Case Report: A Typical Lyme Disease With Uncommon Neurologic Presentation

Abstract

Lyme disease is a very common infectious disease worldwide. The seventh cranial nerve palsy occurred in 9% of Lyme disease cases and the majority of them present as unilateral facial palsy. We present a rare case of bilateral facial palsy in Lyme disease due to Borrelia burgdorferi infection. A total of eleven cases of Lyme disease with bilateral facial palsy reported in literature were summarized and compared to our case. The diagnosis and management of Lyme disease with facial nerve palsy were also discussed in this article.

1. Introduction

Lyme disease is a multisystem and multistage infectious disease caused by Borrelia burgdorferi and less commonly by Borrelia mayonii. It is the most commonly reported vector-borne illness in the United States with over 30,000 cases reported annually. 1–3 Although facial nerve palsy is not an uncommon neurologic presentation in the early disseminated stage of the disease, the majority are unilateral. In fact, bilateral facial palsy is an extremely rare condition. We present a case of bilateral facial palsy caused by Lyme disease and reviewed literature on this specific condition.

2. Case

A 32-year-old man presented to the emergency department with four days of rash, headache and one day of right-sided facial weakness. He noticed a large, pinkish rash on the abdomen which was not painful or itchy. Several similar rashes emerged on his legs, trunk, and wrists in the subsequent three days. Meanwhile, the patient had moderate headaches located over the occipital and bilateral parietal areas. One day before presentation, the patient had difficulty in closing the right eyelid and had drooling from the right corner of the mouth when drinking water. He denied fever, chills, chest pain, shortness of breath, nausea, vomiting, abdominal pain, joint swelling or pain. There were no changes in bowel movement or urination and no weakness or sensory changes in the extremities. The patient had no identified allergy to any medicine or food. He lived in a wooded area with his girlfriend, two kids and one indoor cat and often sees deer nearby. However, he could not recall being bitten by a tick and denied recent travel, hiking and sick contact.

On physical examination, the patient had temperature of 36.8 °C, pulse rate of 96 beats per minute, blood pressure of 131/91 mmHg, respiratory rate 16 per minute, and pulse oximetry of 98%. Cranial nerve exam showed diminished wrinkles on the right forehead upon frowning; eyelid closure and cheek puffing were weaker on the right compared to the left; and patient had drop of the right corner of the mouth when smiling. Vision and hearing were normal. Extraocular movement was intact in all six directions bilaterally. Facial sensations were intact bilaterally in all trigeminal dermatomes. The uvula and tongue protrusion were on the midline. Neck was supple with negative Kernig’s sign and Brudzinski’s sign. Strength and sensation were intact in the bilateral upper and lower extremities. All deep tendon reflexes were normal. Multiple oval erythematous rashes were noticed on patient’s trunk and extremities. They were 10–15 cm in diameter with clear margin and were not elevated. Some of the rash showed central clearing (Fig. 1). Examination of other systems were not remarkable.

Fig. 1.

Erythema migrans on left abdomen (A) and left leg (B).

Blood cell count and comprehensive metabolic panel were normal. EKG showed sinus rhythm without evidence of heart block or pericarditis; and non-contrast head CT showed no acute abnormalities. We also performed lumbar puncture. While waiting for serology and CSF analysis results, we started empiric treatment with oral doxycycline 100 mg twice a day for 21 days for early disseminated Lyme disease with right-sided facial nerve palsy, given patient’s history, location in endemic area and the pathognomonic erythema migrans (EM). Patient was discharged home.

Serology showed that Lyme disease antibody IgM and IgG were positive by ELISA. Western blot confirmed positive IgM but showed negative IgG. DNA of Ehrlichiosis chaffeensis, Ehrlishiosis ewingii, and Anaplasma phagocytophilum were negative. CSF was colorless, clear, with specific gravity of 1.005. White cell count was 10/mm³ and no red blood cells were identified. Protein and glucose level were normal. CSF was negative for Lyme disease IgM, IgG and Borrelia Burgdorferi DNA. CSF meningitis viral panel was also negative.

On the 6^th day of treatment with antibiotic, patient came back to the hospital after waking up with left-sided facial weakness. Right sided palsy remained unresolved while erythema migrans (EMs) were partially resolved. Repeated CBC, CMP and head CT were not remarkable. We continued oral doxycycline regimen. On the 17th day of treatment, patient returned to infectious disease clinic for follow-up. His bilateral facial palsy and EMs were all resolved.

