|Ahead of print publication
Lyme neuroborreliosis in a critically ill patient
Rui Alexandre Do Prado Costa1, Maria João Castro Santos Pinto2, José Rogério Bernardo Ruas3, Nilza Carvalho Pinto1
1 Department of Physical and Rehabilitation Medicine, University Hospital Center of São João, Porto, Portugal
2 Department of Neurology, University Hospital Center of São João; Department of Clinical Neurosciences and Mental Health, Faculty of Medicine, University of Porto, Porto, Portugal
3 Department of Infectious Diseases, University Hospital Center of São João, Porto, Portugal
|Date of Submission||22-Apr-2020|
|Date of Decision||07-May-2020|
|Date of Acceptance||04-Jun-2020|
|Date of Web Publication||24-Jul-2020|
Rui Alexandre Do Prado Costa,
Serviço de Medicina Física e de Reabilitação, Centro Hospitalar Universitário de São João, Alameda Prof. Hernâni Monteiro, 4200-319 Porto
Source of Support: None, Conflict of Interest: None
Lyme neuroborreliosis (LNB) is a nervous system infection caused by the species of the spirochete Borrelia. A woman, with a history of right facial palsy, was admitted to the physical and rehabilitation medicine (PRM) unit for the rehabilitation of presumed sequalae of aneurism rupture and neuromuscular weakness related to critical illness. She presented in the PRM unit with flaccid quadriparesis, right facial nerve paresis, bulbar paresis, aphonia, dysphagia, and hypoactive deep-tendon reflexes. Electromyography (EMG) revealed multifocal neuropathy and serologic tests revealed positive for Borrelia (IgG). The patient was treated with 2 weeks of intravenous ceftriaxone with a marked improvement in the following days. LNB diagnosis was made due to the clinical presentation, positive Borrelia serology, and great improvement with targeted therapy. Neuromuscular weakness related to critical illness was the main differential diagnosis; however, weakness in bulbar and facial musculature and the EMG findings were not consistent with this diagnosis.
Keywords: Aneurysm, facial paralysis, lyme neuroborreliosis, mononeuropathies
| Introduction|| |
Lyme disease is caused by a tick-borne spirochete of the Borrelia species and can have neurological manifestations as result of dissemination of the bacterium, termed Lyme neuroborreliosis (LNB), which occur in about 10% of patients. At least 80% of European patients present with facial palsy (FP) and radiculitis, but neuroborreliosis has to be considered in a wide spectrum of neurological diseases.
This is a case report of LNB diagnosed after ruptured intracranial aneurysm in a patient with a history of peripheral FP.
| Case Report|| |
A 54 year-old woman presented with a history of presumed unresolved idiopathic peripheral right FP since 2015, arterial hypertension, obesity and dyslipidemia. In January 2019, she was admitted to the emergency room with the sudden onset of explosive holocranial headache and vomiting. Computed tomography angiography revealed a ruptured saccular aneurysm localized on the anterior cerebral artery. She was submitted to microsurgical clipping and then transferred to the intensive care unit (ICU). After weaning of sedation, she remained unresponsive. Cerebral magnetic resonance imaging (MRI) revealed multiple small areas of cytotoxic edema [Figure 1] and electroencephalogram (EEG) evidenced severe diffuse encephalopathy. She stayed in prolonged coma for 6 weeks (Glasgow Coma Scale 6). By week 7, the level of consciousness improved in eye-opening response and ability to obey to some simple commands.
|Figure 1: Brain magnetic resonance imaging 6 days after admission. Multiple small areas of cytotoxic edema were found in both centrum semiovale, left striatum nucleus, frontoparietal and right occipital cortical regions, left hemiprotuberance and left cerebellar hemisphere (the latter two not shown). Those areas did not show contrast enhancement and magnetic susceptibility, being suggestive of recent ischemia. Epicranial hematoma at parieto-frontal region due to the surgical intervention is also shown on the images. (a) T2-fluid attenuation inversion recovery-weighted (FLAIR) image. (b) T2-weighted image. (c) Diffusion-weighted image. (d) Apparent diffusion coefficient-image|
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By week 9, she was transferred to inpatient physical and rehabilitation medicine (PRM) unit of our hospital. At the admission in this unit, she had a functional independence measure (FIM) score of 20 out of 126. She had cognitive slowing, poor collaboration, right peripheral FP, bulbar weakness with severe hypophonia and dysphagia, flaccid tetraparesis (muscle strength in medical research council scale <3 in all limbs), hypoactive deep-tendon reflexes, and absent head and trunk control. Despite several weeks of continuous rehabilitation, neurological examination did not improve and she remained totally dependent in all the activities of daily living (ADL). Initial laboratory testing only revealed a mild elevation of erythrocyte sedimentation rate (37 mm/h) [Table 1]. A second EEG continued to indicate the same level of severe diffuse encephalopathy. Nerve conduction studies and electromyography (NCS/EMG) evidenced severe axonal asymmetric mononeuropathy multiplex in all four limbs [Table 2], [Table 3], [Table 4], [Table 5]. At this point, an extensive etiological study of mononeuropathy multiplex was pursued. Blood serological tests [Table 1] revealed positivity for IgG and negativity for IgM antibodies for Borrelia (confirmed by Western blot). Other infectious and immunological causes were ruled out [Table 1]. Cerebrospinal fluid analysis [Table 1] only revealed unspecific mild hyperproteinorrhachia; it was negative for Borrelia antibodies and other infectious agents.
