Article, Neurology

Bilateral facial nerve paralysis in the outpatient setting; the need for follow-up in the face of a serious pathology

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American Journal of Emergency Medicine

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Bilateral facial nerve paralysis in the outpatient setting; the need for follow-up in the face of a serious pathology?


Bilateral facial nerve paralysis is an extremely rare disease with an annual incidence of 1 in 5 million cases. Although unilateral facial nerve paralysis is often a benign illness, bilateral facial nerve palsy is a harbinger of more serious pathology. We present the case of a 25-year-old man who presented with alternating left then right facial weakness. We diagnosed him with bilateral facial nerve palsy and scheduled further diagnostic tests, but he was lost to follow-up. Given the severity of the differential diagnosis of bilateral facial nerve paralysis, diagnostic tests must be done at time of initial presentation. The self-resolving nature of this symptom makes follow-up difficult in this unique subset of patients.

Bilateral facial nerve paralysis is a rare disease. It is often the sign of a more serious underlying pathology such as leukemia, sarcoidosis, and HIV. Unilateral facial nerve paralysis is often idiopathic or due to a self-limiting Viral illness. The severity of the differentials of bilateral facial nerve palsy makes thorough diagnostic testing essential at time of presentation as demonstrated by this case. This presenting sign is not only rare but also serious and should be treated accordingly. Given the self-resolving nature of facial nerve paralysis, follow-up appointments present a challenge, and diagnostic tests should be done at initial presentation.

A 25-year-old man with no medical history presented to the emer- gency department (ED) with a 2-day history of subjective fever and generalized body numbness and tingling accompanied by headache. The patient reported a 5-pack-year history of smoking with occasional alcohol use. He stated being sexually active with 1 monogamous female partner with whom he has been in a 1.5-year relationship. He reported no surgical history and has no known drug allergies. In the ED, the patient received complete blood count, basic metabolic panel, liver function tests, Urine analysis, blood culture, and chest radiograph, all of which showed no abnormal findings. The patient refused admission at this visit and was discharged. The patient returned in 7 days to the ED complaining of back pain and new-onset left eye pain with accompa- nying facial paralysis. The patient was diagnosed with left-sided Bell’s palsy. Lumbar puncture was performed which revealed no pathology. Testing for herpes simplex Virus (HSV) 1 and 2, Venereal Disease Re- search Laboratory (VDRL) test, and Enterovirus was performed and

? This case report highlights an extremely rare disease and discuses important differen- tial diagnosis.

sent to the laboratory. The patient declined testing for HIV. The patient was started on Valtrex, prednisone, Robaxin, Naproxen, and Lacri-Lube and discharged home. Results from laboratory work showed no reactiv- ity to HSV1, 2, VDRL, or Enterovirus. Fourteen days after diagnosis of uni- lateral Bell’s palsy, the patient returned complaining of continued back pain radiating to the legs and persistent migraines accompanied by a right-sided facial droop. On physical examination, the patient was in no distress and was in good physical shape. Cranial nerves II to XII were intact on the patient’s left side. Examination of the right side of the patient’s face revealed inability to smile, wrinkle forehead, and shut the right eye. Cardiac, pulmonary, and Abdominal examination re- sults were all within normal limits. Neurological examination revealed that the patient was alert and oriented to person, place, and time. The patient showed normal gait. Motor strength was appropriate in both upper and lower extremities bilaterally. Reflexes and sensory examina- tion results were normal in all extremities. The time between initial pre- sentation of left-sided Bell’s palsy and the subsequent right-sided facial paralysis was 2 weeks. Using the diagnostic criteria, a diagnosis was made of bilateral Bell’s palsy. The patient was scheduled to return to outpatient clinic for further diagnostic workup. Despite multiple attempts to contact both the patient and his family, the patient missed his appointment and was lost to follow-up.

