An Approach to a patient with paraplegia

Dr. Sadia Tareq HMO MU-1

Paraplegia is an impairment in motor and or sensory functions of the lower limbs and is caused

by injury or disease that damages the spinal cord.

Causes of paraplegiaCompressive• Epidural , Intradural or Intramedullary neoplasms• Epidural Abcess• Epidural Haemorrahage• Haematomyelia• Vertebral Neoplasms – metastasis , myeloma• Herniated or Prolapsed Intervertebral Disc• Cervical Spondylosis• Fracture or Dislocation of vertebra- trauma

osteoporosis, paget’s desease

Non Compressive Infectious and post infectious• Viral- polio virus, Varicella Zoster, Herpes Simplex 1 and 2,

Cytomegalovirus, HIV,HTLV-1, rabies virus,Ebstein –Barr Virus, Influenzae virus, mumps, measles,rubella virus

• Bacterial-Mycobacterium Tuberculosis, Treponema Pallidum, Borellia(Lymes disease) ,Listeria, Mycoplasma Pneumoniae

• Parasitic- Schistosomiasis , Toxoplasmosis Inflammatory and Immune mediated • Transverse Myelitis• Multiple Sclerosis • SLE • Sarcoidosis

• Vasculitis(with p-ANCA )• Sjogren’s Syndrome• Behcet’s syndrome• Mixed connective tissue disease Metabolic• Vitamin B 12 Deficiency• Electrolytic Imbalance- hypokalemia,hyperkalemia, hyponatraemia, hypernatraemia,hypercalcaemia Vascular-• Infarction of anterior or posterior spinal arteries• Arteiovenous Malformation• Antiphospholipid Syndrome and other hypercoagulable

states

Developmental• Syringmyelia • Meningomyelocele

Neuromuscular junction disorders• Myasthenia Gravis• Lambert-Eaton Myasthenic syndrome

Peripheral Nerve Disorders• GBS • Peripheral Neuropathy

Clinical Evaluation of a paraplegic patient The following questions needs to be asked during history Mode of Onset • Acute- TM ,Trauma,GBS, Infarction, Infections, Epidural

haematoma,metastases.• Subacute- Pott’s disease, Epidural abcess, Tumours,

Post infectious• Chronic- Spondylosis, MS, Tabes Dorsalis H/O trauma , fall, RTA H/O Backache,Root pains,Radiation of the pain and

aggravating factors Motor or sensory symptoms and symmetry of the

symptoms

Gait Bladder,Bowel and sexual dysfunction (retention

incontinence and sensation,impotence) H/O exposure and STI H/O infections ie, chicken pox, influenzae ,mumps

measles,TB etc H/O vaccination -anti rabies and polio vaccine H/O fever ,seizures,loss of conciousness, headache,

vomitting and features of raised Intracranial Pressure. Dietary Habits -vegeterians ,non vegeterians, alcohol

Features and Important syndromes of spinal cord disease• The extent of the paralysis depends on the level at which

the damage occurs.• Main features of transverse damage at each level of the

spinal cord are as follows:

Cervical cord

Junction of medulla and spinal cord

Fatal due to the involvement of adjacent medullary vasomotor and respiratory centers.

Upper cerical (C1- C3) Quadriplegia and weakness of the diaphragm

C4-C5 Quadriplegia

C5-C6 Loss of power and reflex in

biceps

C7 Weakness of finger and wrist extensors and triceps

C8 Finger and wrist flexion impaired

Horner’s syndrome (Accompanies a cervical cord leision at any level)

Miosis Ptosis Facial hypohidrosis

Thoracic Cord Leisons are localised by the sensory

level on the trunk and midline.Leg weakness and disturbances of bowel and bladder are present.

Lumber CordL2-L4 Paralysis of flexion & adduction of

the thigh, extension of the knee.Loss of patellar reflex.

L5-S1 Paralysis of foot & ankle movement, flexion of the knee & extension of thigh. Loss of ankle jerk.

Sacral Cord Leisions in the sacral segment gives rise to conus or cauda equina syndrome.

