An 18-year-old girl hailing from Kozhikode, South India, with no past history of any illness, visited our hospital with complaints of fever of 1 week duration. She also had headache that was increasing in intensity and aggravated in the early morning. She also had two episodes of vomiting. She complained of double vision on the third day of admission, which was followed by asymmetric weakness of both proximal distal muscles of all four limbs. There was no history of sensory disturbances and bladder or bowel dysfunction. On examination, her vitals were stable. There was no pallor, icterus, clubbing, lymphadenopathy, rashes or eschar on general examination. Neurological examination on admission revealed normal speech, intelligence and memory. There was no papilledema. She developed bilateral lateral rectus palsy (Figure 1A and B), with normal upgaze and papillary reflexes. Other cranial nerves were within normal limits. Motor system examination showed normal muscle bulk. Muscle groups in all limbs showed hypotonia with grade 3/5 power in the right lower limb, grade 2/5 power in the left lower limb and grade 4/5 power in both upper limbs. All deep tendon reflexes were sluggish. Sensory system examination was normal. There was neck stiffness with other signs of meningeal irritation. Rest of the systems were within normal limits. Her clinical course is summarized in Figure 2.
Initial investigations revealed normal hemogram, with an elevated erythrocyte sedimentation rate (ESR) of 95 mm in the first hour. Blood sugar, serum electrolytes and renal and liver function tests were within normal limits. Computerized tomography of brain was within normal limits.
With a history of fever and headache and findings of meningitis, lumbar puncture was done, which showed an opening pressure of 190 mm of cerebrospinal fluid (CSF). CSF analysis revealed a CSF total count of 30 cells with 80% lymphocytes. CSF sugar was normal, and protein was mildly elevated (60 mg/dL [normal 20-40 mg/dL]). Bacterial cultures were sterile. CSF adenosine deaminase and culture for Mycobacterium tuberculosis were negative. India ink stain for cryptococci was negative. Cytology did not show any malignant cells. CSF viral serology turned out to be positive for JE virus with positive IgM antibodies specific for the same insignificant titer. Autoimmune markers in CSF (NMDAR, LGI1, Caspr2, AMPAR [GluR1 and GluR2 subunits] and GABA-B-R) were negative.
Magnetic resonance imaging (MRI) of the brain showed hyperintensities in bilateral thalami and in the limbic region, suggestive of limbic encephalitis (Figure 3). MRI of the spine showed T2-weighted hyperintense signal intensities in the spinal cord caudally till the L1 level, predominantly involving the anterior horn cells, and rostrally, they extended upto pons, suggestive of longitudinal myelitis (Figure 4). Electroencephalogram displayed slowing of delta waves in the frontotemporal region.
The patient was treated with supportive measures. However, she deteriorated with the development of altered sensorium and intermittent hypothermia. Oral, axillary, rectal temperature revealed a temperature <94 F on multiple occasions. Electrocardiogram at the time of hypothermia showed bradycardia, prolonged QT interval and Osborn wave (Figure 5). Other causes of hypothermia such as hypothyroidism and hypoadrenalism were ruled out with normal thyroid function tests and fasting serum cortisol levels. Warm saline infusions and intermittent rewarming were given at the time of hypothermia. She developed intractable seizures and autonomic instability and succumbed to her illness after 2 weeks of hospital care.
Written informed consent was obtained from the parents of the patient for the publication of this report and accompanying images.