Sneddon's syndrome with granulomatous leptomeningeal infiltration.
BACKGROUND
There is limited neuropathologic information available from cases of Sneddon's syndrome in which strokes are associated with livedo reticularis. Pathogenesis of the syndrome is controversial, although current opinion favors a coagulopathy, often with antiphospholipid antibodies. We describe a case lacking antiphospholipid antibodies but having a granulomatous infiltration of the leptomeninges.


METHODS
The patient presented at age 29 with stroke, livedo reticularis, essential hypertension, and Raynaud's phenomenon. Assessment uncovered no underlying disease, including absent antiphospholipid antibodies. A leptomeningeal biopsy showed granulomatous infiltration.


CONCLUSIONS
The findings suggest that an inflammatory process plays a role in at least some cases of Sneddon's syndrome.
Boortz-Marx
R L
RL
Department of Neurology, Hennepin County Medical Center, Minneapolis, MN 55415.
Clark
H B
HB
Taylor
S
S
Wesa
K M
KM
Anderson
D C
DC
D000328:Adult; D001099:Arachnoid / Q000473:pathology*; D002493:Central Nervous System Diseases / Q000473:pathology; D002544:Cerebral Infarction / Q000473:pathology*; D005260:Female; D006099:Granuloma / Q000473:pathology*; D006801:Humans; D006973:Hypertension / Q000473:pathology; D010841:Pia Mater / Q000473:pathology*; D011928:Raynaud Disease / Q000473:pathology; D017445:Skin Diseases, Vascular / Q000473:pathology*; D013577:Syndrome