Age: 19.0
Sex: M
Vaccine: UNK
Symptoms: Encephalomalacia, Endotracheal intubation, Faecal calprotectin increased, Flow cytometry, Fungal test negative
Clinical narrative: #Acute demyelinating encephalomyelitis is the adverse event. He received an influenza vaccination 10/28 in the setting of a recent URI which had resolved per his report/filing out of the flu shot screen record.  11/3 /24 he presented to his hospital with R hemiparesis and cerebral edema. He was transferred to  hospital and then to another Hospital, where he spent 3 months. He transferred to Medical Center 2/4/25 for ongoing care.  19 y/o man without significant past medical history who developed an acute disseminated encephalomyelitis in Nov 2024 with prolonged hospitalization at Hospital now transferred to for continuity of care.   Following prodrome of respiratory infection and flu vaccination, patient developed progressive encephalopathy. He was discovered to have a acute, multifocal, tumefactive demyelinating lesions complicated by cerebral edema requiring decompressive craniectomy and CSF diversion. Extensive work-up at tertiary medical center with multiple sub-specialties to include neuroimmunology, rheumatology and infectious disease was unrevealing after investigating several infectious, autoimmune, inflammatory, neoplastic, genetic, toxic causes. Patient received several bouts of immunosuppressive treatment to include IVMP, IVIG, PLEX, rituximab, tocilizumab and cyclophosphamide which were, per review of imaging and records, able to control the progressive inflammation as evidenced by resolution of the areas of enhancement. Subsequent imaging revealed evolving cystic encephalomalacia involving the bilateral cerebellum, right pons, bilateral thalami, and extensive L>R subcortical white matter.Treatments received at hospital: - PLEX #1 (11/9), PLEX #2 (11/10), PLEX #3 (11/12), PLEX #4 (11/14), PLEX #5 (11/16), PLEX #6 (11/18), PLEX #7 (11/20)  - IV Methyiprednisolone 1 gm x 7 days  - Meropenum x 7 days      - 11/20 prednisone course discontinued and started IV dexamethasone  - 11/20 s/p IV tocilizumab (1st dose)  - 11/21 s/p IT rituximab (1st dose) + IT decadron (1st dose)  - 11/28 s/p IT rituximab (2nd dose) + IT decadron (2nd dose) and initiated on IV cyclophosphamide 2 mg/kg IV daily (1st dose)  - 11/29 2nd dose of Tocilizumab  - 12/2 cyclophosphamide dose held as ANC <750 - 12/2 per Genetics, genome sequencing report resulted negative, will plan for reanalysis in 2 years - 12/2 repeat bMRI W WO contrast obtained to assess for response to recent treatments (last done on 11/17) with significant encephalomalacia in bilateral basal ganglia and R pons with concern for poor prognosis - 12/3 MRI results discussed extensively with family - 12/4 Rheumatology recommend IV tocilizumab monthly (next dose 12/29) -12/5 Allergy/immunology recommend Bactrim for PJP ppx given CD4 ct < 200 - 12/8 Patient transferred from NNICU to step down unit. Overnight bromocriptine started for fevers/tachycardia - 12/12 minor head trauma after hitting right side of head on bed rail during coughing fit. Staple came lose from scalp. CT head stable. Exam stable. Low concern for significant injury. NSGY replaced staple. -- 12/13 ongoing neuro-storming with fevers/tachycardia. Currently on SCH APAP, ibuprofen, bomocriptine, gabapentin. PRN morphine for breakthrough fevers. BLE doppler without evidence - 12/17 rheumatology sign-off -12/23 LP. CSF w N4>9, P65, G55, RBC 537>0. Had planned for cytokine testing but lab was - 12/26 Brain MRI WWO with VWI - new right parietal lobe enhancement, persistent left parietal and left BG enhancement; no evidence of vasculitis - 12/30 Neurosurgery decided against repeat biopsy. 3rd dose Tocilizumab given. Started on IVMP 250mg daily x5 days. Plan for shunt/cranioplasty approximately 1/9.