A few diseases are sometimes linked with Chiari malformation, such as: 

Aseptic meningitis – Inflammation of the linings of the brain and spinal cord and does not have a bacterial cause. This can occur after any surgery that requires the opening of the brain coverings. During surgery, some blood cells may enter the cerebral spinal fluid. After surgery, as these cells breakdown the products of cellular break down irritate the inside lining of the meninges. This can cause a headache and fever and is treated with steroid therapy. 

Basilar invagination – This is when the upper end of the spine, or C2 vertebra, sticks into the skull. This puts harmful pressure on the brain stem.

Connective tissue disorders – Connective tissue supports many body parts, such as the skin, muscles and ligaments. When connective tissue is faulty, it is usually due to faulty collagen. Collagen is a protein that works like glue in the body. It makes connective tissue strong and stretchy. One of the connective tissue diseases that might cause CM is Ehlers-Danlos Syndrome (EDS). EDS is a genetic disease that makes joints too mobile, skin too stretchy and tissue too fragile. There are six different types of EDS categorized by the symptoms they cause. Treatment includes physical and occupational therapy to learn how to avoid injury.


Dysautonomia - Dysautonomia is a term for a group of diseases that include postural orthostatic tachycardia syndrome (POTS), multiple system atrophy, autonomic failure, and autonomic neuropathy. In these conditions the autonomic nervous system (ANS) does not work properly. Dysautonomia is most often a type of neuropathy affecting the nerves that carry information from the brain and spinal cord to the heart, bladder, intestines, sweat glands, pupils, and blood vessels; although it has many causes, not all of which classify as neuropathic. (Wikipedia)

Hydrocephalus - This occurs when the ventricles of the brain hold too much cerebrospinal fluid (CSF). Treatment includes placing a shunt to drain the fluid.

Intracranial hypertension - This can also be known as pseudotumor cerebri, a condition characterized by CSF pressure in the brain always being too high. It can cause headache, nausea, vomiting and vision problems. The vision problems are due to optic nerve swelling. Optic nerve swelling can be found by an eye exam. A spinal tap measures pressure, but longer-term pressure monitoring may be needed. Surgery and a hospital stay are needed for pressure monitoring. Treatments include medical therapies to reduce CSF production or a shunt to drain CSF.

Myelomeningocele - A birth defect that occurs when the vertebrae do not fully form and remain open allowing the spinal cord to protrude. The condition is also known as a type of spina bifida. Surgical repair is usually required.


Neuropathic pain syndrome - Patients experiencing pain caused by damage to the central nervous system are said to have neuropathic pain syndrome. The symptoms are burning pain and abnormal feelings. Neuropathic pain syndrome is hard to treat, but responds best to medication that treats
neuropathic pain.


Postural orthostatic tachycardia syndrome - (POTS, also known as postural tachycardia syndrome) is a condition in which a change from the supine position to an upright position causes an abnormally large increase in heart rate, called tachycardia. Other symptoms of an orthostatic nature—occurring in response to upright posture—may accompany the tachycardia.


Scoliosis - Scoliosis, a side-to-side curving of the spine, is one of the most common first symptoms in pediatric Chiari patients over 3 years old. Early and quick treatment of CM in a child with fast progressing scoliosis can halt progress of the spine curving. These children also should have an MRI of the spine to assist in ruling out syringomyelia (SM), which may cause scoliosis.

Spina bifida - When a person is born with a spinal canal that did not close during development, he or she is said to have spina bifida. There are many different types of spina bifida, ranging from spina bifida occulta, which is benign, to myelomeningocele, which is severe. Most patients with myelomeningocele will have Chiari II.

Syringobulbia - This is a syrinx, or cyst, in the brain stem.

Syringomyelia - This is a disease in which there is a syrinx, or cyst, in the spinal cord.

Tethered Spinal Cord Syndrome - This is a disorder in which the spinal cord is abnormally attached to a structure within the spine and is causing harmful traction on the spinal cord.


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Cortical Chauvinism

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