Arnold-Chiari syndrome is a severe neurological disorder where the base of the brain or the cerebellum, extends out of the skull and moves down into the spinal cord, causing increased pressure on both the brain and spine resulting in a variety of symptoms. It is called so because it was initially identified by Hans Chiari in the 1890’s.
What are the other names for Arnold-Chiari syndrome?
- Chiari Malformation (CM)
- Arnold-Chiari Malformation (ACM)
- Tonsillar Herniation
- Tonsillar Ectopia
- Hindbrain Herniation
What are the causes of Arnold-Chiari syndrome?
- Altered Cerebrospinal Fluid Dynamics: This altered CSF dynamics causes brain to descend out of skull such as hydrocephalus or intracranial hypertension.
- Small Posterior Fossa: The most common cause is if the rear of the skull is very small for the normal-sized brain, hernia of the brain results.
- Tethered Cord (still controversial): Atypical anchoring of the spinal cord at the base may also lead to hernia of the brain.
- Mass in the brain: This could displace parts of the brain out through the base of the skull.
- Genetic conditions such as an Ehlers-Danlos variant
What are the symptoms of Arnold-Chiari syndrome?
The chief symptom is a persistent headache at the back of the head precipitated by straining and coughing.Symptoms may develop at any age. However, trauma may play a role in causing symptoms.The symptoms are caused due to compression of brain and spinal tissue, disrupted flow of cerebrospinal fluid and elevated pressure in the brain.Some people with Chiari malformations are asymptomatic, implying they have no symptoms.There may be even cognitive effects of Chiari, but these are not well understood.
How is Arnold-Chiari syndrome diagnosed?
There is no particular, objective, definitive test for symptomatic Chiari. The diagnosis is established on a blend of factors and finally is an informed opinion on whether symptoms seen are due to herniated cerebellar tonsils.
Chiari is often missed and misdiagnosed as other diseases.
The diagnostic tests include-
- MRI
- cine MRI other tests
- Neurological Exam & Symptoms History
What is the treatment of Arnold-Chiari syndrome?
Surgical treatment is the mainstay for Arnold-Chiari syndrome.
- Craniectomy- A piece of the skull is removed, so the brain may be positioned back in its place.
- Laminectomy- Part of one or more vertebrae are removed.
- Duraplasty- The covering of the brain is opened and a patch is sewn in to make it bigger.
- Tissue removal- Cerebellar tonsils are sometimes cauterized.
- Plate insertion- Mesh is inserted where skull was removed.
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Disclaimer: The content provided here is meant for general informational purposes only and hence SHOULD NOT be relied upon as a substitute for sound professional medical advice, care or evaluation by a qualified doctor/physician or other relevantly qualified healthcare provider.
