[PPT] - RAIN 2016: Challenging Cases Tiresome weakness Megan Richie, MD PowerPoint Presentation

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RAIN 2016: Challenging Cases

Tiresome weakness

Megan Richie, MD Department of Neurology

Disclosures

None

Sunday morning

You are on call and your neurosurgery colleagues have a consult. 61 year old man with hypertension and pre-diabetes

Friday: Lifted a heavy tire  back pain
Saturday: Woke up and could barely urinate

‒ Right foot was numb from the knee down

He presented to an OSH where CT showed “DJD” and he was given 2 doses dexamethasone & transferred to neurosurgery. You are consulted to help interpret his MRI findings.

History

PMH: HTN, prediabetes Medications: Amlodipine, Losartan, Aspirin SH: Former smoker. Married. FH: Father, grandfather with cancer. Sister with diabetes.

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Examination

Vitals: T 36.4C, BP 131/71, HR 80, RR 17, SpO2 94%
Medical: No apparent distress, well-groomed
Mental status: A&O x 4, fluent speech, excellent historian
CN: VFFTC, PERRL, EOMI, face symmetric, T/P midline
Motor: Normal bulk/tone. 5/5 in upper extremities and LLE. Rectal tone intact.
Sensory: Reduced light touch, pinprick in groin, buttocks, and RLE below the
knee. Intact vibration at toes.
Reflexes: 2+ in UE but absent at bilateral patellae & Achilles. Toes down.
Coordination: Intact FNF

IP Hams Quads TA Gastr Right 4+ 4 4+ 2 2

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Q1 – Where is the problem?

A. A B. B C. C D. d 2/15/2019

A B C D

45% 31% 16% 7%

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T1-Pre T1-Post Radiology report

1. Abnormal T2 hyperintensity in the distal thoracic cord/conus

medullaris, ventral involvement significantly greater than dorsal. The cord is slightly expanded in the areas of signal abnormality with possible subtle enhancement.

2. Multilevel degenerative changes of the lower lumbar spine, most

pronounced at L4-L5 where there is mild canal stenosis and severe right neural foraminal narrowing. Additional notable severe left foraminal narrowing at L5-S1.

Q2 – What at the top of your differential?

A. Idiopathic transverse myelitis
B. Demyelinating lesion
C. Infectious myelitis
D. Spinal cord compression
E. Spinal cord infarct

F. Dural AV fistula

G. Intramedullary neoplasm

19% 3% 2% 16% 13% 44% 3%

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Q3 – What is your next step in management?

A. Methylprednisolone 1g IV
B. Neurosurgical intervention
C. Spinal fluid analysis
D. MRI brain and total spine
E. A and C

F. C and D

8% 2% 53% 19% 13% 6%

Case continuation

Lumbar puncture performed
WBC 0
RBC 3
Glucose 90
Protein 54
No oligoclonal bands
IgG index 0.6
MRI brain and spine performed

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DWI FLAIR T1 Post T2

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Q4 – What would be your next diagnostic test?

A. Body PET/CT
B. CSF HSV PCR
C. CSF cytology and flow cytometry
D. Flexion/extension imaging
E. MR Diffusion sequences of cord

F. Biopsy of conus lesion

11% 8% 11% 46% 8% 16%

Key features

61 year old man with hypertension, pre-diabetes
Acute onset back pain after heavy lifting
Right leg weakness: Pyramidal + S1
Light touch, pinprick > vibration sensory loss
Imaging findings:
T7-T8 disk herniation without cord signal
Ventral, expansile T2 lesion of conus medullaris
Left L4-5 and Right L5-S1 neural foraminal narrowing
Bland CSF studies

Reminder of your differential diagnosis

Idiopathic transverse myelitis Demyelinating lesion Infectious myelitis Spinal cord compression Spinal cord infarct Dural AV fistula Intramedullary neoplasm

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DWI ADC

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Spinal cord infarction

Acute (minutes to hours) motor / sensory deficits, back pain
+/- Vascular clinical syndrome

‒ Anterior spinal artery: Bilateral pain/temperature & Motor ‒ Posterior spinal artery: Unilateral vibration/proprioception

Multiple causes
Aortic disease (aneurysm, dissection, trauma, surgery)
Vascular disease (atherosclerosis, cocaine, sickle cell)
Embolism (thrombotic, fibrocartilagenous)
Hypoperfusion (hypotension, cardiac arrest, diving)

