Case of the Month
Section Editor: Nicholas Stence, MD
Children's Hospital Colorado, Aurora, CO
April 2020
Next Case of the Month Coming May 5...
Unilateral Lymphocytic Vasculitis
- Background:
- Rare and severe disease
- Mean age is 50 years
- Men are affected twice as often as women.
- Lymphocytic vasculitis (lymphocytic angiitis) is a subtype of primary angiitis of the central nervous system (PACNS).
- The etiology is unknown, although there is an association with viral infections and lymphoma.
- Clinical Presentation:
- Headache and encephalopathy are most frequent.
- Less than 20% present with stroke symptoms.
- Key Diagnostic Features:
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MRI can show nonspecific findings, and usually not all signs are visible.
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MRI findings are usually bilateral, although extensive unilateral cases have been described.
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Possible findings are:
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Confluent white matter abnormalities, vasogenic edema
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Infarcts, usually in multiple vascular territories
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Enhancing cortical “masses”
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Peripheral distribution, with sparing of the brain stem and cerebellum
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Subarachnoid and intraparenchymal hemorrhages
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"Beading" of vessels/multiple focal regions of vessel narrowing
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- Histopathology
- Can show inflammation and necrosis of blood vessel walls and perivascular lymphocytic infiltration
- Differential Diagnoses
- Infarction
- Mitochondrial encephalopathy, lactic acidosis, and strokelike episodes (MELAS)
- Reversible cerebral vasoconstriction syndrome
- Septic embolisms
- Encephalitis
- Other vasculitides
- Malignant tumor
- Treatment:
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Prednisone infusion of 1000 mg/day (3 days), followed by oral prednisone in decreasing dosages
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Monthly cyclophosphamide infusion (6 months)
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Without treatment, the disease is usually progressive and might be fatal.
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