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Objective:To study peripheral nerve morphology in patients with transthyretin familial amyloid polyneuropathy (TTR-FAP) using high-frequency ultrasonography (HFUS), and to evaluate the value of HFUS in diagnosis of TTR-FAP.Methods:Thirty-eight patients with TTR-FAP diagnosed by gene detection and 23 normal controls from June 2015 to June 2021 in Peking University First Hospital were enrolled in this study. Consecutive ultrasonography scanning was performed in 6 pairs of nerves of bilateral limbs with 30 sites. The cross sectional area (CSA), CSA variability and inter-nerve CSA variability data of the two groups were retrospectively calculated and compared.Results:Compared with the normal controls, TTR-FAP patients showed larger CSA values at most nerve sites of both upper and lower limbs, and there were statistically significant differences at M1(median nerve) [8.55 (6.90, 9.40) mm 2vs 10.05 (9.10, 14.10) mm 2, Z=5.58, P<0.001], M3 (median nerve) [(6.98±1.66) mm 2vs (9.29±2.30) mm 2, t=6.28, P<0.001], M5 (median nerve) [(8.91±1.81) mm 2vs (14.33±4.20) mm 2, t=9.84, P<0.001], U5 (ulnar nerve) [(6.20±1.93) mm 2vs (9.34±2.85) mm 2, t=7.31, P<0.001], Sci1 (sciatic nerve) [(53.50±17.24) mm 2vs (79.74±20.75) mm 2, t=7.57, P<0.001], Sci2 (sciatic nerve) [(53.66±14.21) mm 2vs (73.98±19.21) mm 2, t=6.82, P<0.001] and Tib (tibial nerve) [(31.05±8.43) mm 2vs (46.29±13.14) mm 2, t=7.84, P<0.001] sites. There was no statistically significant difference in CSA at each site among the different subtypes and disease severity of TTR-FAP patients ( P>0.05). There was no statistically significant difference in CSA-variability of the median and ulnar nerves between the patients with TTR-FAP and the normal controls ( P>0.05). The side-to-side difference ratio of intra-nerve CSA variability of the ulnar nerve in the patients with TTR-FAP was smaller than that of the normal controls (1.15±0.10 vs 1.46±0.43, t=3.43, P=0.002), whereas no statistically significant difference of that in the median nerve was found between the two groups ( P>0.05). Conclusions:The most pronounced peripheral nerve thickening in the proximal limb segments with no signs of asymmetric distribution or lateralization is confirmed by HFUS in TTR-FAP patients and should be regarded as a marker of TTR-FAP. HFUS has clinical value in diagnosis of peripheral neuropathy in TTR-FAP patients.
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BACKGROUND - Peripheral neuropathies are the most common neuromuscular disorder seen by neurologists. Nerve ultrasonography is a recently emerging tool for visualizing the structure, architecture and vascularity of peripheral nerves. OBJECTIVES -To study the Clinical correlation between Nerve conduction studies and Neurosonography in peripheral neuropathies. METHODOLOGY - All patients with peripheral neuropathy were included in the study from February 2016 to January 2018. RESULTS -The most common aetiologies for peripheral neuropathy were diabetes and leprosy. Nerve ultrasound showed corresponding changes in the above conditions. CONCLUSION – Nerve ultrasound is useful in peripheral neuropathy and can complement other diagnostic investigations.
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The ulnar dorsal aspect of the hand is predominantly innervated by the dorsal ulnar cutaneous nerve with variable input from the superficial radial cutaneous nerve. This cross innervation can cause difficulty in interpreting low amplitude sensory nerve action potential for the dorsal ulnar cutaneous nerve particularly when facing suspected ulnar neuropathyat the elbow. In three subjects with low dorsal ulnar cutaneous sensory nerve action potential amplitude due to cross over with the superficial radial nerve, we compared amplitude with nerve circumference and fascicular count as measured by ultrasound. Dorsal ulnar cutaneous nerve circumference was significantly smaller where there was low sensory nerve action potential amplitude and showed fewer fascicles. Nerve ultrasonography may be a useful additional test modality to determine if low dorsal ulnar cutaneous nerve amplitude is physiological.
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Humeral medial epicondyle fractures constitute around 15% of pediatric elbow fractures. Up to 60% occur in association with elbow dislocations. Knowledge of potential imaging pitfalls when examining acute elbow fractures in children contributes significantly to accurate diagnosis. Nevertheless, management of missed pediatric medial epicondyle fractures has rarely been reported. We present an 11-year-old boy with a neglected and severely displaced medial epicondyle fracture with concurrent ulnar nerve palsy. We performed neural decompression, fragment excision, and muscular and capsuloligamentous reconstruction of the medial elbow. This study demonstrates that the surgical outcome of a late presenting fracture can be satisfactory in terms of function and neural recovery. It also underscores the importance of careful interpretation of elbow imaging including normal anatomic variants.