Neurologic manifestations of progressive systemic sclerosis
L. Averbuch-Heller, I. Steiner and O. Abramsky
Department of Neurology, Hadassah University Hospital, Jerusalem, Israel.
Neurologic involvement in progressive systemic sclerosis is considered
uncommon. We retrospectively examined the prevalence and nature of
neurologic complications in 50 patients with progressive systemic
sclerosis. In 20 (40%), neurologic abnormalities were detected, with a
total of 28 neurologic manifestations. All levels of the central and
peripheral nervous system were affected: muscle (22%), peripheral nerve
(18%), spinal cord (8%), and brain (6%). Of note were the presence of
myelopathy in four patients and inclusion-body myositis in two. In 10
patients (20%), no definable cause of the neurologic dysfunction could be
identified, apart from progressive systemic sclerosis. Thus, neurologic
presentations of progressive systemic sclerosis are much more common than
previously reported and may be due to direct involvement of the nervous
system by a primary pathologic process in a significant number of patients.