Department of Neurology, University Medical Center Groningen, University of Groningen, Groningen, the Netherlands.
Department of Neurology, Erasmus MC, University Medical Center Rotterdam, Rotterdam, the Netherlands.
Eur J Paediatr Neurol. 2023 May;44:28-36. doi: 10.1016/j.ejpn.2023.03.002. Epub 2023 Mar 22.
Acute flaccid paralysis (AFP) is characterized by rapidly progressive limb weakness with low muscle tone. It has a broad differential diagnosis, which includes acute flaccid myelitis (AFM), a rare polio-like condition that mainly affects young children. Differentiation between AFM and other causes of AFP may be difficult, particularly at onset of disease. Here, we evaluate the diagnostic criteria for AFM and compare AFM to other causes of acute weakness in children, aiming to identify differentiating clinical and diagnostic features.
The diagnostic criteria for AFM were applied to a cohort of children with acute onset of limb weakness. An initial classification based on positive diagnostic criteria was compared to the final classification, based on application of features suggestive for an alternative diagnosis and discussion with expert neurologists. Cases classified as definite, probable, or possible AFM or uncertain, were compared to cases with an alternative diagnosis.
Of 141 patients, seven out of nine patients initially classified as definite AFM, retained this label after further classification. For probable AFM, this was 3/11, for possible AFM 3/14 and for uncertain 11/43. Patients initially classified as probable or possible AFM were most commonly diagnosed with transverse myelitis (16/25). If the initial classification was uncertain, Guillain-Barré syndrome was the most common diagnosis (31/43). Clinical and diagnostic features not included in the diagnostic criteria, were often used for the final classification.
The current diagnostic criteria for AFM usually perform well, but additional features are sometimes required to distinguish AFM from other conditions.
急性弛缓性麻痹（AFP）的特征是迅速进展的肢体无力伴低肌张力。它有广泛的鉴别诊断，包括急性弛缓性脊髓炎（AFM），一种罕见的类似脊灰的疾病，主要影响幼儿。AFM 与其他 AFP 病因的鉴别可能很困难，尤其是在疾病发病初期。在这里，我们评估了 AFM 的诊断标准，并将 AFM 与儿童其他急性无力的病因进行比较，旨在确定有区别的临床和诊断特征。
应用 AFM 的诊断标准对一组急性起病的肢体无力儿童进行评估。根据阳性诊断标准进行的初始分类与基于替代诊断提示特征和与专家神经病学家讨论的最终分类进行比较。将分类为确定、可能或疑似 AFM 或不确定的病例与替代诊断的病例进行比较。
在 141 例患者中，最初分类为确定 AFM 的 9 例患者中的 7 例在进一步分类后保留了该标签。对于可能的 AFM，这是 11 例中的 3 例，对于可能的 AFM，这是 14 例中的 3 例，对于不确定的 AFM，这是 43 例中的 11 例。最初分类为可能或疑似 AFM 的患者最常见的诊断为横贯性脊髓炎（16/25）。如果最初的分类不确定，格林-巴利综合征是最常见的诊断（31/43）。未包含在诊断标准中的临床和诊断特征，通常用于最终分类。
目前的 AFM 诊断标准通常表现良好，但有时需要额外的特征来区分 AFM 与其他疾病。
