Nerve ultrasound can identify treatment‐responsive chronic neuropathies without electrodiagnostic features of demyelination

Abstract

Introduction We present a case series of six treatment‐naive patients with clinical phenotypes compatible with chronic inflammatory demyelinating polyneuropathy and multifocal motor neuropathy without electrodiagnostic features of demyelination but with abnormal peripheral ultrasound findings who responded to treatment. Methods All six patients underwent a complete set of ancillary investigations, including extensive nerve conduction studies. We also performed standardized nerve ultrasound of median nerves and brachial plexus as part of a larger effort to evaluate diagnostic value of sonography. Results Nerve conduction studies did not show conduction block or other signs of demyelination in any of the six patients. Sonographic nerve enlargement was present in all patients and was most prominent in proximal segments of the median nerve and brachial plexus. Treatment with intravenous immunoglobulin resulted in objective clinical improvement. Discussion Our study provides evidence that nerve ultrasound represents a useful complementary diagnostic tool for the identification of treatment‐responsive inflammatory neuropathies.

Sporadic hereditary neuropathies misdiagnosed as chronic inflammatory demyelinating polyradiculoneuropathy: Pitfalls and red flags

“…Neuroimaging is included in the supportive criteria for the diagnosis of CIDP, 2 and is gaining increasing role in the diagnostic workup of neuropathies. 3,[19][20][21] Ultrasound, being easily accessible, non-invasive and reproducible, is often used in CIDP, also in light of its diagnostic power in inflammatory neuropathies also when compared with MRI. 22 Peculiar US patterns in some demyelinating hereditary neuropathies (diffuse homogeneous increase of CSA in the entire length of the nerves), clearly different from those found in typical CIDP, 5,23 proved to be useful in our cohort (patients #4, 5) suggesting the correct diagnosis.…”

“…Neuroimaging may be helpful in the diagnostic workup. 3,5,[19][20][21][22][23][24] ORCID Marta Campagnolo https://orcid.org/0000-0002-6394-7168…”

“…Neuroimaging is included in the supportive criteria for the diagnosis of CIDP, and is gaining increasing role in the diagnostic workup of neuropathies . Ultrasound, being easily accessible, non‐invasive and reproducible, is often used in CIDP, also in light of its diagnostic power in inflammatory neuropathies also when compared with MRI .…”

“…Transient or lack of benefit from immunomodulatory therapies should lead to consider a hereditary neuropathy. Neuroimaging may be helpful in the diagnostic workup …”

Pathophysiology of Chronic Inflammatory Demyelinating Polyneuropathy: Insights into Classification and Therapeutic Strategy

“…Patients with these variable subtypes of CIDP, as defined in the EFNS/PNS criteria, are diagnosed comprehensively on the basis of symptoms/signs, electrodiagnostic criteria, and other supportive criteria. Nevertheless, a recent development in neuroimaging techniques enabled the discovery of patients suspected of having chronic inflammation of the peripheral nerve similar to that observed in CIDP but that did not fulfill its EFNS/PNS electrodiagnostic criteria [11,24]. In addition, some patients with CIDP initially manifest an acute, but not chronic, disease onset mimicking Guillain-Barré syndrome (GBS) [25].…”