Is it Guillain-Barré or Acute-onset CIDP?

Professor of Clinical Neurology, Weill Cornell Medical College

Dr. Rubin reports no financial relationships relevant to this field of study.

SYNOPSIS: Acute inflammatory demyelinating polyneuropathy and acute-onset chronic inflammatory demyelinating polyneuropathy (CIDP) may present with identical clinical pictures and can be differentiated only with the passage of time. CIDP will have a slower course of progression and may involve relapses.

SOURCE: Alessandro L, Rueda JMP, Wilken M, et al. Differences between acute-onset chronic inflammatory demyelinating polyneuropathy and acute inflammatory demyelinating polyneuropathy in adult patients. J Peripher Nerv Syst 2018;23:154-158.

Weakness in acute inflammatory demyelinating polyneuropathy (AIDP) develops over several days, reaching maximal deficit within four weeks. However, neurological deficits in chronic inflammatory demyelinating polyneuropathy (CIDP) evolve at a slower pace, progressing over at least a two-month period. Although this temporal demarcation appears straightforward, in clinical practice, differentiating the two disorders early may be difficult, as some CIDP patients, usually children or young adults, present with an acute- or subacute-onset CIDP (A-CIDP) that resembles AIDP and will be recognized only with the passage of time. Might it be possible clinically to differentiate AIDP from A-CIDP earlier?

Alessandro et al conducted a retrospective review of the records of 119 patients, 18 years of age or older, diagnosed with AIDP between January 2006 and July 2017 at the Center for Research on Neuroimmunological Diseases at Raúl Carrea Institute for Neurological Research in Buenos Aires, Argentina. Data collected included medical history; vaccinations within a month prior to disease onset; HIV status; motor, sensory, and autonomic dysfunction; cerebrospinal fluid (CSF) analysis; and electrodiagnostic testing. Patients were classified as AIDP or A-CIDP based on published¹ and electrophysiologic criteria. All patients were followed for at least one year. A statistical analysis comprised Student’s t test, Fisher’s test, and Pearson’s χ2 test.

Of the 119 patients’ records reviewed, 91 satisfied criteria for inclusion in the study, 77 with AIDP and 14 with A-CIDP, of which 69% were male. Mean age (43 years and 55.5 years, respectively) was not significantly different between the two groups. In addition, no difference was found for median Medical Research Council sum score, sensory disturbances, autonomic dysfunction, cranial nerve involvement, CSF cytoalbuminogenic dissociation, HIV status, neoplastic or autoimmune disease, preceding infectious disease or vaccination, rate of respiratory dysfunction, need for mechanical ventilation, hemodynamic instability, intensive care monitoring, or death. However, diabetes mellitus was seen more often in A-CIDP (29% vs. 8%; P = 0.4), as were proprioceptive abnormalities (83% vs. 28%; P < 0.001) and sensory ataxia (46% vs. 16%; P = 0.01). First relapse in A-CIDP patients occurred eight weeks after initial acute presentation, with most (11/14) evolving into CIDP, and few into single (n = 2) or relapsing-remitting
(n = 1) disease. Based on this retrospective analysis, AIDP and A-CIDP essentially are indistinguishable.

COMMENTARY

First described in 1958 by Austin as a fluctuating, motor-predominant polyneuropathy responsive to corticosteroid treatment, the term chronic inflammatory polyneuropathy (demyelinating was added later) was introduced by Dyck in 1975 in a landmark paper describing 53 personally examined patients. Segmental demyelination, typically paranodal, and remyelination, the pathologic hallmarks, are best seen on teased nerve fiber preparations, with inflammatory, frequently perivascular, infiltrates. Chronic demyelination and remyelination result in onion bulb formation. Although some have suggested that diabetes is a risk factor for developing CIDP, at least two epidemiologic studies have shown this is not the case. In addition to the classic presentation of symmetrical polyneuropathy, subtypes include multifocal CIDP, an upper-limb-predominant asymmetric neuropathy, also known as Lewis-Sumner syndrome or MADSAM (multifocal acquired demyelinating sensory and motor neuropathy), with focal motor and sensory conduction blocks, mildly elevated protein (not as high as classic CIDP), and responsive to corticosteroids (unlike multifocal motor neuropathy with conduction block, which does not) and intravenous immunoglobulin. CIDP may affect only sensory nerve roots, chronic inflammatory sensory polyneuropathy, resulting in sensory ataxia and numbness with mild pain and without weakness.²

REFERENCES

1. Willison HJ, Jacobs BC, van Doorn PA. Guillain-Barre syndrome. Lancet 2016;388:717-727.
2. Dyck PJB, Tracy JA. History, diagnosis, and management of chronic inflammatory demyelinating polyradiculoneuropathy. Mayo Clin Proc 2018;93:777-793.