Lyme Neuroborreliosis Presenting with Isolated Intracranial Hypertension: A Case Report Open Access

Lyme borreliosis is a tick-borne disease caused by Borrelia burgdorferi [1]. It is primarily transmitted to humans through the bite of infected Ixodes or “deer” ticks [1]. Eastern Canada and Northeast USA have seen an increase in Lyme disease in recent years. Nova Scotia, Canada, has an incidence of 85.6 per 100,000 population, which is the highest in North America and second only to Central Europe [2, 3]. While most cases are restricted to early localized disease with erythema migrans, about 20% manifest early disseminated disease with arthritis, multiple erythema migrans lesions, or neurological symptoms (often facial palsy, radiculopathy, or meningitis), an entity called Lyme neuroborreliosis (LNB) [1].

Early LNB frequently manifests with both peripheral nervous system and central nervous system (CNS) involvement. Early symptoms include painful meningopolyradiculitis, unilateral or bilateral facial paresis (Bannwarth’s syndrome if combined with lymphocytic pleocytosis), cranial neuritis, or meningitis [1]. In contrast, late-stage LNB may result in more severe, less frequent neurological outcomes such as encephalomyelitis, bladder dysfunction, and neuropathies [4]. In this report, we present a case of a 6-year-old female who had documented early localized Lyme disease but presented with early disseminated disease with intracranial hypertension (IH) as the only sign of LNB.

The patient was cared for at a tertiary pediatric hospital in Nova Scotia, Canada. A detailed review of the patient’s medical records, clinical presentations, laboratory results, and imaging studies was conducted, and written consent was obtained from the parents during the hospital admission.

A previously healthy 6-year-old female presented with complaints of binocular horizontal diplopia, headache, fever, and malaise in the context of a prior tick exposure. Forty-seven days prior to hospitalization, the patient was noted to have an embedded tick on her left temple just below the hair line, which was removed by her parents (Fig. 1). Two weeks later, the patient developed a fever and nonspecific complaints that in retrospect could have been secondary to diplopia or pulsatile tinnitus. One month after the tick removal, the patient presented with fatigue, headache, fever, stomach pain, and erythema migrans rash on the right flank (Fig. 1). She was started on doxycycline in a community hospital. After the initial dose, she vomited, experienced severe headaches, stomach pain, lethargy, and pallor and was switched to amoxicillin under direction of a pharmacist. Nine days later, the patient developed increased fatigue and diplopia and was noted by her parents to have decreased abduction of left eye. Assessment by a local optometrist noted left eye optic disc swelling for which the patient was sent to the emergency department for further assessment. A head computed tomography was normal. Ceftriaxone was started empirically to treat possible Lyme meningitis, and the patient was admitted to hospital.

Ophthalmological assessment revealed visual acuity of 20/20 in both eyes. She had full visual fields on confrontation and pupils were equal and reactive. She had an incomitant intermittent esotropia of 8 prism diopters on distance fixation, slightly worse on left gaze, and an exophoria of 4 prism diopters on near fixation. There were no obvious limitations on extra-ocular movements including abduction of both eyes. Fundus examination showed bilateral optic disc swelling, grade I on the right and grade II on the left (Frisen) (Fig. 2). Optical coherence tomography of the retinal nerve fiber layer showed increased thickness in both eyes.

Neurological assessment of cranial nerves was positive for mild left eye sixth nerve palsy. Otherwise, cognitive, language, motor, tone, coordination, gait, reflex, and sensory systems were unremarkable. Magnetic resonance imaging (MRI) and venography of the head demonstrated findings in keeping with IH including flattening of the posterior sclera, tortuosity and edema of the optic nerve, and a partially empty sella (Fig. 3). There were no signs of venous occlusion. The lumbar puncture (LP) showed an opening pressure of 36 mm Hg (normal values for age of 11.5 cm of water to 28 cm of water). Cerebrospinal fluid (CSF) revealed pleocytosis (23 units × 10⁶/L, normal range 0–7 units × 10⁶/L) and detection of CSF IgG Borrelia antibodies (1.4 antibody index, reference range 0.6–1.2, from Mayo Clinic Laboratories, compliant with Clinical Laboratory Improvement Amendments requirements) with higher CSF titers than in the peripheral blood, confirming a definite diagnosis of LNB based on the European Federation of Neurological Societies (EFNS) 2010 guidelines [5]. The parents had kept the partially engorged Ixodes scapularis tick, which tested positive via positive PCR for Borrelia burgdorferi.

The treatment consisted of high doses of acetazolamide (500 mg TID) to achieve symptom control. The patient was treated with 2 days of ceftriaxone on admission, changed to a 12-day outpatient course of doxycycline for LNB. The papilledema gradually improved over the course of few weeks with complete resolution by the 2-month follow-up visit (Fig. 2), at which time the acetazolamide was weaned without recurrence of symptoms or papilledema.

