Chiari Malformation (CM) is a neurological disorder in which part of the cerebellum, the lower rear part of the brain, protrudes into the spinal canal. It is divided into four types, numbered 1 through 4, according to the severity of the condition. Chiari 2 and 3 are the two most common types.
Chiari 2 Malformation
Chiari 2 malformation (CM2) is the most common form of the condition, accounting for 85% of all cases. This type of CM usually occurs in infants and young children, and is caused by a malformation in the area of the brain known as the cerebellar vermis. The cerebellar vermis is the part of the brain responsible for balance and coordination. In CM2, the cerebellar vermis does not form correctly, pushing the lower parts of the cerebellum and brainstem into the spinal canal. This creates a blockage of CSF (cerebral spinal fluid) flow, leading to a buildup of pressure in the skull and spinal column. Symptoms of CM2 include headaches, balance problems, poor coordination, and difficulty swallowing.
Chiari 3 Malformation
Chiari 3 malformation (CM3) is a rare form of the condition, accounting for less than 10% of all cases. This type of CM usually occurs in infants and young children, and is caused by a malformation in the area of the brain known as the cerebellar tonsils. The cerebellar tonsils are the lower parts of the cerebellum which protrude into the spinal canal. In CM3, the cerebellar tonsils are enlarged, pushing further into the spinal canal and blocking CSF flow. This leads to a buildup of pressure in the skull and spinal column, which can cause a range of symptoms such as headaches, balance problems, poor coordination, and difficulty swallowing.
The main difference between Chiari 2 and 3 is the location of the malformation. In Chiari 2, the malformation occurs in the cerebellar vermis, while in Chiari 3, the malformation occurs in the cerebellar tonsils. In both cases, the malformation leads to a blockage of CSF flow, leading to a buildup of pressure in the skull and spinal column.