3448 Stroke November 2014 examination, suggesting CARASIL. The patients do not have hypertension or diabetes mellitus, which are the major risk factors for sporadic CSVD. Diffuse thinning of hair without hairline recession beginning during the teenage years or when patients are in their 20s has been recognized in 9 of 12 families (mean age, 16.7 years; range, 0–27 years). Pubic hair loss and body hair loss have not been reported. Acute mid-to lower-back pain has been noticed at a mean age of 24.9 years (range, 14–39 years). Mood changes (apathy and irritability), pseudobulbar palsy, hyper-reflexia, Babinski sign, and urinary incontinence are frequently observed. Motor and cognitive functions slowly decline, and 7 of 13 patients needed wheelchairs by 30 to 40 years of age. An acute ischemic stroke event has been reported in 23.1%, and no hemorrhagic stroke events have been reported. Five of 13 patients (38.5%) have experienced horizontal nystagmus. Two of 13 patients (15.4%) have experienced advanced-stage seizures. Obvious migraines have not been reported in these patients, and there has been no skin color change in the extremities. Retina and kidney involvement have not been reported; however, in other small vessel diseases, involvement of retinal vessels or involvement of renal dysfunction has been reported.