facial palsy distinguishing bells vs Lyme; new tick problem with B. miyamotoi

 Facial palsies are quite common and can be associated with Lyme disease or attributed to Bell’s palsy, however the treatments for these conditions are very different. This study developed a clinical risk tool to help differentiate the cause of the facial palsy (see facial palsy bells vs lyme NeuroClinPrac2025 in dropbox or doi.org/10.1212/CPJ.0000000000200476)

 

Details:

-- they accessed the records of 285 patients with facial palsies who were seen in the Massachusetts Eye and Ear Infirmary (MEEI) between October 18, 2005 and December 13, 2021, having first been seen in various primary care, acute care, and emergency care facilities throughout the US before being referred to this site

-- inclusion criteria for the study included those with a rapid onset of unilateral flaccid facial paralysis, with full evolution of weakness over 72 hours and onset of recovery of facial tone and movement occurring within four months

-- exclusion criteria included  middle ear disease, evidence of tumor or an infectious process affecting the facial nerve, evidence of an active underlying autoimmune or inflammatory disease associated with facial palsy, and evidence of bilateral facial palsy, or a vesicular rash (herpetic), or evidence of head and neck cancer or other neurologic disease affecting motor nerves

 

-- determination of the diagnoses:

    -- for Lyme disease: laboratory evidence of infection, including positive two-tier Lyme disease (LD) antibody testing on a blood sample, or positive CSF testing for the production of intrathecal antibody to B. burgdorferi, or positive PCR testing for detection of B. burgdorferi in blood or CSF. Cases were also included in the LDFP (Lyme disease-associated facial paralysis) group if the patient had been diagnosed with LDFP by an infectious diseases specialist in the setting of ambiguous laboratory results.

    -- for Bells’ palsy: Cases were otherwise classified as BP (Bell’s palsy)

-- 76 patients were felt to have LDFP, and 209 were felt to have BP

    -- Bell’s palsy patients: 60% female, 3% <18 years old/16% 18-30 yo/20% 31- 50yo/19% 51-60yo/ 15% 60-70yo/5% 71-80yo/1% 81-90yo

        -- 0% had radiculitis/transverse myelitis, 2% rash, 0.5% fever, 0.5% nuchal rigidity, 4% fatigue, 18% headache, 55% retroauricular pain or otalgia, 0.5% arthralgia, 20% dysgeusia

    -- LDFP patients: 46% female, 21% <18 years old/20% 18-30 yo/15% 31- 50yo/12% 51-60yo/9% 60-70yo/5% 71-80yo/1% 81-90yo

        -- 4% radiculitis/transverse myelitis, 38% rash, 47% fever, 28% nuchal rigidity, 41% fatigue, 72% headache, 42% retroauricular pain or otalgia, 33% arthralgia, 16% dysgeusia

    -- in addition, Lyme disease was predominantly in June and July, and Bell’s palsy was spread across the year

 

-- based on these patients, the authors were able to develop a clinical risk assessment tool called “FACE DROPS” from the noted pertinent differences between signs and symptoms of LDFP and BP at the time of patient presentation

 

Results:

-- Risk tool developed with seven clinical criteria, and the associated relative weights of the presence of these criteria:

    -- fever (at least 100.4F) +8 to the score

    -- aches (arthralgia/myalgia) +6

    -- cephalgia (headache) +3

    -- exhaustion (unusual fatigue) +4

    -- dermatomal or radicular pattern (transverse myelitis or radiculitis) +4

    -- otalgia or postauricular pain -1

    -- stiff neck (nuchal rigidity) +3

-- FACE DROPS score to differentiate the etiologies:

    -- diagnosis of BP: ≤4

        -- ≥93.5% accuracy

    -- diagnosis of LDFP: ≥7

        -- ≥96.0% accuracy

    -- moderate scores of 5-6 leaned towards LDFP, but with accuracy of only 71.4%

-- of note, nearly one in 5 patients with LDFP had scores of 4 or less

-- 4 patients with BP had scores of 6-8, suggesting LDFP. All of these patients were in the 6th to 8th decade of life

 

Commentary:

-- overall, only about 5% of patients living in endemic Lyme disease areas have facial palsy associated with the acute Lyme disease infection, though facial palsy is the most common manifestation of neurologic effects of Lyme disease in the US. LDFP is typically unilateral but can be bilateral (the latter being excluded from this study)

-- Lyme disease may well be accompanied by systemic symptoms, erythema migrans (EM), and other additional neurologic manifestations such as meningitis, transverse myelitis, or radiculitis

