A 56-year-old man comes to your office for two “spells” he’s had in the past three days, each lasting approximately 20-30 seconds before resolving spontaneously. The first was blurry vision in both eyes without other vision changes. He had just woken up at the time, but states that the blurry vision was very different from the experience of just being “sleepy.” The second spell consisted of tingling in his left hand. He denies that he had any weakness or change in speech or balance. He feels back to normal currently. Show His physical exam is normal so you make a plan for outpatient work-up with MR brain, EKG, laboratory studies, and a carotid duplex, thinking that the episodes were likely too brief and non-specific to represent a true transient ischemic attack (TIA). Before he leaves the office, he experiences another episode of the same blurry vision that he had a few mornings ago. You re-enter the exam room and observe left beating horizontal nystagmus in neutral gaze, which was not there previously. On rightward gaze the nystagmus becomes rotatory. He also tells you that now he has non-pulsatile ringing in his right ear, which also just started. You send him to the ED by ambulance and visit him after the initial workup is done. The Figure shows a cut from the MRI obtained in the ED. What does the MRI reveal? [[{"type":"media","view_mode":"media_crop","fid":"59730","attributes":{"alt":"","class":"media-image","id":"media_crop_5190071503385","media_crop_h":"0","media_crop_image_style":"-1","media_crop_instance":"7528","media_crop_rotate":"0","media_crop_scale_h":"0","media_crop_scale_w":"0","media_crop_w":"0","media_crop_x":"0","media_crop_y":"0","style":"width: 204px; height: 259px;","title":" ","typeof":"foaf:Image"}}]] Jan Bailey was an energetic 70-year-old who enjoyed hiking, biking and cross-country skiing — strenuous activities that kept her young as she embraced retirement with her husband, Wally.MORE FROM MICHIGAN: Sign up for our weekly newsletter So nothing could have prepared the Northville, Michigan, couple for the next few years as Bailey, now 73, experienced ongoing health issues. The confusion was compounded because the condition was not immediately diagnosed. It all started in the fall of 2014. Bailey awoke very early with severe vertigo. Feeling unstable and nauseous, she was taken by ambulance to the emergency room of the closest hospital. She was examined, given medication to help alleviate her vertigo and sent home. Still experiencing vertigo, her primary doctor sent her to an ear, nose and throat specialist who diagnosed her with Ménière’s disease, an inner ear condition that causes dizziness, hearing loss and ringing in the ears, known as tinnitus. When Bailey lost feeling in her left foot in April 2015, she knew her symptoms were the result of something more serious. Taken to a nearby emergency room, she was diagnosed with an ischemic stroke and put on blood thinners to prevent a recurrence. Spotting recurrent strokeRecurrent episodes of weakness in her legs, arms and face, along with loss of balance, led Bailey to Michigan Medicine, where a team of neurologists performed a number of tests to determine the exact cause of the strokes she was experiencing. SEE ALSO: Think You’re Having a Stroke? Every Minute Counts Under the care of vascular neurologist Mollie McDermott, M.D., Bailey ultimately had a brain biopsy in March 2016, which revealed primary central nervous system (CNS) vasculitis as the cause of her issues. CNS vasculitis is a rare disease that causes inflammation of the small arteries and veins in the brain or spinal cord, which make up the CNS. The inflamed vessel wall can block the flow of oxygen-containing blood to the brain, resulting in a loss of brain function or stroke. The disease affects fewer than three individuals per 1 million, regardless of sex or age, and is associated with an overactive immune system that attacks the blood vessels in the brain. Symptoms of CNS vasculitis can include:
“Most patients diagnosed with this rare condition are treated collaboratively with neurology, rheumatology and neurosurgery,” says McDermott. “And, like Jan, most are put on immunosuppressant drugs. “In Jan’s case, CNS vasculitis led to dozens of strokes and sensory abnormalities in her trunk, arms and legs,” McDermott says, noting that the outcomes of patients with CNS vasculitis are typically poor and include limb weakness, gait disturbance and significant cognitive issues. “Jan, however, has done very well, in part because she’s incredibly motivated to stay active and participate in physical therapy. She’s been working very hard.” One long-lasting effect: Bailey has experienced a loss of proprioception (position sense) in her legs. “My feet don’t always know where they are and what they’re doing,” she says. But she isn’t giving up. The retired engineer works out at a nearby gym for two hours, twice a week, with a goal of maintaining her balance and mobility. She says she’s fortunate to have a committed neurologist for her medical care and a dedicated husband of 48 years by her side for moral and physical support.
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