The Chiari Symptom Profile: development and validation of a Chiari-/syringomyelia-specific questionnaire.

Objective: The Chiari Symptom Profile (CSP) was developed to provide a quantitative assessment of self-reported Chiari (CMI)-related symptoms and their impact on the individual's quality of life.

Methods: The instrument was developed in three phases. Phase I consisted of instrument development using exploratory item analysis from the literature and direct patient evaluations.

Posterior fossa decompression and reconstruction in adolescents and adults with the Chiari I malformation.

Objective: The purpose of this paper is to review the current and historical surgical approaches to treating Chiari I malformation in adolescents and adults.

Methods: A PubMed literature review and the author's own surgical experiences serve as a basis for discussing the variability in the surgical treatment of the Chiari I malformation.

Results: The most common treatment of the Chiari I malformation is posterior fossa decompression, which can range from a minimally invasive extradural approach to durotomy, duraplasty, internal decompression, and cranioplasty.

Phenotypic definition of Chiari type I malformation coupled with high-density SNP genome screen shows significant evidence for linkage to regions on chromosomes 9 and 15.

Chiari type I malformation (CMI; OMIM 118420) is narrowly defined when the tonsils of the cerebellum extend below the foramen magnum, leading to a variety of neurological symptoms. It is widely thought that a small posterior fossa (PF) volume, relative to the total cranial volume leads to a cramped cerebellum and herniation of the tonsils into the top of the spinal column. In a collection of magnetic resonance imagings (MRIs) from affected individuals and their family members, we measured correlations between ten cranial morphologies and estimated their heritability in these families.

Arachnoid cyst resulting in tonsillar herniation and syringomyelia in a patient with achondroplasia. Case report.

Achondroplasia has been associated with varying degrees of cervicomedullary and spinal compression, although usually in the pediatric population. Large arachnoid cysts have also been found to result in tonsillar herniation and syringomyelia. The authors present the case of a patient with achondroplasia who presented with symptoms of foramen magnum compression and syringomyelia, and who was subsequently found to have a large posterior fossa arachnoid cyst.

Prospective analysis of self-perceived quality of life before and after posterior fossa decompression in 112 patients with Chiari malformation with or without syringomyelia.

Object: The purpose of this prospective study was to determine if there was a difference in the self-perceived quality of life (QOL) before and after surgery among patients with Chiari I malformations with or without syringomyelia. Most patients with Chiari I malformations report improvement in their QOL after decompression surgery; however, specific outcome data have not been empirically studied in this patient population.

Methods: One hundred seventy-two consecutive patients who underwent posterior fossa decompression based on neuroimaging evidence of a Chiari I malformation with or without syringomyelia were prospectively offered participation in the study.

Evolution of the brain: from behavior to consciousness in 3.4 billion years.

Once life began as single-cell organisms, evolution favored those able to seek nutrients and avoid risks. Receptors sensed the environment, memory traces were laid, and adaptive responses were made. Environmental stress, at times as dramatic as the collision of an asteroid, resulted in extinctions that favored small predators with dorsal nerve cords and cranially positioned brains.

Prospective analysis of presenting symptoms among 265 patients with radiographic evidence of Chiari malformation type I with or without syringomyelia.

Objective: To characterize the presenting symptoms among persons with radiographic evidence of Chiari malformation (CM) type I in an effort to help the primary care provider determine accurate diagnosis and treatment.

Methods: The symptoms of CM can often be vague and ambiguous, leading to misdiagnosis. Patients who had radiographic evidence of CM with or without syringomyelia were prospectively studied.

Determination of death by neurological criteria.

Until 1968, when an ad hoc Harvard Medical School Committee published a landmark paper calling for determination of death using neurological rather than cardiovascular criteria, death was considered to have occurred when the heart irreversibly ceased beating. Since that time, every jurisdiction in the country has come to accept through law or court decision neurological criteria to define death. The authors review the issue of death by neurological criteria in light of current guidelines and recent advances.