De Michele G, Filla A, Striano S, Iorio L, Merola B, De Rosa M, Lombardi G, Campanella G
Clinica Neurologica I e II, Facoltà di Medicina II, Napoli.
Riv Neurol. 1987 Nov-Dec;57(6):328-32.
We describe the association of cerebellar ataxia and hypogonadism in four female patients. Clinical, neurophysiological, neuroradiological and endocrinological features are reported. Post-mortem findings in one case are also described. We show in our patients that, beside the cerebellum, both the supratentorial white matter and the peripheral nerve are involved. We suggest that this association can be considered as a "multiple system atrophy". Furthermore, since hypogonadism is hypergonadotropic in three patients and hypogonadotropic in one, the endocrine pattern appears to be heterogeneous.
我们描述了4例女性患者中小脑共济失调与性腺功能减退的关联。报告了其临床、神经生理学、神经放射学和内分泌学特征。还描述了1例患者的尸检结果。我们的患者显示,除小脑外,幕上白质和周围神经均受累。我们认为这种关联可被视为一种“多系统萎缩”。此外,由于3例患者性腺功能减退为高促性腺激素性,1例为低促性腺激素性,内分泌模式似乎是异质性的。