Rare Manifestation of Sjögren’s Syndrome as Unilateral Facial

Paralysis: A Case Report and Literature Review

2nd Lt Emily A. Montgomery, MS, MSC, USAF *; CPT Jagatkumar A. Patel, MD, MC, USA †;
CPT Rachel E. Boone, MD, MC, USA †; MAJ Jeffrey C. Teixeira, MD, MBA, MC, USA *,†;
MAJ Aurora G. Vincent, MD, FACS, MC, USA *,‡; LTC Marc H. Hohman, MD, FACS, MC, USA*,†

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ABSTRACT Herein, we present a unique case of Sjögren’s syndrome (SS) first presenting as facial palsy, as well
as a literature review of case reports describing SS-associated facial paralysis. A PubMed search for papers containing
the keywords Sjögren’s syndrome or Sjögren’s disease, as well as facial paralysis, facial paresis, facial palsy, or Bell’s
palsy, was performed. Articles not in English and cases of SS not involving facial paralysis were excluded. Appropriate
articles were reviewed for patient demographics and symptoms of SS, including laterality of facial paralysis, cranial nerve
involvement, and comorbid diseases. House–Brackmann grades were annotated based on either assignment by individual
case reports or the authors’ descriptions when sufficient details were present. Of 43 peer-reviewed articles found, 14 were
both in the English language and provided adequate information on a total of 16 patients with facial paralysis and SS
diagnosis. Ultimately, SS and other systemic autoimmune disorders should be considered in the differential diagnosis of
patients presenting with insidious onset facial paralysis.

INTRODUCTION initially presented with 1.5 years of slowly progressive right-

Sjögren’s syndrome (SS) is one of the most common
autoimmune disorders in the United States, bearing clas-
sic associated symptoms of xerostomia and xerophthalmia
(“sicca complex”) because of inflammatory infiltration of sali-
vary and lacrimal glands.1 While exocrine glands throughout
the body may be affected and cause other symptoms to accom-
pany the classic presentation, the sicca complex may be subtle
and its presence is not required for the diagnosis of SS.1
Although central nervous system manifestations are less
common (1%-5%) than the sicca complex, peripheral ner-
vous system symptoms are not unusual in SS (∼25%).2 The
involvement can be unifocal or multifocal, with cranial nerve
palsies affecting women at a rate of 20.8:1 compared to men.2
The trigeminal nerve is the most commonly affected cranial
nerve in SS, and isolated facial nerve palsy in the absence of
other symptoms in SS is quite rare.2 Herein, we describe a
case of SS diagnosed after presenting with progressive facial
palsy in the absence of sicca complex symptoms.
METHODS
Case Report
A 20-year-old female with a history of recurrent otitis media,
xerophthalmia, chronic arthralgia, and cold-induced urticaria
* Department of Surgery, Uniformed Services University of the Health
Sciences, School of Medicine, Bethesda, MD 20814, USA
† Otolaryngology-Head & Neck Surgery, Madigan Army Medical Center,
Tacoma, WA 98431, USA
‡ Otolaryngology-Head & Neck Surgery, Eisenhower Army Medical
Center, Fort Gordon, GA 30905, USA
The views expressed are those of the authors and do not reflect the official
policy of the Department of the Army, the DoD, or the U.S. Government.
Published by Oxford University Press on behalf of the Association of
Military Surgeons of the United States 2022. This work is written by (a) US
Government employee(s) and is in the public domain in the US.
sided facial paresis to House–Brackmann grade III (Fig. 1).
There were no other neurological deficits. Brain and skull base
magnetic resonance imaging and subsequent temporal bone
computed tomography scanning did not reveal any masses or
lesions along the course of the facial nerve. Electromyogra-
phy demonstrated fewer motor units on the right side with
a decreased amplitude, a longer duration, and fast firing.
Electroneuronography indicated 40% action potential degen-
eration in the right orbicularis oculi muscle when compared
to the left side and 60% degeneration in the right nasalis.
The patient was subsequently evaluated with
anti-Sjögren’s-Syndrome-related antigen A (SSA/Ro) and
anti-Sjögren’s-Syndrome-related antigen B (SSB/La) titers,
which were negative; however, antinuclear antibody titers
were elevated (1:640 speckled; 1:1280 spindle). She then
underwent a right lower lip minor salivary gland biopsy,
which demonstrated multifocal lymphocytic sialadenitis, con-
sistent with the diagnosis of SS. The patient received a 4-week
course of prednisone 40 mg daily and started hydroxychloro-
quine 400 mg daily, based upon recommendations from the
rheumatology service.
In addition to medical management, her paralytic lagoph-
thalmos was addressed surgically because of persistent eye
pain, particularly with cold wind and showering. A 1.2-g
low-profile rigid platinum weight was placed in a pretarsal
position, and a lateral tarsal strip canthopexy was performed
as well. At the 2-week follow-up, the patient was very pleased
with the improvement in her eye closure. At the 6-month
follow-up, she reported sustained improvement in her ocu-
lar symptoms, and her facial paralysis remained stable on
hydroxychloroquine (Fig. 2).
Literature Review
This project was deemed to meet exempt human research
criteria by the Madigan Army Medical Center Human

