Bannwarth's syndrome and a surgicaldilemma: An uncommon zoonosis with a rare presentation

Abstract

Lyme's disease also known as Erythema chronicum Migrans, is a multisystem infectious disease caused by the spirochete “Borrelia burgdorferi” which is transmitted by “Ixodes” tick, with both specific and nonspecific manifestations. Nervous system involvement occurs in 10%–15% of untreated patients and typically involves lymphocytic meningitis, cranial neuritis, and/or polyradiculitis. Here, we are reporting an interesting and challenging case of Neuro-Borreliosis in a young officer cadet, meeting the description for Bannwarth's syndrome and presenting initially as a surgical emergency followed by paraparesis. The diagnosis was finally clinched based on clinical profile of Chronic Myeloradiculopathy with focal myositis in the setting of recent outdoor camping, and confirmed by demonstrating high IgG antibody titres in serum and Cerebro spinal fluid (CSF). The officer cadet was treated successfully with a 6 week course of Ceftriaxone and Doxycycline, and went back to full training.

Introduction

Lyme disease is usually seen in temperate hilly regions of Northern India.¹ Typical symptoms include fever, headache, fatigue and a characteristic skin rash called erythema migrans. If treatment is delayed, infection can spread to the joints, heart and nervous system.² This case report illustrates an unusual case of neuroborreliosis from Southern India (Kerala).

Case report

An 18-year-old male patient was first hospitalised in a peripheral hospital on January 29, 2017, with pain in the right inguinal region for 6 h. He was provisionally diagnosed with acute appendicitis based on clinical and ultrasound abdomen findings and managed conservatively with Intravenous (IV) fluids and antibiotics for 7 days. He responded well to therapy and was discharged after 1 week, and an elective appendicectomy was planned after 6 weeks. He reported back to the hospital on March 15, 2017, for aggravation of pain and discomfort in the right inguinal region. Clinically, his examination was unremarkable, and a fresh ultrasonography (USG) abdomen revealed evidence of thickening of the ileocaecal region (Tubercular etiology). No appendicular lump was visualised. He was transferred to a tertiary care hospital on March 20, 2017, for further management. Clinical examination at this centre was again unremarkable, and contrast enhanced computerized tomography (CECT) of the abdomen revealed indirect right inguinal hernia and chronic epididymitis (Fig. 1).

Fig. 1.

Contrast enhanced computerized tomography (CECT) abdomen showing right indirect inguinal hernia (red arrow).

Laparoscopic mesh repair was performed for hernia, and antibiotics were given for 1 week for epididymitis. His workup for tuberculosis (TB) was negative. He reported relief from pain after surgery and was discharged. He reported back to the hospital within 3 weeks with recurrence of pain in both inguinal regions, radiating to the perineum and reported a weight loss of 6 kg, along with complaints of low-grade intermittent fever and difficulty in walking. A detailed history revealed features of proximal weakness in both lower limbs along with severe radicular pains in the both inguinal regions (right more than left), radiating to medial aspects of the thigh and spreading up to the perianal region. He had mild urgency and precipitancy of urine but no incontinence. Neurological evaluation revealed normal higher functions, speech and cranial nerves. Motor examination revealed Ashworth grade 2 spasticity in both the lower limbs with grade 4 + weakness in the hip flexors and adductors on both sides. The knee power was normal, but he had grade 4− weakness of the ankle dorsiflexors on the right side. Reflex examination in the upper limbs was normal. Lower limb reflexes were brisk, except for the left knee jerk, which was absent. His plantar reflex was extensor on the right side and equivocal on the left side. His abdominal and cremasteric reflexes were absent bilaterally. His sensory examination revealed hyperaesthesia in bilateral T12 and L1 dermatome, with tenderness over the muscles of the perineum and inner thigh region. Vibration and joint position sense were preserved.

A clinical diagnosis of chronic myeloradiculopathy with focal myositis was considered. Myelon involvement was suggested by bladder urgency along with spastic paraparesis and pyramidal signs in the lower limbs. Radicular involvement was suggested by chronic pain and hyperaesthesia in a dermatomal distribution (T12 and L1), along with proximal lower limb weakness and absent knee jerk on the left side. Myositis was suggested by muscle pain and tenderness in the groin and medial thigh areas. In view of a chronic presentation with fever, our broad differentials included, tubercular arachnoiditis, neurosarcoidosis, lymphomatous meningeal infiltration and a remote possibility of spinal carcinomatosis.

His lab evaluation revealed an Erythrocyte Sedimentation Rate (ESR) of 35 mm fall after 1 hour. Complete blood counts, liver and renal functions were normal. Anti nuclear antibodies (ANA) and Anti neutrophil cytoplasmic antibodies (ANCA) profiles were negative. His viral serology for Human immunodeficiency virus (HIV), Hepatitis B surface antigen (HBsAg), Hepatitis C virus (HCV), Cytomegalovirus (CMV) and Herpes simplex virus (HSV) was negative. Cerebro spinal fluid (CSF) analysis was normal, and CSF Adenine deaminase (ADA) was 04 IU/ml. His radiological evaluation with CECT of the chest and abdomen was essentially normal. Contrast magnetic resonance imaging (MRI) of the brain and whole spine along with both lumbosacral plexuses was normal (Fig. 2). Special Short tau inversion recovery (STIR) images of the thigh showed inflammation and oedema in the perineum and adductors of the thigh (Fig. 3). His bone marrow examination, serum protein electrophoresis and a whole body positron emission tomography (PET) scan were also normal.

Fig. 2.

(a and b) Postcontrast T1-weighted magnetic resonance (MR) images of the spine with no abnormal enhancement evident.

Fig. 3.

Short tau inversion recovery (STIR) axial images of the upper thighs showing abnormal hyperintensities in the medial compartment of both thigh muscles (red arrow).

On detailed questioning, he gave history of attending an outdoor camp in his training area (in Kerala), in the month of January 2017. There, he had sustained an insect bite with skin rash in the right groin region, which resolved in 1 week. Thus, a possibility of Lyme disease with myeloradiculitis and myositis was considered. Serology for Lyme IgG antibodies was positive in both serum and CSF (five times elevated). The patient was started on ceftriaxone injection 2 gm IV 12 hourly (BD) for 4 weeks along with doxycycline capsule 100 mg BD for 8 weeks. He showed rapid improvement in pain and fever within 2 weeks of therapy, with full motor recovery in 8 weeks.

Discussion

This case was unusual in its presentation as the patient had repeated radicular inguinal pain as the first symptom. This was initially misdiagnosed and managed as appendicitis and later as inguinal hernia. The common initial central nervous system (CNS) manifestations of Lyme disease are aseptic meningitis, cranial or peripheral neuropathy and sometimes fluctuating meningoencephalitis. The absence of any of these typical symptoms probably resulted in the diagnostic dilemma in this case.

In addition to facial palsies, severe and painful meningoradiculitis of the cauda equina (Bannwarth's syndrome) is particularly characteristic and is reported more commonly in Europe.³ This case clinically had myeloradiculopathy, which itself is rare, and the radicular pain with paraparesis and bladder symptoms was similar to those described in Bannwarth's syndrome. Radiculopathy affects no more than 5% of the 30,000 patients with Lyme disease reported to the centers for disease control and prevention (CDC) annually.⁴ Lyme neuroborreliosis involving spine and radicles has been mentioned in the literature as a possibility, but we could find only one case report of myeloradiculopathy.⁵ Parenteral cephalosporins or benzylpenicillin is recommended to be used for at least 4 weeks in patients with Lyme disease with severe systemic involvement or neurological complications.⁶

Conclusion

Myeloradiculopathy and focal myositis are rare manifestations of neuroborreliosis. This case report highlights the need for detailed history taking to reach a correct diagnosis in difficult cases. When suspected clinically, the diagnosis can be established with reasonable certainty using an enzyme linked immunosorbent assay (ELISA)-based antibody test available in serum and CSF.⁷ The key to treatment is the duration of antibiotic therapy, which has to be given for 4–8 weeks depending on clinical presentation.

Conflicts of interest

The authors have none to declare.