Case of the Week
Section Editors: Matylda Machnowska1 and Anvita Pauranik2
1University of Toronto, Toronto, Ontario, Canada
2BC Children's Hospital, University of British Columbia, Vancouver, British Columbia, Canada
Sign up to receive an email alert when a new Case of the Week is posted.
July 10, 2014
Neurosyphilis
- Background: Syphilis is classified as primary, secondary, tertiary, or quaternary. Tertiary lesions are caused by obliterative small vessel endarteritis, which usually involves the vasa vasorum of the CNS. Tertiary syphilis comprises 3 types: neurosyphilis, cardiovascular syphilis, and late benign (ie, gummatous) syphilis.
- Neurosyphilis is divided into 2 general categories: (1) early involvement of the CNS limited to the meninges and (2) parenchymal involvement. Pathology of meningovascular syphilis is endarteritis with perivascular inflammation. This causes fibroblastic proliferation of the intima; thinning of the media; fibrous and inflammatory changes in the adventitia; and lymphocytic and plasma cell infiltration.
- Less commonly, neurosyphilis can present as limbic encephalitis. It should be considered a differential diagnosis of mesiotemporal signal changes on MRI.
- Clinical Presentation: The most common presentation of meningovascular syphilis is a stroke syndrome in a relatively young adult involving the middle cerebral artery (most common) or the branches of the basilar artery (second most common). A subacute encephalitic prodrome is present, with headache, vertigo, and psychological abnormalities. The CSF usually reveals elevated protein levels, lymphocytic pleocytosis, and a glucose level within the reference range.
- When manifesting as a limbic encephalitis (companion case), patients present with acute/subacute behaviour or mood disorders; recent memory or other cognitive deficits such as aphasia; focal seizures; and inflammatory CSF.
- DDx:
- Meningovascular syphilis: Based on presentation. Differential includes other causes of meningitis and etiologies of stroke.
- Mestiotemporal involvement: Same imaging features as limbic encephalitis. No imaging feature is specific; diagnosis is based on serum and CSF VDRL.
- Rx: Penicillin G (14–21 days). Most patients have only partial recovery, especially those who have symptoms for several months before starting treatment.
Current Issue
Case Collections
