Arnold-Chiari malformation pathophysiology

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Pathophysiology

The most widely accepted pathophysiological mechanism by which Chiari Type 1 Malformations occur is by a reduction or lack of development of the posterior fossa as a result of either congenital or acquired disorders.
The cerebellar tonsils are elongated and pushed down through the opening of the base of the skull (see foramen magnum), blocking the flow of cerebrospinal fluid (CSF).
The brainstem, cranial nerves, and the lower portion of the cerebellum may be stretched or compressed.
Therefore, any of the functions controlled by these areas may be affected. The blockage of CSF flow may also cause a syrinx to form, eventually leading to syringomyelia. Many sufferers turn to the Chiari Institute in Long Island, NY for specialized medical attention and medication.

Other conditions sometimes associated with Chiari Malformation include hydrocephalus,^[1] syringomyelia, spinal curvature, tethered spinal cord syndrome, and connective tissue disorders^[2] such as Ehlers-Danlos syndrome and Marfan Syndrome.

Brain: Arnold-Chiari Malformation: Gross fixed tissue sagittal section brain stem
Brain: Arnold Chiari Malformation: Gross fixed tissue sagittal section cerebrum brainstem and cerebellum
Brain: Arnold Chiari Malformation And Polygyria: Gross fix tissue external view

Brain: Arnold Chiari Malformation: Gross fixed tissue sagittal section brainstem close-up
Brain: Arnold Chiari Malformation: Gross fixed tissue brain stem sagittal section close-up
Brain: Arnold Chiari Malformation: Gross fixed tissue sagittal section brain stem and cerebellum

Brain: Arnold Chiari Malformation: Gross fixed tissue sagittal section brain stem cerebellum and spinal column
Brain: Hydrocephalus Secondary To Arnold Chiari Malformation: Gross fixed tissue three coronal sections cerebral hemispheres
Brain: Arnold Chiari Malformation: Gross fixed tissue cerebellum and brainstem

Spinal cord: Malformation Vertebral Bodies: Gross natural color sagittal section spinal column with malformation in region C7 T1 associated with Arnold Chiari malformation
Brain: Arnold Chiari Malformation; with Hydrocephalus, Type I
Brain: Arnold Chiari Malformation; Type II

Brain: Arnold Chiari Malformation; Tonsilar Herniation of Cerebellum Compressing Medulla
Brain: Arnold Chiari Malformation; Meningomyelocele, Type II, Meningitis
Brain: Arnold Chiari Malformation, Type II; Meningomyelocele, Meningitis, Close-up of Previous one

Brain: Cerebellum Arnold Chiari: Gross fixed tissue flattened cerebellum
Brain: Polymicrogyria: Gross fixed brain lateral view of left cerebral hemisphere case of Arnold Chiari cerebellum