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Arnold-Chiari syndrome

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  • Arnold-Chiari syndrome

    Originally posted by Nazari
    Hi I would like to know about Arnold Chiari Malformation please cause my husband has it for the past 18 years and is getting worse, even though he has been for surgery 11 years ago please help. Thanx

    I am not sure what you know or don't know about Arnold Chiari. Can you give me an idea so that I can begin to answer your questions?


  • #2
    What is Arnold-Chiari Syndrome (sometimes called the Chiari malformation)?
    • This syndrome is often described as a developmental abnormality in which the brainstem is compressed by the cerebellum descending into the foramen magnum (the opening of the skull through which the spinal cord passes). I prefer to think of the condition as one in which the flow of cerebrospinal fluid between the brain and spinal cord is blocked, for a variety of reasons. First reported by Dr. Cleland in 1893, the condition was subsequently described by Austrian pathologist (Hans Chiari) and supplemented by his colleague Dr. Julius Arnold. The Arnold-Chiari malformation (ACM) is often categorized into four types, all associated with the development of syringomyelia (or enlargements of the central canal):
    - Arnold Chiari Malformation I (ACM I). The cerebellum descends into the formamen magnum (the opening of the skull through which the spinal cord passes). This may cause compression of the brainstem and the spinal cord. The fourth ventricle is in normal position.
    - Arnold Chiari Malformation II (ACM II). A syndorme that includes herniation of medulla, cerebellar tonsils, aqueductal stenosis, hydrocephalus, hypogenesis of the corpus callosum, and deformities of the skull.
    - Arnold Chiari Malformation III (ACM III). A syndrome of herniated brainstem where the fourth ventricle is below the foramen magnum and is associated with an encephalocele or myelomeningocoele.
    - Arnold Chiari Malformation IV (ACM IV). Hypoplasia of the cerebellum, sometimes associated with an encephacoele.

    What causes ACM?
    • In children, ACM may result from a development abnormality of the skull and spinal column. Because ACM II is often associated with hydrocephalus, where brain pressure increases, some people think that the herniation of the cerebellum into the foramen magnum is caused by the increased pressure. However, any occlusion of cerebrospinal fluid flow between the brain and spinal cord may lead to hydrocephalus. A large volume of cerebrospinal fluid (CSF) passes between the brain and the spinal cord. It is said that as much as a liter of CSF passes from the brain to the spinal cord daily in most people. The CSF goes through several openings from the fourth ventricle (the lowest ventricle that is a continuation of cystern that eventually become the central canal. If there is any occlusion of the openings allow CSF flow into the subarachnoid space, the CSF is then forced to go through the fourth ventricle and the central canal, resulting in enlargement of the central canal in the spinal cord, a condition that is called syringomyelia. Many people with spinal cord injury know about syringomyelia because it develops after spinal cord injury has occluded the CSF flow at and around the injury site.

    What are the consequences and treatment of the Chiari malformation?
    • An ACM is like a high incomplete spinal cord injury, involving the upper spinal cord and lower brainstem. Many people with ACM I are asymptomatic until some situation aggravates the pressure on the brainstem and spinal cord, producing what appears to be a high spinal cord injury. The treatment is surgical, essentially decompression of the upper spinal cord and lower brainstem. The following picture is one of a girl with Crouzon syndrome (where the skull does not develop properly) and she has a Chiari I malformation (arrow show the cerebellar herniation):

    How is Arnold-Chiari malformation diagnosed?
    • ACM manifests with complaints of headache, neck pain, weakness of the arms and hands (due to the spinal cord compression), and difficulty swalling (due to lower brainstem compression). An MRI usually then shows the descent of the cerebellum into the foramen magnum, the presence of a syringomyelic cyst in the brainstem and spinal cord, and other related problems. In adults, the most common associated symptom is severe pain that may or may not be relieved by surgery.

    Is the Arnold-Chiari Malformation a genetic disorder?
    • The Arnold Chiari Malformation is sometimes associated with problems such as spina bifida, skull development problems such as Crouzon's disease (where the plates of the skull may not fuse or fuse prematurely), and meningomyelocoeles (a condition where the spinal column does not completely close and there are abnormalities of both the arachnoid and spinal cord devleopment). Because of these associations, many doctors believe tha condition may have a genetic origin. In my opinion, while there may be some genetic association with spina bifida and related developmental disorders, the unifying cause of all Arnold-Chiari malformations is occlusion of CSF flow between the brain and spinal cord. I believe that the occlusion may occur from genetic, developmental, mechanical, or even infectious origins.

    Neurosurgery Today has a well-written and illustrated article about Arnold-Chiari Malformation
    Last edited by Wise Young; 05-01-2007, 08:38 AM.


    • #3

      If he has had it for 19yrs adn getting worse ,. You need to see a Dr. ASAP .I bet he needs surgery . Have you had MRIs done over the 18yrs years to see how it has advanced .
      SM/ACM Surgery in 1999

      When the world says "give up"; hope whispers "try one more time"