Di Costanzo Alfonso, Di Salle Francesco, Santoro Lucio, Tessitore Alessandro, Bonavita Vincenzo, Tedeschi Gioacchino
Department of Neurological Sciences, Second University of Naples, Piazza L. Miraglia, 2, 80138 Naples, Italy.
J Neurol. 2002 Sep;249(9):1175-82. doi: 10.1007/s00415-002-0796-z.
We reviewed the brain MRI of 66 patients with the adult form of myotonic dystrophy type 1 (DM1) to evaluate the extent and significance of white matter involvement and to look for a pattern of MRI abnormalities suggestive of DM1. White matter lesions (WMLs) and large Virchow Robin spaces (VRSs) were rated by semiquantitative methods and the signal intensity of white matter superior and posterior to the trigones (WMPST) by reference to standard images. Disease duration was correlated positively with WML and negatively with VRS scores. Patients were divided into four groups according to increasing severity of WMPST involvement: group A with mild WMPST hyperintensity, group B with large VRSs and mild or moderate WMPST hyperintensity, group C with moderate WMPST hyperintensity or mild WMPST hyperintensity with small WMLs, group D with severe WMPST hyperintensity or moderate WMPST hyperintensity with small WMLs. Disease duration, muscular impairment, lobar WMLs and brain atrophy significantly increased from groups A and B (not significantly different) to C and from C to D, while convexity VRSs significantly decreased from group B to C and from C to D. Lobar white matter involvement in DM1 seems progressive during the disease and may be characterized initially by large VRSs or mild WMPST hyperintensity, then by small WMLs or moderate WMPST hyperintensity, and finally by more extensive and confluent WMLs or diffuse white matter hyperintensity and by brain atrophy.
我们回顾了66例成年型1型强直性肌营养不良（DM1）患者的脑部磁共振成像（MRI），以评估白质受累的程度和意义，并寻找提示DM1的MRI异常模式。采用半定量方法对白质病变（WMLs）和大的血管周围间隙（VRSs）进行评分，并参照标准图像评估三角区上方和后方白质（WMPST）的信号强度。病程与WML呈正相关，与VRS评分呈负相关。根据WMPST受累程度的增加，将患者分为四组：A组为轻度WMPST高信号，B组为大VRSs且伴有轻度或中度WMPST高信号，C组为中度WMPST高信号或轻度WMPST高信号伴小的WMLs，D组为重度WMPST高信号或中度WMPST高信号伴小的WMLs。病程、肌肉损伤、脑叶WMLs和脑萎缩从A组和B组（无显著差异）到C组显著增加，从C组到D组进一步增加，而脑凸面VRSs从B组到C组以及从C组到D组显著减少。DM1中的脑叶白质受累在疾病过程中似乎呈进行性，最初可能表现为大VRSs或轻度WMPST高信号，然后是小WMLs或中度WMPST高信号，最后是更广泛且融合的WMLs或弥漫性白质高信号以及脑萎缩。
