(Kiosk 6) Cognitive and Functional Improvement in a Pediatric Renal Transplant Patient with West Nile Virus Neuroinvasive Disease: A Case Report

Case Diagnosis: West Nile Virus Neuroinvasive Disease

Case Description or Program Description: Neuroinvasive West Nile Virus (WNV) is poorly described in the pediatric population - especially in the immunocompromised sub-population. Our case patient developed a progressively worsening movement disorder and had alterations in her consciousness. Through a combination of reduction in immunosuppression and dopamine agonist medications, we saw improvement in responsiveness and movement disorder.

Setting: Inpatient Rehabilitation

Assessment/Results: A 10-year-old female with a single deceased donor renal transplant at 6 years-old for focal segmental glomerulosclerosis on immunosuppression presented with subjective fever, encephalopathy, vertical strabismus, dysarthria, dysmetria, and titubation. MRI Brain was notable for leptomeningeal T2 enhancement and mild cerebellar edema. CSF was positive for WNV IgM and IgG Ab. She was initially treated with IV methylprednisolone. During inpatient rehabilitation, she had bowel/bladder incontinence, global aphasia, and plateaued in participation and tolerance of therapy. Repeat MRI Brain and Spinal Cord was notable for increased patchy T2 hyperintensities in bilateral ventral thalami, bilateral frontal lobe, and cerebellum and cervical ventral root enhancement. She received IVIg and developed jerking movements of her extremities, head, and torso not responsive to propranolol. Methylphenidate was trialed for hypoaroisal, but worsened the movement disorder, as did tetrabenazine. Her arousal and movements improved with amantadine coupled with levodopa-carbidopa. After discussion with renal transplant team, her mycophenolate was weaned leading to further improvement of the movement disorder. Her spasticity and dystonia improved with baclofen and clonazepam. On rehabilitation follow up, she remained dependent for ambulation, required assistance for ADLs, had significant improvement in the movement disorder, and was more awake and alert.

Discussion (relevance): WNV Neuroinvasive disease encompasses patients with meningitis, encephalitis, and acute flaccid myelitis and can present with cerebellitis, cranial nerve palsy, abnormal movement patterns and cardiac and pulmonary decline following neurologic decline. Management is supportive with reduction in immunosuppression and use of IVIg.

Conclusions: Our case patient had WNV neuroinvasive disease and had a functional and cognitive decline that improved with a reduction in immunosuppression and the addition of dopamine agonist medications, a comprehensive rehabilitation program, and intra-disciplinary communication and management.