Facial Diplegia as the Sole Manifestation of Post-Vaccination Guillain-Barre Syndrome: A Case Report and Literature Review

Abstract

Introduction:

Recently, a large study concluded that certain brands of vaccines may increase the risk of Bell’s palsy and Guillain Barre Syndrome (GBS). As to whether vaccination after COVID-19 modify the risk of Bell’s palsy or GBS has not yet been studied.

Case:

Here we report a 35 years old COVID-19 survivor whom in less than 2 weeks after his second dose of inactivated SARS-CoV2 vaccine, developed bilateral facial nerve paralysis. In addition, he had hyperacusis, dysgeusia and decreased lacrimation without any signs of sensory and motor deficits in the limbs. His limb nerve conduction study (NCS) was unremarkable in contrast to bilaterally abnormal facial NCS and blink reflexes. Although he had negative anti-GM1 IgG and IgM antibodies, he has marked albuminocytologic dissociation, classic of acute inflammatory demyelinating polyneuropathy.

Conclusion:

To date, there were no similar case reports which published the occurrence of facial diplegia as sole manifestation of GBS in a post COVID-19 patient who recently completed vaccination. We believe that molecular mimicry, induced by magnified immune response from both COVID-19 and vaccination may have caused the symptom.

Introduction

The number of patients affected by COVID-19 is increasing. Along with this are its neurologic complications, including peripheral neuropathies in the limbs and cranial nerves, thought to be induced by immune mediated mechanisms. ¹ The facial nerves are not spared as reports showed that the risk of unilateral facial paralysis (UFP) is increased 7 times among patients infected with COVID-19. ²

In response to pandemic, millions of vaccines have been rolled out after World Health Organization (WHO) approved their emergency use authorization. Nevertheless, countries are advised to report any adverse events as these are not yet fully seen during trials. Of importance, 7 patients in a phase 3 trial contracted UFP, although longer and larger trials and post hoc analyses disproved any associations. ³ Despite this, a recent study concluded that some vaccines may be associated with increased risk of UFP and Guillain Barre Syndrome (GBS). ⁴ Whether SARS-CoV2 vaccination after COVID-19 modify the risk of UFP or GBS has not yet been studied. Here we report a young patient whom we diagnosed with GBS with facial diplegia as its initial and sole presentation.

Case Report

A 35-year-old male was diagnosed with COVID-19 4 months ago., He received his second dose of inactivated SARS-CoV2 vaccine 10 days prior to his initial symptoms. Eight days prior to admission, he started to have severe throbbing headaches associated with photophobia and lumbar pains, intermittently relieved by intake of analgesics. Five days later, upon awakening, he noted left tongue numbness followed by dysarthria and left facial weakness all occurring within a day. Weakness of the right facial muscles characterized by inability to close the eye, dysarthria and dysgeusia in the right tongue, along with hyperacusis and pain in bilateral mastoids occurred the following day, prompting admission. Despite the facial weakness, there were no deficits noted in the limbs.

The vital signs and systemic physical examination were unremarkable. Despite the inability to contract the frontalis, close both eyes and activate the corners of the mouth fully, the extraocular muscles had full range of motion on both ductions and versions. (Figures 1A-1D) The patient also had difficulty tasting salt and sugar in the anterior portion of the tongue and impaired labial sound production. The rest of the cranial nerve examination was normal. There was no weakness in the extremities. Light touch, vibration sense, position sense and temperature sensations were likewise intact. Although he was hyporeflexic in the pectoralis, bicipital and brachioradialis, the reflexes were normal in the lower extremities. He had no sensory ataxia, and the plantar reflex response was flexor.

Figure 1.

(A-D) These were the patient’s facial muscles during (A) rest, (B) when instructed to look up, (C) when instructed to close eyes, (D), when instructed to pull the corners of his mouth. (Consent was provided by the patient not to censor his eyes in order to illustrate the full facial diplegia).

Baseline laboratories including complete blood count, serum electrolytes, liver and kidney function tests, ECG, chest x-ray and urinalysis were unremarkable. Likewise, nerve conduction studies (NCS) revealed preserved compound muscle action potential (CMAP), sensory nerve action potential (SNAP), motor and sensory nerve conduction velocities and F-wave latencies in the median, ulnar, radial, peroneal, tibial and sural nerves. A repeat NCS done 2 weeks after the initial one revealed the same result. However, facial NCS showed decreased bilateral facial CMAP amplitudes, further supported by blink reflex findings of prolonged bilateral R1’s, R2’s and contralateral R2’s. Despite the absence of the typical ascending paralysis in GBS, he had albuminocytologic dissociation (ACD) (WBC=0, CSF total protein: 331.7 mg/dL, NV: <45mg/dL). Of importance, he had negative anti-GM1 IgM and IgG antibodies. His plain cranial CT scan was unremarkable.

As isolated bilateral Bell’s palsy could not be ruled out, the patient was started on acyclovir 200mg/tablet 5x per day for 10 days and prednisone 60mg/day tapering for 1 month and was referred for facial muscle physical therapy. There was marked improvement after 3 days of treatment. Since the patient was ambulatory, intravenous immunoglobulin or plasmapheresis were not offered.

