Bilateral Facial Nerve Palsy in a Young Woman From West Bengal: Do Not Forget Lyme Neuroborreliosis
Correspondence Address: Source of Support: None, Conflict of Interest: None DOI: 10.4103/0028-3886.325335
Source of Support: None, Conflict of Interest: None
Keywords: Facial nerve palsy, India, Lyme disease, neuroborreliosisKey Message: Lyme neuroborreliosis should be considered more often from now in India. The diagnosis is simple and easy, and so is the treatment. With the prompt institution of therapy, cure is the rule, but unfortunate delay can lead to an ominous outcome.
Lyme disease, caused by the spirochete Borrelia burgdorferi, is the most common tick-borne illness in the United States and Europe., Although it had been considered extremely rare in India, a recent study conducted in Nagarahole and Bandipur in South India surprisingly revealed a high seroprevalence (19.9%) of Borrelia burgdorferi infection in a population at risk (forest workers and staff). Hence, it is imperative that clinicians from nonendemic countries, such as India, should be familiar with its diagnosis and management.
The clinical presentation varies with the disease stage, and neurological manifestations (Lyme neuroborreliosis) are reported in up to 12% of patients with Lyme disease., In Europe and in the United States, it most commonly presents as a triad known as Bannwarth's syndrome (lymphocytic meningitis, radiculoneuritis, and cranial neuropathy),,; however, these manifestations can occur in isolation or in combination.,, Other neurological manifestations include cerebrovascular events secondary to postinfective arteriosclerosis, embolism, vasculitis, hemorrhage, and even cerebral venous sinus thrombosis.
The cases of Lyme neuroborreliosis from India till date are summarized in [Table 1].,,,,,,,, Of note, two cases of bilateral facial nerve palsy as the sole manifestation of Lyme neuroborreliosis have been recently reported from Eastern India.
We herein report a case of a lactating woman with acute onset progressive ascending flaccid tetraparesis that was preceded by a misdiagnosed bilateral facial nerve palsy. She was finally diagnosed to be a case of acute neuroborreliosis, which responded favorably to intravenous and orally administered antibiotics.
A 23-year-old previously healthy puerperal lactating woman from rural West Bengal was brought to the emergency room with rapidly evolving progressive weakness of all four limbs, and lancinating pain over the back of the neck and lower back, radiating to upper and lower limbs, respectively, since past one week. The weakness initially started in the distal lower limbs and quickly progressed proximally, followed by a similar pattern of weakness in the upper limbs and neck, making her helpless and bedridden. Ten days prior to admission, she had developed an episode of fever, which resolved spontaneously within two days without any medical attention. There was no history of associated headache, vomiting, loss of consciousness, seizures, radiating/localized neck pain, muscle pain, stiffness, changes in bladder/bowel habits, respiratory distress, abdominal pain, joint pains, or history of recent travel. She was taking iron, folic acid, and calcium tablets daily. There was no history of similar kinds of ailments in her family or adjoining areas of her residence. Two months back, during the last months of her pregnancy, one day after getting up from bed, she noticed slight deviation of her angle of the mouth toward the left side, along with grossly decreased taste sensation and difficulties in closing eyes, blowing, and whistling, which lasted for 1 month. She was diagnosed with “right-sided Bell's palsy” and treated with methylcobalamine and prednisolone.
Vital parameters, including capillary blood glucose, were normal. Her cognitive functions were intact and she apparently had a “masked face.” Cranial nerve examination revealed asymmetric (right > left) bilateral lower motor neuron–type facial weakness (House-Brackmann Grade II) with reduced taste sensation on both sides of anterior tongue and hyperacusis (tearing was preserved) and weakness of neck flexors (2/5). Motor system examination revealed flaccid hypo- to areflexic tetraparesis (muscle power estimation by the Medical Research Council showed 3/5 in proximal upper and lower limbs and 2/5 in distal upper and lower limbs). Plantar reflexes were flexor bilaterally. Sensory examination revealed reduced pinprick sensations up to both knees and decreased joint position and vibration senses. Autonomic functions were intact. Cerebellar functions and gait could not be tested. Examination of bony cranium and spine were normal. There were no signs of meningeal irritation or papilledema.
Acute evolving flaccid ascending symmetric tetraparesis with weak neck flexors and bilateral asymmetric lower motor neuron–type sequential facial paresis with apparently intact autonomic functions led us to think of (1) brainstem involvement, (2) acute anterior horn cell damage following some viral infections, (3) acute motor predominant poly-(radiculo)-neuropathy, (4) acute precipitation of neuromuscular junction transmission disorders, and (5) acute myopathies (mostly inflammatory/infectious).
