Barbeau A
Can J Neurol Sci. 1982 May;9(2):95-8. doi: 10.1017/s0317167100043766.
We present a working classification of recessively inherited ataxic syndromes based on the use of simple tools available to every clinician: a good history (particularly pinpointing the age of onset) and a good neurological examination (simplified to the verification of the presence of ataxia, deep tendon reflexes in the knee, optic nerve, retinal and/or 8th nerve signs). In the three groups of disorders (non progressive, intermittent or progressive) patients can be hyper/normo reflexic, or they can be hypo/areflexic. Six principal types of progressive ataxic disorders are further delineated by the age of onset. Sub-types depend on the presence of absence of eye and ear signs, whereas eponymic or regional denominations are used only for simplicity while awaiting exact delineation of the biochemical defects.
我们基于每位临床医生都可使用的简单工具，提出了一种隐性遗传性共济失调综合征的实用分类方法：详细的病史（尤其要明确发病年龄）和全面的神经系统检查（简化为检查是否存在共济失调、膝部深腱反射、视神经、视网膜及/或第8对脑神经体征）。在三组疾病（非进行性、间歇性或进行性）中，患者的反射可能亢进/正常，也可能减弱/消失。根据发病年龄进一步划分出六种主要类型的进行性共济失调疾病。亚型则取决于是否存在眼部和耳部体征，而在等待对生化缺陷进行精确界定之前，仅为简便起见使用以人名或地区命名的名称。
