Hemi-section of Spinal Cord: Etiology & Clinical Features of Paraplegia (2nd Year, Jan 2025)

PARAPLEGIA Dr fawad jan Assistant professor neurology MBBS, FCPS neurology Learning outcome Learning outcome Describe the clinical features of brown Sequard syndrome. Describe the etiology, clinical features, investigations and management of patient with parap...

PARAPLEGIA Dr fawad jan Assistant professor neurology MBBS, FCPS neurology Learning outcome Learning outcome Describe the clinical features of brown Sequard syndrome. Describe the etiology, clinical features, investigations and management of patient with paraplegia. Brown Sequard Syndrome Clinical features 1 dcml 3 st 2 cs 1 dcml 2 cs 3 st 2 cs 3 st 1 dcml Clinical signs in brown-sequard syndrome Deficit Cause of clinical deficit Ipsilateral below Motor Spastic weakness Lateral column/ cortical spinal system level of lesion Deep tendon reflexes increased Lateral column/cortical spinal system Babinski sign Pyramidal tract in the lateral column Sensory Vibration, position sense, and all other Posterior column proprioceptive sensory modalities Contralateral Sensory Loss of pain and temperature Lateral spinothalamic tract below level of sensation lesion Beginning one or two segments below the level of the transaction/ compression Ipsilateral at the Motor Band of weakness atrophy and anterior root and or the anterior horn level of lesion fasciculations Motor/sensory Depression of deep tendon stretch afferent or efferent components reflexes involved in the monosynaptic stretch reflex arc Sensory Ipsilateral loss of all sensory modalities Damage to the dorsal root and Leading to a dermatomal pattern dorsal horn of sensory loss. Clinical signs in brown -sequard syndrome Deficit Cause of clinical deficit Ipsilateral below Motor Spastic weakness Lateral column/ cortical spinal system level of lesion Deep tendon reflexes increased Lateral column/cortical spinal system Babinski sign Pyramidal tract in the lateral column Sensory Vibration, position sense, and all other Posterior column proprioceptive sensory modalities Contralateral Sensory Loss of pain and temperature Lateral spinothalamic tract below level of sensation lesion Beginning one or two segments below the Level of the transaction/ compression Ipsilateral at the Motor Band of weakness atrophy and anterior root and or the anterior horn level of lesion fasciculations Motor/sensory Depression of deep tendon stretch Afferent or efferent components involved reflexes in the monosynaptic stretch reflex arc Sensory Ipsilateral loss of all sensory modalities Damage to the dorsal root and dorsal Leading to a dermatomal pattern of horn sensory loss. PARAPLEGIA  Paraplegia is defined as weakness or paralysis affecting both legs due to damage to the spinal cord. This can manifest as spastic paraplegia, with increased muscle tone, or flaccid paraplegia, with decreased muscle tone, and may also involve sensory and autonomic dysfunction. The severity, time course, and other associated symptoms depend on the level and type of spinal cord damage. Feature Flaccid Paraplegia Spastic Paraplegia Etiologies Spinal cord injury (SCI) at or below the conus Hereditary spastic paraplegia (HSP) medullaris Guillain-Barré syndrome (GBS) Multiple sclerosis (MS) Poliomyelitis Spinal cord injury (SCI) above the conus medullaris Acute flaccid myelitis (AFM) Cerebral palsy (CP) Cauda equina syndrome Stroke Clinical Features Decreased muscle tone (hypotonia) Increased muscle tone (hypertonia) Absent tendon reflexes (areflexia) Exaggerated tendon reflexes (hyperreflexia) Muscle atrophy Muscle spasms Fasciculations Clonus Flaccid paralysis Spastic paralysis Management Acute stabilization (respiratory, Physical therapy to manage spasticity cardiovascular) Oral antispasticity agents (e.g., baclofen, Physical therapy to maintain muscle strength tizanidine) Prevention of complications (e.g., pressure Botulinum toxin injections for focal spasticity ulcers, UTIs) Addressing underlying cause (e.g., Intrathecal baclofen pump for severe immunotherapy for GBS) spasticity Supportive care (e.g., assistive devices) Surgical interventions (e.g., selective dorsal rhizotomy) Etiology Traumatic 73% trauma ETIOLOGY Non-traumatic 27% 1. Motor vehicle 1. Spinal tumors 29 % compressive accidents 55 % 2. Spinal stenosis 29 2. Fall 34 % % Non- compressive 12 % 3. Gunshot wounding 3. Transverse myelitis 8% 1. Multiple sclerosis (MS): 18 % 4. Diving 2% 29% 4. Vascular origin 16 5. Sports injury 1% 2. Idiopathic myelitis: 19% % Spinal cord 3. Sarcoidosis: 16.5% 5. Spinal arachnoiditis Compressive 88% (intradural, 4. Spinal cord infarction: 14% 5% intramedullary) 5% 5. Human T-cell lymphotropic 6. Syringomyelia 3 % Meninges (intradural, virus (HTLV) myelopathy: Vertebral 80% extramedullary) 15% Tumours (e.g. 4.8% 1. glioma, 6. Radiation myelopathy: 4% 1. Trauma (extradural) 7. Neuromyelitis optica 2. ependymoma, 2. Intervertebral disc spectrum disorder 3. metastasis) prolapse 1. Tumours (e.g. (NMOSD): 3.8% 3. Metastatic carcinoma 1. meningioma, 8. HIV-associated vacuolar e.g. 2. neurofibroma, myelopathy: 1.4% i. breast, 3. ependymoma, 9. Paraneoplastic ii. prostate, 4. metastasis, myelopathy: 1.21% iii. bronchus 5. lymphoma, 10.Vitamin B12 deficiency 4. Myeloma 6. leukaemia) myelopathy: 0.7% 5. Tuberculosis 2. Epidural abscess Causes of spinal cord compression Site Frequency Causes I. Vertebral 80% 1. Trauma (extradural) 2. Intervertebral disc prolapse 3. Metastatic carcinoma e.g. i. breast, ii. prostate, iii. bronchus 4. Myeloma 5. Tuberculosis II. Meninges (intradural, 15% 1. Tumours (e.g. extramedullary) 1. meningioma, 2. neurofibroma, 3. ependymoma, 4. metastasis, 5. lymphoma, 6. leukaemia) 2. Epidural abscess III. Spinal cord (intradural, 5% 1. Tumours (e.g. glioma, ependymoma, metastasis) intramedullary) trinsic diseases of the spinal cord Type of disorder Condition Clinical features Congenital Diastematomyelia (spina bifida) Features variably present at birth and deteriorate thereafter LMN features, deformity and sensory loss of legs Impaired sphincter function Hairy patch or pit over low back Incidence reduced by increased maternal intake of folic acid during pregnancy Hereditary spastic paraplegia Onset usually in adult life Autosomal dominant inheritance usual Slowly progressive UMN features affecting legs > arms Little or no sensory loss Infective/inflammatory Weakness and sensory loss, often with pain, developing over hours to days Transverse myelitis due to viruses (HZV), schistosomiasis, HIV, MS, sarcoidosis UMN features below lesion Impaired sphincter function Paraneoplastic May predate tumour diagnosis Vascular Anterior spinal artery infarct due to Abrupt onset atherosclerosis, aortic dissection, embolus Anterior horn cell loss (LMN) at level of lesion UMN features below Spinothalamic it sensory loss below lesion but dorsal column sensation spared Spinal AVM/ dural fistula Onset variable (acute to slowly progressive) Variable LMN, UMN, sensory and sphincter disturbance Symptoms and signs often not well localised to site of AVM Neoplastic Glioma, ependymoma Weakness and sensory loss often with pain, developing over months to years UMN features below lesion in cord; additional LMN features in conus Impaired sphincter function Metabolic Progressive spastic paraparesis with proprioception loss Vitamin B12 deficiency (subacute combined Absent reflexes due to peripheral neuropathy degeneration) ± Optic nerve and cerebral involvement Copper deficiency Excess dietary Zinc Nitrous oxide toxicity Modifies vitamin B12 metabolism Degenerative Relentlessly progressive LMN and UMN features, associated bulbar weakness Motor neuron disease No sensory involvement Gradual onset over months or years, pain in cervical segments Syringomyelia Anterior horn cell loss (LMN) at level of lesion, UMN features below it Suspended spinothalamic sensory loss at level of lesion, dorsal columns preserved (AVM arteriovenous malformation; HIV human immunodeficiency virus; HZV