By week 13 LNB diagnose was assumed, and the patient was treated with ceftriaxone 2 g IV for 2 weeks. A marked improvement occurred in the following weeks: right peripheral FP practically resolved; there was a marked, yet partial recovery of muscle strength and normalization of muscle tone and the intensity of deep-tendon reflexes. She was able to progress from enteral to oral feeding, recovered speech ability and walking capacity with minor left foot drop, and functional autonomy in all ADL, requiring minor third person assistance. At discharge of PRM Unit, the FIM score was 98 out of 126. The patient had complaints of paresthesia involving the distal left lower limb that ameliorated with gabapentin 300 mg daily.
| Discussion|| |
This case of LNB was diagnosed in the context of a ruptured aneurysm with manifestations of the peripheral nervous system. We hypothesize that the first known manifestation was the right FP, misdiagnosed as Bell's palsy. Attending to the findings of neurological examination, the presence of mononeuropathy multiplex, positive Borrelia IgG and the marked improvement after ceftriaxone administration, we considered LNB diagnosis. Although no epidemiologic context was known and no previous history compatible with Erythema migrans was reported, the development of FP years earlier suggests LNB at that time. Multifocal inflammatory neuropathy is common throughout the course of Lyme disease and reversible peripheral nerve abnormalities, with an electrophysiological pattern of axonal damage, occur in one-third of patients with late LNB. Peripheral nerve disease in Lyme disease can be adequately treated with oral antibiotic.
Arterial aneurysms have been reported in patients with Lyme disease, posing a possible causal relation, mediated by acquired degenerative process, particularly vasculitis, despite being controversial.,,
The findings in brain MRI, consisting of multiple lesions suggestive of ischemia, can be explained by the cardiovascular risk factors present in this patient.
The heterogeneity among the strains of Borrelia species seems to be the main factor causing differences in the clinical expression of human Lyme borreliosis. Genospecies identification was unfortunately not performed once methods for that purpose were not available in our hospital.
In this case, the main differential diagnosis was neuromuscular weakness related to critical illness, since the patient stayed in the ICU for several weeks, the first two mechanically ventilated, developing flaccid tetraparesia and hyporreflexia. Nevertheless, weakness in bulbar and facial musculature is not common in this condition, and the concurrent encephalopathy and the NCS/EMG findings do not support this diagnosis.
It is known that immune dysregulation occurs in critically ill ICU patients and the use of corticosteroids may increase spirochetal load. Those factors could explain the marked exacerbation of LNB in this patient during ICU stay. Spirochaetes that survive in localized niches in untreated patients for several years may bypass innate and adaptive immune mechanisms in situ ations of immune dysregulation, such as this context of critical care.
Natural history of the disease and antibiotic therapy are the determinant on the functional recovery of those patients and rehabilitation professional must be aware that possible posttreatment symptoms with sensorial and/or motor deficits can occur like in this case.
Patients with FP are common in the rehabilitation field and lack of recovery should be cause for concern for professionals. An extensive medical investigation should be performed in these cases, especially if associated with other manifestations. FP in Lyme disease is often misdiagnosed as Bell's palsy, as the preceding rash may not occur, or may have been missed. Moreover, misuse of corticosteroid in Lyme disease-associated FP may lead to worse long-term facial function outcomes.
We would like to acknowledge Dr. Fernando Parada, head of Physical and Rehabilitation Department and Dr. Maria José Festas, coordinator of the inpatient rehabilitation unit, for supporting the management of the presented clinical case.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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[Table 1], [Table 2], [Table 3], [Table 4], [Table 5]