-- 25% of patients with BP and 50% of patients with LDFP have permanent and disfiguring facial sequelae, post-paralytic facial palsy (PPFP), or gustatory epiphora (excessive tearing associated with eating or drinking)

    -- symptoms of PPFP include synkinesis (involuntary muscle movement occurring when an intended muscle movement is performed), impaired expression, gustatory epiphora or rhinorrhea, and facial hypertonicity leading to discomfort

-- treatment of BP: corticosteroids, which lead to shortened PPFP courses

-- treatment of LDFP: antibiotics, with some suggestion that corticosteroids may actually worsen outcomes. For example a study of 51 patients found that those treated with antibiotics plus steroids for LDFP had worse outcomes than those given antibiotics alone: https://pubmed.ncbi.nlm.nih.gov/27598389/

-- the real value of having a risk tool as in this current study is for patients presenting with facial palsy who do not have cutaneous EM indicating Lyme disease and, for those without the typical rash, before the development of a clear and  measurable antibody response to the infecting organism Borrelia burgdorferi. 

    -- the issue here is that testing for Lyme disease is a bit fraught:

        -- serologic test results are negative in 10 to 15% of patients at the time of LDFP diagnosis, and false positives can occur

        -- and, there may well be a delay in obtaining the results

-- of note, though Borrelia burgdorferi is a predominant organism in the United States causing Lyme disease, in Eurasia there are two other Borrelia species (B. afzelii and B. Garinii), and their clinical presentations are somewhat different than in the US, with much more radiculitis symptoms

 

-- though the results of this study are impressive for their accuracy in differentiating BP from LDFP, given the importance of an accurate diagnosis and subsequent appropriate therapy, the study did find that nearly one in 5 patients with LDFP had scores of 4 or less on their risk calculator and 4 older patients with BP had scores of 6-8, so the authors suggest the following in patients presenting with facial paralysis:

    -- laboratory testing for Lyme disease is appropriate in patients with acute facial paralysis in endemic areas for Lyme disease (assuming that there is not clear clinical evidence of Lyme disease, as with EM rash)

    -- empiric antimicrobial treatment for LD is reasonable while testing results are pending in these areas where Lyme disease is endemic during Lyme disease season.

    -- for patients who have scores of 5 or 6, empiric treatment for both Lyme disease and Bell’s palsy is reasonable while testing for Lyme disease, perhaps best with triple therapy including steroids, antivirals, and antibiotics (in general the data are not so impressive for antivirals, though they are often used in severe cases)

        -- given that steroids might undercut the benefit of antibiotics in patients with Lyme disease, these authors recommend that the duration of prescribed antibiotic therapy be significantly longer (not defined) than the steroid course in order to ensure eradication of the spirochete and avoiding steroid-induced immunosuppression

   -- in patients who have a moderate or high score and a negative Lyme disease serologic testing, they do recommend repeating the serologic testing to be sure

 

-- and, by the way, there is a new tick-borne illness rearing its face in the Northeast (as well as north-central US and areas in Canada), having made the journey from Europe, and previously from Japan in 1995, Russia in 2011, and now here: Borrelia miyamotoi (see https://www.journalofinfection.com/action/showPdf?pii=S0163-4453%2824%2900260-3 and https://www.cdc.gov/relapsing-fever/about/about-htrf.html). a few comments:

    -- the ticks involved include those associated with other tick-borne diseases (and one can get more than one of the diseases at the same time....), including Ixodes (and in particular the blacklegged ticks Ixodes scapularis and the Western I. pacificus) , which also carries Borrelia burgdorferi (associated with Lyme disease), tick-borne encephalitis virus, Anaplasma phagocytophilum, Neoehrlichia mikurensis, Rickettsia and Babesia species

    -- the B. miyamotoi spirochete is actually a pretty impressive mimic of my signature: 

                

    -- B. miyamotoi is of the Borrelia family, known for causing relapsing fever, and is associated with deer ticks, "hard ticks"; their natural reservoirs include deer mice, voles, woodrats, squirrels, and shrews in North America, whereas the other Borrelia  relapsing fever diseases are from "soft" ticks and louses

    -- unlike Lyme disease, the B. Miyamoto spirochete is already in the salivary glands (vs Lyme, where it takes up to 72 hours to cycle though and infect us), so B. miyamotoi infection is transmitted within the first 24 hours after the bite

    -- and, this rather evolutionarily-advanced spirochete is also passed vertically from the adult ticks to their larvae though its eggs, unlike the circuitous route of the Lyme spirochete.

