CEREBRAL AUTOSOMAL DOMINANT ARTERIOPATHY WITH SUBCORTICAL INFARCT AND LEUCOENCEPHALOPATHY (CADASIL) SYNDROME. A. Alghadhan 1 , A. Hamad 2 ; 1 SUNY at Stony Brook, Port Jefferson, NY; 2 NUMC, Eastmeadow, NY LEARNING OBJECTIVES: -recognize cerebral autosomal dominant arteriopathy with subcortical infarct and leucoencephalopathy (CADASIL) in the differential diagnosis for normal pressure hydrocephalus. CASE INFORMATION: 53 years old white female presented with progressive cognitive dysfunction and dementia, gait disturbance, urinary incontinence over the last 3 years. She has long standing history of migraine. 3 years ago, she had a computed tomography (CT) of the brain that showed dilated ventricles and labeled as normal pressure hydrocephalus and the patient refused treatment. Family history was significant for early dementia and migraine in the mother. Her physical exam showed severe dementia, severe ataxia on standing or walking despite normal muscular strength. Sensory exam was normal. Laboratory tests were unremarkable. Head CT and MRI showed severe atrophy with severe dilated ventricles and severe periventricular white matter disease. Skin biopsy showed characteristics PAS positive granular material in the media and narrowing of the lumen of the vessels which is typically seen in cerebral autosomal dominant arteriopathy with subcortical infarct and leucoencephalopathy (CADASIL). The patient was placed in a nursing home after explaining the nature of the disease to the family. DISCUSSION: CADASIL can be considered as a new disease that had been described recently (1996) in few families. It predominantly affects the small vessels of the brain. The transmission is autosomal dominant linked to chromosome 19. The clinical features include migraine with or without aura (the gene for familial type of migraine is located on the same chromosome), strokes or stroke-like episodes, psychiatric symptoms and dementia. In our case the presentation was similar to the normal pressure hydrocephalus symptoms. We want physicians to be aware of this similarity as the management is completely different. year-old women presented to the hospital with nonhealing ulcers on her breast, thighs and toes. She developed systemic lupus at a young age that ultimately caused complete renal failure. She underwent renal transplant; however, she reinitiated hemodialysis after failure of the transplant. Her examination revealed necrotic ulcers in the left upper breast region. Radiologic evaluation of the breast, hands and chest revealed significant calcifications throughout the body. This pattern revealed systemic calciphylaxis. She underwent debridement and nerve block for pain control. She was dismissed; however, she eventually died three months after dismissal. DISCUSSION: Nonhealing ulcers are a common source of morbidity in hospitalized patients. Common etiologies include ischemia from vascular causes, chronic edema, and small vessel disease. Uncommon etiologies like calciphylaxis differ from traditional chronic ulcers. The skin appears violaceous with a plaque-like or a nodular appearance. The ulcers cause extreme pain independent of size or appearance. The pathogenesis of calciphylaxis remains unknown. Biochemical tests often reveal abnormal calcium or phosphorus levels. Treatment options often include subtotal parathyroidectomy in some patients. Normalization of calcium and phosphorus remains the primary focus of medical treatment. The chronic wounds are often aggressively de Âbrided and antibiotics are used in cases with infections. The use of immunosuppression for calciphylaxis has had mixed results. Despite aggressive wound care and calcium management, the prognosis of calciphylaxis remains poor at 60% morality. Further research needs to be performed on this uncommon yet potentially lethal illness.