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Related Experiment Videos

Scleroderma, stroke, optic neuropathy: a rare association.

C P Das1, S Prabhakar, V Lal

  • 1Department of Neurology, Postgraduate Institute of Medical Education and Research, Chandigarh - 160 012, India. dascp@glide.net.in

Neurology India
|February 11, 2003
PubMed
Summary

Scleroderma, a rare autoimmune disease, can affect the central nervous system, causing stroke-like symptoms such as hemiparesis and seizures due to large vessel vasculitis. Early treatment with various medications can lead to patient improvement.

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Area of Science:

  • Neurology
  • Rheumatology
  • Vascular Medicine

Background:

  • Scleroderma is a chronic autoimmune disease characterized by hardening and tightening of the skin and connective tissues.
  • Central nervous system (CNS) involvement in scleroderma is rare, often presenting with subtle or nonspecific symptoms.
  • Large vessel vasculitis is an uncommon but serious complication of systemic sclerosis.

Observation:

  • A patient with known scleroderma presented with acute neurological deficits including right hemiparesis, focal seizures, and optic atrophy.
  • Physical examination revealed digital gangrene but no peripheral nerve or muscle involvement.
  • Magnetic Resonance Imaging (MRI) demonstrated multifocal infarcts in both cerebral hemispheres.

Findings:

  • MR angiography indicated compromised blood flow in the bilateral carotid arteries with compensatory collateralization from the posterior circulation.

Related Experiment Videos

  • The patient's neurological symptoms improved with a treatment regimen including phenytoin, nifedipine, antibiotics, and immunosuppressants.
  • The case highlights the potential for significant CNS vascular complications in scleroderma.

    • Implications:

    • This case underscores the importance of considering CNS vasculitis in scleroderma patients presenting with neurological symptoms.
    • Early diagnosis and multimodal treatment are crucial for managing these rare but severe manifestations.
    • Further research into the pathogenesis and optimal management of CNS involvement in scleroderma is warranted.