Cheiro-oral-pedal syndrome as the presenting symptom of brainstem cavernous malformation: a case report

Abstract

The rare cheiro-oral-pedal syndrome (COPS) is characterized by sensory disturbances around the corner of the mouth, and in the hand and foot of the same side. The causative lesion is located in the thalamocortical projections, thalamus or brainstem and is usually due to ischemic or hemorrhagic stroke. We report a case of a patient with brain stem cavernous malformations presented as pure COPS with additional sensory disturbance in the thorax. We report this case to raise awareness of these very rare syndromes and demonstrate that mildly presenting symptoms can be caused by an underlying devastating condition.

Cheiro-oral-pedal syndrome of the pons and the role of imaging in diagnosis and management

“…The differential diagnosis begins to narrow towards cerebral or brainstem infarction, intracranial hemorrhage, brain tumor, migraine, and unique presentation of encephalopathy/encephalitis (if corresponding history applies), and referral for high quality imaging and interpretation from a neuroradiologist is the preferred next step in evaluation [2]. Since the location and deterioration of COS/COPS cannot be predicted by clinical symptoms alone, COS/ COPS should be considered an emergent condition for aggressive investigation until the fatal cause is substantially excluded [3].…”

“…Etiologies of cheiro-oral-pedal syndrome (COPS) are ischemic stroke, hemorrhagic stroke, and less commonly: neoplasm, intracranial bypass complications, cervical cord disorder, vascular malformation, aneurysm, infection/abscess, seizure, middle cerebral artery (MCA) stenosis, dermoid cyst, stereotactic surgery, drug, and other idiopathic causes [1]. Reported areas of involvement in COPS include the capsulestriatum, thalamus, medulla oblongata, corona radiata, pons, fronto-parietal cortex, multiple sites, or other unknown sites [2,3]. Six rare cases have pointed to spinal cord involvement after findings of aggravated paresthesia when flexion and extension manipulations to the neck were performed [2].…”

“…Six rare cases have pointed to spinal cord involvement after findings of aggravated paresthesia when flexion and extension manipulations to the neck were performed [2]. Risk factors contributing to COPS include hypertension, hyperlipidemia, diabetes mellitus, trauma, and vessel malformations [3].…”

“…Symptoms are further classified as type I, type II, type III, or type IV depending on the localization and size of the infarct as discussed earlier. Type I involves sensory impairment to the perioral area and ipsilateral hand/foot, type II involves perioral and hand/foot sensory deficits bilaterally (typical bilateral), type III is a sensory impairment in either the perioral area or the hand/foot bilaterally with the other area having unilateral impairment (atypical bilateral), and type IV being a sensory impairment confined to the perioral area and the contralateral hand/foot in a crossed pattern [3,5].…”