DISCLOSURES No relevant disclosures RAIN 2018 - Difficult Cases A - - PowerPoint PPT Presentation

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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


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Zuckerberg San Francisco General

2/16/18

Nicole Rosendale, MD

RAIN 2018 - Difficult Cases

A case of migrating leukoencephalopathy

Zuckerberg San Francisco General

Presentation Title 2

DISCLOSURES

  • No relevant disclosures

Zuckerberg San Francisco General

Presentation Title 3

Patient MJ – Initial Presentation

  • 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

Presentation Title 4

Patient MJ – Initial Presentation

  • She presented to a local hospital and was admitted for evaluation
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Zuckerberg San Francisco General

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Patient MJ – Initial Presentation

  • 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

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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

Zuckerberg San Francisco General

Presentation Title 7

Patient MJ – Second Presentation

  • She was treated with methylprednisolone 1g IV for 5 days

followed by an oral prednisone taper with no significant improvement in her symptoms

  • During her treatment course, a serum NMO test returned

positive, so she was referred to UCSF Neuro-Immunology Clinic for further evaluation

Zuckerberg San Francisco General

Question

The serum assay for aquaporin 4 antibody detection is:

A.

  • A. more sensitive than specific

B.

  • B. more specific than sensitive

C.

  • C. equally sensitive and specific

D.

  • D. neither sensitive nor specific

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44% 10% 11% 35%

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Zuckerberg San Francisco General

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Patient MJ

  • On initial evaluation in the UCSF Neuro-Immunology Clinic,

she reported progression in her symptoms, as well as new episodes of nausea and vomiting.

  • She was admitted to the hospital from clinic for further

management.

Zuckerberg San Francisco General

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Patient MJ – Admission Examination

  • GENERAL: well appearing, comfortable, neck supple
  • NEURO:
  • MS: Awake, alert, not clearly hard of hearing but had difficulty

comprehending what was being said, able to name “pen”, ”thumb” and ”glasses” but not “knuckles”; followed 1-step commands inconsistently and was unable to perform digit span or 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

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Patient MJ – Inpatient Evaluation

Zuckerberg San Francisco General

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Patient MJ – Inpatient Evaluation

  • CSF
  • 7 WBC (91% L, 9% M),

protein 24 mg/dL, glucose 67 mg/dL

  • IgG index 0.6
  • No unique oligoclonal bands
  • Negative:
  • HSV PCR
  • VZV PCR, IgG & IgM
  • CMV PCR
  • WNV IgM/IgG
  • AFB & fungal culture
  • VDRL
  • Cytology & flow cytometry
  • Universal microbial DNA screen
  • Serum – all negative
  • Aquaporin-4-Ab
  • HIV
  • Treponema
  • Coccidiodes
  • Brucella
  • Bartonella
  • Leptospira
  • ANA, dsDNA, ANCA,

C3/C4, beta-2- glycoprotein, lupus anticoagulant screen

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Zuckerberg San Francisco General

Question

What would be the next step?

A.

  • A. Repeat large volume LP for cytology

B.

  • B. Finish steroid taper and monitor clinically

with no further diagnostic studies

C.

  • C. CT angiogram

D.

  • D. Brain biopsy

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9% 55% 34% 2%

Zuckerberg San Francisco General

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Patient MJ – Inpatient Evaluation

  • Steroids were held to increase the diagnostic yield
  • She underwent an uncomplicated brain biopsy of the left

inferior parietal lobule

  • The neurosurgeon described the tissue as friable with frank
  • verlying subarachnoid hemorrhage

Zuckerberg San Francisco General

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Patient MJ – Biopsy Results

1. H&E stain with inflammatory infiltrate of leptomeningeal vessels. 2. Verhoeff’s (EVG) stain demonstrating arteriolar wall breakdown associated with inflammatory infiltrate 3. A CD3 stain highlighting small round T-cells that predominate 4. CD45 stain confirms paucity of B-cells within the inflammatory component

Zuckerberg San Francisco General

Question

The treatment for primary angiitis of the central nervous system is:

A.

  • A. prednisone

B.

  • B. cyclophosphamide

C.

  • C. azathioprine

D.

  • D. rituximab

E.

  • E. A, B & C

F.

  • F. all of the above

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3% 23% 36% 21% 11% 5%

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Zuckerberg San Francisco General

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Patient MJ- Treatment & Follow-Up

  • She was started on steroids and azathioprine
  • One month later, she presented to a local hospital with

recurrent seizure and worsened aphasia

  • Levetiracetam dose was increased and she was switched

from azathioprine to cyclophosphamide

  • She has continued to do well on cyclophosphamide with near

full recovery of language and improvement in her vision to 20/30 OU

Zuckerberg San Francisco General

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Primary angiitis of the central nervous system (PACNS)

  • A rare and challenging diagnosis
  • Differential is often broad, and includes:
  • Infectious etiologies of CNS vasculitis
  • Reversible cerebral vasoconstriction syndrome (RCVS)
  • Systemic vasculitides with CNS involvement
  • Brain biopsy remains the gold standard
  • Recommendation to increase yield: open wedge biopsy of

leptomeninges, cortex and subcortical white matter from a radiographically active area

Zuckerberg San Francisco General

Presentation Title 19 20

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