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

2/15/2019 Disclosures None RAIN 2016: Challenging Cases Tiresome weakness Megan Richie, MD Department of Neurology Sunday morning History You are on call and your neurosurgery colleagues have a consult. PMH: HTN, prediabetes Medications: Amlodipine, Losartan, Aspirin 61 year old man with hypertension and pre-diabetes • Friday : Lifted a heavy tire  back pain SH: Former smoker. Married. • Saturday : Woke up and could barely urinate ‒ Right foot was numb from the knee down FH: Father, grandfather with cancer. Sister with diabetes. 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. 1

2/15/2019 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. IP Hams Quads TA Gastr Right 4+ 4 4+ 2 2  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 Q1 – Where is the problem? A A. A B. B C. C D. d B 45% 31% C D 16% 7% 2/15/2019 2/15/2019 2

2/15/2019 T1-Pre T1-Post 10 Presentation Title and/or Sub Brand Name Here 2/15/2019 Radiology report Q2 – What at the top of your differential? 1. Abnormal T2 hyperintensity in the distal thoracic cord/conus A. Idiopathic transverse myelitis medullaris, ventral involvement significantly greater than dorsal. The 44% B. Demyelinating lesion cord is slightly expanded in the areas of signal abnormality with possible subtle enhancement. C. Infectious myelitis D. Spinal cord compression 2. Multilevel degenerative changes of the lower lumbar spine, most E. Spinal cord infarct 19% 16% pronounced at L4-L5 where there is mild canal stenosis and severe F. Dural AV fistula 13% right neural foraminal narrowing. Additional notable severe left foraminal narrowing at L5-S1. G. Intramedullary neoplasm 3% 3% 2% 3

2/15/2019 Q3 – What is your next step in management? Case continuation  Lumbar puncture performed A. Methylprednisolone 1g IV • WBC 0 B. Neurosurgical intervention 53% • RBC 3 C. Spinal fluid analysis • Glucose 90 D. MRI brain and total spine • Protein 54 E. A and C 19% • No oligoclonal bands F. C and D 13% • IgG index 0.6 8% 6% 2%  MRI brain and spine performed DWI T2 FLAIR T1 Post 15 Presentation Title and/or Sub Brand Name Here 2/15/2019 16 Presentation Title and/or Sub Brand Name Here 2/15/2019 4

2/15/2019 Q4 – What would be your next diagnostic test? Key features A. Body PET/CT  61 year old man with hypertension, pre-diabetes B. CSF HSV PCR 46%  Acute onset back pain after heavy lifting C. CSF cytology and flow cytometry • Right leg weakness: Pyramidal + S1 D. Flexion/extension imaging • Light touch, pinprick > vibration sensory loss E. MR Diffusion sequences of cord  Imaging findings: 16% F. Biopsy of conus lesion • T7-T8 disk herniation without cord signal 11% 11% 8% 8% • 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 DWI ADC Infectious myelitis Spinal cord compression Spinal cord infarct Dural AV fistula Intramedullary neoplasm 20 Presentation Title and/or Sub Brand Name Here 2/15/2019 5

2/15/2019 Spinal cord infarction Spinal cord infarction: Imaging findings  Acute (minutes to hours) motor / sensory deficits, back pain  May be initially normal but delayed MR reveals abnormality • +/- Vascular clinical syndrome  Expansile T2 hyperintensity ‒ Anterior spinal artery: Bilateral pain/temperature & Motor • +/- Diffusion restriction ‒ Posterior spinal artery: Unilateral vibration/proprioception • +/- enhancement (subacute)  Distinct patterning  Multiple causes • ASA: Ventral predominant • Aortic disease (aneurysm, dissection, trauma, surgery) ‒ Anterior horns, central grey, white matter • Vascular disease (atherosclerosis, cocaine, sickle cell) ‒ Owl’s-eye/ Snake-eye pattern; pencil-like • Embolism (thrombotic, fibrocartilagenous) • PSA: Dorsal, unilateral • Hypoperfusion (hypotension, cardiac arrest, diving) Posterior spinal artery Posterior radicular artery Spinal segmental artery Anterior spinal artery Intercostal artery Anterior radicular artery Zalewski N et al. 2019 6

2/15/2019 Q5 – Why did this patient have a cord infarct? Fibrocartilaginous embolism  5.5% of spinal cord infarctions A. Aortic dissection 25% 24% B. Atherosclerotic disease 21%  Intervertebral disks are classically considered avascular. However, C. Fibrocartilaginous embolism disk material can gain access via: 16% D. Trauma 1) Revascularization 13% E. Hypoperfusion event 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 Diagnosing Fibrocartilaginous Embolism Exclude Exclude trauma & inflammatory disease Establish myelopathy compression with by CSF +/- MR CT/MRI 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 Yadav N et al. 2018 7

2/15/2019 Take-home points UCSF Neurohospitalist Program  Consider cord infarct in patients with acute myelopathy and dissociated sensory loss Challenging case?  Collateral spinal cord vasculature leads to variable presentations  Diffusion-weighted images of the spine are helpful in the evaluation of cord infarction (415) 353 – 9166  CSF analysis and gadolinium sequences can help exclude other causes of acute myelopathy  Causes of cord infarct include aortic disease, other vascular UCSF Transfer Center disease, hypoperfusion, and embolism  Fibrocartilagenous embolism may be an underappreciated cause of cord infarct and can occur remote to the site of disk herniation 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. 32 2/15/2019 8