Diseases of the Spinal Cord. Disease description Many conditions constitute neurologic emergencies because of Severity on presentation (quadriplegia,

Diseases of the Spinal Cord

Disease description

Many conditions constitute neurologic emergencies because of Severity on presentation (quadriplegia,

sensory deficits, spinal shock)Potential reversibility

Hallmark: presence of horizontally defined level below which motor, sensory and autonomic function is impaired

Approach to a Patient

1. Determine level of lesion

2. Identify special anatomic patterns, if any

3. Determine time frame of the disease

4. Differentiate compressive from non-

compessive lesions

Determining level of the lesion Sensory level

Using pinprick or cold stimulusLevel indicates damage to the spinothalamic

tract 1-2 segments above a unilateral lesion or at the level of the bilateral lesion

Determining level of the lesionMotor level

Damage to the descending corticospinal tractParaplegia or quadriplegiaUpper motor neuron syndrome

Autonomic abnormalitiesSweatingBladder or bowel dysfunction

Determining level of lesionLevel of Lesion Manifestations

Cervical Horner’s syndrome at any levelUpper cervical: quadriplegia, diaphragm weakness

Thoracic Localized by the sensory level on the trunk or midline back pain, if presentBeevor’s sign (T9-T10 lesion leading to paralysis of lower abdominals, thus upward movement of umbilicus on abdominal contraction)

Lumbar Lesions at L5-S1 abolish ankle jerk

Sacral/conus medullaris

Bilateral saddle anesthesia with urinary retention, fecal incontinence with lax anal tone, impotence

Determining level of the lesionUpper level of the lesion may be

determined with segmental signsHyperalgesia or hyperpathiaFasciculations or muscle atrophyHypo- or areflexia

Special patterns

Special patterns

Brown-Sequard syndrome Ipsilateral weakness (corticospinal), loss of

joint and position sense (posterior column)Contralateral loss of pain or temperature

sense 1-2 levels below the lesion (spinothalamic)

Unilateral segmental signs

Special patterns

Central cord syndromeDamge to gray matter and crossing

spinothalamic tracts near central canalArm > leg weaknessLoss of pain and temperature sense with

intact light touch, joint position and vibration sense (dissociated sensory loss)

Special patterns

Anterior spinal artery syndrome Extensive bilateral deficits below the level of the

lesion, with intact vibration and position senseForamen magnum syndrome

Damage to decussating pyramidal tract fibers of the legs

“Around the clock” pattern of weakness with suboccipital pain

Special patterns

ExtramedullaryRadicular painEarly sacral sensory loss, spastic leg

weaknessIntramedullary

Sacral sparing

Time-frame of the disease

Acute or severe transverse lesions may initially present as spinal shock (flaccidity, areflexia) in the first few days or weeks, rather than upper motor neuron signsMay be mistaken for acute severe

polyneuropathy or stratified to have more severe damage than actual

Compressive vs noncompressive myelopathy

Compressive (mass)TumorEpidural abscess or

hematomaHerniated discVertebral pathology

Non-compressive (intrinsic cord lesions)Vascular Inflammatory Infectious

Tumors

Extradural

Intradural

Intramedullary

Epidural abscess

Epidural hematoma

Compressive myelopathies

Neoplastic spinal cord compression: Epidural neoplasms

Mostly metastatic (breast, lung, prostate, kidney, lymphoma, myeloma)

Thoracic cord most common except for prostate and ovarian cancers (lumbosacral)

Pain is an early symptom, awakens patient at night and is worse with movement

MRI is useful

Neoplastic spinal cord compression: EpiduralTherapy

Glucocorticoids Local radiotherapy Treatment of underlying tumor

Fixed motor deficits of >12 hours do not usually improve and > 48 hours, prognosis is poor

Neoplastic spinal cord compression: IntraduralMostly slow-growing and benignMeningiomas, neurofibromas, also chordoma,

lipoma, dermoid, sarcomaPresent with radicular sensory symptoms

followed by asymmetric progressive cord syndrome

Therapy is surgical resection

Neoplastic spinal cord compression: Intramedullary

Present as central cord or hemicord syndromes of the cervical region

Ependymoma, hemangioblastoma or low-grade astrocytoma in adults

Secondary metastatic lesions are also common

Spinal epidural abscess

Triad: midline dorsal pain, fever, progressive limb

weaknessRisk factors:

impaired immune status, intravenous drug abuse, infections of skin or otehr tissues

