Cheiro-oral syndrome: A reappraisal of the etiology and outcome.

Objective: This is a review of our cases and published literature on cheiro-oral syndrome (COS), to better understand its localization, etiology and outcome. Methods: In addition to our database, we reviewed the medical database (including PUBMED, BIOSIS, EMBASE, and SCOPUS) and other sources, searched by the keyword of "cheiro-oral". The definition of COS was a subjective or an objective sensory disturbance confined to the perioral area and the finger(s)/hand without a detectable abnormality in mental, motor or cerebellar function. Only cases of COS where the clinicoanatomic correlation could be identified by neuroimaging study, autopsy or stereotatic surgery was included. Results: There were a total of 174 patients; 85 patients from our database, 76 patients from medical database, and 13 patients from other sources. They were 111 men and 63 women. Their age ranged from 12 to 85 years; average being 58.2 years. Stroke is the leading etiology and constituted 74% of the patients. The most common location of lesion was thalamus, followed by pons and cortex. Classical unilateral COS was seen in 81% of patients, atypical COS in 19%. Whereas the lesions were from cortex to cervical spinal cord in unilateral COS, atypical COS was associated with lesions in pons or medulla oblongata. An early deterioration was seen in 16.5% of patients, especially in large cortical infarction and subdural hemorrhage. Structural lesions were found in 85% of patients. Conclusion: Classical unilateral COS do not have a high localizing value, the atypical COS is associated with lesion in pons or medulla. [ABSTRACT FROM AUTHOR]