Pain is not usually considered a symptom of chronic relapsing demyelin
ating polyneuropathy. We report two patients with chronic demyelinatin
g polyneuropathies in whom clinical and electrophysiological worsening
was associated with the development of deep and distressing upper lim
b aching discomfort. One patient had a MGUS-associated hypertrophic de
myelinating and prednisone-dependent polyneuropathy, followed over a c
ourse of two and a half years. His discomfort regularly predicted elec
trophysiological relapse of neuropathy, before more obvious clinical s
igns had emerged. Resolution of the discomfort also predicted subseque
nt clinical and electrophysiological improvement. Upper limb pain may
be an important feature of early relapse in some patients with demyeli
nating polyneuropathies. Standardized serial electrophysiological test
ing in patients with chronic demyelinating polyneuropathy can be an im
portant management tool in conjunction with clinical signs and symptom
s.