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FIGURE 1. House–Brackmann grade III facial paralysis, preoperative views. Note the subtly incomplete eye closure with full effort, inferior malposition of
the right lower eyelid, and smile asymmetry.

FIGURE 2. Six-month postoperative views, after right upper eyelid loading and lateral tarsal strip canthopexy. Note the stable smile asymmetry but improved
eye closure and lower eyelid position.

Research Protections Office. The authors conducted a lit- DISCUSSION

erature search in PubMed on May 12, 2022, using the
search strategy keywords: “((Sjögren’s syndrome) OR (Sjö-
gren’s disease)) AND ((facial palsy) OR (facial pare-
sis) OR (facial paralysis) OR (Bells palsy) OR (Bell’s
palsy)).” Case reports and case series reporting SS patients
with facial nerve involvement published in English were
included. Two authors (E.A.M. and J.A.P.) independently
screened 43 titles, abstracts, and full texts of relevant
articles.
RESULTS
Sixteen reports of facial paralysis associated with SS were
identified in the literature. Fifteen cases were female, and
nine involved unilateral facial nerve palsy. All of the cases
with unilateral facial paralysis were associated with comorbid
autoimmune diseases, involvement of other cranial nerves, or
both. Table I describes the 16 other reports of facial paralysis
in SS.
Sjögren’s syndrome is a chronic autoimmune disorder
characterized by lymphocytic infiltration of exocrine glands,
leading to the classic symptoms of xerostomia and xeroph-
thalmia. These symptoms, however, can be subtle and
may not be present in all patients. In fact, approx-
imately 20% of patients with SS do not report sicca
symptoms.2
Numerous extraglandular manifestations have been
described in patients affected by SS as well. The involvement
of the peripheral nervous system is frequently encountered,
and the prevalence of these symptoms ranges from 10% to
60% because of, in part, broad variations in diagnostic cri-
teria for neuropathies.3 When cranial nerves are involved,
there is a predilection for the trigeminal nerve, manifesting
most commonly as neuropathy of the maxillary branch.1,3
Transient cranial neuropathies of III, VI, VII, VIII, IX, and
X have been reported as well.2 Facial nerve involvement is
unusual, with only 16 reports identified in the literature before

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 Facial Palsy in Sjögren’s Syndrome

ours, 15 of which were female, and 9 of which involved
unilateral facial nerve palsy (Table I). However, all of the
cases with unilateral facial nerve palsy involved comorbid
autoimmune diseases, involvement of other cranial nerves,
or both.
Our patient ultimately met the criteria for the diagno-
sis of SS based on salivary gland biopsy findings and ele-
vated antinuclear antibody titers in the absence of the classic
facial palsy should raise concern for a neoplastic process,
and even relapsing and remitting unilateral paralysis may
indicate a benign tumor, such as a geniculate ganglion heman-
gioma.4 When multiple cranial nerves are involved, partic-
ularly bilaterally, systemic processes, such as autoimmune
and infectious diseases, including coronavirus disease, must
be considered. Another important entity that should be con-
sidered in a differential diagnosis of patients with unilateral

