Arpa J, López-Pajares R, Cruz-Martínez A, Palomo F, Ferrer T, Caminero A B, Rodríguez-Albariño A, Alonso M, Lacasa T, Nos J
Servicio de Neurología, Hospital La Paz, Madrid.
Neurologia. 1995 Aug-Sep;10(7):288-96.
Forty subjects were clinically examined using scales for cerebellar, pyramidal, parkinsonian, and mental status and by quantitative evaluation of neuroimages. The patients were classified into two groups: cerebellar-plus and "pure" cerebellar syndromes. Patients with "pure" cerebellar syndrome were diagnosed as autosomal dominant cerebellar ataxia III (ADCA III) or "pure idiopathic" late-onset cerebellar ataxia (ILOCA) in this series. Patients with cerebellar-plus syndrome were diagnosed as multiple system atrophy (MSA), subclassified as either ILOCA-plus, ADCA I, ADCA II or autosomal recessive LOCA. We have used visual (VEP), brainstem auditory (BAEP) and somatosensory (SEP) evoked potentials in order to establish their diagnostic validity. Cerebellar-plus syndrome and "pure" cerebellar syndrome showed overlapping VEP, BAEP and SEP abnormalities. VEP P100 latency, however, shows a certain ability to differentiate between the two groups (p = 0.08) and appears useful in distinguishing between sporadic cerebellar-plus syndromes (MSA or ILOCA-plus) and "pure" cerebellar syndromes (p < 0.02). The incidence of prolonged N9-N13 latency was significantly higher in the latter subgroup (p < 0.04) as well. Within cerebellar-plus syndromes, VEP, BAEP and SEP abnormalities were more frequent in inherited cases (ADCA I and II, along with autosomal recessive LOCA) than in sporadic ones. The most apparent differences were a higher incidence of abnormal BAEPs at brainstem level (p < 0.002), and of both peripheral and possible central SEP impairment in hereditary cerebellar-plus syndrome than in sporadic cerebellar-plus syndrome (p < 0.03). EP investigation is useful to a certain extent in differentiating between some variants of LOCA.
使用小脑、锥体束、帕金森病和精神状态量表对40名受试者进行临床检查，并对神经影像进行定量评估。患者被分为两组：小脑加综合征组和“单纯”小脑综合征组。在本系列中，“单纯”小脑综合征患者被诊断为常染色体显性遗传性小脑共济失调III型（ADCA III）或“单纯特发性”迟发性小脑共济失调（ILOCA）。小脑加综合征患者被诊断为多系统萎缩（MSA），再细分为ILOCA加型、ADCA I型、ADCA II型或常染色体隐性遗传性迟发性小脑共济失调（LOCA）。我们使用视觉诱发电位（VEP）、脑干听觉诱发电位（BAEP）和体感诱发电位（SEP）来确定它们的诊断有效性。小脑加综合征和“单纯”小脑综合征表现出VEP、BAEP和SEP异常的重叠。然而，VEP的P100潜伏期在区分两组方面具有一定能力（p = 0.08），并且在区分散发性小脑加综合征（MSA或ILOCA加型）和“单纯”小脑综合征方面似乎很有用（p < 0.02）。后一组亚组中N9 - N13潜伏期延长的发生率也显著更高（p < 0.04）。在小脑加综合征中，VEP、BAEP和SEP异常在遗传性病例（ADCA I型和II型，以及常染色体隐性遗传性LOCA）中比散发性病例更常见。最明显的差异是脑干水平BAEP异常的发生率更高（p < 0.002），以及遗传性小脑加综合征比散发性小脑加综合征在外周和可能的中枢SEP损害方面都更高（p < 0.03）。诱发电位检查在一定程度上有助于区分LOCA的一些变体。
