Most individuals with DADA2 experience strokes in infancy or early childhood. DADA2 was discovered by researchers at the National Institutes of Health ( NIH ). If untreated, the disorder is often fatal, ending in failure of vital organs.ĭeficiency of adenosine deaminase 2 ( also known as DADA2)ĭADA2 is a rare, genetic form of vasculitis that is caused by a mutation in the CECR1 gene and can occur in more than one family member. In some instances, the disease can recur after a few years. Damage to the peripheral nerves with neuropathy is more common than damage to the brain and spinal cord tissue. As the disease progresses the kidneys may fail and high blood pressure may develop rapidly. The onset of this rare disease can occur at any age but most often appears between the ages of 40 and 60. Most individuals with the disorder are female, with disease onset between the ages 15 and 40. The disorder can cause partial to complete disability and can be fatal if left untreated. It also may cause heart attacks and damage to the intestines. This disease affects large arteries such as the aorta. It may cause strokes, headaches, dizziness, a feeling of cold or numbness in the limbs, problems with memory and thinking, and visual disturbances. The disorder can affect anyone of any age but peaks about age 50 and is most often seen in males. It is fatal if left untreated. This rare disorder, which is confined to the brain and spinal cord, typically develops slowly and includes headache, dementia, behavioral changes, pain, sensory abnormalities, and tremor. Stroke, transient ischemic attack, multiple mini-strokes, and seizures can occur. Primary angiitis of the central nervous system (also known as granulomatous angiitis)

Tenderness in the temporal arteries or the scalp.

Pain in the jaw or tongue when chewing or swallowing.

Visual problems, including blurred or double vision, or sudden vision loss.

Untreated temporal arteritis can cause strokes and even death. Giant cell arteritis affects the aorta, which brings blood to every branch of the body, including the arms, legs, and head. The temporal artery (found on both sides of the head and running across the temple) and the ophthalmic artery that supplies the eyes are often affected. Giant cell arteritis typically occurs in people age 50 years and older. Giant cell arteritis (also known as temporal arteritis or cranial arteritis) Many forms of vasculitis can affect the brain, spinal cord, and nerves, including:

Problems with the kidneys or other organs.

Blood in the inflamed blood vessel can clot (thrombosis), blocking blood flow and causing ischemic stroke.

Cerebral aneurysms (weak spots on a blood vessel in the brain that balloon out) can burst and spill blood into surrounding tissue (hemorrhagic stroke).

How vasculitis in the brain leads to stroke:

Vasculitis can cause strokes, blood clots, swelling, and other conditions that affect the brain and nervous system and other organs. In the brain and elsewhere, the weakened vessel can burst, causing bleeding into surrounding tissues.

Inflammation from vasculitis can cause the walls of blood vessels to weaken, stretch, thicken, and swell or develop scarring, which can narrow the vessel and slow or completely stop the normal flow of blood. Vasculitis can affect blood vessels of any type, size, or location, including those in the central and peripheral nervous systems, where it affects the blood vessels that nourish the brain, spinal cord, and peripheral nerves. A vasculitis syndrome may begin suddenly or develop over time. Vasculitis is inflammation of blood vessels (veins, arteries, and capillaries) that carry blood through the body. What is vasculitis in the nervous system?

Charge syndrome blood vessels