Targeted biopsy of the ulnar nerve for amyloidosis with potential implications of a diagnostic and nondiagnostic biopsy: illustrative case.

Background: MRI-guided targeted fascicular biopsy has been a valuable technique for diagnosing idiopathic neuropathies. It has an 80% yield and a minor permanent morbidity rate of 5%.

Observations: A 48-year-old woman with a history of localized amyloidosis to the orbital muscle 2 years earlier presented with new neurological symptoms. Extensive evaluations including skin, muscle, and distal cutaneous nerve biopsies were negative, and empirical treatment trials did not improve her condition. An MRI-guided targeted biopsy of the right ulnar nerve in the distal arm was planned. During surgery, a medial triceps branch arising from the ulnar nerve was identified and was biopsied along with a neighboring fascicle of the ulnar nerve to the flexor carpi ulnaris. The pathology of the ulnar nerve fascicle but not the contiguous triceps branch showed amyloid (AL [kappa] subtype on mass spectroscopy). The patient was started on treatment.

Lessons: The positive biopsy of the ulnar nerve fascicle allowed targeted treatment of the neural amyloidosis. While the negative biopsy of the triceps branch could be attributed to sampling error, it could also be due to the fact that this branch found within the ulnar nerve is thought to originate from the radial nerve. https://thejns.org/doi/10.3171/CASE25484.