        -- which unfortunately for us means that B. miyamotoi can be transmitted by larval ticks, which have their peak activity in August, a month or two after the peak activity of the nymphal and adult tick (ie, for those of us who are hikers or field-meanderers in the northeast who have gotten used to the tick and tick-carrying disease risks ending in early July, the bite season goes on....)

    -- at this point in the US, the prevalence of B. miyamotoi in Ixodes ticks ranges from 1-3%, which seems likely to increase given the increased ease and shortened timeframe of transmission 

    -- the symptoms in immunocompetent people are similar to other tick-borne diseases with headache, myalgia, arthralgia, malaise/fatigue. but there is no rash like Lyme disease. many infected people are asymptomatic or have mild symptoms, and recurrent fever happens in about 9%

    -- immunocompromised  individuals (eg immunoglobulin or other B-cell deficiencies) are at risk of developing neurologic complications (meningoencephalitis)

    -- labs: 50% have mild to moderate leukopenia, thrombocytopenia, elevated ALT and AST levels and high CRP; there is a specific PCR test for the spirochete (though i don't know how available that is)

    -- likely that we will often need to treat this empirically. which is pretty easy, since doxycycline 100mg twice a day for 14 days works, as it does for several other tick-borne illnesses

 

Limitations:

-- these were all patients who came to a large referral center and may have been sicker or otherwise different from patients seen/treated in a primary care setting, limiting generalizability of the results. The results of the FACE DROPS risk assessment needs to be validated in a primary care setting with a large number of patients.

-- the FACE DROPS scoring tool does not include a few pertinent criteria: for example, Lyme disease is much more common during the warm months (though we do see occasional cases at other times) and is clearly more common in areas of endemic Lyme disease.

    -- Since these researchers did not assess the change in interpretation of the scores by including the issue of endemicity of the region or season, we do not know their effect in changing the accuracy of the risk tool. For example, the 93.5% recorded accuracy for Bell’s palsy associated with a low score might well approach 100% in patients presenting from October to May or in areas of low Lyme disease endemicity

    -- The authors intentionally did not include the presence of the EM rash for Lyme disease since its presence is considered diagnostic of Lyme disease and therefore LDFP in endemic region, since most patients do not recall being bitten by a tick, and the rash is often overlooked or lacking (especially in patients with darker skin pigment). also, almost half of the infected Lyme disease patients have abdominal symptoms, mostly nausea or vomiting (again not in their risk calculator)

 

So:

-- it was notable above that:

    -- only 38% of those with Lyme disease in thi study had a rash (though typically there is a rash in about 80%, but it may not be noticed). The identification of the rash is lower in people with darker skin

    -- appropriate targeted therapy is important to prevent complications

-- FACE DROPS only applies to those getting facial palsy in the US, since Lyme disease-related facial palsy has a different presentation in Eurasia

-- what seem to be reasonable general recommendations from this study(though FACE DROPS really needs to be validated in a larger sample of primary care patients):

    -- treat the patient for Lyme disease if their clinical presentation is clear (eg erythema migrans rash)

    -- those with FACE DROPS scores of 4 or lower have a 93.5+% chance of having BP (only 4 patients, all at least in their 6^th decade of life who had BP scores of 6-8), suggesting that these patients overall should be treated with steroids for highly likely BP (though lab tests for Lyme disease is reasonable if in endemic area and especially in the tick season)

    -- those with FACE DROPS scores of 7 or higher have a 96.7+% % chance of having LDFP and should be treated with antibiotics (though it seems that a few in their 6^th plus decade of life may have BP)

    -- and, for those with FACE DROPS scores  of  5 or 6 (71.4% change of having LDFP), it is reasonable to treat patients with the triple therapy as above (antibiotics, antivirals, and steroids, with the antibiotics extending significantly beyond the steroid treatment time-frame) and repeat the Lyme disease serology if negative on the first round

 

-- and, the big issue: scientific research is being devastated by the current president and his sycophants (including RFK, Jr). all of us know that there needs to be ongoing and intensifying research to deal with older infections that are becoming antibiotic-resistant and present a huge international threat (eg see the WHO report from 2017: https://gmodestmedblogs.blogspot.com/2017/03/antibiotic-resistant-bacteria-of-concern.html), and, of course, the predictable emergence of new organisms that will lead to more pandemics in the future

    -- it was quite notable that Paul Sax in his ID blog noted that the NIH was ending support for HIV clinical guidelines next summer, which have been used internationally to update clinicians (and many of us clinicians taking care of HIV patients are not ID-trained, do not read all of the current ID literature, and rely on these guidelines to present and clarify new, important information for HIV care). He suggested we send comments to the Feds on this awful decision. i went on their website that day and the Feds had already shut down their link to leave comments...... 

 

geoff

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