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Título

A university neuromuscular center experience in chronic autoimmune demyelinating polyneuropathy diagnostic workup

Resumo

Background:
Chronic autoimmune neuropathies are a heterogeneous spectrum of disorders with distinct pathophysiology, phenotypes and treatment. This group accounts for both economic and quality of life burden. Misdiagnosis remains common, especially for chronic autoimmune demyelinating polyneuropathy (CIDP) variants, which is why diagnostic criteria have recently been reviewed. Widespread interest in immune neuropathies relies on the fact that they are treatable conditions.
Goal:
An analysis of our university hospital cohort of patients presenting with CIDP variants, aiming to contribute to diagnostic workup debate.
Methods:
A cohort of 46 suspected CIDP patients was identified in our neuromuscular outpatient clinic, after thorough chart review from 2018 to June/2022. Patients underwent analysis based on EFNS/PNS diagnostic criteria.
Results:
Out of 46 suspected acquired demyelinating neuropathy patients, 9 fulfilled criteria for multifocal motor neuropathy and 2 were diagnosed with metabolic neuropathy, and were all excluded from further analysis. Out of 35 remaining patients, 14 are female and 21 male. 12 patients presented with relapsing forms, 3 progressive, 11 secondary progressive, 8 clinical remission and 1 still unidentified.
According to 2021 ENFS/PNS electrodiagnostic criteria, 20 patients were classified as definite CIDP and 7 as possible CIDP. 8 did not fulfill criteria.
Twenty three patients presented with typical CIDP, 2 with multifocal CIDP, 1 with focal CIDP and 1 with sensory CIDP. Median time for diagnosis after initial symptoms was 53 months.
Conclusions:
Accurate diagnosis and full understanding of different clinical presentations guide therapeutic management and prognosis. They range from multifocal to diffuse, from sensory to motor, from benign to severe and from relapsing to progressive forms.
Out of our cohort of clinically suspected and treatment responsive patients, 77.14% fulfilled criteria for possible diagnosis of CIDP, which reinforces importance of supportive criteria when diagnosis is uncertain. Sensibility may be reduced for focal and sensory forms, besides severe cases with axonal loss.
Most frequent clinical course was relapsing. 85.2% of patients showed typical presentations.
CIDP diagnosis remains a challenge, which is evidenced by the prolonged time lapse between initial symptoms and definite diagnosis.

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Doenças Neuromusculares