To report a novel case of symptomatic, biopsy proven, mass spectrometry confirmed, wild type transthyretin amyloidosis (ATTRwt) in nerve tissue.

A 94-year-old man with history of spinal stenosis presented to neurology clinic with a subacute on chronic progressive, upper limb predominant, weakness along with numbness and tingling paresthesia.  Electromyography (EMG) revealed multiple mononeuropathies involving bilateral median nerves at the wrists, bilateral ulnar nerves, and bilateral distal radial nerves superimposed upon a mild length-dependent, sensorimotor, axonal predominant, peripheral neuropathy along with multilevel lumbosacral radiculopathies.  Extensive serology for causes of neuropathy and multiple mononeuropathies returned unremarkable.  A diagnostic right superficial radial nerve biopsy was performed and showed congophilic material within a small epineurial vessel wall in the nerve tissue.  Amyloid typing by mass spectrometry was performed and revealed ATTRwt.  TTR gene sequencing returned normal.  The patient was diagnosed with ATTRwt neuropathy.

This case confirms the presence of ATTRwt deposition directly in nerve tissue as the likely cause of the patient’s large fiber and multiple mononeuropathies expanding our current understanding of ATTRwt associated disease.  Proving the direct association of ATTRwt and neuropathy could open up amyloid specific treatments for this disease.