A complete blood cell count and hepatic, renal, and thyroid function tests were unremarkable except mild elevation of transaminases. Urinalysis revealed mild proteinuria (2+ in urine dipstix). Glycemic indices were within normal limits, and so were electrolytes, arterial blood gases, and muscle enzymes (creatine kinase, aldolase, and lactate dehydrogenase). C-reactive protein and erythrocyte sedimentation rate were elevated. Magnetic resonance imaging of the brain and spinal cord with gadolinium contrast was normal. A nerve conduction study, coupled with needle electromyography, on Day 10 after the onset of limb weakness, revealed a sensorimotor (motor more than sensory) axonal and demyelinating poly-(radiculo)-neuropathy. Cerebrospinal fluid (CSF) study revealed lymphocytic pleocytosis, with raised protein levels and low glucose levels (white blood cell count = 60/μL, protein = 90 mg/dL, glucose = 21 mg/dL [capillary blood glucose = 103 mg/dL]).
Causes of acute motor predominant poly-(radiculo)-neuropathies were searched for. Metabolic causes were ruled out as she was euglycemic, nonazotemic, and the urine sample tested for total porphyrins and toxins were negative. Drugs and toxins were reasonably ruled out as she never had any relevant exposure to organophosphorus, heavy metals, or hexacarbon compounds, and she had no addiction. Connective tissue disorders with autoimmune/vasculitic neuropathies were ruled out as antinuclear antibodies panel, serum angiotensin-converting enzyme, and vasculitis panel were unremarkable. Critical illness polyneuropathy was ruled out from the historical analysis. Thus, we were left with either infectious or paraneoplastic neuropathies. Relevant tests for SARS-CoV-2, hepatitis B, hepatitis C, human immunodeficiency virus, cytomegalovirus, Epstein–Barr virus, Campylobacter jejuni, Hemophilus influenzae, and Tropheryma whipplei were negative. Serum protein electrophoresis with immunofixation failed to reveal any paraproteinemia. A contrast-enhanced computed tomographic scan of neck, thorax, abdomen, and pelvis could not find any neoplastic lesion or suspicious lymphadenopathy. IgM antibodies to Lyme disease by enzyme immunoassay were positive as well as real-time polymerase chain reaction for detection of Borrelia DNA.
We initiated treatment with intravenous ceftriaxone (2 g/day) and oral azithromycin (500 mg/day) for 14 days. Pregabalin and acetaminophen were prescribed for relief of radicular pain. With regular physiotherapy, her deficits improved significantly, and she could walk unaided after three months of discharge from the hospital. At the sixth month of follow-up, she had no demonstrable neurological deficit.
Only 10 cases of Lyme neuroborreliosis have currently been reported from India.,,,,,,,, Among the different presentations, infective meningitis and meningoencephalitis were the commonest clinical pictures.,,,,,,,, Of note, four cases from India had lower motor neuron–type facial paresis.,, Our case also had an asymmetric lower motor neuron–type bilateral facial paresis, which was misdiagnosed by her treating physicians as right-sided Bell's palsy leading to a delay in diagnosis. Low CSF glucose level, as found in this case, is one of the rarely described findings in Lyme neuroborreliosis., Particularly because Lyme neuroborreliosis is eminently treatable, it should be considered in the workup of patients with meningitis with hypoglycorrhachia, apart from considering other more commonly known causes such as tubercular, fungal, protozoal, mumps, malignancy, lupus, and sarcoid. Involvement of neuroretina had also been reported in two patients., A noteworthy finding is that in none of the previously published Lyme neuroborreliosis cases,,,,,,,,, including the current one, erythema chronicum migrans was reported or observed. Therefore, Lyme neuroborreliosis should be suspected even in the absence of this pathognomonic dermatological manifestation, if the clinical picture is suggestive enough. Our case had cranial neuropathy as the presenting manifestation, which unfortunately escaped diagnosis culminating in more severe painful tetraparesis due to sensorimotor poly-(radiculo)-neuropathy. However, with an apt clinical approach and relevant investigations, the diagnosis was established without further delay, and antibiotic treatment helped in apparently rapid cure.
Borrelia burgdorferi reaches the central nervous system either through a hematogenous spread using several transporters and adhesion molecules, crossing the blood–brain barrier by a complex transcellular passage or by retrograde transport from peripheral nerves. Despite incessant research over so many decades, the exact pathogenesis of Lyme neuroborreliosis remains elusive. There are several hypotheses. First, the spirochetes downregulate their surface protein expression and take refuge in the extracellular matrix using complement-neutralizing proteins. Second, they could secrete soluble antigens and stay protected from recognition by the body's immune system. Finally, Borrelia burgdorferi possesses an armament for active suppression of host immunity and prevention of complement-mediated damage. Neurological manifestations may be due to direct cytotoxicity, autoimmune-mediated molecular mimicry, and secretion of cytotoxic cytokines by affected leukocytes and glial cells. In addition, Borrelia burgdorferi may also trigger inflammation that leads to glial/neuronal apoptosis, neurodegeneration, and demyelination.
The possibility of Lyme neuroborreliosis should be considered more often from now on as in the last year, four cases with the kindred clinical syndrome have been described from a so-called “nonendemic zone.” Diagnosis is simple and easy, and so is the treatment. With the prompt institution of therapy, cure is the rule, but unfortunate delay can lead to an ominous outcome.
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