Bilateral cheiro-oral syndrome

Eight cases of pure bilateral cheiro-oral syndrome (COS) are reported. The location and etiology of lesion were well defined in six cases, including pontine infarct in three, and brainstem hemorrhage, unilateral thalamic infarct and bilateral subdural hematoma in one patient each respectively. Neuroimaging and neurophysiological studies were normal in another two patients. Taken together with the previous five reported cases of bilateral COS, pons is the most frequent site for presentation even in the absence of associated brainstem signs.