Arnold-Chiari malformation type 2 (ACM2) is a rare congenital disorder that affects the development of the brain and spinal cord. It is caused by a malformation of the cerebellum and brainstem, which is the part of the brain that controls movement, coordination, and balance. ACM2 is characterized by the downward displacement of the cerebellar tonsils through the foramen magnum, the opening at the base of the skull. This displacement can lead to a variety of symptoms, such as difficulty swallowing, chronic headaches, neck pain, dizziness, and impaired coordination. In some cases, ACM2 can also cause hydrocephalus, a buildup of fluid in the brain.
The components of ACM2 include the following:
• Cerebellar tonsillar herniation: This is the downward displacement of the cerebellar tonsils through the foramen magnum.
• Hydrocephalus: This is a buildup of cerebrospinal fluid in the brain, which can lead to increased pressure and damage to the brain.
• Syringomyelia: This is a chronic disorder characterized by fluid-filled cavities in the spinal cord, which can cause pain, weakness, and muscle stiffness.
• Chiari I malformation: This is a malformation of the cerebellum that can cause headaches, neck pain, and impaired coordination.
• Occipitalization of the atlas: This is a malformation of the vertebrae at the base of the skull, which can cause narrowing of the spinal canal and compression of the nerves.
• Atlantoaxial instability: This is an instability of the vertebrae at the base of the skull, which can cause pain and difficulty in movement.
• Arnold-Chiari malformation type 2: This is the most severe form of ACM, which can cause severe neurological symptoms.
In addition to these components, ACM2 is often accompanied by other medical problems, such as respiratory difficulties, spina bifida, and scoliosis. Treatment for ACM2 typically involves surgical decompression of the brainstem and cerebellum, as well as medications to control symptoms. In some cases, physical therapy and rehabilitation may also be beneficial.