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2/13/2015 Disclosures None RAIN Conference 2015 Case Presentations Maulik Shah, MD UCSF Neurohospitalist Division Case #1, continued Case #1: Rapidly progressive weakness He was at home with family stable on pain 18 year old man

slide-1
SLIDE 1

2/13/2015 1

RAIN Conference 2015 Case Presentations

Maulik Shah, MD UCSF Neurohospitalist Division

Disclosures

  • None

Case #1: Rapidly progressive weakness

  • 18 year old man who developed diarrheal illness

and colitis while traveling in India in July

  • GI illness improved but then he developed

persistent and diffuse joint pains, oral ulcers, hair loss, frequent headache, and intermittent fever

  • Upon return to US, had work-up for tropical

diseases which was unrevealing

  • Labs notable for new normocytic anemia and ESR

> 100

Case #1, continued

  • He was at home with family stable on pain

medications until October, when he was taken to Emergency Department with worsening headache and high fever

  • He was admitted and the next day started to

complain of tingling and weakness in his feet than quickly progressed to his thighs over few hours

  • Noted to be areflexic
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SLIDE 2

2/13/2015 2

Lumbar Puncture Results

  • The patient had lumbar puncture at that time
  • Normal opening pressure
  • WBC 21 (61% neutrophils)
  • Glucose 52 (serum 126)
  • Protein 388

Question 1

How would you manage this patient at this time?

  • A. Start antibiotics (Vancomycin and

Ceftriaxone) at meningeal dosing and Acyclovir

  • B. Start antibiotics, Acyclovir, and

steroids

  • C. Start high dose steroids
  • D. Start IVIg
  • E. Obtain urgent EMG/NCS

S t a r t a n t i b i

  • t

i c s ( V a n c

  • m

. . . S t a r t a n t i b i

  • t

i c s , A c y c l

  • v

i . . S t a r t h i g h d

  • s

e s t e r

  • i

d s S t a r t I V I g O b t a i n u r g e n t E M G / N C S

26% 18% 21% 30% 4%

Case #1, continued

  • The patient was started on antibiotics and

Acyclovir

  • Over next 24 hours, his flaccid weakness and

tingling progressed to involve all limbs

  • He developed respiratory failure requiring

intubation as well as worsening anemia requiring transfusion

  • He was then started on IVIg and was

transferred to UCSF Neurology service

Initial Neurologic Examination

  • Intubated on ventilator in the ICU
  • MENTAL STATUS awake and alert, able to shake head to

answer questions and follows midline commands

  • CRANIAL NERVES vfftc, PERRL, full extra-ocular

muscles, full facial strength with eyelid closure, and able to lift his head out of bed

  • MOTOR flaccid paralysis of arms and legs
  • SENSORY absent over limbs, trunk, buttocks and back

but intact over upper neck and face

  • COORD no tremor or involuntary movements
  • REFLEXES absent throughout in limbs with intact jaw

jerk

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SLIDE 3

2/13/2015 3

Question #2 What diagnostic testing would you pursue

at this time?

  • A. MRI Cervical and Total Spine
  • B. Repeat lumbar puncture
  • C. Urgent EMG/NCS
  • D. CT Cervical Spine

M R I C e r v i c a l a n d T

  • t

a l . . . R e p e a t l u m b a r p u n c t u r e U r g e n t E M G / N C S C T C e r v i c a l S p i n e

60% 7% 26% 8%

Repeat Lumbar Puncture

  • WBC 198 (76% neutrophils)
  • Glucose 26
  • Protein 233
  • CSF gram stain negative
  • Prior CSF and blood cultures negative to date

Question #3

Which diagnosis is LEAST likely at this time given neurologic examination, CSF and imaging findings?

