多囊性腦室周邊腦白質軟化症二病例報告

Polycystic Periventricular Leukomalacia-Two Cases Report-

周國燊(Kuo-Sun Chow) ； 張志岳(Chin-Youeh Chang) ； 呂道南(Tao-Nan Lu)

《Acta Paediatrica Sinica》 27卷6期 (1986/12) Pp. 570-575

摘要

本院於1985年3月及8月共發現兩個多囊性腦室周邊腦組織軟化症病例。此2例的腦部超音波掃描，在側腦室邊緣四週，呈現囊狀及腔洞的變化，大的囊狀損傷中間存有中隔的形成。電腦斷層攝影呈現低的放射線透過性(low attenuation)，而臨床症狀均顯示有明顯的腦性麻痺現象。本文提出報告並加以討論。

並列摘要

Periventricular leukomalacia (PVL), a perinatal hypoxic-ischemic insult of the neonatal cerebrum, is located in the deep white matter adjacent to the external angle of the lateral ventricles. It may lead to the development of areas of cystic degeneration varying from a few small cysts to multiple cysts of various sizes separated by septa.
PVL is the second most frequent lesion of the infant brain following hemorrhage into the germinal matrix or ventricles. Its incidence in surviving infants has not been established. In a neonatal autopsy series, its incidence is 7-22%. The frequency is higher in infants weighing less than 1500gm at birth (34%).
The predisposing factors are severe hypoxia and ischemia, which in turn lead to inadequate perfusion of the watershed areas and development of infarction and necrosis.
The role of CT scan for diagnosis is controversial, and its use is of little value until the cystic lesions reach a relatively large size.
All infants exhibited abnormal neurologic signs following convulsive disorders, and had unequivolcal signs of mental retardation and severe motor and sensory deficits months after patient discharge.

並列關鍵字

polycystic periventricular leukomalacia (PVL)