3. Discussion

We present an early disseminated Lyme disease with bilateral facial nerve palsy. Bilateral facial palsy is facial paralysis or paresis affecting both sides of the face, with onset being either completely simultaneous or the involvement of the opposite side within 30 days from the onset of the first side.4,5 The patient had a right facial palsy followed by left facial nerve palsy 6 days later met the criteria. The bilateral facial palsy was most likely secondary to Lyme disease. A complete and prompt resolution of palsy upon doxycycline treatment supports the diagnosis.

Bilateral facial palsy is exceedingly rare (0.3–3% of all facial palsies) with an incidence of only one in 5,000,000 cases.6 Unlike unilateral facial paralysis, which is most often idiopathic (a.k.a. Bells palsy), bilateral facial nerve paralysis most commonly has an identifiable cause. A literature review on bilateral facial palsy identified autoimmune, infectious and traumatic reasons are most common, causing 33.74%, 27.61% and 8.59% of cases respectively; while 15.64% of total cases were idiopathic.7 Other etiologies include metabolic, congenital and iatrogenic. 6,7 Borrelia Burgdorferi infection is the most common infectious cause of bilateral facial palsy, followed by HIV, EBV and VZV infection which composed of 13.19%, 3.37%, 1.84%, and 1.53% of the total case respectively.7 In addition, ruling out lifethreatening conditions, such as leukemia or Guillain-Barre syndrome (GBS), is important and requires prioritized investigations.8

Lyme disease may present a broad spectrum of clinical manifestations and severity with the involvement of multiple systems. Early localized disease presents as single erythema migrans. Early disseminated stage may involve multiple EMs, neurological, cardiac and ocular symptoms; while the late disseminated stage usually involved arthritis in large joints. The nervous system is the third most commonly involved organ system after skin and joints and is found in 10–15% of infected individuals.9 Lymphocytic/monocytic meningitis, facial nerve palsy, and radiculoneuritis are classic acute manifestations which may present in combination or alone. Seventh cranial nerve palsy occurred in 9% of Lyme disease cases; among them, 28–29.5% are bilateral.10,11

We summarized cases of Lyme disease with bilateral facial palsy reported in the literature in Table 1. Our case is the only one that reported the presence of EM at the time of initial presentation. Interestingly, the contralateral facial palsy can develop even 3 days–6 days after the start of antibiotic treatment as shown in Diaz et al.’s case12 and our case. The palsy possibly results from neuritis due to immune response to B. burgdorferi infection; and this inflammatory process does not fully correlate to antibiotic treatment. It is also worth noting that two very senior patients (age 80 and 93 years) with little outdoor activities were found to have bilateral facial palsy due to Lyme disease.13,14 Even though differential diagnosis like stroke, malignancy, and diabetes probably rank higher in this age group, Lyme disease should also be considered.

Table 1.

Summary of Lyme disease with bilateral facial palsy.^a

Author (Year)	Age, Gender	Tick exposure (last 3 months)	Onset of CNVII palsy	Interval	Other symptoms	Serology	CSF		Treatment (duration)	Resolution of CN VII palsy
							Cytology/chemistry	Microbiology
Das (2021)15	25, M	Not reported	25 days	Simultaneous	None	IgM (+), IgG (+)	Lymphocytosis + normal protein	Normal	p.o. doxycycline (21 days)	Complete
	27, M	Not reported	27 days	27 days	None	IgM (+), IgG (−)	Normal	Normal	p.o. doxycycline (21 days)	Complete
Diaz (2019)12	60, M	Denied	1 day	4 days	3 weeks of back pain, bilateral extremities weakness, hand numbness, transaminitis, unintentional weight loss.	IgM (+), IgG (+)	Lymphocytosis + elevated protein	IgM (+), IgG (+	) iv ceftriaxone 2 g (28 days)	Complete
Gilchrist (1993)16	25, M	Lives in wood area, saw tick on his body	1 week	1 week	4 weeks of right ear and scalp pain, dysgeusia, lymphadenopathy, and fever	IgM (+), IgG (+)	Lymphocytosis + elevated protein	Not reported	iv ceftriaxone 2 g (21 days)	Partial
Greenberg (2013)17	52, M	tick on ear 2 weeks ago	2 weeks	Simultaneous	2 weeks of headache, left neck pain with radiation to left arm/shoulder, hoarseness	IgM (+)	Lymphocytosis + elevated protein	Not reported	iv ceftriaxone	Complete
Hagemann (2009)18	54, M	new tick bites	A few days	Simultaneous	none	IgM (−), IgG (+)	Lymphocytosis + elevated protein	IgM (−), IgG (+)	iv ceftriaxone	Partial
Méreaux (2020)13	93, F	No	1 week	1 week	4 weeks of leg pain, fatigue; hyponatremia	IgM (+), IgG (−)	Lymphocytosis + elevated protein	IgG (+)	iv ceftriaxone 2 g (28 days)	Complete
Nagata (2014)14	80, M	No	1 week	n/a	1 week of weakness of extremities, vesicoureteral disturbance; SIADH	IgM (+)	Lymphocytosis + elevated protein	Not reported	iv ceftriaxone 2 g (21 days)	Worsened
Renaud (2018)19	10, M	Saw ticks locally	1 day	Simultaneous	Spike fever and evanescent rash 3 weeks ago	IgM (+), IgG (+)	Lymphocytosis + elevated protein	Not reported	iv ceftriaxone (28 days)	Complete
Vanzieleghem (1998)20	7, M	Suspected tick bite 1 month ago	1 day	Simultaneous	1 week of headache, bilateral conjunctivitis	IgM (+), IgG (+)	Elevated protein	DNA (−)	iv ceftriaxone (14 days)	Complete
Wong (2020)21	16, M	Lives in wood area	4 days	1 day	8 days of headache	IgM (+), IgG (+)	n/a	Note done	po doxycycline	Not reported
Our case (2023)	32, M	Lives in wood area, saw deer	1 day	7 days	4 days of headache, EM	IgM (+), IgG (+)	Normal	IgM (−), IgG (−)	po doxycycline (21 days)	Complete

