臨床神経学

<シンポジウム(3)―14―4>小血管病の謎に迫る:CARASIL病態研究の最前線

脳小血管病におけるNeurovascular Unitの障害

伊藤 義彰, 鳥海 春樹, 海老根 妙子, 畝川 美悠紀, 山田 哲, 此枝 史恵, 小泉 健三, 冨田 裕, 鈴木 則宏

慶應義塾大学神経内科〔〒160―8582 東京都新宿区信濃町35〕

Among many conditions causing small vessel diseases, lipohyalinosis is the leading pathology next to microatheroma. Lipohyalinosis affects penetrating arteries distally than microatheroma. Proliferation of smooth muscle cells may occlude the lumen, reducing the blood flow and inducing lacunar infarction. In contrast, fibrinoid necrosis of smooth muscle cells in the media may weaken the vascular constriction, increasing the perfusion pressure in the capillary and damaging the blood brain barrier which can induce white matter lesion. Neurovascular unit (NVU) is a concept that neurons, astrocytes, and vessels function as a unit to support neuronal activity. NVU is involved in the maintenance of synapse, transmitter, energy metabolism, blood-brain barrier, and blood flow. Change in neuronal activity is transmitted to capillaries through NVU, where the information is collected along vessels proximally and regulates blood flow (proximal integration model). CARASIL and CADASIL both affect vascular smooth muscle cells, resulting in vascular dilatation, damaging NVU, and inducing white matter lesion. Occlusion of the affected vessels, causing cerebral ischemia, under these diseases is relatively infrequent. Similarity in pathophysiology between hypertensive arteriolar disease and the familial angiopathy may indicate that injury to NVU may indicate the common pathophysiology of white matter lesions.
Full Text of this Article in Japanese PDF (386K)

(臨床神経, 52:1365−1368, 2012)
key words:CARASIL,CADASIL,高血圧性細動脈疾患,白質病変,微小循環障害

(受付日:2012年5月25日)