DISCLOSURES No relevant disclosures RAIN 2018 - Difficult Cases A case of migrating leukoencephalopathy Nicole Rosendale, MD 2/16/18 Zuckerberg San Francisco General Zuckerberg San Francisco General 2 Presentation Title Patient MJ – Initial Presentation Patient MJ – Initial Presentation She presented to a local hospital and was admitted for evaluation 42 year old woman with episodic migraine and hypothyroidism who developed new, intractable right-sided headaches Headaches were refractory to her typical over the counter abortive Her husband also witnessed a few episodes of shaking with decreased responsiveness concerning for seizure Zuckerberg San Francisco General 3 Presentation Title Zuckerberg San Francisco General 4 Presentation Title 1
Patient MJ – Initial Presentation Patient MJ – Second Presentation After a few months, she developed a new, intractable left-sided headache followed by rapidly progressive bilateral vision impairment and hearing loss CSF: 1125 WBC (81% L, 13% N) - protein 125 mg/dL - glucose 69 mg/dL - Treated with IV acyclovir and levetiracetam Her headache improved, and she was discharged home Zuckerberg San Francisco General Zuckerberg San Francisco General 5 Presentation Title 6 Presentation Title Question Patient MJ – Second Presentation The serum assay for aquaporin 4 antibody detection is: She was treated with methylprednisolone 1g IV for 5 days A. more sensitive than specific A. followed by an oral prednisone taper with no significant B. more specific than sensitive B. 44% improvement in her symptoms C. equally sensitive and specific C. During her treatment course, a serum NMO test returned 35% positive, so she was referred to UCSF Neuro-Immunology D. neither sensitive nor specific D. Clinic for further evaluation 11% 10% Zuckerberg San Francisco General Zuckerberg San Francisco General 7 Presentation Title 8 Presentation Title 2
Patient MJ Patient MJ – Admission Examination GENERAL: well appearing, comfortable, neck supple NEURO: On initial evaluation in the UCSF Neuro-Immunology Clinic, MS: Awake, alert, not clearly hard of hearing but had difficulty - she reported progression in her symptoms, as well as new comprehending what was being said, able to name “pen”, episodes of nausea and vomiting. ”thumb” and ”glasses” but not “knuckles”; followed 1-step She was admitted to the hospital from clinic for further commands inconsistently and was unable to perform digit span or management. calculations CN: able to count fingers and detect movement bilaterally, blink to - threat was intact bilaterally, pupils were equal and briskly reactive to light with no rAPD, fundoscopic exam was normal bilaterally, extraocular movements were full, facial sensation and strength were intact, able to hear finger rub bilaterally (L<R) Motor, sensory, reflex, coordination and gait exam were - unremarkable Zuckerberg San Francisco General Zuckerberg San Francisco General 9 Presentation Title 10 Presentation Title Patient MJ – Inpatient Evaluation Patient MJ – Inpatient Evaluation CSF Serum – all negative 7 WBC (91% L, 9% M), - Aquaporin-4-Ab - protein 24 mg/dL, glucose 67 HIV - mg/dL Treponema IgG index 0.6 - - Coccidiodes No unique oligoclonal bands - - Brucella Negative: - - Bartonella HSV PCR - Leptospira VZV PCR, IgG & IgM - CMV PCR ANA, dsDNA, ANCA, - C3/C4, beta-2- WNV IgM/IgG glycoprotein, lupus AFB & fungal culture anticoagulant screen VDRL Cytology & flow cytometry Universal microbial DNA screen Zuckerberg San Francisco General Zuckerberg San Francisco General 11 Presentation Title 12 Presentation Title 3
Question Patient MJ – Inpatient Evaluation What would be the next step? Steroids were held to increase the diagnostic yield A. Repeat large volume LP for cytology A. She underwent an uncomplicated brain biopsy of the left B. Finish steroid taper and monitor clinically B. 55% inferior parietal lobule with no further diagnostic studies The neurosurgeon described the tissue as friable with frank C. CT angiogram C. 34% overlying subarachnoid hemorrhage D. Brain biopsy D. 9% 2% Zuckerberg San Francisco General Zuckerberg San Francisco General 13 Presentation Title 14 Presentation Title Question Patient MJ – Biopsy Results The treatment for primary angiitis of the central nervous system 1. H&E stain with is: inflammatory infiltrate of leptomeningeal vessels. 36% 2. Verhoeff’s (EVG) stain A. prednisone A. demonstrating arteriolar B. cyclophosphamide wall breakdown B. associated with 23% C. azathioprine 21% C. inflammatory infiltrate 3. A CD3 stain highlighting D. rituximab D. 11% small round T-cells that E. A, B & C E. predominate 5% 3% 4. CD45 stain confirms F. all of the above F. paucity of B-cells within the inflammatory component Zuckerberg San Francisco General Zuckerberg San Francisco General 15 Presentation Title 16 Presentation Title 4
Primary angiitis of the central nervous Patient MJ- Treatment & Follow-Up system (PACNS) A rare and challenging diagnosis She was started on steroids and azathioprine One month later, she presented to a local hospital with Differential is often broad, and includes: recurrent seizure and worsened aphasia Infectious etiologies of CNS vasculitis - Levetiracetam dose was increased and she was switched Reversible cerebral vasoconstriction syndrome (RCVS) - from azathioprine to cyclophosphamide Systemic vasculitides with CNS involvement - She has continued to do well on cyclophosphamide with near full recovery of language and improvement in her vision to Brain biopsy remains the gold standard 20/30 OU Recommendation to increase yield: open wedge biopsy of - leptomeninges, cortex and subcortical white matter from a radiographically active area Zuckerberg San Francisco General Zuckerberg San Francisco General 17 Presentation Title 18 Presentation Title Thank you for your attention! Acknowledgements: Megan Richie, MD Arturo Montano, MD Patrick Hullett, MD, PhD Giselle Lopez, MD, PhD Bruce Cree, MD, PhD Maulik Shah, MD Zuckerberg San Francisco General 19 Presentation Title 20 5
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