To describe an uncommon presentation of distal extracranial internal carotid artery (ICA) dissection which can lead to misdiagnosis and delay in treatment.

Carotid artery dissections typically present with ipsilateral head or neck pain, Horner syndrome and anterior circulation stroke symptoms if distal embolism has occurred. However, a subset of patients with distal ICA involvement and pseudoaneurysm or intramural hematoma formation can present with compressive lower cranial neuropathies, of which hypoglossal involvement is the most common, rendering the diagnosis difficult.  

We report on three middle-aged patients with distal cervical ICA dissection that developed lower cranial neuropathies including moderate-to-severe dysphagia as their main presenting symptom.  All patients had some degree of ipsilateral head/neck pain and dysarthria. One patient had a Horner syndrome and pronounced hoarseness; two developed hypoglossal nerve palsy.  Swallow evaluation including modified barium swallow and/or fiberoptic endoscopic evaluation of swallowing (FEES) revealed oropharyngeal dysphagia. CT angiogram showed distal ICA dissection with pseudoaneurysm and/or intramural hematoma in all patients. None suffered acute cerebral infarction. In one patient, the dysphagia led to aspiration pneumonia and almost 20 pound weight loss requiring gastrostomy tube placement. All patients had been seen in an emergency or urgent care setting at least once prior to presenting to neurological attention, and were treated for presumed dental or upper respiratory infections.

In patients presenting with head or neck pain, dysphagia, dysarthria and hoarseness, distal ICA dissection with compressive lower cranial neuropathies should be considered. Timely diagnosis and initiation of antithrombotic therapy is critical for stroke prevention. Complete swallow evaluation and consideration of a nasogastric or gastrostomy tube may be necessary to prevent aspiration pneumonia and significant weight loss.