Discussion

The inability of our patient to fully contract his facial muscles, in combination with decreased lacrimation, hyperacusis and decreased taste sensation would localize the lesions proximal to or in the geniculate ganglion of both facial nerves. Although bilaterally prolonged R1, R2 and contralateral R2’s in blink reflex may also be seen in patients with bilateral trigeminal nerve pathology, the decreased CMAP amplitudes in the facial nerve NCS and the absence of facial sensory deficit render this less likely. A brainstem lesion would result into multiple cranial nerves and limbs weakness in addition abnormal cranial imaging, which were never seen in our patient.

Although facial diplegia is one of the common symptoms in GBS, this has been rarely reported as its initial and sole presentation. The normal NCS of both the initial and repeat studies supports the absence of limb involvement. ⁵ Although the ACD implies polyradiculoneuropathy, the location of the facial nerve’s involvement in the subarachnoid space may elevate the albumin in the CSF in the background of normal white blood cell count. ⁶ Several cases have been reported with facial diplegia as the initial manifestation of GBS, most of them however, were eventually followed by symmetric ascending limb weakness and hyporeflexia, which are absent in our patient.^7–10 To evaluate for an axonal GBS variant, anti GM1 antibodies were tested. ⁵ As these were negative, we suspect that our patient had acute inflammatory demyelinating polyneuropathy. Other differentials include neoplasms such as leukemias and lymphomas which may also affect the facial nerves, but these usually present less rapidly and with similar involvement of the 8^th cranial nerve. Sarcoidosis has not been reported yet in the Philippines.

Recent studies suggest that COVID-19 and SARS CoV-2 vaccines, particularly those using inactivated virus, may increase the risk of UFP.^2,4 Among those given with inactivated SARS-CoV2 vaccines, 67 cases of UFP per 100 000 person years compared to 43 cases per 100 000 person years when BNT162b2 vaccine was used, was observed. ⁴ However, a nested case-control study comparing 298 patients with UFP and 1181 controls showed that those given with inactivated SARS-CoV2 vaccine had odds of developing UFP 2.8 times that of the controls. ⁴ Consistent with previous reports, the use of BNT162b2 seems not to increase the risk of UFP when compared to controls. ³ Most patients had left sided UFP and had their deficits within 21 days (93%), but none had bilateral facial nerve involvement.^2–4 It was also unknown if they included subjects with previous COVID-19 infection.

Smaller studies suggest that the incidence of UFP may be increased by COVID-19 alone by 6.8 times compared to vaccinated uninfected individuals. ² In 2 case series of patients with COVID-19 who developed Bell’s palsy, all had unilateral involvement days after testing positive using SARS-CoV2 RT-PCR testing.^11,12 Similar to post-vaccination Bell’s palsy, it is postulated that immune-mediated segmental demyelination, molecular mimicry of host molecules by the possible viral or vaccine antigens, or bystander activation of dormant autoreactive T-cells and reactivation of latent herpes simplex virus type 1 (HSV-1) infection of the geniculate ganglion may be the culprit.^4,8,13 Likewise, in unvaccinated patients with COVID-19, the hypercoagulability induced thrombosis of the vasa nervorum, direct invasion of the virus or ACE-2 induced neuronopathy may also cause the deficit. ¹¹ However, as with other ischemic neuropathies, asymmetric involvement is pathognomonic and the temporality from infection to the ictus should be significantly shorter than 4 months. In this case, we suspect that after suffering from COVID-19, our patient developed antibodies directed against SARS-CoV2 virus, which after the second dose of inactivated SARS-CoV2 vaccine, may have exponentially increased. Through molecular mimicry, these autoantibodies could have later induced bilateral segmental facial nerve demyelination.

Unlike UFP, isolated bilateral facial paralysis is a rare phenomenon, occurring only in 1 in every 5 million individuals, representing 23% of all patients with known or unknown causes of facial paralysis. Although its prognosis is good, its aesthetic and functional implications may significantly affect the daily quality of life. In addition, the alternative etiologies like brainstem infarct, leukemia, lymphoma, sarcoidosis among others carry poorer prognosis hence extensive work up should be done to rule them out.

Conclusion

The use of SARS-CoV2 vaccine has been associated with increased incidence of UFP. However, there have been no reports about the occurrence of bilateral facial paralysis as the initial and sole manifestation of post-vaccination GBS among patients who had prior COVID-19. We believe that molecular mimicry, induced by magnified immune response from both COVID-19 and vaccination may have caused the condition.

Ethics

This research has been reviewed and approved the Philippine General Hospital Research Ethics Board.

Abbreviations

ACD: Albuminocytologic dissociation; CMAP: Compound motor action potential; COVID-19: Coronavirus disease 2019; GBS: Guillain Barre Syndrome; NCS: Nerve conduction study; SNAP: Sensory nerve action potential; UFP: Unilateral Facial paralysis; WHO: World Health Organization

ORCID iD

Mario B. Prado https://orcid.org/0000-0003-0465-0943

Karen Joy B. Adiao https://orcid.org/0000-0001-5777-5171