  • A. Neuromyelitis optica (NMO)
  • B. SLE Myelitis
  • C. Acute Disseminated

Encephalomyelitis

  • D. West Nile Encephalomyelitis
  • E. VZV Myelitis

N e u r

  • m

y e l i t i s

  • p

t i c a ( N M O ) S L E M y e l i t i s A c u t e D i s s e m i n a t e d E n c . . . W e s t N i l e E n c e p h a l

  • m

y e l i t i s V Z V M y e l i t i s

28% 10% 14% 13% 36%

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SLIDE 4

2/13/2015 4

Multple Sclerosis Neuromyelitis optica ADEM SLE Sjogren’s Sarcoid Vasculitis HSV CMV EBV VZV HIV WNV (AHC) HTLV (not always inflammatory) Enterovirus 71 Fungal: cocci, histo Tuberculosis Mycoplasma Lyme Syphilis Lymphoma Intramedullary tumors:

  • Ependymoma
  • Astrocytoma
  • Hemangioblastoma

Paraneoplastic Vascular (Ischemic) Dural AV fistula Vitamin B12 deficiency Nitrous Oxide toxicity Vitamin E deficiency Copper deficiency Adrenoleukodystrophy / Adrenomyeloneuropathy HIV vacuolar myelopathy MRI of Spine CSF Analysis: Elevated WBC or IgG index or OCB

Additional Serologic Work-up

  • HIV negative
  • ESR > 100
  • ANA 1:160
  • DS-DNA titers > 1:10,000 titer
  • Low C3 and C4 complement levels
  • Normal lupus anti-coagulation and anti-

phospholipid antibody panels

  • CSF HSV, VZV PCR negative

SLE Myelitis

  • Occurs in 1% of patients with neuropsychiatric SLE,

most commonly in women

  • Can be the first presentation of SLE
  • Longitudinally extensive > 3 segments in 70% of cases,
  • ften holo-cord involvement
  • In one study of 75 patients with neuropsychiatric SLE,
  • nly one had +NMO antibody

– Patient had longitudinally extensive myelitis – Increased incidence of +NMO antibody in SLE and other rheumatologic disorders in patients with myelitis and/or

  • ptic neuritis
  • Two clinical phenotypes in terms of presentation

Grey and White Myelitis

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SLIDE 5

2/13/2015 5

SLE Myelitis: Clinical Subtypes

  • Grey matter myelitis: rapid onset weakness

(often within 6 hours) with LMN findings of flaccidity and hypo-reflexia on presentation

– First symptom was usually acute urinary retention – LMN signs persist over time

  • White matter myelitis: UMN findings of

spasticity and hyper-reflexia

Question #4

How would treat this patient at this time?

  • A. High dose steroids and Cyclophosphamide

for next few months, then Azathioprine long-term

  • B. Plasmapheresis and prolonged steroid taper
  • C. High dose steroids and then Rituximab if

NMO returns positive

  • D. High dose steroids and Cyclophosphamide

for next few months, then Rituximab if NMO returns negative

H i g h d

  • s

e s t e r

  • i

d s a n d C . . . P l a s m a p h e r e s i s a n d p r

  • .

. . H i g h d

  • s

e s t e r

  • i

d s a n d t . . . H i g h d

  • s

e s t e r

  • i

d s a n d C . . .

27% 13% 24% 37%

SLE Myelitis: Treatment

  • No randomized controlled trials, most data from
  • utcomes of case series
  • European League Against Rheumatism

recommendations

– Early high-dose steroids and ideally Cyclophosphamide (although LP will often be concerning for infection) – Anticoagulation in those with +APLS antibodies – Reasonable to use plasmapheresis in severe cases

  • Several case reports suggest efficacy of Rituximab

– NMO association and recurrent myelitis

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SLIDE 6

2/13/2015 6

Prognosis: Grey vs. White

  • Grey matter myelitis: despite getting more immune

therapy, mean EDSS 8.0 (range 8-10)

– Irreversible paraplegia – Wheelchair bound

  • White matter myelitis mean EDSS 6.0 (range 2-10)

– Dependent on ambulatory assistive device to walk

  • Patient was treated with IV steroids and Cyclophosphamide
  • Our patient’s arm strength improved and he was extubated
  • Continued on six months of Cyclophosphamide and then

