Non-spondylotic Myelopathies

Anthony Chiodo, MD, MBA

University of Michigan Health System

AAPMR Meeting 2015, Boston

• Acute: –Vascular, decompression sickness, transverse myelitis, hemorrhage, MS

• Chronic–B-12, tumor, HSP, MND, post-polio, radiation, MS

• Anterior:

• Posterior: MS, Friedrich, B12, HIV

• Central: Syrinx

• Motor: Motor neuron, HSP, HTLV, West Nile

Cases that change the surgical plan

• Hemorrhage

• Abscess

• Tumor

• Tethered Cord/Syrinx

• Spinal Cord Herniation

HOLD THE SURGERY!!

WHY?

• Multiple Sclerosis

• Transverse Myelitis

• Motor Neuron Disease

• Vascular

• Hereditary

• Others: HTLV, B12, West Nile, post-polio, radiation

Non-Traumatic SCI

• Older

• More medical co-morbidities

• Male to female 1.7:1

• Psychosocial impact of stress, anxiety and depression mirror those with SCI

Etiology of NT-SCIMcKinley, Archives, 1999

• 39 percent of 220 patients admitted to acute rehab

• 46% Non-spondylotic– Tumor 26% – Ischemia 8.2% – Infection 7% – Transverse myelitis 4.7%

Etiology in NT-SCICitterio, Archives, 2004.

• Inflammatory/Infection 19.5%

• Vascular 25.1%

• Neoplastic 25.1%

• Degenerative 18.6%

• Other 11.8%

Other

• Syrinx

• Epidural Hematoma

• Radiation Myelopathy

Level of Injury in NT-SCI Tetra

CompleteTetra

Incomp

Para Complete

Para Incomp

TM 8 8 11 36

Vascular 1 7 25 48

Neoplasm 1 9 17 54

Degen 1 25 6 28

Other 1 11 8 18

Total 12

3.7%

60

18.6%

67

20.7%

184

60%

Non-traumatic SCI is Incomplete

• Also, more paraplegia than tetraplegia

• Less secondary conditions – Spasticity– Deep vein thrombosis– Autonomic dysreflexia

• Comorbidities and generalized deconditioning significantly affect functional outcome

Case

• 25 year old right man presents with fall

• In retrospect, some change in gait for several months with dragging the right leg

• No sensory or cranial nerve complaints

• No UE complaints

• No bowel or bladder issues

• Reflexes 2+ upper extremities, 3+ knees, 3+ ankles, right extensor and left flexor plantar response

• UE and LLE strength intact, RLE with hip flexors 3, gluteals 4, knee extensors 5, knee flexors 4, ADF 4

• Pin sensation intact

• 15 second vibration sense loss in LE’s, normal in UE’s

• EMG/NCS RLE is normal

• MRI cervical spine revealed multiple stir enhanced lesions in the mid and upper cervical spine and brainstem

• Corona radiata lesions noted in brain MRI without atrophy or mass lesions.

Multiple Sclerosis

• Sensory changes 33%

• Optic neuritis 20%

• Weakness 13%

• Gait disorder 5%

• Balance disorder 3%

• Psychiatric 3%

• Speech or swallowing 3%

• Mixed 15%

Case

• A 58 year old Caucasian female presents with a three week history of progressive left greater than right arm parathesias and weakness

• Presentation precipitated by urinary incontinence and inability to sense need to void

Neurological Exam

• Manual muscle testing –LUE: deltoid 2/5, triceps 4/5, biceps 2/5, finger extensors 3/5, and finger flexors 4/5.

–RUE: deltoid 5/5, triceps 5-/5, biceps 3/5, finger extensors 4/5, finger flexors 5/5

–LE: all muscles tested are 4/5 except left dorsiflexion is 5/5.

• Reflexes: 0/4 bilateral biceps and brachioradialis, 3/4 bilateral patella. Plantar responses are flexor bilaterally.

• Normal tone and muscle bulk is noted throughout.

• Diminished light touch and pinprick are found in the arms in the C5 throught T1 distribution: Normal trunk and LE’s.

• Proprioception is decreased in the upper extremities and there is an absence of vibration sense in all four extremities

• 3mm left pupil and a 5 mm right pupil with symptoms of a left Horner’s syndrome with mild ptosis.

MRI Imaging

• BRAIN: Multiple scattered foci of abnormal T2 and FLAIR hyperintensities throughout the periventricular and subcortical white matter bilaterally.

• SPINE: Low T1 and high T2 signal changes in central portion of the cord from the lower medulla to the superior T7 level.

