Abstract Details

Title Mysterious case of recurrent cryptogenic strokes in a young female: A rare case of Parry Romberg Syndrome

Topic Cerebrovascular Disease and Interventional Neurology

Presentation(s) P1 - Poster Session 1 (9:00 AM-5:00 PM)

Poster/Presentation
Number 129

Objective To present a patient with varying neurological manifestations leading to the diagnosis of Parry Romberg syndrome (PRS).

Background Strokes in young adults are uncommon and present a diagnostic challenge. PRS is a rare multisystem connective tissue disorder linked to linear scleroderma with various neurological complications including progressive hemifacial atrophy, headaches, seizures, deafness, trigeminal neuralgia and rarely ischemic strokes.

Design/Methods Case Presentation: 43-year-old right-handed woman with history of scleroderma was seen in vascular neurology clinic for recurrent cryptogenic strokes at the ages of 37 and 42, recurrent episodes of Bell’s palsy involving left and right sides, and focal seizures. Prior MRI brain at the time of stroke showed acute infarcts in the left MCA and PCA distributions. Recent MRI brain showed left hemispheric atrophy with ex-vacuo dilation and encephalomalacia in the left posterior parietal and occipital lobes with punctate areas of contrast enhancement. Work up for cerebral vasculitis including conventional angiogram, serological markers and CSF examination was grossly unremarkable except mildly increased CSF protein. Hypercoagulable workup, echocardiogram and cardiac monitoring were grossly unremarkable. Further examination showed left hemifacial atrophy, and a lesion consistent with linear scleroderma over her chest.

Results In contrast to two cases reported in the literature with known diagnosis of PRS who subsequently had ischemic stroke in their 30’s, here we present a case with initial neurological presentation being recurrent cryptogenic stroke with subsequent diagnosis of PRS based on clinical features and neuroimaging findings.

Conclusions Conclusion:
PRS is a rare disease entity and its neurologic manifestations are often under recognized by the clinicians. Further research is proposed to better understand the mechanisms for the stroke and potential treatment options.

Authors/Disclosures
Vijaya Lakshmi Valaparla, MD PRESENTER	Dr. Valaparla has nothing to disclose.
Xiangping Li, MD, FAAN (University of Texas Medical Branch (UTMB))	Dr. Li has nothing to disclose.
Chilvana V. Patel, MBBS, FAAN	Dr. Patel has nothing to disclose.
Arun S. Chhabra, MD	Dr. Chhabra has stock in Pfizer. Dr. Chhabra has stock in Biogen. Dr. Chhabra has stock in Johnson and Johnson. Dr. Chhabra has stock in Medtronic.
Hashem Shaltoni, MD	Dr. Shaltoni has nothing to disclose.
Anand V. Patel, MD (University of Texas Medical Branch)	Dr. Patel has nothing to disclose.

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