Abstract
Chiari disease (or malformation) is in general a congenital condition characterized by an anatomic defect of the base of the skull, in which the cerebellum and brain stem herniate through the foramen magnum into the cervical spinal canal. The onset of Chiari syndrome symptoms usually occurs in the second or third decade (age 25 to 45 years). Symptoms may vary between periods of exacerbation and remission. The diagnosis of Chiari type I malformation in patients with or without symptoms is established with neuroimaging techniques. The most effective therapy for patients with Chiari type I malformation/syringomyelia is surgical decompression of the foramen magnum, however there are non-surgical therapy to relieve neurophatic pain: either pharmacological and non-pharmacological. Pharmacological therapy use drugs that act on different components of pain. Non-pharmacological therapies are primarly based on spinal or peripheral electrical stimulation.It is important to determine the needs of the patients in terms of health-care, social, educational, occupational, and relationship issues, in addition to those derived from information aspects, particularly at onset of symptoms.Currently, there is no consensus among the specialists regarding the etiology of the disease or how to approach, monitor, follow-up, and treat the condition.It is necessary that the physicians involved in the care of people with this condition comprehensively approach the management and follow-up of the patients, and that they organize interdisciplinary teams including all the professionals that can help to increase the quality of life of patients.
Highlights
For several decades, the eponyms Arnold and Chiari have been used as synonyms to define conditions with ectopia of the cerebellar tonsils below the level of the posterior edge of the foramen magnum
Chiari syndrome is a developmental malformation of the occipital mesodermal somites that can be associated to syringomyelia and hydrocephalus
The most extreme form consists of the herniation of structures of the lower cerebellum, the cerebellar tonsils, and brain stem through the foramen magnum, in such a way that parts of the brain enter the spinal canal, thickening and compressing it
Summary
The eponyms Arnold and Chiari have been used as synonyms to define conditions with ectopia of the cerebellar tonsils below the level of the posterior edge of the foramen magnum. Spinal stimulation has demonstrated its efficacy in certain types of neuropathic pain, this is not the case for central pain, and there is no evidence available in patients with Chiari syndrome Since this therapy is free of major side effects, and any side effect that could occur is reversible, it is an option to be considered before using more questionable therapeutic approaches. A combined medical and psychological intervention is required for each patient, with the major objectives of reducing the psychosocial impact of the disease and improving the patient’s quality of life In this context, psychotherapy is a therapeutic element that should be considered mandatory in the comprehensive management of patients with Chiari syndrome and syringomyelia. This can be a disabling condition, and may cause full disability in some patients, often requiring legal support to claim economic aid, special
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