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Neurologic manifestations of progressive systemic sclerosis
L Averbuch-Heller1, I Steiner, O Abramsky
1Department of Neurology, Hadassah University Hospital, Jerusalem, Israel.
Archives of Neurology
|December 1, 1992
Summary
Neurologic complications in progressive systemic sclerosis are more common than previously thought. Forty percent of patients showed neurological abnormalities, suggesting direct nervous system involvement.
Area of Science:
- Neurology
- Rheumatology
- Systemic Autoimmune Diseases
Background:
- Progressive systemic sclerosis (PSS), a systemic autoimmune disease, is historically associated with uncommon neurologic involvement.
- This study aimed to determine the actual prevalence and characteristics of neurological complications in PSS patients.
Observation:
- A retrospective analysis was conducted on 50 patients diagnosed with progressive systemic sclerosis.
- Neurologic abnormalities were identified in 20 patients (40%), manifesting as 28 distinct neurologic events.
- Affected areas included muscle (22%), peripheral nerve (18%), spinal cord (8%), and brain (6%).
Findings:
- Myelopathy was observed in four patients and inclusion-body myositis in two.
- In 20% of cases, no cause for neurologic dysfunction other than PSS was identified.
- The findings indicate that neurologic manifestations in PSS are significantly more frequent than previously reported.
Implications:
- Progressive systemic sclerosis may directly involve the nervous system in a substantial number of patients.
- Increased awareness and investigation of neurologic complications in PSS are warranted.
- These findings could lead to revised diagnostic and treatment strategies for PSS patients with neurologic symptoms.