Causes: Hematogenous spread Direct extension

Spinal epidural abscessCausative organsims

Staph. AureusTuberculosis Gram-neg bacilli, Strep, anaerobes, fungi

Lab testsMRIHigh cervical MRI (to rule out concomittant

meningitis)Blood culture

Spinal epidural abscess

TreatmentDecompressive laminectomy with

debridement withEmpirical, then culture-guided, antibiotics for

>4weeks

Spinal epidural hematoma

Acute focal or radicular pain with variable cord findings

Risk factors: anticoagulation, trauma, tumor or blood dyscrasia

Labs MRI, CT

Therapy is surgical decompression and correction of underlying problem

Spinal cord infarction Inflammatory and

immune myelitis Systemic inflammatory

diseasesSLE

Demyelinating myelopathyMultiple sclerosis

Post-infectious myelitis Acute infectious

myelitis

Noncompressive myelopathies

Spinal cord infarction

“Watershed” infarcts T3-T4 and boundary zones between anterior

and posterior spinal artery territoriesRapidly progressive weakness and spasticity

with little sensory changeUsually associated with hypotension

Spinal cord infarctionAnterior spinal artery infarct

Anterior cord syndromeParaplegia or quadriplegia, dissociated sensory

loss, loss of sphincter controlOnset may be sudden or progressive over

hoursSharp midline back pain Initial spinal shock

Systemic inflammatory disordersMostly due to SLE, specially with

antiphopholipid antibodiesCSF may be normal or show mild

lymphocytic pleocytosisMay respond to high dose steroids and

cyclophosphamide

Demyelinating myelopathyMultiple sclerosis may present as myelitis

Mild swelling and edema of the cordMultifocal areas of abnormal T2 signal on MRIMild pleocytosis, oligoclonal bandHihg dose steroids, plasma exchange

Neuromyelitis opticaNo oligoclonal bandsMay respond to anti-CD20 (rituximab)

Post-infectious or post-vaccinal myelitisOrganisms implicated: EBV, CMV,

mycoplasma, influenza, measles, varicella, rubeola, mumps

Autoimmune disorder triggered by infection and not due to direct infection of spinal cord

Treatment glucocorticoids,plasma exchange

Acute infectious myelitis

Poliomyelitis, herpes zosterBacterial or mycobacterial myelitis

(abscess)Schistosomiasis

Spondylitic myelopathy

Vascular malformations of the cord and dura

SyringomyeliaSubacute combined

degenerationTabes dorsalisFamilial spastic

paraplegia

Chronic myelopathies

Spondylitic myelopathy

Most common cause of gait problems in the elderlyEarly neck and shoulder stiffness, later radicular

painCord compression in <1/3 of patients

Spastic paraparesis, paresthesia Reduced vibratory sense, (+) Romberg sgin Dermatomal sensory loss & decreased tendon reflex in

the arms, intrinsic hand muscle atrophy Hyperreflexia in the legs

Vascular malformation of the cord & duraSlowly progressive or intermittent

myelopathy with incomplete sensory, motor or bladder disturbance

Spinal bruitsLabs : high resolution contrast MRI, CT

myelogram, selective spinal angiographyTherapy: endovascular embolization

Syringomyelia

Developmental cavitary expansion of the cervical cord, often associated with Chiari type I malformation

Classic presentation is central cord syndrome (dissociated sensory loss, areflexi weakness of the arms)

Symptoms progress as the syrinx expand

Subacute combined degenerationVitamin B12 deficiencySubacute paresthesia in hands & feet,

loss of vibration and position sense, progressive spastic & ataxic weaknessDiffuse, symmetric myelopathyPredominant involvement of posterior and

lateral tractsAssociated peripheral neuropathy

Subacute combined degenerationLabs: macrocytic RBCs, low B12

concentrationTherapy: replacement with intramuscular

1000ug vitamin B12 at regular intervals or subsequent oral treatement

Tabes dorsalis

Complication of syphilisFleeting and lancinating pains in the legs,

wtih ataxia due to loss of position senseLoss of leg reflexes Impaired vibratory and position senseRomberg’s signArgyll Robertson pupils

Differential: diabetic polyradiculopathy

Familial spastic paraplegia

Genetic defects of >20 different loci presenting as slowly progressive myelopathy Progressive spasticity and leg weakness Absent or mild sensory symptoms Sphincter disturbances + nystagmus, ataxia, optic atrophy

Onset maybe from infancy to middle adulthoodTherapy: symptomatic for spasticity

Summary

Spinal cord conditions may present catastrophically but many have treatable causes

Determine the level of the lesion and identify special patterns in the presentation to derive a diagnosis

Address the conditions promptly

Thank you.