Case of the Month: June, 2011, Page 2
Answer: Arnold Chiari Malformation Type 1. Also known as Chiari I Malformation
- Pointed cerebellar tonsils ≥ 5 mm below foramen magnum
- (+/-) Syringohydromyelia (14-75%)
- "Tight foramen magnum" with small/absent cisterns
- (+/-) Elongation of fourth ventricle, hindbrain anomalies
- Oblique tonsillar folia (like "sergeant's stripes")
Some people with Chiari-I have no symptoms. But in others, signs and symptoms may include:
- Headaches, especially those brought on suddenly by coughing, sneezing or straining
- Neck pain
- Unsteady gait
- Swallowing difficulties
- Slurred speech
Because cerebrospinal fluid can't drain properly, excess fluid may build up in the brain (hydrocephalus). This usually requires the surgical placement of a shunt system to divert cerebrospinal fluid to another part of the body.
Overview Of Chiari Malformations:
- Chiari I = downward displacement of cerebellar tonsils below foramen magnum (> 5 mm); unrelated to Chiari II malformation.
- Chiari II = abnormal neurulation leads to a small posterior fossa, caudal displacement of brainstem and herniation of tonsils and vermis through the foramen magnum; myelomeningocele.
- Chiari III = encephalocele and Chiari II findings (rare). Chiari IV = severe cerebellar hypoplasia (rare).
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