Summary
Definition
History and exam
Key diagnostic factors
- numbness
- weakness
- pain
- sicca symptoms
- parotid gland enlargement
- rash, ulcerations, or pigment changes
- wheeze, cough, other pulmonary signs
- fever, weight loss, and malaise
Other diagnostic factors
- predisposing conditions causing vasculitis, inflammation, or other nerve damage
Risk factors
- age over 50 years
- sarcoidosis
- hepatitis C
- cryoglobulinemia
- hepatitis B
- connective tissue disease
- primary vasculitis
- drug use
- HIV infection
- non-HIV, non-hepatitis infections
- recreational intravenous drug use
- genetic predisposition
Diagnostic investigations
1st investigations to order
- electromyogram (EMG)
- CBC with differential
- erythrocyte sedimentation rateĀ (ESR)
- C-reactive protein (CRP)
- serum creatinine
- serum glucose
- cryoglobulins
- serum complement
- hepatitis B surface antigen
- hepatitis C antibodies or RNA
- anti-HIV antigens or HIV RNA
- Lyme disease antibodies
- cytoplasmic and perinuclear antineutrophil cytoplasmic antibodies (c-ANCA and p-ANCA)
- rheumatoid factor
- antinuclear antibodies (ANA)
- anti-double-stranded (ds) DNA
- anti-SSA or SSB antibodies
- serum angiotensin-converting enzyme
- protein electrophoresis and immunofixation
- chest x-ray
- urinalysis
- muscle and nerve biopsy
Investigations to consider
- anti-Smith (anti-Sm) antibodies
- anti-topoisomerase I (anti-Scl 70) and anti-centromere (ACA) antibodies
- skin biopsy
- lip biopsy
- anti-Hu antibodies
- cerebrospinal fluid analyses
- CT of chest, abdomen/pelvis
- positron emission tomography (PET) scan of chest, abdomen, or pelvis
- conventional angiography
- magnetic resonance angiography
Treatment algorithm
Contributors
Authors
Clinical Neurologist
Colorado Springs Neurological Associates
Colorado Springs
CO
Disclosures
KRS declares that he has no competing interests.
Professor of Neurology
Penn State College of Medicine
Hershey
PA
Disclosures
MJK declares that he has no competing interests.
Dr Kevin Scott and Dr Milind Kothari would like to gratefully acknowledge Dr Jenice Robinson, the previous contributor to this topic. JR declares that she has no competing interests.
Peer reviewers
Professor and Chairman
Department of Neurology
The George Washington University Medical Center
Washington
DC
Disclosures
JJK declares that he has no competing interests.
Assistant Professor of Neurosciences
Hotchkiss Brain Institute
University of Calgary
Alberta
Canada
Disclosures
CT declares that he has no competing interests.
Consultant Neurophysiologist
East Kent Hospitals NHS Trust
Canterbury
Kings College Hospital NHS Trust
London
UK
Disclosures
JB declares that he has no competing interests.
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