Disease Information for Arnold-Chiari syndrome (Clinical Manifestations)

ACM Arnold Chiari malformat, ACM Arnold Chiari malformation, Arnold Chiari syndrome, Arnold Chiari, Arnold Chiari Deformity, ARNOLD CHIARI MALFORMATION, Arnold Chiari obstruction, Arnold Chiari Syndrome, Arnold Chiari syndrome (disorder), CEREBELLOMEDULLARY MALFORMATION SYNDROME, Chiari malformation, Chiari malformation (disorder), Chiari's malformation, Deformity Arnold Chiari, Malformation Arnold Chiari, obstruction Arnold Chiari, Syndrome Arnold Chiari

A group of congenital malformations involving the brainstem, cerebellum, upper spinal cord, and surrounding bony structures. Type II is the most common, and features compression of the medulla and cerebellar tonsils into the upper cervical spinal canal and an associated MENINGOMYELOCELE. Type I features similar, but less severe malformations and is without an associated meningomyelocele. Type III has the features of type II with an additional herniation of the entire cerebellum through the bony defect involving the foramen magnum, forming an ENCEPHALOCELE. Type IV is a form a cerebellar hypoplasia. Clinical manifestations of types I-III include TORTICOLLIS; opisthotonus; HEADACHE; VERTIGO; VOCAL CORD PARALYSIS; APNEA; NYSTAGMUS; swallowing difficulties; and ATAXIA. (From Menkes, Textbook of Child Neurology, 5th ed, p261; Davis, Textbook of Neuropathology, 2nd ed, pp236-46)<br>

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Arnold-Chiari Malformation; ACM; Arnold-Chiari Malformation; Cerebellomedullary Malformation Syndrome <br>

Disorder Subdivisions:Chiari Type I (Chiari Malformation I);Chiari Type II (Chiari Malformation II).<br>

Discussion:Arnold-Chiari malformation is a rare malformation of the brain that is sometimes, but not always, apparent at birth; It is characterized by abnormalities in the area where the brain and spinal cord meet that cause part of the cerebellum to protrude through the bottom of the skull (foramen magnum) into the spinal canal; This interferes with the flow of cerebral spinal fluid to and from the brain, leading to accumulation of cerebral spinal fluid in the empty spaces of the spine and brain; The portion of the cerebellum that protrudes into the spinal canal can become elongated and is called the "cerebellar tonsils" because it resembles the tonsils;<br>

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There are four types of Chiari malformation. Depending on the type, the impact on the affected individual may range from mild to severe[NORD 2005]<br>

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