Conus syndromeBilateral saddle anesthesia(S3-S5)Bowel and bladder dysfunctionImpotenceLoss of bulbocavernosus (S2-S4) and anal (S4-S5) reflexesMuscle strength is normal

Cauda Equina Syndrome

Mass leisons in the lower spinal canal

Low back pain or radicular pain Assymetric leg weakness and

sensory loss Reflexes are absent in the lower

limbs Sparing of bowel and bladder

functions

Produces mixed clinical picture with both syndromes.

Spinal Cord Level relative to vertebral bodies

Spinal Cord Level Corresponding Vertebral Body

Upper Cervical Same as cord levelLower cervical 1 level higher Upper thoracic 2 levels higherLower Thoracic 2 to 3 levels higher

Lumber Sacral Coccygeal

T10-T12T12-L1L1

Brown Sequard Syndrome• Characterised by Ipsilateral

weakness(corticospinal) and loss of joint position and vibration sense

(posterior coloumns) and contralateral loss of pain and temperature sense(spinothalamic) one or two levels below the leision.

• Common causes – MS Glioma Trauma Myelitis Postradiation myelopathy

Central Cord Syndrome-• Arm weakness more than leg weakness• Dissociated sensory loss ie loss of pain and

temperature sense in cape distribution over shoulders,lower neck and upper trunk but intact light touch,joint position and vibration sense in these regions.

• Causes – Trauma Syringomyelia Tumours Anterior Spinal Artery Ischeamia

Anterior Spinal Artery Syndrome• Cause-Occlusion or diminished flow to anterior

spinal artery .• All spinal cord functions (motor ,sensory and

autonomic ) lost below the level leision except the vibration and postion sense.

Foramen Magnum Syndrome• Compressive leision near the Foramen Magnum

causes weakness of ipsilateral arms ,then legs followed by contralateral arms and then legs (‘around the clock pattern’) and suboccipital pain spreading to neck and shoulders.

Intramedullary and Extramedullary Syndromes• Extramedullary leisions- radicular pain is prominent

early sacral sensory loss and spastic weakness in the legs. Extramedullary leisions can further be distinguished as having extradural and intradural origins. Extradural are usually malignant and intradural are benign and have a longer duration of symptoms.

• Intramedullary leisions – produce poorly localized burning pains and perineal and sacral sensations are intact. Spastic weakness of legs appears later.

Distinguishing between Compressive and Non Compressive Myelopathies

• Epidural compressions due to malignancies or abscesses causes warning signs of neck or back pain,that precedes the development of paralysis, where as the non compressive aietiologies usually produce myelopathies without antecedent symptoms.

Compressive Myelopathies Neoplastic Spinal Cord Compression• Most neoplasms are epidural in origin resulting from

metastasis.• Any malignant tumours can metastasize to spinal coloumn,

most common sites being metastasis from lungs ,breasts, kidneys,prostate ,lymphoma ,blood cell dyscrasia.

• Thoracic cord is commonly involved ,with the exceptions of prostate and ovaries,which metastasize in the sacral and lumber vertebrae,due to spread through the Batson’s venous plexus in the anterior epidural space.

• Retroperitoneal neoplasms especially lymphomas and sarcomas enter the spinal canal through the intervertebral foramina and produce radicular pain and other signs of root involvement prior to cord compression.

• Pain is the initial symptom which is either aching and localised or sharp and radiating in nature.It worsens with movement,coughing or sneezing and awakes the patient at night. A recent onset of persistent back pain especially in the thoracic spine should prompt consideration of vertebral metastasis,as it is uncommonly involved in spondylosis.

• Intradural mass leision are slow growing and benign.• Meningiomas and Neurofibromas mostly accounts for

intradural leisions. Meningiomas are located posterior to the thoracic cord or near the foramen magnum. Neurofibromas are tumours of nerve sheath and arise in the dorsal root.

Spinal Epidural Abscess-• Presents with the triad of midline dorsal pain ,fever and

progressive limb weakness.• Pain is aching ,constant and present over the spine or in

a radicular pattern.• Duration of pain is 2 weeks or less before the

presentation but can be longer.• Fever is accompained by increased WBC count and ESR

and progression is rapid once weakness appears.

