Taly A B, Prasad A, Vasanth A, Shankar S K, Nagaraja D
Department of Neurology, National Institute of Mental Health & Neuro Sciences, Bangalore, India.
Acta Neurol Scand. 1991 Nov;84(5):398-402. doi: 10.1111/j.1600-0404.1991.tb04976.x.
Sensory ataxia as the chief manifestation of acute neuropathy is rather rare. Of the 224 cases of acute polyneuropathy seen over 6 years (1984-1990) only 10 patients (M:F 3:7) had disabling ataxia as the presenting feature. Their ages ranged from 14-61 years. Antecedent febrile illness was present in 6 patients and the peak deficit evolved over 2-25 days. Severe ataxia, paresthesia, distal areflexia and predominant joint sense loss were common to all, motor weakness was either absent or insignificant. CSF was acellular and revealed elevated protein in 3 subjects. All patients had electrophysiological evidence of severe sensory neuropathy with mild or no motor neuropathy. Sural nerve biopsy in one patient showed loss of large, as well as small, diameter myelinated fibres, secondary demyelination, but no evidence of inflammation. At follow up marginal to moderate improvement in ataxia was noted in only 5 patients. Absence of ophthalmoplegia and motor weakness, poor prognosis and characteristic electrophysiological and histopathological observations suggest that acute ataxic neuropathy may be a distinct entity.
以感觉性共济失调为急性神经病变主要表现的情况相当罕见。在6年(1984 - 1990年)间所见的224例急性多发性神经病变中,仅有10例患者(男:女为3:7)以致残性共济失调为首发特征。他们的年龄在14至61岁之间。6例患者病前有发热性疾病,最大功能缺损在2至25天内进展。所有患者均有严重共济失调、感觉异常、远端反射消失及主要关节位置觉丧失,运动无力要么不存在,要么不明显。3例患者的脑脊液无细胞,蛋白升高。所有患者均有严重感觉神经病变的电生理证据及轻度或无运动神经病变。1例患者的腓肠神经活检显示大直径和小直径有髓纤维均减少、继发性脱髓鞘,但无炎症证据。随访时仅5例患者的共济失调有轻微至中度改善。无眼肌麻痹和运动无力、预后差以及特征性的电生理和组织病理学表现提示急性共济失调性神经病变可能是一种独特的疾病。