Chiron C, Dulac O, Bulteau C, Nuttin C, Depas G, Raynaud C, Syrota A
Neuropediatric Department, Hospital Saint-Vincent-de-Paul, Paris, France.
Epilepsia. 1993 Jul-Aug;34(4):707-15. doi: 10.1111/j.1528-1157.1993.tb00450.x.
Focal cortical disturbances are frequent sequelae in West syndrome (WS) even though it is a generalized epileptic syndrome. Functional neuroimaging was used to determine whether focal perfusion abnormalities exist at WS onset and change during evolution. We studied regional cerebral blood flow (rCBF) at different stages of WS. Mean CBF (mCBF) and rCBF were measured using SPECT (single photon emission computed tomography) and 133Xe in 13 WS patients: at onset (20 cases), just after steroids (17 cases), and after a mean follow-up of 2 years (26 cases). At WS onset, interictal mCBF was increased as the result of foci of hyper- and hypoperfusion, which were, respectively, mainly located in the frontal and posterior cortex. Just after steroid therapy, mCBF decreased without any focal predominance. During follow-up, hypoperfused foci remained unchanged whereas the frontal hyperperfused foci decreased after spasm control. Our results show that focal abnormalities are present at WS onset. Focal hypoactivity could reflect a cortical lesion responsible for WS and focal hyperactivity could play a role in the persistence of generalized epilepsy.
尽管韦斯特综合征（WS）是一种全身性癫痫综合征，但局灶性皮质功能障碍却是其常见的后遗症。功能性神经影像学被用于确定在WS发病时是否存在局灶性灌注异常以及在疾病发展过程中这些异常是否会发生变化。我们研究了WS不同阶段的局部脑血流（rCBF）情况。使用单光子发射计算机断层扫描（SPECT）和133Xe对13例WS患者在不同阶段测量了平均脑血流（mCBF）和rCBF：发病时（20例）、使用类固醇治疗后即刻（17例）以及平均随访2年后（26例）。在WS发病时，由于高灌注和低灌注灶的存在，发作间期mCBF增加，这些病灶分别主要位于额叶和后皮质。在类固醇治疗后即刻，mCBF下降且无任何局灶性优势。在随访期间，低灌注灶保持不变，而额叶高灌注灶在痉挛得到控制后减少。我们的结果表明，在WS发病时存在局灶性异常。局灶性低活性可能反映了导致WS的皮质病变，而局灶性高活性可能在全身性癫痫的持续存在中起作用。