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sicca complex; however, the presenting symptom leading
to her evaluation was an isolated unilateral facial palsy.
While she did complain of a dry right eye, the unilaterality
and postoperative resolution indicate that her xerophthalmia
was related to lagophthalmos rather than SS lacrimal gland
involvement. When compared to reports of facial paralysis
and SS in the literature, our patient’s report remains unique,
given her unilateral facial nerve involvement in the absence
of other cranial neuropathies or systemic comorbidities
beyond SS.
The few reported cases of facial palsy in SS have gen-
erally involved an acute presentation with some degree of
subsequent recovery (Table I). These are consistent with the
classic presentation of relapsing and remitting cranial neu-
ropathies in autoimmune diseases.4 Our patient, however,
had a slowly progressive and sustained palsy. Progressive
facial paralysis accompanied by other cranial neuropathies
is Ramsay Hunt syndrome, although this tends to present
acutely.5
The variability of associated systemic symptoms in
patients with SS suggests a complex pathophysiologic mech-
anism. It has been postulated that the underlying pathogenesis
may be vasculitis, an extension of inflammatory lympho-
proliferation, or a combination of both. Given our patient’s
course, her failure to improve with corticosteroids, and her
lack of facial nerve enhancement on magnetic resonance
imaging, it may be that the site of injury was quite proximal,
albeit not cortical, given the weakness of the frontalis muscle.
Her presentation remains peculiar. A variety of immuno-
suppressive and anti-inflammatory treatments for SS have
been investigated, but more research into pharmacological
remedies is needed.3

TABLE I. Review of the Literature Regarding Sjögren’s Syndrome Patients With Facial Nerve Paresis

Age
Year First author Sex (years) Laterality CN Comorbid disease HB grade

2022 Montgomery F 20 Unilateral VII None III

2021 Isik6 F 59 Bilateral VII Rheumatoid arthritis Unk
2021 Maturu7 F 33 Bilateral VIIa Idiopathic central pontine Unk
myelinolysis
2021 Neumann2 F 62 Unilateral (VII) and VII and VIII SNHL VI
bilateral (VIII)
2020 Alinezhad8 F 32 Bilateral VII None VI
2020 Li9 F 47 Bilateral VIIb MG and Hashimoto’s thyroiditis Unk
2019 Shima10 M 70 Unilateral III and VII Systemic lupus erythematosus IV
2019 Wei11 F 69 Bilateral VII Unilateral hearing impairment III
since childhood
2018 Gorodet- F 60 Unilateral VII Rosai–Dorfman disease and nodal VI
skiy12 marginal zone lymphoma
2015 Birnbaum13 F 32 Unilateral VII and VIII None Unk
F 47 Unilateral VII and VIII Rheumatoid arthritis Unk
F 40 Unilateral VII and VIII None Unk
2009 Ashraf14 F 47 Unilateral VI, VII, VIII, IX, X, Cranial meningitis and Unk
and XII hypophysitis
2001 Hadithi15 F 41 Unilateral III, V, and VII Autoimmune hypothyroidism Unk
1993 Moll16 F 51 Unk VII and VIII None Unk
1992 Alfaro- F 41 Unilateral (motor) V and VII MCTD, pericarditis, and pleural Unk
Giner17 and bilateral effusion
(sensory)
1989 Uchihara18 F 48 Bilateral VII None Unk
Results from the literature search that met the inclusion criteria (SS diagnosis, facial paralysis involved, and English language).
Abbreviations: CN, cranial nerve affected; HB, House–Brackmann; MCTD, mixed connective tissue disease; MG, myasthenia gravis; unk, unknown.
a Bulbar palsy also described and implicates one or more of CNs IX–XII.
b Diplopia also described and implicates one or more of CNs III, IV, and VI.