MRI Spine –Cord compression MRI Spine- Epidural tubercular abscess

Risk Factors include- Impaired immune status-Diabetes Mellitus, Renal

failure, Malignancies, Alcoholism, IV Drug AbuseSkin and other tissue infections-furunculosis,dental and

pharygeal abscess, bacterial endocarditis, decubitus ulcers.

Iatrogenic complications from Lumber puncture , Epidural Anaesthesia,Spinal surgery.

• Organisms commonly implicated are Staphylococcus aureas, Streptococcus, Mycobacterium tuberculosis, Mycoplasma Pneumonaie,gram negative bacilli , anaerobes, fungi.

Spinal Epidural Haematoma-• There is haemorrhage into the epidural space

causing acute focal or radicular pain followed by spinal cord or conus medularis disorder.

• Predisposing factors- Anticoagulation, trauma, tumour, blood dyscrasia

Haematomyelia –• Haemorrage into the substance of spinal cord.• Causes- Trauma ,vascular malformation, Vasculitis

due to SLE or PAN,bleeding disorder, spinal neoplasms.

• Presents as acute painful transverse myelopathy.

Non-Compressive Myelopathies Most frequent causes of non compressive acute

transverse myelopathies(ATM) are Spinal Cord Infarction, Systemic disorders, Infectious, Demyelinating diseases and Idiopathic Transverse Myelitis.

Spinal Cord Infarction-• Spinal cord ischaemia can occur at any level . T3-T4

and at boundary zones between anterior and posterior spinal artery territories are at greatest ischaemic risk from systemic hypotension.

• Acute infarction in ant. spinal territory produces anterior spinal artery syndrome with a sudden and dramatic onset within minutes to few hours.

• There is a sharp midline or radiating back pain localised to the area of ischaemia.

• Initially there is spinal shock later hyperreflexia and spasticity develops.

• Posterior spinal artery infarction is less common and results in loss of joint position and vibration sense.

• Spinal Cord Infarction is associated with the following-Aortic AtherosclerosisDissecting Aortic AneurismsSevere Hypotension Cardiogenic EmboliVasculitis due to collagen vascular diseases esp SLE,

Antiphosholipid Antibody Syndrome

• Cord Infarction due to presumed thromboembolism does not need acute anticoagulation unless there is unusual TIA or infarction with a progressive course. Antiphospholipid antibody syndrome is treated with anticoagulation.

Systemic Disorders-• Myelitis also occurs in certain immune mediated

disorders such as SLE, Sarcoidosis,Sjogren’s syndrome ,Behcet’s syndrome,Mixed Connective Tissue Diseases,Vasculitis with perinuclear antineutrophilic cytoplasmic antibody(p-ANCA).

• ATM may be the presenting symptom of SLE and antiphospholipid antibodies are present in nearly 2/3 rd of SLE –associated ATM.

• ATM may be preceded or followed by optic neuritis (Neuromyelitis Optica).

• Recurrent episodes of myelitis are usually due to immune mediated diseases such as SLE or sarcoids,demyelinating diseases or infection with HSV-2.

Demyelinating diseases-• Multiple sclerosis may present with myelitis, particularly

in individuals of Asian or African ancestry. • Neuromyelitis optica (NMO) is a demyelinating

syndrome consisting of a severe myelopathy associated with optic neuritis which is often bilateral and may precede or follow myelitis by weeks or months.It is also associated with SLE and antiphospholipid antibodies and also with other connective tissue diseases.

• A brain MRI is most helpful in determining the likelihood that a case of myelitis represents an initial attack of MS. A normal scan indicates that the risk of evolution to MS is low, whereas the finding of multiple periventricular T2-bright lesions indicates a much higher risk.

•The CSF may be normal, but more often there is a mild pleocytosis with several hundred mononuclear cells per microliter. oligoclonal bands are variable, but when bands are present, a diagnosis of MS is more likely. These bands are generally absent in neuromyelitis optica.

