Nerve Transfer for Intrinsic Hand Dysfunction in Brown-Vialetto-Van Laere Syndrome: Expanding the Role of SETS in Motor Neuronopathies

Abstract

Brown-Vialetto-Van Laere syndrome (BVVLS) is a rare neurodegenerative disorder associated with progressive cranial and spinal motor neuron involvement. While typically managed conservatively, select patients may present with focal peripheral deficits amenable to surgical intervention. We report the first known case of intrinsic hand dysfunction in BVVLS treated using a supercharged end-to-side (SETS) anterior interosseous-to-ulnar motor nerve transfer. Electroneuromyography revealed isolated postganglionic ulnar motor dysfunction with preservation of the median nerve, supporting surgical candidacy. The procedure resulted in restored hand function within 6 months and sustained improvement at 5-year follow-up. This case highlights the potential for SETS nerve transfer to expand beyond compressive neuropathies and serve as a targeted strategy in progressive motor neuron disorders with preserved donor pathways.