herpes zoster virus; LMN = lower motor neuron; MS = multiple sclerosis; UMN = upper motor = = = neuron) CLINICAL FEATURES Symptoms of spinal cord compression Pain Localized over the spine or in a root distribution, aggravated by coughing, sneezing or straining Sensory Paresthesia, numbness or cold sensations, especially in the lower limbs, which spread proximally, often to a level on the trunk Motor Weakness, heaviness or stiffness of the limbs, most commonly the legs Sphincters Urgency or hesitancy of micturition, leading eventually to urinary retention Signs of spinal cord compression Cervical, above C5 Upper motor neuron signs and sensory loss in all four limbs Diaphragm weakness (phrenic nerve) Complete lesions above C5 requires intubation in 100 % of patients Cervical, C5–T1 Lower motor neuron signs and segmental sensory loss in the arms; upper motor neuron signs in the legs Respiratory (intercostal) muscle weakness Thoracic cord Spastic paraplegia with a sensory level on the trunk Weakness of legs, sacral loss of sensation and extensor plantar responses Cauda equina Spinal cord ends approximately at the T12/L1 spinal level and spinal lesions below this level can cause lower motor neuron signs only by affecting the cauda equina  8 3 6 7 5 4 1 2 Patterns of motor loss according to the anatomical site of the lesion. INVESTIGATION Investigation of acute spinal cord syndrome 1.Magnetic resonance imaging of spine or myelography 2.Plain X-rays of spine 3.Chest X-ray 4.Cerebrospinal fluid 5.Serum vitamin B12  Spinal cord compression  Patients with a history of acute or subacute spinal cord syndrome should be investigated urgently.  The investigation of choice is MRI, as it can define the extent of compression and associated soft-tissue abnormality.  Plain X-rays may show bony destruction and soft-tissue abnormalities.  Routine investigations, including chest X-ray, may provide evidence of systemic disease.  If myelography is performed, o CSF should be taken for analysis; o in cases of complete spinal block, this shows a normal cell count with a very elevated protein causing yellow discoloration of the fluid (Froin syndrome). o The risk of acute deterioration after myelography in spinal cord compression means that the neurosurgeons should be alerted before it is undertaken.  Where a secondary tumor is causing the compression, needle biopsy may be required to establish a tissue diagnosis. Magnetic resonance image showing cervical cord compression (arrow) in cervical spondylosis. 1. Axial magnetic resonance image of thoracic spine. 2. A neurofibroma (N) is compressing 3. the spinal cord (SC) 4. and emerging in a ‘dumbbell’ fashion through the vertebral foramen into the paraspinal space. 1. Computed tomographic myelogram 2. of cervical spine at the level of C2 3. showing bony erosion of vertebra by a metastasis (arrow). Intrinsic diseases of the spinal cord  Investigation of intrinsic disease starts with imaging to exclude a compressive lesion.  MRI  provides most information about structural lesions, such as diastematomyelia, syringomyelia or intrinsic tumors.  Non-specific signal change may be seen in the spinal cord in inflammatory or infective conditions and metabolic disorders such as vitamin B12 deficiency.  Lumbar puncture or blood tests  may be required to make a specific diagnosis. MANAGEMENT Specific Therapy Treat The Cause  Spinal cord compression  Treatment and prognosis depend on the nature of the underlying lesion.  Benign tumours  should be surgically excised,  good functional recovery can be expected unless  a marked neurological deficit has developed before diagnosis. Extradural compression due to malignancy  is the most common cause of spinal cord compression in developed countries  has a poor prognosis.  Useful function can be regained if treatment, such as radiotherapy,  is initiated within 24 hours of the onset of severe weakness or sphincter dysfunction;  management should involve close cooperation with both oncologists and neurosurgeons.  