Infectious and Post Infectious-• Many viruses have been associated with an acute

myelitis such as Herpes zoster , HSV types 1 and 2, EBV, CMV,Polio virus ,Rabies Virus. HSV-2 produces recurrent sacral myelitis in association with outbreaks of genital herpes mimicking MS.

• Bacterial and mycobacterial myelitis are less common than viral causes and mostly form abscesses.

• Schistosomiasis is an important cause of parasitic myelitis in endemic areas. The process is intensely inflammatory and granulomatous, caused by a local response to tissue-digesting enzymes from the ova of the parasite. Toxoplasmosis causes a focal myelopathy,especially in AIDS patients.

• Postinfectious myelitis begins as the patient appears to be recovering from an acute febrile infection, or in the subsequent days or weeks, but an infectious agent cannot be isolated from the nervous system or spinal fluid and is presumed that it represents an autoimmune disorder triggered by infection and is not due to direct infection of the spinal cord.

• Pott’s disease (spinal TB) involves 2 or adjacent vertebrae and their intervertebral discs and commonly affects the lower thoracic and upper lumbar vertebrae in adults. In advanced cases collapse of vertebral bodies results in kyphosis (gibbus). It can present with psoas abscess. Paraplegia is a frequent complication of Pott’s due to compression of the cord by the abscess or leision.

Idiopathic Transverse Myelitis- • In ¼th of cases no underlying cause is identified and later

manifest with additional symptoms of systemic immune mediated disease like SLE or a demyelinating disorder.

Chronic Myelopathies- Spondylitic myelopathies-• Spondylitic myelopathy is one of the most common

causes of gait difficulty in the elderly. • Initial symptoms are neck and shoulder pain with

stiffness; impingement of bone and soft tissue overgrowth on nerve roots results in radicular arm pain, mostly in a C5 or C6 distribution. Compression of the cervical cord produces a slowly progressive spastic paraparesis which is asymmetrical and accompanied by paresthesias in the feet and

hands.Dermatomal sensory loss in the arms, atrophy of intrinsic hand muscles, increased deep-tendon reflexes in the legs, and extensor plantar responses are common. Urinary urgency or incontinence occurs in advanced cases.

Vascular Malformations- These are uncommon and treatable cause of progressive myelopathies. AVMs are located posteriorly along the surface of the cord or within the dura. Typical presentation being a middle aged man with progressive myelopathy that worsens slowly or intermittently with periods of remissions.

Retrovirus Associated Myelopathies-• The myelopathy associated with the human T cell

lymphotropic virus type I (HTLV-I), formerly called tropical spastic paraparesis, is a slowly progressive spastic syndrome with variable sensory and bladder disturbances.

• The onset is insidious and illness slowly progressive. The presentation may resemble primary progressive MS .Diagnosis is made by demonstration of HTLV-I–specific antibody in serum by enzyme-linked immunosorbent assay (ELISA), confirmed by radioimmunoprecipitation or western blot analysis.

• A progressive myelopathy may also result from HIV infection, characterized by vacuolar degeneration of the posterior and lateral tracts, resembling subacute combined degeneration.

Chronic Myelopathy of Multiple Sclerosis-• A chronic progressive myelopathy is the most frequent

cause of disability in both primary progressive and secondary progressive forms of MS. Involvement is typically bilateral, asymmetrical and produces motor, sensory, and bladder/bowel disturbances.

Fixed motor disability is due to extensive loss of axons in the corticospinal tracts. The symptoms are not simply due to demyelination.

Subacute Combined Degeneration (Vitamin B12 Deficiency)- This treatable myelopathy presents with subacute

paresthesias in the hands and feet, loss of vibration and position sensation. and a progressive spastic and ataxic weakness. Loss of reflexes due to an associated peripheral neuropathy in a patient who also has Babinski signs, is an important diagnostic clue. The myelopathy is diffuse, signs are symmetrical. The diagnosis is confirmed by the finding of macrocytic red blood cells, a low serum B12,Conc., elevated serum levels of homocysteine and methylmalonic acid, and in uncertain cases a positive Schilling test .

