doi: 10.1016/s0090-3019(00)00218-4.
To evaluate clinical and MR features of de novo lesions (DNL) in the familial form of cerebral cavernous malformation (CCM) in 40 patients belonging to 29 unrelated non-Hispanic families.
Forty patients followed up by serial cerebral MR examinations were included in this retrospective study. First and last available MR examinations were retrospectively analyzed and compared for each patient to diagnose DNL. Gradient-echo (GRE) sequences were performed in only 11 of the 40 patients and were not considered for this study. Incidence of DNL was evaluated in terms of lesions/patient-year. All DNL were characterized by their clinical and MR features (location, size, type). Type of CCM was determined according to the classification of Zabramski (1994). Patient groups with and without DNL were compared for sex, age, number of pre-existing CCMs, and follow-up.
Twenty-three DNL were recorded in 11 patients (27.5%) and the incidence was 0.2 lesions/patient-year (mean follow-up = 3.2 years). All but one DNL were asymptomatic. Twenty DNL were supratentorial and three were infratentorial. Mean diameter was 8 mm (2-35 mm). Six DNL were classified as type 1 (subacute hemorrhage), six as type 2 (hemorrhages and thromboses of varying ages) and 11 as type 3 (chronic hemorrhage with hemosiderin staining). No statistical difference between groups was found in terms of sex, age, or number of pre-existing CCMs. On the other hand, duration of follow-up was significantly longer in the group with DNL.
The occurrence of DNL seems to be a hallmark of the familial form of CCM in non-Hispanic families as well as in Hispanic families. Such DNL are usually asymptomatic and are mainly classified as type 3 (chronic hemorrhage with hemosiderin staining). Within the limits of the retrospective study design and potential selection bias introduced by the varying indications for MR scanning, it does seem that DNL may occur at any time in the lifespan of CCM patients, and occurrence does not seem to depend on age, sex, or the total number of pre-existing lesions.
评估29个非西班牙裔无关家庭中40例家族性脑海绵状畸形（CCM）患者新发病灶（DNL）的临床和磁共振（MR）特征。
本回顾性研究纳入40例接受系列脑部MR检查随访的患者。对每位患者的首次和最后一次可用MR检查进行回顾性分析和比较，以诊断DNL。40例患者中仅11例进行了梯度回波（GRE）序列检查，本研究未考虑该序列检查结果。根据病灶/患者年评估DNL的发生率。所有DNL均根据其临床和MR特征（位置、大小、类型）进行特征描述。CCM的类型根据Zabramski（1994年）的分类确定。比较有和无DNL的患者组的性别、年龄、既往CCM数量和随访情况。
11例患者（27.5%）记录到23个DNL，发生率为0.2个病灶/患者年（平均随访时间 = 3.2年）。除1个DNL外，其余均无症状。20个DNL位于幕上，3个位于幕下。平均直径为8mm（2 - 35mm）。6个DNL被分类为1型（亚急性出血），6个为2型（不同时期的出血和血栓形成），11个为3型（伴有含铁血黄素沉着的慢性出血）。在性别、年龄或既往CCM数量方面，两组之间未发现统计学差异。另一方面，有DNL的组随访时间明显更长。
DNL的出现似乎是非西班牙裔家庭以及西班牙裔家庭中家族性CCM的一个标志。此类DNL通常无症状，主要分类为3型（伴有含铁血黄素沉着的慢性出血）。在回顾性研究设计的局限性以及MR扫描不同适应证所带来的潜在选择偏倚范围内，似乎DNL可能在CCM患者的任何年龄段出现，且其发生似乎不取决于年龄、性别或既往病灶总数。
