A 48-year-old male presented with a four-day history of acute onset progressive weakness of the lower limbs. The weakness started in the right lower limb and then progressed to involve the left lower limb leading to an inability to get out of the bed without support within 4 days of onset of symptoms. He also complained of sensory loss below the level of the upper abdomen with tingling and paresthesia in bilateral lower limbs. This was associated with a constant, deep-seated, ill-defined pain with dysthesias without any positional variation in the lower back and both lower limbs suggestive of a funicular pain. He also complained of urinary and stool retention. He had a history of fever with mild headache from 3 days prior to the onset of the weakness which persisted till admission (7 days). There were no significant past interventions, medical or family history. He belonged to the lower socio-economic strata and was a rickshaw puller by occupation. On examination, he was febrile and had a catheter in situ. He had hypotonia and motor weakness of lower limbs. The power was 2/5 in the right and 3/5 in the left lower limb, with absent deep tendon/superficial reflexes (anal/bulbo-cavernous) and mute plantar. He had brisk tendon reflexes in both the upper limbs. There was also complete loss of pain and temperature sensation below the T6 level. The sensorium, cognition, optic disc, cranial nerves, cerebellar system, and power in upper limbs were normal on examination. With this history and examination, the possibility of an acute transverse myelitis was considered.
The routine investigations including blood and urine cultures were negative. The contrast enhanced magnetic resonance imaging (CEMRI) of cervical-dorso-lumbar spine showed a T2W/STIR hyperintensity in the spinal cord extending from C7 to D11 vertebral level with partial enhancement, suggestive of a longitudinal extensive transverse myelitis (LETM). The CEMRI brain showed multiple T2W/FLAIR hyperintense lesions in the left frontal, right parietal and left temporal, periventricular white matter, pons, medulla and bilateral cerebellar lobes without any diffusion restriction or post contrast enhancement (Figure 1). Contrast enhanced MRI orbit did not reveal any significant abnormality. With these imaging findings, the diagnosis was revised to an encephalomyelitis. Infective, inflammatory, and demyelinating causes for LETM were investigated. Serum Antinuclear Antibody [ANA], Anti-Neutrophil Cytoplasmic Antibodies [ANCA] and Serum Neuromyelitis optica [NMO], Myelin oligodendrocyte glycoprotein [MOG] antibodies were negative. ELISA for serum viral markers of Hepatitis B, Hepatitis C and HIV were also negative. Serum Acetylcholinesterase (ACE) was normal [40 U/L]. Pathergy test was negative. Contrast enhanced computer tomography of chest and abdomen (CECT) did not reveal any abnormality. Visual evoked potential (VEP) showed bilateral normal P100 latency. The CSF examination showed decreased glucose, i.e., 67 mg/dl against the corresponding random blood sugar of 152 mg/dl and increased protein of 133 mg/dl with a cell count of 70 cells/mm3 (i.e., lymphocytes-56 cells/mm3, neutrophils-14 cells/mm3). No atypical cells were found. CSF gram stain, Ziehl-Neelsen [ZN] stain, India ink and CSF cultures were non-contributory. CSF neuroviral panel (Measles, Mumps, Epstein Barr, Parvo B19, Enterovirus, Varicella zoster, West Nile, Herpes simplex viruses) was also negative. CSF Cartridge based Nucleic Acid Amplification Test [CBNAAT] for Tuberculosis and Venereal Disease Research Laboratory [VDRL] was negative. However, CSF antigen was positive for Cryptococcus neoformans (CRAG) by lateral flow assay. Hence, a diagnosis of cryptococcal encephalomyelitis presenting as a LETM was made based on the clinical presentation and investigations. As the patient was immunocompetent and did not have any occupational exposure, detailed evaluation for any presence of cryptococcus in lungs/sinuses and CD4 counts for immunodeficiency were done but were found to be normal.
The clinical team started the patient on 4 weeks of induction therapy with intravenous liposomal amphotericin B at 5 mg/kg [250 mg] every 24 h and oral fluconazole 200 mg every 8 hourly. After 3 weeks of antifungal therapy his motor power improved to 4/5 and 5/5 in the right and left lower limbs, respectively. His bladder and bowel symptoms resolved completely. His sensory loss resolved, although he had occasional paresthesia. He was discharged in a stable condition with an advice to continue oral fluconazole 400 mg/day for 12 months. The patient is in follow up, he had no side effects, tolerated the medicines and on subsequent examination has now regained full functional capacity. The follow up imaging done after 6 months showed near complete resolution of lesions (Figures 2, 3).