Hypocupric Myelopathy• This recently described myelopathy is identical to

subacute combined degeneration. Low levels of serum copper are found and often there is also a low level of serum ceruloplasmin.

Tabes Dorsalis- • Tabes Dorsalis is a late manifestation of syphilis which

presents as demyelination of posterior coloumns ,dorsal roots and dorsal root ganglia.

Others-• Primary lateral sclerosis is a degenerative disorder

characterized by progressive spasticity with weakness, accompanied by dysarthria and dysphonia and bladder symptoms. Sensory function is spared.

• Familial Spastic Paraplegia , Adrenomyeloneuropathy are genetic disorders where there are progressive spastic paraparesis.

• Toxic Causes include Lathyrism- due to ingestion of chickpeas containing B-

N-oxaloaminoalanine(BOAA). Inhalation of nitrous oxide Investigations• MRI of spinal cord with and without contrast • CSF studies • Blood culture for isolation of organisms,RPR for HIV IgG ,IgM antibody for Enteroviruses, Mumps, Measles

Rubella; Brucella, Chlamydia, Bartonella,schistosomal antibodies.

• CBC with ESR• ANA,Anti DsDNA• Rheumatoid factor, Anti-SSA , Anti –SSB, Complement

levels• p-ANCA, Antimicrosomal antibody• Stool for ova and parasites, Nasopharygeal swab cultures• Antiphospholipid and anticardiolipin antibodies• If Sjögren syndrome suspected Schirmer test salivary gland scintography salivary/ lacrimal gland biopsy.• Sarcoidosis- Serum angiotensin-converting enzymes Serum Ca & 24 hr urinary ca Chest x-ray ,CT & Lymph node biopsy.

• Demyelinating disease- Brain MRI ,CSF oligoclonal bandsNeuromyelitis optica antibody (aquaporin-4)• Vascular causes- CT myelogram; Spinal angiogram.

Rehabilitation of Spinal Cord Disorders• The prospects for recovery from an acute

destructive spinal cord lesion fade after ~6 months. There are currently no effective means to promote repair of injured spinal cord tissue. The primary goals are development of a rehabilitation plan framed by realistic expectations and attention to the neurologic, medical, and psychological complications that commonly arise.

• Detrusor spasticity is treated with anticholinergic drugs (oxybutinin, 2.5–5 mg qid) or tricyclic antidepressants with anticholinergic properties (imipramine, 25–200 mg/d).

• Urinary dyssynergia may be managed with the alpha-adrenergic blocking agent terazosin hydrochloride (1–2 mg tid or qid), with intermittent catheterization, or, if that is not feasible, by use of a condom catheter in men or a permanent indwelling catheter. Surgical options include the creation of an artificial bladder by enterocystoplasty . Bladder areflexia due to acute spinal shock or conus lesions is best treated by catheterization.

• Bowel regimens and disimpaction are necessary in most patients to ensure at least biweekly evacuation and avoid colonic distention or obstruction.

• Patients with acute cord injury are at risk for venous thrombosis and pulmonary embolism. During the first 2 weeks, use of calf-compression devices and anticoagulation with heparin (5000 U subcutaneously every 12 h) or warfarin (INR, 2–3) are recommended. In cases of persistent paralysis, anticoagulation should be continued for 3 months.

• Prophylaxis against decubitus ulcers should involve frequent changes in position in a chair or bed, the use of special mattresses, and cushioning of areas where pressure sores often develop, such as the sacral prominence and heels. Early treatment of ulcers with careful cleansing, surgical or enzyme debridement of necrotic tissue, and appropriate dressing and drainage may prevent infection of adjacent soft tissue or bone.

• Spasticity is aided by stretching exercises to maintain mobility of joints. Baclofen (15–240 mg/d in divided doses) is effective; Diazepam is useful for leg spasms that interrupt sleep (2–4 mg at bedtime). Tizanidine (2–8 mg tid), an alpha2 adrenergic agonist, is another option. For nonambulatory patients, the direct muscle inhibitor dantrolene (25–100 mg qid) may be used, but it is potentially hepatotoxic.