^aDatabase: PubMed; Search strategy: ((((lyme disease) OR (disseminated lyme disease)) OR (Lyme neuroborreliosis)) OR (Borrelia burgdorferi infection)) OR (Borreliosis) AND (((((bilateral facial palsy) OR (bilateral facial nerve palsy)) OR (bilateral cranial nerve VII palsy)) OR (bilateral facial paralysis)) OR (contralateral facial palsy)) OR (contralateral facial paralysis). Manual screening of case reports on adult Lyme disease with bilateral cranial nerve VII palsy was performed.

Epidemiology, patient’s history, and physical examination are crucial in early diagnosis of Lyme disease. Patients with suspected Lyme disease should undergo serologic testing for antibodies against B. burgdorferi. However, clinicians should be aware of the variable sensitivity and the waiting time of the serology study. For early disseminated Lyme disease, the traditional algorithm of enzymelinked immunosorbent assay (ELISA) followed by Western blot (WB) has sensitivity of 60.7–73.0% and the modified algorithm composed of two sequential ELISA with different targets has sensitivity of 60.7–100%.22,23 Therefore, a diagnose made clinically should warrant empiric antibiotic treatment with pending initial serology or repeated serology at a later stage when immunity is better mounted.

Oral doxycycline is the treatment of choice for patients with acute cranial neuropathy (particularly facial nerve palsy) and/or other neurological manifestations such as meningitis, sensory or motor radiculoneuropathy when hospitalization is not required. The duration of treatment is 14–21 days. For patients who are more acutely ill requiring inpatient care or for patients with parenchymal involvement of the brain or spinal cord (such as encephalitis or myelitis), ceftriaxone 2 mg IV daily for 14 days is preferred.24 It is also worth noting that corticosteroids are not recommended in Lyme disease-associated facial nerve palsy, which is different from the treatment of Bell’s palsy.24

The prognosis of bilateral facial palsy in Lyme disease is good. Most cases have complete resolution of bilateral facial palsy within 1 month or after the antibiotic treatment (Table 1). Partial resolution was observed in two cases; one case continued to have left-sided palsy after 4 months,16 while the other patient was not able to blow his hunting horn despite of some improvement.18 One case reported worsening symptoms in the context of co-existing T-cell lymphoma.14

4. Conclusion

Bilateral facial palsy is an extremely rare condition whose common etiologies include autoimmune, infection and trauma. In Lyme disease, bilateral facial palsy comprises of less than one-third of all facial palsy which, in turn, is observed in only 9% of cases. Most cases in the literature report the copresentation of bilateral facial palsy with lymphocytic meningitis, radiculoneuropathy or systemic symptoms in Lyme disease. We present the first case of Lyme disease with isolated bilateral facial palsy and pathognomonic EMs on initial presentation. Detailed history and physical examination finding were critical in aiding early diagnosis and treatment in our case. All the symptoms resolved completely on the 17^th day of a 21-day treatment course with oral doxycycline.