Case Summary
A 34-year-old Nigerian gentleman, with no significant past medical or surgical history, presented with one-day history of sudden-onset numbness and heaviness of the tongue. This was associated with drooling of saliva, loss of taste, slurring of speech, and the inability to close both eyes completely. The symptoms were persistent since onset and had never occurred before. He denied any earache, hearing difficulties, tinnitus, recent trauma to head or neck, or any other weakness or numbness in other parts of the body. There was no history of recent fever, flu-like illness, nausea, vomiting, diarrhea, headache, neck stiffness, photophobia, alteration of the level of consciousness, or abnormal jerky body movements. He has been living in Qatar for the last 2 years and has never traveled to Europe or North America. There was no contact with sick persons, insect bites, or use of any herbal supplements. He has never smoked, used alcohol or any illicit drugs. Initial evaluation showed that he was afebrile with a temperature of 36.2oC with heart rate of 81 beats per minute, the respiratory rate of 19 breaths per minute, a blood pressure of 117/69 mmHg and oxygen saturation of 98% on room air. On general examination, he was not pale or icteric. On neurological examination, no visual field defects or loss in visual acuity was noted. The pupils were equal and reactive to light. There was full range of eye movements and no diplopia, but bilateral lagophthalmos was present (Figure A). Bell’s phenomenon was also noted, where the eyes rolled upwards when the patient attempted to close his eyes. He was unable to clench his teeth, blow, whistle, or frown his forehead (Figure B). He was unable to show his teeth and the nasolabial folds were inapparent bilaterally. Facial sensations and jaw strength were normal on both sides. Hearing, palatal tongue movements, head rotation, and shoulder shrug were intact. Ankle jerk was absent bilaterally, and all other deep tendon reflexes were diminished (1/4). Bulk, tone, motor strength, and sensation were normal in all four extremities. The remaining systemic examination was unremarkable.
Routine laboratory examinations and blood cultures were unremarkable (Table 1). Serum assays for anti-neutrophil antibody (ANA), anti-double-stranded DNA antibody (anti-dsDNA), and anti-neutrophil cytoplasmic antibody (ANCA), were in the normal range. Polymerase chain reaction (PCR) examination of the blood for herpes and cytomegalovirus (CMV) viruses, viral capsid antigen for EBV, quantiferon TB, serological tests of HIV, and Lyme disease were all negative. Analysis of cerebrospinal fluid showed albuminocytological dissociation with no evidence of infection (Table 2). A non-contrast CT scan of the head showed no intra or extracranial pathology. MRI brain with contrast and intracranial MRA were normal except for an incidental finding of a small nasopharyngeal retention/ Thornwaldt cyst. Chest X-Ray showed normal lung parenchyma, heart, and mediastinal silhouette. CT chest and whole-body positron emission tomography (PET) CT scan did not reveal any evidence of underlying lymphoma, malignancy, sarcoidosis or other paraneoplastic causes. Electromyography (EMG) and nerve conduction velocity (NCV) revealed a demyelinating type of facial palsy (Table 3). He was started on intravenous immunoglobulin therapy at 0.4 grams/ kilogram for 5 days, along with ocular lubricants, eye patches, and physical therapy.
Follow-up at one month showed significant improvement with complete resolution and thus ability to close his eyes (figure C) and frown his forehead (figure D). However, he still had inapparent right nasolabial fold with mild deviation of the right side of the mouth (Figure E).