Diseases of the Spinal Cord. Disease description Many conditions constitute neurologic emergencies...
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Transcript of Diseases of the Spinal Cord. Disease description Many conditions constitute neurologic emergencies...
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.