Spinal cord infarction: Imaging findings

May be initially normal but delayed MR reveals abnormality
Expansile T2 hyperintensity
+/- Diffusion restriction
+/- enhancement (subacute)
Distinct patterning
ASA: Ventral predominant

‒ Anterior horns, central grey, white matter ‒ Owl’s-eye/ Snake-eye pattern; pencil-like

PSA: Dorsal, unilateral

Zalewski N et al. 2019

Intercostal artery

Spinal segmental artery Posterior radicular artery Anterior radicular artery Anterior spinal artery Posterior spinal artery

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Q5 – Why did this patient have a cord infarct?

A. Aortic dissection
B. Atherosclerotic disease
C. Fibrocartilaginous embolism
D. Trauma
E. Hypoperfusion event

21% 24% 13% 16% 25%

Fibrocartilaginous embolism

5.5% of spinal cord infarctions
Intervertebral disks are classically considered avascular. However,

disk material can gain access via: 1) Revascularization 2) Schmorl’s nodes 3) Persistent childhood disk vasculature

Fibrocartilaginous material from nucleus pulposus migrates into

nearby vasculature to embolize to a spinal cord vessel

May also travel to lung, brain, vertebrae, ribs

Yadav N et al. 2018

Diagnosing Fibrocartilaginous Embolism

Establish myelopathy Exclude trauma & compression with CT/MRI Exclude inflammatory disease by CSF +/- MR enhancement Establish Cord infarction

Major criteria
Vascular distribution by exam
Vascular distribution by imaging
Adjacent vertebral or disk infarction
Minor criteria
New neck or back pain
Clinical nadir < 4 - 8 hours
Initial normal MRI that later shows cord lesion

Establish Fibrocartilaginous embolism

Temporal relation to lifting or minor neck/back injury
Presence of degenerative disk disease
2 or fewer vascular risk factors (HTN, DM, PAD, Age > 60, smoking,

prior stroke)

Yadav N et al. 2018

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Take-home points

Consider cord infarct in patients with acute myelopathy and

dissociated sensory loss

Collateral spinal cord vasculature leads to variable presentations
Diffusion-weighted images of the spine are helpful in the evaluation
f cord infarction
CSF analysis and gadolinium sequences can help exclude other

causes of acute myelopathy

Causes of cord infarct include aortic disease, other vascular

disease, hypoperfusion, and embolism

Fibrocartilagenous embolism may be an underappreciated cause of

cord infarct and can occur remote to the site of disk herniation

UCSF Neurohospitalist Program

Challenging case?

(415) 353 – 9166

UCSF Transfer Center

References

1. AbdelRazek MA, Mowla A, Faroog S, Silvestri N, Sawyer R, Wolfe G. Fibrocartilaginous embolism: A comprehensive review of an under-studied cause of spinal cord infarction and proposed diagnostic criteria. J Spinal Cord

Med. 2016;39(2):146-54.

2. Rigney L, Cappellen-Smith C, Sebire D, Beran RG, Cordato D. Nontraumatic spinal cord ischaemic syndrome. J Clin Neurosci. 2015 Oct;22(10):1544-9. 3. Toro-Gonzalez G, Navarro-Roman L, Roman GC, Cantillo J, Serrano B, Herrera M, Vergara I. Acute ischemic stroke from fibrocartilaginous embolism to the middle cerebral artery. Stroke 1993 May;24(5):738-40. 4. Yadav N, Pendharkar H, Kulkarni GB. Spinal cord infarction: Clinical and radiological features. J Stroke Cerebrovasc Dis. 2018 Oct;27(10)2810-2821. 5. Zalewski NL, Rabinstein AA, Krecke KN, Brown RD Jr, Wijdicks EFM, Weinshenker BG, Kauffmann TJ, Morris JM, Aksamit AJ, Bartleson JD, Lanzino G, Blessing MM, Flanagan

EP. Characteristics of spontaneous spinal cord infarction and proposed diagnostic criteria.

JAMA Neurol 2019;76(1):56-63. 6. Zalewski NL, Rabinstein AA, Wijdicks EFM, Petty GW, Pittock SJ, Mantyh WV, Flanagan EP . Spontaneous posterior spinal artery infarction: An under- recognized cause of acute myelopathy. Neurology. 2018 Aug 28;91(9):414- 417.

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