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Diagnosis: Chiari Malformation

Arnold-Chiari malformation is a neurological condition that causes compression of the brainstem and the spinal cord at the base of the skull. Symptoms of Chiari malformation, as it is commonly known, include headache, neck pain, fatigue, swallowing difficulties and numbness and tingling of the extremities. It is diagnosed with a special magnetic resonance imaging (MRI) scan.

Chiari, CFIDS and FM
Some have speculated that Chiari malformations may be responsible for some cases of chronic fatigue and immune dysfunction syndrome (CFIDS) and fibromyalgia (FM) because of their similar symptoms. Others believe that some people with Chiari malformations have been misdiagnosed with CFIDS or FM because standard MRI films only pick up the most obvious Chiari malformations, when lesser degrees of compression might also cause symptoms. It has not been suggested that Chiari malformation is a cause of CFIDS in general, rather that it may contribute to CFIDS symptoms or be misdiagnosed as CFIDS or FM.

Characteristics
Chiari malformation is a structural abnormality in which the bony area at the base of the skull (the posterior fossa) is too small to comfortably contain the cerebellum and the spinal cord. Cervical stenosis, a related condition, involves compression of the upper spinal column, and can occur alone or in association with a Chiari malformation. In both cases, important tissues are squeezed, causing pain and impeding the transfer of signals between the brain and the body.

Diagnosis
The development of MRI scans has greatly improved the diagnosis of Chiari malformation, as neurologists and radiologists can physically see the compression on film. Chiari is readily diagnosed when the cerebellar tonsils extend several millimeters below the posterior fossa into the spinal column. Confusion occurs when a patient with symptoms of Chiari has a lesser degree of cerebellar herniation. Because some patients with a Chiari malformation on MRI have no abnormalities on the neurological examination and few symptoms, seeing a Chiari malformation on the MRI does not necessarily indicate a need for treatment. Physicians generally obtain an MRI to look for Chiari malformation only if symptoms and physical findings on the neurological examination are potentially consistent with this disorder.

Treatment
Surgery to create more space at the base of the skull and relieve pressure is the standard treatment for Chiari malformation. Techniques for doing this differ among neurosurgeons and the optimal method for treating Chiari malformation is the subject of debate. 

As with all surgeries, patients should discuss the pros and cons carefully with their doctors. This is especially true when considering a form of brain surgery. CFIDS patients who have undergone decompression surgery have reported mixed results , with some experiencing resolution of many symptoms, others showing little to no improvement and occasional patients reporting worsening symptoms. 

Other resources
From The CFIDS Chronicle: