Lyme disease in north India: a case for concern

Introduction

Lyme disease, rarely seen in India, is caused by a tick-borne (Ixodes) spirochete, Borrelia burgdorferi. Ticks become infected with the spirochete while feeding on the infected animal carriers such as mice, squirrels, birds, etc. and transmit the disease to humans incidentally.

Case report

A 20-year-old man presented with recurrent high-grade fever and headache. Investigations including cerebrospinal fluid (CSF) examination and imaging performed after three weeks of onset were normal. Steroids and antibiotics were administered for one week, but the symptoms persisted. Subsequently, acute onset painless loss of vision occurred. Ophthalmological examination being normal, the treatment was continued. One week later, he developed acute onset symmetrical quadriparesis. Generalised hyper-pigmented erythematous lesions (0.5 cm) (Figure 1), bilateral abducens palsy, 4/5 power in right lower limb, bilateral hypoactive jerks and flexor plantars, and meningeal irritation were the positive findings elicited. OPEN IN VIEWER

A clinical diagnosis of meningo-encephalitis was made. Old white matter ischemic changes were visible on MRI of the brain (Figure 2). CSF opening pressure was high as was its protein content (214 mg/dL). Serum angiotensin converting enzyme (ACE) levels were normal; PCR-TB and ANA were negative. Computed tomography (CT) of the thorax revealed soft tissue density in the posterior segment of the right upper lobe measuring 5.1 cm × 4.1 cm limited by major fissure inferiorly, multiple bilateral small nodules, mediastinal and para-tracheal lymphadenopathy (Figure 3). The endoscopic ultrasound-guided subcarinal lymph node cytology was inconclusive. Serology for Lyme (IgM and IgG by EIA) was positive but negative for cryptococcus and Brucella. PET-CT reported hypermetabolic right upper lobe mass, bilateral cervical, mediastinal, axillary lymphadenopathy along with diffuse hypermetabolism in spleen and the bone marrow, suggestive of a lymphoproliferative disorder prompting a bone marrow examination that was hypocellular. OPEN IN VIEWER

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Figure 3.

Contrast-enhanced thorax CT showing consolidation in RUL with parenchymal nodules.

Intravenous ceftriaxone, vancomycin, oral doxycycline and steroids and mannitol to reduce intracranial pressure were initiated. With persistence of symptoms after two weeks of treatment, anti-tuberculous treatment (ATT) was initiated. Nearly three weeks later, the patient experienced multiple episodes of generalised tonic clonic seizures. Bilateral VIth cranial nerve palsy and bilateral papillitis were evident; power in both lower limbs was reduced to 3/5 with absent deep tendon reflexes with bilateral flexor plantars. The investigations suggested acute liver injury and ATT was withheld. IgM was again positive for Borrelia sp. in CSF.

Anti-epileptics, steroids and intravenous doxycycline were administered for two weeks. Headache and fever subsided and upper limb weakness improved; however, blindness and lower limb weakness persisted. Fundus examination subsequently revealed bilateral optic atrophy. The patient was discharged from the hospital and is regularly being followed up without any change in his clinical status.

Discussion

Slowly progressive encephalitis, bilateral papillitis progressing to optic atrophy, bilateral abducens palsies, quadriparesis and seizures, dermatological and pulmonary lesions, lymphadenopathy and hypocellular marrow were suggestive of an infectious or an autoimmune disease. As polymerase chain reaction for Borrelia sp. is not performed in our country, the diagnosis was made by positive serology on two occasions. Also, clinical improvement in the patient following treatment with doxycycline supports the diagnosis.

While 13% seroprevalence has been reported in the healthy population in north-east Indian states, ¹ reports of Lyme disease from the neighbouring north Indian states of Haryana ² and Himachal Pradesh ³ have been restricted to dermatological lesions and lymphadenopathy. We report Lyme disease with multi-system involvement and skin manifestations for the first time from India. The utilisation of injectable over oral doxycycline in suspicious cases with neurological manifestations is also emphasised.

Evidence of the presence of the ticks and the reservoir over a couple of decades and reports of the disease clustered over the last 3–4 years from non-endemic regions may be the harbinger of a potentially dangerous outbreak in the near future. Lack of knowledge about the disease, its slow progression, multi-system involvement, atypical manifestations and lack of widespread availability of diagnostic testing of the disease are the main challenges in such an eventuality.