• Peripheral cord enhancement from the C2 to T4 vertebral body level.

Laboratory Results

• Westergren Sed Rate 27, C3 183, C4 33, ANA positive with titer 1:1280 and homogeneous, IgG index 0.63, Anti-ds DNA 24.6, Anti-cardiolipin positive, Beta-2 glycoprotein negative, Anti-SM negative, Anti-RNP negative, Anti-RO/Anti-SSA negative, Anti-LA/Anti SSB negative.

• CSF protein 180, glucose 76 without pleiocytosis, oligoclonal bands and with negative cultures.

Transverse Myelitis

• Female to male ratio of 4:1

• Peaks in the second and fourth decades

• Time course of progression is greater than 48 hours and less than six weeks

• At nadir, half have paraplegia; all have neurogenic bladder; and 80-94% have sensory symptoms

• One third recover, one third have neurological deficits, and one third have plegia

• Poor recovery is predicted by rapid progression, back pain and spinal shock

Differentiating MS from TM

• Asymmetric clinical findings

• Predominant sensory symptoms

• MR lesions extending over fewer than two spinal segments

• Abnormal brain MRI

• Oligoclonal bands in the CSF

Transverse Myelitis

• Rheumatological

• Idiopathic

• A 47-year-old white female presented with a several week history of gait abnormality

• Stiffness and balance difficulties

• Presents to ED with difficulty with urination

• Physical examination revealed a T6 incomplete pin level with lower extremity spasticity, brisk reflexes, and extensor plantar responses

Magnetic resonance imaging

• Abnormal high T2 and low T1 signals extending from T2-T11.

• Abnormal signal was somewhat more pronounced in the right side and central aspects of the cord

• Post-contrast enhancement.

CSF Analysis

• Laboratory tested showed cerebrospinal pleiocytosis with 11% polymorphonucleocytes, 7% lymphocytes, and 82% histiocytes.  

• Glucose was 152 and protein 74, albumin and IgG were elevated.  

• Viral and bacterial studies were negative.  

• No aberrant lymphoid cells and negative for neoplasm.

• Myelin basic protein and oligoclonal bands were normal.

Serum testing

• C-reactive protein of 1.9 mg/dl

• Rheumatoid factor of 104 IU/ml

• Nuclear antibody titer of greater than 1:2560 speckled

• Extractable nuclear antibodies ro and la were positive

Sjogren’s Syndrome Treatment

• Monthly course of pulsed cyclophosphamide

• Subsequent oral cyclophosphamide therapy

• Complete resolution of her neurological symptoms

Transverse MyelitisSellner, Spinal Cord, 2009.

Long Term Problems with Transverse Myelitis

• Myoclonus–Episodic increase in tone–Involuntary–Lasts seconds to minutes–Painful–Responds to anti-convulsants, intrathecal baclofen

• Recurrence–Patients with immunological disorders (Sjogrens, SLE)

Case

• 35 year old elbowed in his upper thoracic apins during a basketball game

• Over the course of several days, he developed cape-like dysesthesias in his arms and hands, mlide weak grip, elbow extensors, hip flexors and knee extensors. He developed hyperreflexia in the legs with ankle clonus.

MRI scan

• T2 enhancement from C3-6 with edema and associated stenosis

• Result: emergent laminectomy, regained bowel and bladder continence and ambulation to 100 feet with assistive device.

But not the end of the story

• Two weeks later, he presented with inability to walk and recurrent bladder dysfunction

• MRI shows unchanged mass with improved cord compression

• CT chest: mediastinal and hilar lymph nodes

• Bronchoscopy of lung nodules reveal non-necrotizing granulomas

Case

• 60 year old with a four month history of progressive right arm weakness

• Started when it was hard to put objects in the overhead bin

• Progressed to include the hand

• ROS shows a couple of falls and some vague stiffness in the left hip

• No pain or sensory symptoms

• EMG of right UE showed normal NCS, active denervation in the deltoid, biceps, brachioradialis, triceps

• MRI showed left C5-6 DDD with foraminal narrowing

• Offered epidural injection but she deferred and was seen for a second opinion

• Thoughts??

