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An Important Statement From the ASAP Medical Advisory Board:



The hindbrain herniation syndrome or the so-called Chiari I malformation was initially thought to occur in approximately 1 in 2,000 individuals.  Epidemiological survey has shown that this is probably even much more common.  The familial incidence is approximately 6-7%.

The abnormality consists of a downward descent of the lower portion of the cerebellum called the cerebellar tonsils.  In this case, the tonsils reside more than the accepted 3-4 mm below the exit of the skull and as such may cause abnormal changes that reflect compression of the brain stem and abnormal brain fluid dynamics.  This is a not a fatal condition.  The surgical treatment is very satisfactory. 

In the very young, failure to thrive, headaches and scoliosis are the major presenting symptoms.  In later life, as the child gets older, scoliosis and other symptoms are evident.  We would refer you to the ASAP.org list of symptoms.  Only in unusual circumstances such as serious head or neck injury could the Chiari I abnormality lead to a serious situation. 

Every neurosurgical training program in the United States addresses this issue.  Most neurosurgeons as well as neurologists are familiar with the symptoms, the signs and the recognized treatment. 

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