Azathioprine

  • Remains paraplegic with distal hand weakness and

bowel/bladder dysfunction

Case #2: An attack of ataxia

  • 84 year old woman with rheumatic heart disease

and hypertension who experienced abrupt onset

  • f difficulty holding playing cards
  • Her voice then became slurred and slowed and
  • ver next few hours she was unable to walk

without support

  • Went to Emergency Department and was felt to

have an acute stroke although MRI Brain showed no evidence of ischemia

  • Discharged home with adjustment of antiplatelet

and initiation of statin

Case #2, continued

  • At home, she had progressive symptoms

– Double vision – Recurrent falls, unable to ambulate even with walker

  • Additional review of symptoms

– 25 lb weight loss due loss of appetite – URI while visiting Hawaii two months prior

  • Patient provided permission for video to be used for teaching purposes
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SLIDE 7

2/13/2015 7

Question #5

Given patient’s examination, the imaging shown has most definitively ruled out which of the following etiologies?

  • A. Acute or subacute cerebellar stroke
  • B. Auto-immune cerebellitis
  • C. Post-infectious viral cerebellitis
  • D. Creutzfeldt-Jakob Disease
  • E. Miller Fisher variant AIDP

A c u t e

  • r

s u b a c u t e c e r e b e . . . A u t

  • i

m m u n e c e r e b e l l i t i s P

  • s

t

  • i

n f e c t i

  • u

s v i r a l c e r e b . . . C r e u t z f e l d t

  • J

a k

  • b

D i s e a s e M i l l e r F i s h e r v a r i a n t A I D P

82% 4% 3% 6% 5%

Lumbar Puncture

  • Clinical examination was felt to be most

consistent with pan-cerebellopathy

  • CSF was collected as next part of diagnostic work-

up and revealed

– WBC 30 (81% lymphs) – Protein 130 – Glucose 78

  • CSF cytology and flow cytometry negative
  • CSF EBV, HSV, VZV PCR negative
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SLIDE 8

2/13/2015 8

Question #6

Which CSF or serum antibody is most likely to be positive in this case when considering patient’s demographic background and CSF results?

  • A. Anti-GQ1b antibody
  • B. Anti-TPO and anti-thyroglobulin

antibodies

  • C. Anti-GAD antibodies
  • D. Anti-Tr antibodies
  • E. Anti-Yo antibodies

Anti-GQ1b antibody Anti-TPO and anti-thyr... Anti-GAD antibodies Anti-Tr antibodies Anti-Yo antibodies

22% 8% 55% 4% 12%

Anti-Yo Paraneoplastic Cerebellar Degeneration

  • Almost exclusively occurs in women, mean age of

65 years old

  • Pan-cerebellar disorder with truncal and

appendicular ataxia, dysarthria or dysphagia, and nystagmus

  • Acute to subacute onset (range of 1 day to 16

weeks)

  • Cognitive and psychiatric symptoms in 10-30%
  • MRI Brain usually normal, atrophy in chronic

courses

Question #7

Which of the following neoplasm is most associated with anti-Yo paraneoplastic antibodies?

  • A. Hodgkin’s lymphoma
  • B. Prostate cancer
  • C. Ovarian cancer
  • D. Lung adenocarcinoma

H

  • d

g k i n ’ s l y m p h

  • m

a P r

  • s

t a t e c a n c e r O v a r i a n c a n c e r L u n g a d e n

  • c

a r c i n

  • m

a

2% 33% 64% 0%

slide-9
SLIDE 9

2/13/2015 9

Anti-Yo: Treatment and Prognosis

  • Treatment against malignancy is mainstay,

however syndrome may pre-date detection of malignancy by 1-2 years

  • Case series using steroids or plasmapheresis

with majority of patients having no significant improvement

  • Case reports of efficacy with

Cyclophosphamide or Rituximab but not clear if long-term prognosis changes

  • Thank you for attention
  • UCSF Neurohospitalist Division and Fellowship

– S. Andrew Josephson – Vanja Douglas – John Betjemann – Megan Richie – Maulik Shah

  • Transfer Center at UCSF: 415-353-9166