This case presents a 6-year-old patient with binocular horizontal diplopia and papilledema due to IH as the primary manifestation of LNB. Neuroborreliosis is a neurological manifestation of Lyme disease that is most often caused by Borrelia garinii, followed by Borrelia afzelii and Borrelia burgdorferi [4]. A comparative study indicated greater CSF inflammation in cases involving B. garinii, yet no significant difference in clinical presentation between the strains has been noted [4]. Children are found to be at a higher risk of developing LNB due to the anatomical distribution of tick bites near the CNS, such as the ears, neck, and head [4].

LNB can present with a wide range of neurological symptoms, with Bannwarth’s syndrome being the clinical hallmark. This case report illustrates an unusual presentation of LNB, where IH was the predominant symptom. IH as a complication of LNB is rare. Additionally, the pathophysiology of IH in LNB is not yet fully understood. The first reported case of IH secondary to Lyme disease was by Raucher et al. [6] in 1985 followed by two case series in 1998 and 2016 [6‒8]. Following that, a meta-case series in 2021 reviewed 46 cases with evidence of papilledema being associated with LNB, noting that 87% of cases were in pediatric patients [9]. Headache was the most common presenting feature. A diagnosis of IH was confirmed in 86% of patients based on an elevated opening pressure on LP [9]. Approximately 60% of these patients had normal MRI or computed tomography findings, but half of those tested for intrathecal Borrelia antibodies were positive [9]. More recently, in 2024, a retrospective study involving 44 pediatric patients with papilledema secondary to Lyme disease revealed the papilledema was usually bilateral and most patients had symptoms such as headache, diplopia, and nausea [10]. With a median presentation time of 20 days from symptom onset, most cases were classified as early LNB [10]. The study emphasized that papilledema could occur with or without pleocytosis, elevated opening pressure on LP, or secondary findings of IH on imaging [10]. These findings highlight the challenges of diagnosing IH in children with LNB and differentiating it from conditions such as lymphocytic meningitis or LNB associated optic neuritis [11].

In this case, the symptoms encountered following the initiation of doxycycline were most likely representative of the Jarish-Herxheimer reaction, which would have best been managed by continuing doxycycline with oral non-steroidal anti-inflammatories as needed. MRI findings were consistent with IH but not specific to LNB. The patient’s diplopia and abducens palsy were considered to be secondary to IH as opposed to LNB as they are very common in patients with IH and their resolution corresponded with the successful treatment of her IH with acetazolamide [12]. Previously reported MRI findings in LNB include cerebral white matter foci, edematous lesions, and meningeal or nerve root enhancement [13]. Given the limitations of MRI, an LP remains a crucial diagnostic tool with measurement of CSF opening pressure when suspicion of IH exists. Features on LP that support the diagnosis of LNB include pleocytosis, elevated protein levels, and intrathecal antibody synthesis [14]. In this case, the elevated opening pressure on LP, pleocytosis, and the presence of higher CSF: serum Borrelia antibody titers showcasing possible intrathecal antibody synthesis supported a diagnosis of IH secondary to LNB.

The treatment of LNB in the pediatric population generally favors oral doxycycline, which is effective against the meningitis, cranial neuritis, and radiculitis manifestations [15]. Intravenous antibiotics are reserved for patients with CNS parenchymal involvement, severe neurological symptoms, or those unresponsive to oral therapy [15]. Although concerns about doxycycline causing tooth staining in children persist, it is the preferred treatment. Other antibiotics, such as intravenous penicillin, ceftriaxone, and cefotaxime, are alternative options [15]. For patients presenting with LNB-associated IH, acetazolamide can be administered to reduce intracranial pressure until the underlying LNB is fully resolved and to alleviate symptoms of diplopia [9]. Rarely, patients with severe IH may require neurosurgical intervention, such as serial LPs, lumbar drain placement, or ventriculoperitoneal shunting.

This case report is limited by the non-LNB-specific MRI findings and that it focuses on 1 patient. Larger studies are required to confirm the prevalence of IH as a manifestation of LNB.

This case report presents a rare instance of IH as an isolated sign of Lyme neuroborreliosis. Given the increasing prevalence of Lyme in endemic areas, it is important to consider LNB in pediatric patients who present with IH, even in the absence of concurrent clinical signs of Lyme disease. The CARE Checklist has been completed by the authors for this case report, attached as online supplementary material (for all online suppl. material, see https://doi.org/10.1159/000546097).

This retrospective review of patient data did not require ethical approval in accordance with national guidelines. Written informed consent was obtained from the parents of the patient for publication of the details of their medical case and images.

The authors have no conflicts of interest to declare.

This study was not supported by any sponsor or funder.

Dax Bourcier and Joyce Beshara: conceptualization and design. Dax Bourcier, Joyce Beshara, Griffin Pauli, Tyler Henry, Richard Huntsman, Bashar M. Bata, and Tobias R. Kollmann: interpretation. All authors (Dax Bourcier, Joyce Beshara, Griffin Pauli, Tyler Henry, Michael Peckford, Richard Huntsman, Bashar M. Bata, and Tobias R. Kollmann): clinical data acquisition, contribution to separate parts of the article draft, critical review for intellectual content, approval of the final version, and agree to be accountable for all aspects of the work. Artificial intelligence was not used in the production of this article.

All data generated or analyzed during this study are included in this article and its online supplementary material files. Further inquiries can be directed to the corresponding author.