The natural course of cerebral lesions in Sneddon syndrome
A. Tourbah, J. C. Piette, M. T. Iba-Zizen, O. Lyon-Caen, P. Godeau and C. Frances
Federation de Neurologie, Groupe Hospitalier Pitie-Salpetriere, Paris, France.
OBJECTIVES: To characterize the clinical, biological, and neuroradiological
findings in Sneddon syndrome; to correlate magnetic resonance imaging
abnormalities with disability, presence of hypertension and other vascular
risk factors, presence of heart valvulopathy on echography, and titer of
antiphospholipid antibodies; and to compare these findings in
antiphospholipid-positive and antiphospholipid-negative patients. DESIGN:
Retrospective review of the records of 32 consecutive patients with livedo
reticularis and neurological events, followed up in our institution between
January 1991 and August 1995. PATIENTS: Twenty-six patients (20 women and 6
men) who had at least 1 cerebral ischemic arterial event associated with
generalized and pathological livedo reticularis. RESULTS: The age at the
first cerebral ischemic event ranged from 22 to 58 years. Motor deficit was
the most frequent sign (found in 73% of cases). Disability was found in
50%, systemic hypertension in 65%, heart valvulopathy in 61%, and
antiphospholipid antibodies in 42% of cases. Patients were classified in 6
groups according to magnetic resonance imaging findings. No correlation was
found between the presence of hypertension or other vascular risk factors,
valvulopathy, antiphospholipids, and magnetic resonance imaging
abnormalities. There was no significant difference between
antiphospholipid-positive and antiphospholipid-negative patients except for
the presence of antinuclear antibodies. There was a significant correlation
between the extent of magnetic resonance imaging abnormalities and
disability. CONCLUSION: The severity of the disease seems to be correlated
with magnetic resonance imaging aspects, but not to the presence of
antiphospholipid antibodies. Magnetic resonance imaging may help to
understand the natural course of the cerebral involvement of the disease.
