Malformations of the craniocervical junction (Chiari type I and syringomyelia: classification, diagnosis and treatment).
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 neuropathic pain: either pharmacological and non-pharmacological. Pharmacological therapy use drugs that act on different components of pain. Non-pharmacological therapies are primarily 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.
Fernández
Alfredo Avellaneda
AA
Carlos III Health Institute, Sinesio Delgado n. 6 (pavilion 12), 28029 Madrid, Spain. alfavel@gmail.com
Guerrero
Alberto Isla
AI
Martínez
Maravillas Izquierdo
MI
Vázquez
María Eugenia Amado
ME
Fernández
Javier Barrón
JB
Chesa i Octavio
Ester
E
Labrado
Javier De la Cruz
Jde L
Silva
Mercedes Escribano
ME
de Araoz
Marta Fernández de Gamboa Fernández
MF
García-Ramos
Rocío
R
Ribes
Miguel García
MG
Gómez
Carmen
C
Valdivia
Joaquín Insausti
JI
Valbuena
Ramón Navarro
RN
Ramón
José R
JR
D001139:Arnold-Chiari Malformation / Q000145:classification / Q000150:complications / Q000175:diagnosis / Q000523:psychology / Q000628:therapy*; D006801:Humans; D013595:Syringomyelia / Q000209:etiology