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Chiari Malformation

Chiari Malformation is a cause of recurrent, severe headaches, usually provoked by cough or straining or exercise. The presentation can, rarely, be more dramatic with Thunderclap Headache.

A recent case report describes the Neck-Tongue Syndrome in association with a type I Chiari.

The diagnosis is usually made on an MRI brain scan, which shows that the cerebellar tonsils (the lowest lying part of the brain within the skull) have fallen further down into the hole at the base of the skull called the foramen magnum (foramen = hole, magnum = big).

Surgery to free up space at the foramen magnum is usually performed if headache can be attributed to crowding at the foramen magnum.

An potential cause of an apparent malformation is Spontaneous Intracranial Hypotension, and this should be considered if symptoms suggest that intracranial pressure is low - for example postural headache, tinnitus.

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