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首页> 外文期刊>Neuropathology: official journal of the Japanese Society of Neuropathology >Pseudopolyneuritic form of ALS revisited: clinical and pathological heterogeneity.
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Pseudopolyneuritic form of ALS revisited: clinical and pathological heterogeneity.

机译：ALS的伪多神经病形式：临床和病理异质性。

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摘要

Pseudopolyneuritic form of ALS is a subtype of ALS characterized by distal weakness of the unilateral lower limb and absence of Achilles tendon reflex (ATR) at disease onset. Recognition of this form of ALS is important for clinicians because the combination of distal weakness of the lower limb and absence of ATR usually suggests peripheral neuropathy. We reviewed the clinical records of 42 autopsy-proven sporadic ALS cases and found three cases that showed onset of weakness of the unilateral lower limb with distal dominance and absence of ATR. The disease duration in the three cases was 2, 3 and 19 years, respectively. The clinical features of the patient with a course of 19 years had been restricted to lower motor neuron signs. Histopathologically, consistent findings in the three cases were severe motor neuron loss throughout the whole spinal cord, with relative preservation of the hypoglossal nucleus. Reflecting this finding, TDP-43-positive neuronal cytoplasmic inclusions in the spinal cord were sparse in two cases, and absent in a third. In the patient showing a clinical course of 19 years, mild corticospinal tract degeneration appeared to correspond to the absence of upper motor neuron signs and prolonged disease duration. In this case only, Bunina bodies were not demonstrated. In this study, we clarified the clinical and pathological heterogeneity of this form of ALS.
机译：ALS的假多神经病形式是ALS的一种亚型，其特征是疾病发作时单侧下肢的远端无力和无跟腱反射（ATR）。对这种形式的ALS的识别对临床医生很重要，因为下肢远端无力和ATR缺乏通常会提示周围神经病变。我们回顾了42例经尸检证实的散发性ALS病例的临床记录，发现3例表现为单侧下肢无力发作，远端占优势且无ATR的病例。三例病例的病程分别为2、3和19年。病程为19年的患者的临床特征仅限于下运动神经元体征。在组织病理学上，这三例病例的一致发现是整个脊髓严重运动神经元丢失，并伴有舌下核的相对保留。反映了这一发现，脊髓中TDP-43阳性神经元胞浆内含物稀疏，三例中无。在临床病程为19年的患者中，轻度的皮质脊髓束变性似乎对应于上运动神经元体征的缺乏和疾病持续时间的延长。仅在这种情况下，未展示Bunina的尸体。在这项研究中，我们阐明了这种形式的ALS的临床和病理学异质性。

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