Case Discussion Late summer: Right leg started to occasionally give - PowerPoint PPT Presentation

2/9/2017 Case • 56 year old right-handed woman • History of non-insulin dependent diabetes mellitus • Lifelong smoker Case Discussion • Late summer: Right leg started to occasionally give out Michael Wilson, MD, MAS • After 2 months: started to fall and had difficulty keeping up with the kids as a playground monitor UCSF MS and Neuroinflammation Center • After 6 months: right leg became numb and developed lower abdominal pain • Husband insisted they go to the hospital when she had to crawl to the bathroom Case • Bladder ultrasound showed severe urinary retention 1

2/9/2017 Case Case • Received high dose methylprednisolone • CSF • Improved from not moving her right leg to some movement • WBC 130 (96% lymphocytes, 4% monocytes) • Left leg remained numb • RBC 5 • Went to inpatient rehab for a month with suspected diagnoses of • Total protein 83 ADEM or multiple sclerosis • Glucose 45 • Persistent enhancement on MRI at 10 months after onset • One unique oligoclonal band • IgG index 0.57 • 14 months after onset • New inability to move her left leg • Persistent enhancement on MRI • 15 months after onset: Evaluated at UCSF Case Inpatient Evaluation • CSF Differential Diagnosis? Additional Work-Up? • WBC 8 (87% lymphocytes, 13% monocytes) • ?Autoimmune demyelination • RBC 1 • Total protein 99 • ?Malignancy • Glucose 92 • ACE < 5 • ?Vasculitis • HSV, EBV, VZV PCRs neg • ?Infection • NMO, HTLV-I/II, Lyme, RPR, VDRL, FTA-ABS neg • Cytology and flow cytometry negative • Whole body PET/CT: unremarkable • Spinal angiogram: unremarkable • Mayo autoimmune panel sent on blood and CSF 2

2/9/2017 Case 21 months • No clinical response to high dose solumedrol • Completed 5 cycles of plasma exchange with mild improvement in lower extremity strength and sensation • Discharged on a high dose prednisone taper • Mycophenolate mofetil initiated with goal dose of 1000mg BID after a 6 week ramp up • Despite maximal mycophenolate mofetil dosing, neuroinflammation persisted on subsequent MRIs What is the Differential Diagnosis? • ?Autoimmune demyelination • ?Malignancy • ?Vasculitis • ?Infection Courtesy of Drs. Giselle Lopez and Andy Bollen 3

2/9/2017 CD3 CD20 Epilogue • 22 months • PLEX repeated • High dose prednisone taper • Oral cyclophosphamide with maximal dose of 100mg daily • 24 months • Slightly improved lower extremity motor exam • Decreased swelling and enhancement in the brain and spinal cord Courtesy of Drs. Giselle Lopez and Andy Bollen 28 months What is the next treatment? • ?PLEX • ?High does steroids • ?Rituximab • ?Other immunosuppressive therapy 4