J Korean Neurol Assoc.
2000 Jan;18(1):102-105.
Polyneuropathy in Progressive Systemic Sclerosis
- Affiliations
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- 1Department of Neurology, Chungbuk National University Hospital .
Abstract
- Progressive systemic sclerosis (PSS) is a chronic inflammatory disease of the connective tissue
which affects the skin and other internal organs. Involvement of the peripheral nervous system is rare, although
muscle abnormalities as a component of PSS are well recognized. 29-year-old (patient 1) and 64-year-old
(patient 2) women had PSS for 3 and 5 years, respectively. They complained of having a tingling sensation in the legs.
Examination showed reduced strength in the distal muscles, bilateral hypesthesia for pain and vibration below
the knees and reduced or absent tendon reflexes in the lower limbs. Sensorimotor neuropathies were
demonstrated by decreased motor conduction velocities and pro-longed distal sensory latencies.
Sural nerves were of normal thickness. The density of myelinated fibers was not decreased and axonal
degeneration was scanty or absent. Characteristic findings were intimal hypertrophy and perivas-cular
inflammatory infiltrates in both patients. Inflammatory cell invasions in the peripheral nerve is extremely
are in patients with PSS. It may be suggested that an inflammatory or immune mechanism is another contributing
factor to polyneuropathy in PSS as well as microangiopathy.