Multiple Sites Ultrasonography of Peripheral Nerves in Differentiating Charcot–Marie–Tooth Type 1A from Chronic Inflammatory Demyelinating Polyradiculoneuropathy

Abstract

IntroductionMultiple sites measurement of cross-sectional areas (CSA) by ultrasound was performed to differentiate Charcot–Marie–Tooth type 1A (CMT1A) and chronic inflammatory demyelinating polyradiculoneuropathy (CIDP).MethodsNine patients with CMT1A, 28 patients with CIDP, and 14 healthy controls (HC) were recruited prospectively. Consecutive ultrasonography scanning was performed from wrist to axilla on median and ulnar nerves. CSAs were measured at 10 predetermined sites of each nerve.ResultsCMT1A had significantly larger CSAs at all sites of median and ulnar nerves (p < 0.01). In CMT1A, CSAs increased gradually and homogeneously from distal to proximal along the nerve, except potential entrapment sites. CIDP displayed three different morphological patterns, including mild enlargement in 15 patients, prominent segmental enlargement in 12, and slight enlargement in 1, among which different treatment responses were observed. All patients with mild nerve enlargement treated with intravenous immunoglobulin were responsive (7/7), while less than half of those with prominent segmental enlargement (3/7) were responsive (p < 0.01).DiscussionConsecutive scan along the nerve and multiple sites measurement by ultrasound could supply more detailed morphological feature of the nerve and help to differentiate CMT1A from CIDP.

Biomarker Supervised G-CSF (Filgrastim) Response in ALS Patients

“…Cytokines are mediators of the immune communication that may cross the blood-brain barrier (BBB) and provide a mechanism by which the peripheral immune system may directly influence the CNS ( 30 ). In a recent study, we demonstrated a pro-inflammatory immune response with elevated inflammatory cytokines both in serum during disease and post-mortem in the CNS of ALS patients ( 31 ). However, immune response in ALS cannot be clearly dichotomized to a purely pro- or anti-inflammatory state, as cytokines are often pleiotropic, and their function may change over time and depend on concentration and specific disease context.…”

“…Initial TNF-beta (LTA, lymphotoxin-alpha) levels negatively correlated with survival and were found at higher levels in shorter surviving ALS patients upon treatment initiation. G-CSF application led to reduction in TNF-beta, a pro-inflammatory cytokine and common cell death effector found to be increased in ALS sera ( 31 ). TNF-alpha was borderline increased in patients with shorter survival (trend) and G-CSF led to an increase in its serum levels.…”

“…TNF-alpha was borderline increased in patients with shorter survival (trend) and G-CSF led to an increase in its serum levels. TNF-alpha is elevated in ALS ( 31 , 43 – 46 ) and correlates with disease duration ( 47 ). But its role in ALS in unclear and the two TNF-alpha receptors, either associated with inflammation or neuroprotection, have opposing effects concerning survival in ALS ( 48 ).…”

Serial nerve ultrasound and motor nerve conduction studies in chronic inflammatory demyelinating polyradiculoneuropathy

“…Recently, nerve ultrasound has been used to detect morphological abnormalities of the peripheral nerves in CIDP. Most studies have been cross‐sectional and have shown different patterns of nerve morphological changes in patients with CIDP, including moderate cross‐sectional area (CSA) enlargement, segmental prominent enlargement, and little enlargement . However, few studies have reported how nerve morphology changes over time in CIDP.…”

“…Most studies have been cross-sectional and have shown different patterns of nerve morphological changes in patients with CIDP, including moderate cross-sectional area (CSA) enlargement, segmental prominent enlargement, and little enlargement. 2,3 However, few studies have reported how nerve morphology changes over time in CIDP. Zaidman et al 4 showed that, in CIDP, enlarged nerves normalized or decreased in size with remission.…”