Rare Case of AAV in Central Nervous System Led to Woman’s Stroke

Woman, 71, had four-day history of chest pain, lethargy, and limb weakness

Margarida Maia, PhD avatar

by Margarida Maia, PhD |

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An elderly woman was diagnosed with ANCA-associated vasculitis (AAV) after having an ischemic stroke, a condition wherein a blood clot cuts off blood supply to a part of the brain, a case study reported.

It’s rare that AAV manifests in the central nervous system, which is made up of the brain and spinal cord, making the case is “an important reminder that acute ischemic stroke can be the presenting manifestation of AAV,” wrote the researchers in their report, “Ischaemic stroke as the first presentation of antineutrophilic cytoplasmic autoantibody-associated vasculitis,” which was published in Clinical Case Reports.

AAV encompasses a group of autoimmune disorders marked by blood vessel inflammation. It’s driven by specialized white blood cells called neutrophils that are wrongly activated and primed to attack blood vessels by self-reactive antibodies that patients produce.

An ischemic stroke occurs when blood clots prevent part of the brain from getting enough blood supply, causing tissue damage due to lack of oxygen and nutrients.

Fewer than 15% of people with AAV have involvement of the central nervous system, but those with an AAV diagnosis are at higher risk of having a stroke or other diseases involving the heart or blood vessels.

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A ‘challenging’ AAV diagnosis

However, “the diagnosis of [AAV] in first-episode strokes is particularly challenging, especially in patients lacking features of systemic vasculitis,” wrote the researchers who described the case of a 71-year-old woman who had an ischemic stroke that turned out to be the first symptom of AAV.

The woman presented with a four-day history of chest pain, lethargy (lack of energy), and limb weakness. A CT scan of the brain was normal, but her chest pain and limb weakness worsened two days later.

She scored 15 points on the Glasgow Coma Scale, which measures a person’s level of consciousness after a trauma or injury, meaning she was fully awake and responsive, but required a walker to move about.

On examination, she had dysarthria (difficulty speaking) and the left side of her tongue was weak. She also had severe limb weakness and hyperreflexia (overactive reflexes). Plantar reflexes were upward in both feet, not downward as they are normally.

Blood tests revealed she had eosinophilia, or too many eosinophils — a type of white blood cell. An MRI scan revealed widespread white matter lesions, a sign of inflammation, in both sides of the brain’s outer and inner layers.

These white matter lesions were visible alongside microhemorrhages (small bleeds) and “consistent with features of stroke secondary to vasculitis,” the researchers wrote.

Further blood tests were positive for anti-myeloperoxidase (MPO) antibodies, a type of self-reactive antibody known to drive AAV. She had no other AAV symptoms, such as skin, kidney, or lung involvement, however.

“Without the classical disease course and clinical signs,” her diagnosis was challenging, the researchers noted.

She started treatment with high-dose glucocorticoids and cyclophosphamide to push the disease into remission. Because these medications work by keeping the immune system in check, she was given antibiotics to prevent an infection.

To maintain remission, treatment was switched to the immunosuppressant azathioprine, and glucocorticoids and cyclophosphamide were gradually reduced and withdrawn. She was started on aspirin and atorvastatin to prevent another ischemic stroke.

Her symptoms eased and she was able to move about independently after two weeks of rehabilitation. She continued to be monitored regularly at the time of the report being written.

“Vasculitis is a rare cause of stroke and therefore is easily missed,” the researchers wrote. “Untreated, the risk of recurrent stroke is extremely high; thus, early diagnosis and treatment are mandatory to minimize disability and death.”