Cortical cheiro-oral syndrome: A revisit of clinical significance and pathogenesis

ObjectivesA restricted neurological deficit is not uncommon in patients with cortical lesions. However, in cheiro-oral syndrome (COS) associated with cortical involvement, the topographic disparity of the cheiral and oral representation area is hardly explained by the restricted sensorium at the homolateral mouth angle/lip and finger/hand, with sparing of the facial structures.Patients and methodsIn order to elucidate the factors of clinical significance and pathogenesis in cortical COS, 11 patients with COS associated with contralateral cortical lesions were analyzed, and the cases of similar patients described in the literature were reviewed.ResultsIn our patients, the etiologies of COS were infarct, subdural hematoma, meningioma, and vascular malformation. The lesions were sizable enough to involve areas beyond the postcentral gyrus of the parietal lobe. A cortical sensory sign was detected in only six patients. Their paroxysmal sensorium without exacerbation on stimulus was in contrast to the clinical features of COS associated with thalamic lesions. Five patients suffered worsening of their neurological deficits without a parallel expansion of their infarct or hemorrhage. Removal of the hematoma or tumor in four patients rapidly abolished their sensorium.ConclusionCortical COS is a warning sign of a potentially life-threatening etiology. Since there is a high frequency of exacerbation, COS should be carefully investigated, as a paucity of associated clinical signs was found in our patients. Paroxysmal sensorium signifies possible cortical involvement. Rapid decompression within the “golden period” is encouraged. It seems that the interaction between previously existing vascular compromise, sensory plasticity, and neuronal vulnerability predisposes patients to this peculiar sensory disorder. Epileptogenesis is unlikely. Thus, a reconsideration of COS in clinical practice is warranted.