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

2/9/2017 1

Case Discussion

Michael Wilson, MD, MAS UCSF MS and Neuroinflammation Center

Case

• 56 year old right-handed woman
• History of non-insulin dependent diabetes mellitus
• Lifelong smoker
• Late summer: Right leg started to occasionally give out
• After 2 months: started to fall and had difficulty keeping up with the

kids as a playground monitor

• 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

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Case

• CSF
• WBC 130 (96% lymphocytes, 4% monocytes)
• RBC 5
• Total protein 83
• Glucose 45
• One unique oligoclonal band
• IgG index 0.57

Case

• Received high dose methylprednisolone
• Improved from not moving her right leg to some movement
• Left leg remained numb
• Went to inpatient rehab for a month with suspected diagnoses of

ADEM or multiple sclerosis

• Persistent enhancement on MRI at 10 months after onset
• 14 months after onset
• New inability to move her left leg
• Persistent enhancement on MRI
• 15 months after onset: Evaluated at UCSF

Case

Differential Diagnosis?

• ?Autoimmune demyelination
• ?Malignancy
• ?Vasculitis
• ?Infection

Additional Work-Up?

Inpatient Evaluation

• CSF
• WBC 8 (87% lymphocytes, 13% monocytes)
• RBC 1
• Total protein 99
• Glucose 92
• ACE < 5
• HSV, EBV, VZV PCRs neg
• 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

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Case

• 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

21 months What is the Differential Diagnosis?

• ?Autoimmune demyelination
• ?Malignancy
• ?Vasculitis
• ?Infection

Courtesy of Drs. Giselle Lopez and Andy Bollen

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CD3 CD20

Courtesy of Drs. Giselle Lopez and Andy Bollen

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

28 months What is the next treatment?

• ?PLEX
• ?High does steroids
• ?Rituximab
• ?Other immunosuppressive therapy