Unilateral facial weakness is an alarming symptom for patients and prompts frequent visits to both primary care physicians and emergency rooms. The most common cause for cranial nerve palsy is Bell’s palsy, which makes up 65% to 75% of facial nerve palsy diagnosis [1]. Named after the Scottish surgeon who first defined it, Bell’s palsy is an idiopathic facial nerve paralysis [2]. In the majority of these patients, the incidence is singular with complete spontaneous resolution of 70% of cases and with a resolution of 90% once treatment has been initiated [3]. The cause of facial nerve paralysis is multifaceted. Bell’s palsy is inherently idiopathic and therefore is a diagnosis of exclusion. Common differentials for Bell’s palsy include but are not limited to Borrelia burgdorferi, Guillian-barre syndrome (GBS), Varicella zoster, and Herpes simplex virus. Although unilateral facial weakness is common in the out- patient setting, bilateral facial nerve paralysis is a rare symptom and represents 0.3% to 2% of facial nerve paralysis [4]. For a patient to be di- agnosed with a bilateral facial nerve paralysis, both sides of the face must either be involved simultaneously or within a 4-week span, as was seen in our patient. Unilateral facial nerve paralysis is common, with an incidence of 13 to 34 cases in every 100,000 [5]. Bilateral facial nerve paralysis however is exceedingly rare, with an incidence of 0.3% to 2% [5]. The annual incidence of bilateral facial nerve palsy is 1 per 5 million [6]. Spontaneous resolution of unilateral Bell’s palsy is seen in 85% of patients within 3 weeks of the onset of symptoms [7]. Bilateral facial nerve palsy is often a harbinger of a more serious pathology. In

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only 23% of bilateral palsy is no etiology identified [5]. This however does not mean that a serious Underlying etiology was at the root of the cause. Conversely, spontaneous resolution does not mean resolution of the underlying disease process. The most common viral cause of bilat- eral facial nerve paralysis is the Epstein-Barr virus [8]. Although HSV is the most common viral etiology in unilateral paralysis, it plays a less sig- nificant role in bilateral facial nerve paralysis. In comparison to unilater- al facial paralysis, the differentials in bilateral facial nerve paralysis are more alarming. These differentials include luekemia, sarcoidosis, bacte- rial meningitis, syphilis, leprosy, Moebius syndrome, infectious mono- nucleosis, neurocysistercicosis, traumatic brain injury, mycoplasma pneumonia, HIV, Severe hypertension, and recent linezolid therapy. In a rare subset, it has been reported in pregnant women who are experiencing preeclampsia [2]. With these differentials in mind, this presenting sign should be seen as a clue of a more serious pathology. The differentials for facial paralysis are diverse and can be appropriately stratified based on a good history. Lyme disease is a geographic- dependent cause and represents 36% of facial nerve paralysis, with GBS representing 5% and AIDS at time of seroconversion 0.9% [4]. An in- dividual who works outside in the Northeastern part of the continental United States has a much higher index of suspicion for Lyme disease than someone who works in the Midwest. Although the typical rash of erythema migrans may be present at time of diagnosis, this is not always visible, and its absence does not preclude the viral existence. In patients in whom GBS is suspected, tendon reflexes will be absent and accompanied by weakness in the extremities. Serology can be tested for HIV, syphilis, and HSV, and a peripheral blood smear can rule out leu- kemia. Cerebrospinal fluid can be evaluated for Bacterial meningitis. In patients in whom neurosarcoidosis is expected, serum calcium and a chest radiography are diagnostic. In HIV-infected patients, facial nerve paralysis occurs during acute retroviral syndrome. Acute retroviral syn- drome is reported to affect up to 90% of patients infected with HIV [9]. When HIV-1 is the cause of bilateral facial nerve paralysis, the present- ing sign is seen 4 to 6 weeks before seroconversion [10]. This makes di- agnosis of HIV virus at presentation particularly challenging. The importance of follow-up is stressed in these circumstances where the patients’ symptoms may resolve before seroconversion. Furthermore, the common treatment of facial nerve palsy with corticosteroids may, in a patient with HIV, further increase the severity of an HIV infection. Although the direct pathogenesis of HIV virus resulting in facial nerve paralysis is not fully understood, an immune-mediated inflammatory polyradiculopathy has been suggested [11]. Both the severity of the differentials and their complex diversity make a Detailed history and physical and Laboratory workup at time of initial presentation essential

in all patients presenting with bilateral facial nerve palsy. The self- resolving nature of this presenting sign makes rapid response and evaluation essential. In a patient population who is reluctant to return for follow-up, the severity of the cause should be stressed at initial presentation and diagnostic tests performed at the same visit. With uni- lateral facial nerve paralysis, the standard treatment is herpes antiviral with an oral steroid. The patient is sent home and told to return if the symptoms do not improve. This same approach is insufficient when the presenting sign is bilateral and thus points to a more serious pathol- ogy. We have found that, once symptoms resolve, patients become un- responsive to the importance of further testing and are lost to follow-up. Because of our patient’s unwillingness to return, a true cause of his illness could not be assessed. Although it may be idiopathic, a more serious pathology might be at play.

Benjamin Chaucer

St. Georges University School of Medicine

Corresponding author.

E-mail address: [email protected]

LalanthicaYogendran, MD Lyudmila Rubinshteyn, MD Richmond University Medical Center


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