Arnold Chiari Malformation: Difference between revisions
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Arnold Chiari Malformation, AKA Chiari type II malformation, is one of a group of brain malformations affecting the cerebellum. | Arnold Chiari Malformation, AKA Chiari type II malformation, is one of a group of brain malformations affecting the cerebellum. | ||
They are named after Hans Chiari, the pathologist who first described the whole group of malformations, and another pathologist, Julius Arnold<ref>Schijman (2004). "History, anatomic forms, and pathogenesis of Chiari malformations". Child's nervous system 20 (5): 323</ref>. | They are named after Hans Chiari, the pathologist who first described the whole group of malformations, and another pathologist, Julius Arnold<ref>Schijman (2004). "History, anatomic forms, and pathogenesis of Chiari malformations". Child's nervous system 20 (5): 323</ref>. | ||
It is | It is almost always associated with myelomeningocele. | ||
== <span>C</span>linically Relevant Anatomy<br> == | == <span>C</span>linically Relevant Anatomy<br> == | ||
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Normally the cerebellum and parts of the brain stem sit in an indented space at the lower rear of the skull, above the foramen magnum (a funnel-like opening to the spinal canal). When part of the cerebellum is located below the foramen magnum, it is called a Chiari malformation.<br> | Normally the cerebellum and parts of the brain stem sit in an indented space at the lower rear of the skull, above the foramen magnum (a funnel-like opening to the spinal canal). When part of the cerebellum is located below the foramen magnum, it is called a Chiari malformation.<br> | ||
This condition has skull, dural, brain, spinal, and spinal cord manifestations, including downward displacement of the medulla, fourth ventricle, and cerebellum into the cervical spinal canal, as well as elongation of the pons and fourth ventricle, probably due to a relatively small posterior fossa<ref>Truivit CL, Backovich A. Disorders of brain development. In: Atlas SW, ed. Magnetic Resonance Imaging of the Brain and Spine. 2nd ed. Philadelphia, Pa: Lippincott-Raven; 1996.</ref>. | |||
The brainstem is elongated and displaced into the opening of the base of the skull and into the top of the spinal canal<ref>Stevenson KL. Chiari Type II malformation: past, present, and future. Neurosurg Focus. Feb 15 2004;16(2):E5.</ref>. It is often kinked. The brainstem, cranial nerves and lower portion of the cerebellum may be stretched or compressed. This means that any of the functions controlled by these areas may be affected. | |||
== Clinical Presentation == | == Clinical Presentation == | ||
Many people with Arnold Chiari malformation have no obvious symptoms. | |||
In babies, the most common symptoms are a weak or absent cry, stridor (noisy breathing), arching of the neck, breathing problems, colour change, failure to thrive and feeding or swallowing difficulties.<br> | |||
The blockage of cerebrospinal fluid (CSF) flow may also cause a syrinx to form, eventually leading to syringomyelia. Central cord symptoms such as hand weakness, dissociated sensory loss, and, in severe cases, paralysis may occur. | |||
== Diagnostic Procedures == | == Diagnostic Procedures == | ||
add text here relating to diagnostic tests for the condition<br> | add text here relating to diagnostic tests for the condition<br> | ||
== Differential Diagnosis<br> == | == Differential Diagnosis<br> == | ||
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== Resources <br> == | == Resources <br> == | ||
add appropriate resources here | add appropriate resources here | ||
== Recent Related Research (from [http://www.ncbi.nlm.nih.gov/pubmed/ Pubmed]) == | == Recent Related Research (from [http://www.ncbi.nlm.nih.gov/pubmed/ Pubmed]) == | ||
Revision as of 01:21, 17 September 2014
Original Editor - Wendy Walker
Lead Editors
Introduction[edit | edit source]
Arnold Chiari Malformation, AKA Chiari type II malformation, is one of a group of brain malformations affecting the cerebellum.
They are named after Hans Chiari, the pathologist who first described the whole group of malformations, and another pathologist, Julius Arnold[1].
It is almost always associated with myelomeningocele.
Clinically Relevant Anatomy
[edit | edit source]
Normally the cerebellum and parts of the brain stem sit in an indented space at the lower rear of the skull, above the foramen magnum (a funnel-like opening to the spinal canal). When part of the cerebellum is located below the foramen magnum, it is called a Chiari malformation.
This condition has skull, dural, brain, spinal, and spinal cord manifestations, including downward displacement of the medulla, fourth ventricle, and cerebellum into the cervical spinal canal, as well as elongation of the pons and fourth ventricle, probably due to a relatively small posterior fossa[2].
The brainstem is elongated and displaced into the opening of the base of the skull and into the top of the spinal canal[3]. It is often kinked. The brainstem, cranial nerves and lower portion of the cerebellum may be stretched or compressed. This means that any of the functions controlled by these areas may be affected.
Clinical Presentation[edit | edit source]
Many people with Arnold Chiari malformation have no obvious symptoms.
In babies, the most common symptoms are a weak or absent cry, stridor (noisy breathing), arching of the neck, breathing problems, colour change, failure to thrive and feeding or swallowing difficulties.
The blockage of cerebrospinal fluid (CSF) flow may also cause a syrinx to form, eventually leading to syringomyelia. Central cord symptoms such as hand weakness, dissociated sensory loss, and, in severe cases, paralysis may occur.
Diagnostic Procedures[edit | edit source]
add text here relating to diagnostic tests for the condition
Differential Diagnosis
[edit | edit source]
add text here relating to the differential diagnosis of this condition
Key Evidence[edit | edit source]
add text here relating to key evidence with regards to any of the above headings
Resources
[edit | edit source]
add appropriate resources here
Recent Related Research (from Pubmed)[edit | edit source]
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References[edit | edit source]
References will automatically be added here, see adding references tutorial.
- ↑ Schijman (2004). "History, anatomic forms, and pathogenesis of Chiari malformations". Child's nervous system 20 (5): 323
- ↑ Truivit CL, Backovich A. Disorders of brain development. In: Atlas SW, ed. Magnetic Resonance Imaging of the Brain and Spine. 2nd ed. Philadelphia, Pa: Lippincott-Raven; 1996.
- ↑ Stevenson KL. Chiari Type II malformation: past, present, and future. Neurosurg Focus. Feb 15 2004;16(2):E5.