Motor Neuron Disease

• LUE with mild intrinsic weakness, no LE signs and no UMN signs

• EMG of asymptomatic LUE showed active denervation in left biceps, triceps, deltoid, pronator teres, extensor indicis

• 2+ fasciculations in the left psoas

• 3+ active denervation in the thoracic paraspinal muscles

Presenting Symptoms of ALS

• Weakness presents first 60%: 1/3 arm, 1/3 leg, ¼ bulbar

• Painful muscle cramps (present in over 80% of ALS patients but not initially)

• Muscle stiffness

• Difficulty walking

• Fatigue

Case

• 27 year old diabetic man presents with a six months history of progressive LE weakness

• Started after hurting his back lifting with LBP and spasms in the left thigh for 3-4 weeks

• Progressive burning and sensory loss in LLE

• One month of progressive LE weakness requiring a walkker

• Stabilize for 2 months, then increase RLE weakness and bilateral pain over one month

DIFFERENTIAL DIAGNOSIS

Differential

• CIDP

• Inflammatory myelitis

• Vascular malformation

Physical Examination

• Upper extremity neurological exam normal

• LE reflexes 1+ with flexor plantar response

• RLE strength 1, LLE 2

• T6 pin level, present but decreased below

• Absent RLE vibration sense, severe decrease LLE

• MRI: question lesion at T6 biopsy normal

• CSF studies normal

• EMG mild axonal peripheral polyneuropathy

• Spinal angiography: continuous venous filling of the coronal venous plexus and spotty filling of radicular veins. –No arteriovenous fistula was seen upon spinal artery or intercostal artery injection.

–Dx: Foix-Alajouanine Syndrome: venous infarct

Outcome

• Coumadin for 18 months: no improvement

• Plavix and aspirin

• 40 months later: 2-3 month time frame of symptom improvement spontaneously

• Normal sacral function, normal ambulation, no sensory level

Vascular

• Sinus venous fistula

• Arterio-venous malformations

• Intradural, perimedullary direct arteriovenous fistulae

• Venous infarction (Foix-alajouanine syndrome)

• Embolic stroke

• Thrombotic stroke

• Aortic dissection

• Small vessel ischemia

Time course of neurological deficit

• Infarcts: sudden

• Vascular malformations: step like changes over time–Highly variable presentation and outcome depending degree of spinal cord ischemia

• Hemorrhage: sudden

Case

• A 22 year old presents for gait evaluation • Family Reunion: "you walk just like your mother did when

she was your age." • 1 month ago: Uunable to run, walk upright • 1 week ago: Urinary incontinence

Two years previously, transient gait disturbance with oral oxycodone after emergent appendectomy

• Similar reaction several months ago after an ED visit for migraine

• However, on returning to the ED due to gait difficulties, she was given oral baclofen and diazepam as needed

• Bladder symptoms: urgency and uncertain if completely emptying

• Fully awake and alert.

• Speech and language are normal.

• Cranial nerves are normal.

• Muscle bulk, tone, strength and coordination are normal in the upperextremities.

• Ashworth 0 except 1+ at the ankle plantarflexors

• Strength 5/5 except 4+ ankle dorsiflexion

• No spinal sensory level to PP or light touch, normal vibration sense

• Gait is stooped, on toes

• High arches and developing hammer toes

Work-up

• Brain, cervical and thoracic MRI normal

• SSEP’s normal

• CSF examination normal

• Normal labs including rheumatological evaluation

Radiation Myelopathy

• Typically late onset 9-20 months)

• Associated with more concentrated course

• No clear dose relationship

• Microvascular injury, demyelination

• Bright T2 with gadolinium enhancement

Overview of NT-SCI

• Increasing incidence, relative and actual

• Outcomes are disease specific

• More paraplegia

• Older, with influence on FIM efficiency and FIM level for neurological status

• Impact on etiology associated co-morbidities: CV disease, diabetes mellitus

THANK YOU FOR YOUR ATTENTION

Now I am going to cheat!

• Spondylotic myelolopathy that I am telling you to wait on calling the surgeon

• The case of the lumbar synovial cyst

• 62 year old woman was well until shoveling snow

• Low back and right lower extremity pain.

• No prior episodes of sciatica but intermittent activity related back pain

• No pain in the left leg and no bowel or bladder dysfunction.

• Decreased pain with walking and motion.

• Increased pain first thing in the morning.

Physical Examination

• Deep tendon reflexes 2+

• Strength 5/5

• Pin sensation loss in the right L5 distribution. Strength was intact.

• Straight leg raising was negative.

• Increase in right leg pain with lumbar extension and right lateral bending.

Other Images

• Physical therapy results in a 40% improvement in symptoms

• Right L4-5 and L5-S1 foramenal epidural injections result in no symptom improvement

THANK YOU FOR YOUR ATTENTION