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Abstract Details

Early Onset Cerebral Amyloid Angiopathy in the Context of Perinatal Hydrocephalus and VP Shunt Instrumentation
Cerebrovascular Disease and Interventional Neurology
P9 - Poster Session 9 (5:30 PM-6:30 PM)
CAA occurs when amyloid-beta peptides are deposited in cerebral blood vessels, resulting in recurrent intracerebral hemorrhages (ICH), lacunar infarcts, and microbleeds.

Here, we describe a case of early-onset cerebral amyloid angiopathy (CAA) and review predisposing factors.


A 29-year-old man born with a history of Arnold-Chiari malformation with spina bifida and hydrocephalus, requiring perinatal ventriculoperitoneal shunt placement, and migraine who developed transient paresthesias in the right face and arm numbness prompting stroke workup was found to have a left thalamic lacunar ischemic stroke, microhemorrhages and superficial siderosis in the right temporal/occipital lobes, left frontal lobe and ventriculomegaly on brain MRI. He was discharged from another facility on aspirin and clopidogrel and 4-months later developed a right frontal ICH. He was discharged aspirin, and one year later presented to our facility with a left frontal lobe ICH. The aspirin was discontinued, and he was discharged to rehab. He returned 2-months later with re-bleed in the left frontal lobe.  He is now aphasic, wheelchair-bound, and requires multiple anti-epileptic medications for post-stroke epilepsy. During his multiple hospital admissions, he had an extensive workup including echocardiography, CT angiography, telemetry, MRI brain, MRV, cerebral angiogram, lab workup (serum and cerebral spinal fluid ) that ruled out inflammatory disorders, malignancy, infectious disorders, coagulopathies, and genetic causes of small vessel arteriopathies and CAA.  Brain biopsy demonstrated amyloid-beta deposition with the vessels. Family and other perinatal history were non-contributory.

We diagnosed our patient with probable CAA based on his imaging and brain biopsy results, and hypothesize that his prior perinatal hydrocephalus and neurosurgical instrumentation with the VP shunt placement could have played a role in his development of CAA as a small case series also reported an association of CAA young patients that had prior traumatic brain injury or neurosurgical procedures early in life.

Joshua Augustin Modeste
Mr. Modeste has nothing to disclose.
Isabelle Snider Ms. Snider has nothing to disclose.
Alexis N. Simpkins, MD,PhD,MSCR,FAAN (Cedars-Sinai Medical Center, Dept of Neurology) Dr. Simpkins has received personal compensation in the range of $0-$499 for serving on a Scientific Advisory or Data Safety Monitoring board for National Institute of Neurological Disorders and Stroke Data Safety Monitoring Board. Dr. Simpkins has received research support from NIH/NIA. Dr. Simpkins has received publishing royalties from a publication relating to health care. Dr. Simpkins has received personal compensation in the range of $500-$4,999 for serving as a Speaker with American Heart Association.