This report presents a case of neuroleptospirosis in a 6-year-old girl from New Delhi, India, illustrating the clinical complexities and management strategies involved. The child, born at term through an uncomplicated vaginal delivery as the second child of a non-consanguineous marriage, was previously healthy. She presented with fever, headache, vomiting, and seizures. Her illness began 12 days prior with intermittent high-grade fever that temporarily resolved with oral medication but recurred every few hours. Five days before admission, she experienced a seizure characterized by jerky movements of all four limbs, upward rolling of the eyes, frothing at the mouth, and facial deviation. The episode lasted less than a minute but left her with altered sensorium, manifested by irrelevant speech, unresponsiveness to basic questions, and partial recognition of her family. Neurological examination revealed increased tone in all four limbs—more pronounced in the lower extremities—absent deep tendon reflexes, and mute plantar responses. Additional findings included tongue fasciculations, palatal dystonia, and persistent altered consciousness. Due to repeated seizures and deteriorating neurological status, the child was intubated and placed on mechanical ventilation. Antiepileptic therapy—including a midazolam infusion, phenytoin, levetiracetam, and valproic acid—was initiated but did not halt the progression of symptoms.

Given the rapid progression of her symptoms, various differential diagnoses were considered, including infectious meningitis (bacterial, viral, or fungal), tuberculous meningitis, immune-mediated and autoimmune conditions (such as acute disseminated encephalomyelitis, Guillain-Barre syndrome, or multiple sclerosis), and cerebrovascular causes.

Laboratory investigations revealed lymphocytic pleocytosis and elevated protein levels in the cerebrospinal fluid (CSF), suggesting aseptic meningitis. However, bacterial cultures, tuberculosis cartridge-based nucleic acid amplification test, and a comprehensive meningitis/encephalitis panel were negative. The panel tested for Escherichia coli, Haemophilus influenzae, Listeria monocytogenes, Neisseria meningitidis, Streptococcus agalactiae, Streptococcus pneumoniae, cytomegalovirus, enterovirus, herpes simplex virus 1 and 2, human herpesvirus-6, human parechovirus, varicella zoster virus, and Cryptococcus neoformans/gattii.

Importantly, serology was positive for immunoglobulin M (IgM) antibodies against Leptospira by enzyme-linked immunosorbent assay (ELISA), confirming the diagnosis of neuroleptospirosis. Testing for Leptospira in the CSF could not be performed due to resource limitations. Initial computed tomography imaging of the brain showed no abnormalities; however, subsequent magnetic resonance imaging revealed symmetrical hyperintensities in the substantia nigra of the midbrain on T2-weighted and fluid-attenuated inversion recovery sequences. The lateral ventricles appeared prominent, with dilated temporal horns, indicating central nervous system involvement (Fig. 1).

The child was initially started on intravenous ceftriaxone, acyclovir, and anti-tubercular therapy (ATT); the ATT was discontinued once the tuberculosis workup returned negative. Intravenous immunoglobulin was administered but had minimal effect.

Once Leptospira infection was confirmed, the child was started on doxycycline, which improved her fever as well as her renal and liver function profiles, although her neurological symptoms persisted. Significant clinical improvement was observed only after corticosteroid therapy was introduced. The patient initially received an injection of dexamethasone at 2 mg/kg/day, followed by a transition to oral prednisolone at 2 mg/kg/day.

By the third day of corticosteroid treatment, she demonstrated spontaneous eye-opening, made eye contact, and responded to gestures. By day 5, she was extubated and no longer required supplemental oxygen. Neurological recovery continued with gradual resolution of tongue fasciculations and palatal dystonia. The child was transitioned to oral corticosteroids, and her antiepileptic regimen was reduced to levetiracetam monotherapy. She tolerated oral feeding well and actively participated in oromotor stimulation exercises, which aided her recovery.

Antibiotic therapy remains the cornerstone of leptospirosis treatment, with agents such as penicillin and doxycycline proving effective when administered early. In this case, doxycycline successfully managed systemic symptoms but was insufficient to address the severe neurological complications. The introduction of corticosteroids played a pivotal role in reducing inflammation and promoting rapid neurological recovery, underscoring their importance in managing severe cases of neuroleptospirosis. Supportive care—including mechanical ventilation, seizure management, and rehabilitative therapies such as oromotor exercises—also contributed significantly to her recovery. A multidisciplinary approach ensured comprehensive management of her condition, leading to a favorable outcome.

The decision to administer high-dose steroids was made after a literature review, including a Cochrane review that assessed four clinical studies on the effects of corticosteroids in severe cases of leptospirosis [5].

This case emphasizes the importance of early recognition and treatment of neuroleptospirosis, particularly in pediatric populations. In endemic areas, healthcare providers should remain vigilant for leptospirosis in children presenting with fever and neurological symptoms, especially following potential exposure to contaminated water or animals. The case also highlights the potential role of corticosteroids in managing severe neurological manifestations.

Given the rarity of neuroleptospirosis, establishing a standardized treatment algorithm may be challenging. However, based on our experience, we suggest that in endemic areas, when a patient presents with similar manifestations, an ELISA for Leptospira antibodies should be promptly performed, and Leptospira should be included in the CSF analysis panel.

In conclusion, neuroleptospirosis is a rare but potentially life-threatening complication of leptospirosis. Early diagnosis and prompt initiation of appropriate therapy are crucial to preventing severe complications and improving outcomes. Written informed consent was taken from the patient’s parents.