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 Facial Palsy in Sjögren’s Syndrome
CONCLUSION
A specific etiology will not be identified in the majority of
cases of unilateral facial paralysis (e.g., Bell’s palsy), and
there appears to be an increasing prevalence of facial paralysis
in the age of coronavirus disease and messenger RNA vacci-
nations. However, a high index of suspicion for autoimmune,
infectious, or neoplastic processes should be maintained when
facial paralysis evolves insidiously.4,19 Similarly, when facial
paralysis is followed or accompanied by other cranial neu-
ropathies, clinicians should maintain a high index of suspicion
for systemic pathologies.
FUNDING
None declared.
CONFLICT OF INTEREST STATEMENT
The authors have no conflicts to disclose and have received no financial
support for this work.
REFERENCES
1. Vivino FB, Bunya VY, Massaro-Giordano G, et al: Sjogren’s syn-
drome: an update on disease pathogenesis, clinical manifestations and
treatment. Clin Immunol 2019; 203: 81–121.
2. Neumann M, Quintero J, Shih T, Capitle EM: Not all sicca is Sjögren’s
and not all Sjögren’s is sicca. Cureus 2021; 13(1): e12996.
3. Voigt A, Sukumaran S, Nguyen CQ: Beyond the glands: an in-depth
perspective of neurological manifestations in Sjögren’s syndrome.
Rheumatology (Sunnyvale) 2014; 2014: S4–010.
4. Hohman MH, Hadlock TA: Etiology, diagnosis, and management of
facial paralysis: 2000 patients at a facial nerve center. Laryngoscope
2014; 124(7): e283–93. 10.1002/lary.24542.
5. Crouch AE, Hohman MH, Andaloro C: Ramsay Hunt syn-
drome. In: StatPearls. StatPearls Publishing; 2021. https://www.
ncbi.nlm.nih.gov/books/NBK557409/.
6. Isik K, Morkavuk G, Koc G, Odabasi Z: Sjogren’s syndrome
associated with bilateral peripheral facial paralysis. Neuro-
sciences (Riyadh) 2021; 26(4): 389–91. 10.17712/nsj.2021.4.20210
036.
7. Maturu MVS, Datla AV, Selvadasan V, Dalai S: Rare case of cen-
tral pontine myelinolysis: etiological dilemma. Cureus 2021; 13(11):
e19644.
4 MILITARY MEDICINE, Vol. 00, Month/Month 2022
8. Alinezhad N, Habibagahi Z, Ostovan VR: Bilateral facial palsy as
the first presentation of primary Sjögren’s syndrome: a case report
and review of literature. Acta Neurol Belg 2020; 120(4): 999–1001.
10.1007/s13760-020-01372-9.
9. Li X, Zhao Y, Liao Q, Da Y: Myasthenia gravis coexisting with primary
Sjögren’s syndrome: report of three cases and literature review. Front
Neurol 2020; 11: 939. 10.3389/fneur.2020.00939.
10. Shima N, Murosaki T, Yamamoto S, et al: Simultaneous oculomotor
and facial nerve palsies in a patient with systemic lupus erythematosus
and Sjögren’s syndrome. Case Rep Rheumatol 2019; 2019: 4156781.
Downloaded from https://academic.oup.com/milmed/advance-article/doi/10.1093/milmed/usac272/6699282 by guest on 31 May 2023
11. Wei Z, Jiaying S, Junhong G: Bilateral facial paralysis as a rare neu-
rological manifestation of primary Sjögren’s syndrome: case-based
review. Rheumatol Int 2019; 39(9): 1651–4. 10.1007/s00296-019-
04339-z.
12. Gorodetskiy VR, Klapper W, Probatova NA, Vasilyev VI, Rozhnova
EV: Simultaneous occur-rence of Rosai -Dorfman disease and nodal
marginal zone lymphoma in a patient with Sjögren’s syndrome. Case
Rep Hematol 2018; 2018: 7930823.
13. Birnbaum J: Facial weakness, otalgia, and hemifacial spasm: a
novel neurological syndrome in a case-series of 3 patients with
rheumatic disease. Medicine (Baltimore) 2015; 94(40): e1445.
10.1097/MD.0000000000001445.
14. Ashraf VV, Bhasi R, Kumar RP, Girija AS: Primary Sjögren’s
syndrome manifesting as multiple cranial neuropathies: MRI find-
ings. Ann Indian Acad Neurol 2009; 12(2): 124–6. 10.4103/0972-
2327.53083.
15. Hadithi M, Stam F, Donker AJ, Dijkmans BA: Sjögren’s syndrome:
an unusual cause of Bell’s palsy. Ann Rheum Dis 2001; 60(7): 724–5.
10.1136/ard.60.7.724.
16. Moll JW, Markusse HM, Pijnenburg JJ, Vecht CJ, Henzen-Logmans
SC: Antineuronal antibod-ies in patients with neurologic complica-
tions of primary Sjögren’s syndrome. Neurol 1993; 43(12): 2574–81.
10.1212/WNL.43.12.2574.
́
17. Alfaro-Giner A, Peñarrocha-Diago M, Bagan-Sebasti ań JV: Orofacial
manifestations of mixed connective tissue disease with an uncommon
serologic evolution. Oral Surg Oral Med Oral Pathol 1992; 73(4):
441–4. 10.1016/0030-4220(92)90321-G.
18. Uchihara T, Yoshida S, Tsukagoshi H: Bilateral facial paresis
with Sjögren’s syndrome. J Neurol 1989; 236(3): 186. 10.1007/
BF00314342.
19. Yassin A, Nawaiseh M, Shaban A, et al: Neurological manifestations
and complications of coronavirus disease 2019 (COVID-19): a sys-
tematic review and meta-analysis. BMC Neurol 2021; 21(1): 138.
10.1186/s12883-021-02161-4.