Spinal cord compression due to tuberculosis  is common in some areas of the world  and may require surgical treatment.  This should be followed by appropriate antituberculous chemotherapy for an extended period.  Traumatic lesions of the vertebral column  require specialized neurosurgical treatment. Intrinsic disease According to cause Inflammatory-steroids Subacute combine degeneration----Vitamin B12 Multiple sclerosis---Disease-Modifying Therapies General Care Bladder management General Care Passive physiotherapy The bladder does not empty and urinary helps to prevent contractures. Bowel management retention results. Patients self-catheterize or Severe spasticity, with flexor or Constipation and impaction must be develop reflex bladder emptying, helped by extensor spasms, avoided. may be helped by muscle abdominal pressure. Following acute paraplegia, manual relaxants such as baclofen or Early treatment of urine infections is evacuation is necessary; by botulinum toxin essential. injections. Reflex emptying develops later Chronic kidney disease may develop from chronic obstruction or repeated urinary tract infection. Skin care Rehabilitation Risks of pressure ulcers and their sequelae are serious. Many patients with traumatic paraplegia or The sacrum, iliac crests, greater trochanters, heels and maleoli should be tetraplegia return to self-sufficiency (especially if the level is at C7 or below). inspected frequently. A specialist spinal rehabilitation unit is necessary. Rx Light- weight, specially adapted wheelchairs Meticulous attention must be paid to cleanliness and regular turning. provide independence. Pressure-relieving mattresses are useful initially until patients can turn Tendon transfer operations themselves. may allow functional grip if hands are weak. Autonomic dysreflexia may be a problem. If pressure ulcers develop, plastic surgery may be required. Patients with paraplegia have substantial practical, Pressure palsies, such as those of ulnar nerves, can occur. psychological and sexual needs. Care of the patient with paraplegia Where patients are left with a severe paraplegia, there are several issues in long-term care, and specialist nursing is vital. Bladder management. Bowel function. Skin care. Lower limbs. Rehabilitation. Bladder management. The bladder does not empty and urinary retention results. Patients self-catheterize or develop reflex bladder emptying, helped by abdominal pressure. Early treatment of urine infections is essential. Chronic kidney disease may develop from chronic obstruction or repeated urinary tract infection. Bowel function. Constipation and impaction must be avoided. Following acute paraplegia, manual evacuation is necessary; Reflex emptying develops later Skin care. Risks of pressure ulcers and their sequelae are serious. The sacrum, iliac crests, greater trochanters, heels and maleoli should be inspected frequently. Rx Meticulous attention must be paid to cleanliness and regular turning. Pressure-relieving mattresses are useful initially until patients can turn themselves. If pressure ulcers develop, plastic surgery may be required. Pressure palsies, such as those of ulnar nerves, can occur. Lower limbs. Passive physiotherapy helps to prevent contractures. Severe spasticity, with flexor or extensor spasms, may be helped by muscle relaxants such as baclofen or by botulinum toxin injections. Rehabilitation. Many patients with traumatic paraplegia or tetraplegia return to self-sufficiency (especially if the level is at C7 or below). A specialist spinal rehabilitation unit is necessary. Light- weight, specially adapted wheelchairs provide independence. Tendon transfer operations may allow functional grip if hands are weak. Autonomic dysreflexia may be a problem. Patients with paraplegia have substantial practical, psychological and sexual needs. The recommended font size for medical PowerPoint presentations is between 28 and 34 points in bold. The font should be large enough to be easily read.