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).