Abstract Details

Title Ischemic Stroke in Patients with Scleroderma: Case Reports and Review of the Literature

Topic Neurotoxicology

Presentation(s) P06 - (-)

Poster/Presentation
Number 220

Objective

Background BACKGROUND: Scleroderma or progressive systemic sclerosis (PSS) is a rare disease of unknown etiology resulting in increased collagen deposition and dermal thickening. Stroke or TIA are rare manifestations of progressive systemic sclerosis.

Design/Methods DESIGN/METHODS: Retrospective chart review of patients with scleroderma and stroke.

Results RESULTS: Patient 1: A 39-year-old woman with a 1-year history of scleroderma presented with right arm weakness and sensory symptoms. MRI of the brain showed an acute infarction in the left middle cerebral artery territory. MRA and CTA of the head and neck showed moderate stenosis of the supraclinoid portion of the left internal carotid artery (ICA). Patient 2: A 58-year-old woman with a 15-year history of scleroderma was admitted with left arm and leg numbness and left facial weakness. Initial NIHSS was 2. MRI of the brain showed a right internal capsule infarct. MRA of the head and neck showed no flow limiting stenosis. A surface echocardiogram showed no cardioembolic source. Both patients were started on an anti platelet agent and a statin for secondary stroke prevention.

Conclusions CONCLUSIONS: CNS involvement in scleroderma appears to be a rare phenomenon. Due to a paucity of fibrous tissue, vascular and other immune mechanisms may play an important role in CNS involvement in PSS. The putative pathogenesis of cerebrovascular disease is endothelial changes in the setting of microangiopathy leading to fibrosis and increased tendency for platelet aggregation eventually causing tissue ischemia. The damage to the vascular endothelium decreases the production of prostacyclins, and eventually leads to platelet thrombi formation due to unopposed thromboxane A2 action that resembles the "local factor" theory of Raynaud's phenomenon. More data is needed to determine whether scleroderma is causal in the development of stroke; to better determine the optimum treatment plan for secondary stroke prevention.

Authors/Disclosures
Keyur Patel, MD PRESENTER	No disclosure on file
Muhib Khan, MD, FAAN (Mayo Clinic)	The institution of Dr. Khan has received research support from Mayo Clinic ��ɫ�� Grant . The institution of Dr. Khan has received research support from Mayo Clinic Small Grants .
Brian Silver, MD, FAAN (UMass Memorial Medical Center)	Dr. Silver has received personal compensation in the range of $5,000-$9,999 for serving as an Expert Witness for Various legal firms. Dr. Silver has received intellectual property interests from a discovery or technology relating to health care. Dr. Silver has received publishing royalties from a publication relating to health care. Dr. Silver has received publishing royalties from a publication relating to health care. Dr. Silver has received publishing royalties from a publication relating to health care. Dr. Silver has received personal compensation in the range of $500-$4,999 for serving as a Consultant with Women's Health Initiative. Dr. Silver has received personal compensation in the range of $500-$4,999 for serving as a Consultant with Best Doctors, Inc./Teladoc, Inc.. Dr. Silver has a non-compensated relationship as a Consultant with ABPN that is relevant to AAN interests or activities. Dr. Silver has a non-compensated relationship as a Member, Regional Board of Directors with American Heart Association that is relevant to AAN interests or activities.
Stuart D. Cook, MD, FAAN (Rutgers)	No disclosure on file

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