Neuromuscular and Neuroimmunology Unit, IRCCS Humanitas Research Hospital, via Manzoni 56, 20089 Rozzano, Milan, Italy.
Department of Biomedical Sciences, Humanitas University, Via Rita Levi Montalcini 4, 20072 Pieve Emanuele, Milan, Italy.
J Neurol Neurosurg Psychiatry. 2023 Aug;94(8):614-621. doi: 10.1136/jnnp-2022-331011. Epub 2023 Apr 4.
To assess the ability of the 2021 European Academy of Neurology/Peripheral Nerve Society (EAN/PNS) clinical criteria for chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) to include within their classification the whole spectrum of clinical heterogeneity of the disease and to define the clinical characteristics of the unclassifiable clinical forms.
The 2021 EAN/PNS clinical criteria for CIDP were applied to 329 patients fulfilling the electrodiagnostic (and in some cases also the supportive) criteria for the diagnosis of CIDP. Clinical characteristics were reviewed for each patient not strictly fulfilling the clinical criteria ('unclassifiable').
At study inclusion, 124 (37.5%) patients had an unclassifiable clinical presentation, including 110 (89%) with a typical CIDP-like clinical phenotype in whom some segments of the four limbs were unaffected by weakness ('incomplete typical CIDP'), 10 (8%) with a mild distal, symmetric, sensory or sensorimotor polyneuropathy confined to the lower limbs with cranial nerve involvement ('cranial nerve predominant CIDP') and 4 (1%) with a symmetric sensorimotor polyneuropathy limited to the proximal and distal areas of the lower limbs ('paraparetic CIDP'). Eighty-one (65%) patients maintained an unclassifiable presentation during the entire disease follow-up while 13 patients progressed to typical CIDP. Patients with the unclassifiable clinical forms compared with patients with typical CIDP had a milder form of CIDP, while there was no difference in the distribution patterns of demyelination.
A proportion of patients with CIDP do not strictly fulfil the 2021 EAN/PNS clinical criteria for diagnosis. These unclassifiable clinical phenotypes may pose diagnostic challenges and thus deserve more attention in clinical practice and research.
评估 2021 年欧洲神经病学会/周围神经学会（EAN/PNS）慢性炎症性脱髓鞘性多发性神经病（CIDP）临床标准将疾病临床异质性的全貌纳入其分类的能力，并定义不可分类的临床形式的临床特征。
对符合 EAN/PNS 诊断 CIDP 的电诊断（某些情况下还支持）标准的 329 例患者应用 2021 年 EAN/PNS CIDP 临床标准。对不符合临床标准的每位患者（不可分类）进行临床特征回顾。
在研究纳入时，124 例（37.5%）患者的临床表现不可分类，包括 110 例（89%）四肢某些部位未受累的典型 CIDP 样临床表型（“不完全典型 CIDP”）、10 例（8%）以轻度远端、对称、感觉或感觉运动多发性神经病为特征，局限于下肢伴有颅神经受累（“颅神经为主的 CIDP”）和 4 例（1%）以局限于下肢近端和远端区域的对称感觉运动多发性神经病为特征（“副 paraparetic CIDP”）。81 例（65%）患者在整个疾病随访期间保持不可分类的表现，而 13 例患者进展为典型 CIDP。与典型 CIDP 患者相比，不可分类临床表现的患者 CIDP 程度较轻，而脱髓鞘的分布模式无差异。
一部分 CIDP 患者不符合 2021 年 EAN/PNS 诊断标准。这些不可分类的临床表型可能会带来诊断挑战，因此在临床实践和研究中值得更多关注。
