A new hypothesis for the pathophysiology of symptomatic adult Chiari malformation Type I.

Chiari malformation Type I (CMI) is characterized by herniation of the cerebellar tonsils through the foramen magnum. The pathophysiology of CMI is not well elucidated; however, the prevailing theory focuses on the underdevelopment of the posterior cranial fossa which results in tonsillar herniation. Symptoms are believed to be due to the herniation causing resistance to the natural flow of cerebrospinal fluid (CSF) and exerting a mass effect on nearby neural tissue.

Rare functional genetic variants in COL7A1, COL6A5, COL1A2 and COL5A2 frequently occur in Chiari Malformation Type 1.

Chiari Malformation Type 1 (CM-1) is characterized by herniation of the cerebellar tonsils below the foramen magnum and the presence of headaches and other neurologic symptoms. Cranial bone constriction is suspected to be the most common biologic mechanism leading to CM-1. However, other mechanisms may also contribute, particularly in the presence of connective tissue disorders (CTDs), such as Ehlers Danlos Syndrome (EDS).

Three-Dimensional CT Morphometric Image Analysis of the Clivus and Sphenoid Sinus in Chiari Malformation Type I.

This study evaluated three-dimensional (3D) volumetric image reconstructions to identify morphological differences of the clivus and sphenoid sinus on computed tomography (CT) scans of Chiari malformation type I (CMI) and control subjects. Axial CT images of adult females for 30 CMI subjects and 30 age and body mass index (BMI) matched controls were used for this retrospective study. 3D volumetric reconstructions were created from the bone windows of axial data following image registration for position and orientation correction of the head.

An examination of pain, disability, and the psychological correlates of Chiari Malformation pre- and post-surgical correction.

Background: 50% of patients with Chiari Malformation (CM) report a history of depression; however, rates of other psychological symptoms are unknown. Further, it is unclear whether surgical correction impacts pain, disability, and psychological symptoms.

Objective: /Hypothesis: We examined rates of symptoms in a nationwide sample of CM patients who had (n = 639) and had not (n = 551) undergone surgical correction.

National Institutes of Health Chiari Research Conference: state of the research and new directions.

Objective: To establish the current Chiari knowledge base and develop recommendations for future research.

Methods: Chiari malformation: state of the research and new directions was a two-day, National Institutes of Health sponsored conference. The agenda included review presentations and working groups tasked with developing specific, achievable research recommendations.