Diseases of the Spinal Cord

Disease descriptionMany 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 patternsBrown-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 patternsCentral cord syndrome

Damge to gray matter and crossing spinothalamic tracts near central canal

Arm > leg weaknessLoss of pain and temperature sense with

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

Special patternsAnterior spinal artery syndrome

Extensive bilateral deficits below the level of the lesion, with intact vibration and position sense

Foramen magnum syndrome Damage to decussating pyramidal tract fibers of

the legs “Around the clock” pattern of weakness with

suboccipital pain

Special patternsExtramedullary

Radicular painEarly sacral sensory loss, spastic leg

weaknessIntramedullary

Sacral sparing

Time-frame of the diseaseAcute 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 myelopathyCompressive (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 neoplasmsMostly 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 movementMRI 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: IntramedullaryPresent as central cord or hemicord

syndromes of the cervical regionEpendymoma, hemangioblastoma or low-

grade astrocytoma in adultsSecondary metastatic lesions are also

common

Spinal epidural abscessTriad:

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 abscessTreatment

Decompressive laminectomy with debridement with

Empirical, then culture-guided, antibiotics for >4weeks

Spinal epidural hematomaAcute focal or radicular pain with variable

cord findingsRisk factors: anticoagulation, trauma, tumor

or blood dyscrasiaLabs

MRI, CTTherapy 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 territories

Rapidly progressive weakness and spasticity with little sensory change

Usually 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 myelitisPoliomyelitis, 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 myelopathyMost 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

SyringomyeliaDevelopmental 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 dorsalisComplication 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 paraplegiaGenetic 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

SummarySpinal 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.