A Myel-In, a Long Way to Go (Video)

A 9-year-old girl presented with morning headaches associated with vomiting, gait ataxia, and facial and ocular nerve palsies. Her initial imaging was concerning for possible demyelinating disease. After extensive infectious and rheumatologic work up returned negative, she was treated twice with IVIG and IV steroids with near complete resolution each time. She re-presented, however, with worsening neurologic deficits and imaging revealing focal ischemic infarction in the brainstem as well as new-onset hydrocephalus. A multi-specialty work up was initiated with the aid of consultants in rheumatology, neuroimmunology, and infectious disease without conclusive diagnosis. Brain biopsy revealed gross evidence of infection as well as microscopic evidence of fungus, and non-invasive pathogen testing of plasma established a diagnosis of C. bantiana. Treatment was initiated with systemic voriconazole and intraventricular Amphotericin B. This patient's hospital course spanned nearly 6 months and was complicated by tracheostomy and G-tube placement, multiple EVD and VP shunt revisions, and cardiac arrest requiring prolonged resuscitation. Additionally, fungal cultures continued to grow positive after three months of aggressive treatment, prompting craniotomy and wash out of the 4th ventricle. Today, the patient is continuing her long path to recovery at home with her family. She is bed-ridden with daytime trach collar and is receiving G-tube feeds. Her strength is slowly improving with physical therapy and her personality is returning.