PARAPLEGIA AND SPINAL CORD SYNDROMES Dr. M. A Sofi MD;FRCP(London);FRCPEdin FRCSEdin

Overview of Spinal Cord Information highway between brain and body Extends through vertebral canal from foramen magnum to L1 Each pair of spinal nerves receives sensory information and issues motor signals to muscles and glands Spinal cord is a component of the Central Nervous System while the spinal nerves are part of the Peripheral Nervous System

Functions of the Spinal Cord The spinal cord has two major functions: Carrying information: Spinal cord transmit information from body organs and external stimuli to the brain and send information from the brain to other areas of the body Coordinating reflexes: coordinates reflexes without the involvement of the brain, thus, the spinal cord has both communicative and integrative functions. Reflex actions are automatic, unlearned, involuntary, and inborn responses. These actions are sudden in nature and have a purpose of protecting the individual or his organs from sudden danger

Lateral Coticospinal Tract Anterior Corticospinal Tract Somato-sensory Organization Descending Tracts Pyramidal Tracts Lateral Coticospinal Tract Anterior Corticospinal Tract ExtraPyramidal Tracts Rubrospinal Reticulaospinal Olivospinal Vestibulospinal

Somato-sensory Organization Sensory & Ascending Pathways Ascending tracts Dorsal Column Medial Lemniscus Gracile fasciculus Cuneate fasciculus Spinocerebellar Tracts Posteriors pinocerebellar Anterior spinocerebellar Anterolateral System Lateral spinothalmic tract Anterior spinothalmic tract Spino-olivary tract

Somato-sensory Organization

BLOOD SUPPLY SPINAL CORD

Paraplegia & Spinal cord syndromes Paraplegia is an impairment in motor or sensory function of the lower extremities. It is usually caused by spinal cord injury or a congenital condition such as spina bifida that affects the neural elements of the spinal canal. The area of the spinal canal that is affected in paraplegia is either the thoracic, lumbar, or sacral regions. If all four limbs are affected by paralysis, tetraplegia is the proper terminology. If only one limb is affected, the correct term is monoplegia. Spastic paraplegia is a form of paraplegia defined by spasticity of the affected muscles, rather than flaccid paralysis

Paraplegia & Spinal cord syndromes Spinal shock was first defined by Whytt in 1750 as a loss of sensation accompanied by motor paralysis with initial loss but gradual recovery of reflexes, following a spinal cord injury (SCI) – most often a complete transection. Reflexes in the spinal cord caudal to the SCI are depressed (hyporeflexia) or absent (areflexia), while those rostal to the SCI remain unaffected. ‘Shock' in spinal shock does not refer to circulatory collapse, and should not be confused with neurogenic shock, which is life threatening. Phase Time Physical exam finding Underlying physiological event 1 0-1d Areflexia/Hyporeflexia Loss of descending facilitation 2 1-3d Initial reflex return Denervation supersensitivity 3 1-4w Hyperreflexia (initial) Axon-supported synapse growth 4 1-12m Hyperreflexia, Spasticity Soma-supported synapse growth

Paraplegia & Spinal cord syndromes Classification of etiology UMN type/ Spastic paraplegia LMN type/Flaccid paraplegia: UMN type in state of shock-flaccid Cortical lesion Spinal cord lesion Non-compressive Myelopathy Tumor Falx Cerebri Superior Sagital Sinus Thrombosis Compressive Myelopathy

Classification of etiology COMPRESSIVE MYELOPATHY Paraplegia & Spinal cord syndromes Classification of etiology COMPRESSIVE MYELOPATHY EXTRAMEDULLARY INTRAMEDULLARY Syringomyelia, Ependymymoa, Glioma, Astrocytoma EXTRADURAL INTRADURAL DISC VERTEBRAL Meningoma, Neurofibroma, Arachnoditis

Classification of etiology Non-compressive myelopathies Paraplegia & Spinal cord syndromes Classification of etiology Non-compressive myelopathies NONINLAMMATORY INFAMMATORY INFECTIOUS: VIRAL, BACTERIAL ,FUNGAL PARASTIC AUTOIMMUNE: SLE, SJOGREN, SARCOIDOSIS, BECHET S, MCTD DEMYELINATING: MS,NMO, ADEM, POST VIRAL POST VACCINIAL PARANEOPLASTIC INHERITED: HSP, INHERITED METABOLIC DISORDERS METABOLIC: VIT B12,COPPER,FOLATE ,AIDS ASSOCIATED, VIT E DEFICIENCY TOXIC: CASSAVA, LATHYRISM,FLUOROSIS, SMON, NITROUS OXIDE VASCULAR: ANT SPINAL ARTERY THROMBOSIS, AVM, DURAL AV FISTULA

Paraplegia & Spinal cord syndromes Differences between extradural and intradural lesions Extradural Mnemonic – (3 Ps) Pain present - (root pain & spinal tenderness) Pyramidal involvement – early Protein in CSF high Intradural Dissociated anesthesia Bladder involvement early Not so high protein Symmetrical involvement Trophic ulcers common Determining level of lesion in cord compression Sensory level Motor level Reflex level Root pain – dermatome Type of bladder involvement Sensory level – below that level, sensory impairment of loss Motor level – Beevor’s sign indicates T10 lesion Reflex level – Inverted supinator C5 lesion

Paraplegia & Spinal cord syndromes Vascular disorders of spinal cord Ischemic disorders of spinal cord Primary ischemia: atherosclerosis/vasculitis Secondary ischemia: SOL, disorders of aorta Decompression sickness Spinal hemorrhage: SAH, SDH, EDH, hematomyelia Spinal AVM/Dural AV fistula Inflammatory disorders spinal cord Acute TM: viral, bacterial, fungal, post-infectious Myelitis of chronic disorders: MS Myelitis of systemic disorders: Behcet’s Medular compression: Epidural abscess Subdural abscess Spondilodiscitis

Paraplegia & Spinal cord syndromes Non-inflamatory spinal space occupying lesions Disc prolapse Neoplasms Non-spinal disorders Acute poliradiculitis Guillain Barre Hyper/Hypokalemic paralysis Parasigital cortical syndromes: Bilateral infarctions Toxic or allergic disorders of spinal cord Subacute-myelo-optico- neuropahty (SMON) caused by clioquinol Late myelopathy after chemonucleolysis Elsberg phenomena: In cervical myelopathy there is first weakness ipsilateral arm, then ipsilateral leg then contralateral leg and lastly contralateral arm.

Clinical approach to Spinal cord syndromes What is the onset of paraplegia Is it acute within minutes or hours? Is it sub-acute within days or weeks? Is it chronic within months or years? Was there a history of trauma? Fall from a Height? Road traffic accident? Direct injury to spine?

Any wasting or fasciculations? Clinical approach to Spinal cord syndromes Symmetry of symptoms? Is motor weakness symmetrical? Is sensory symptoms symmetrical? Or they are asymmetrical? Any wasting or fasciculations? Anywhere in the body? Small muscles of the hand? Thigh and gluteal muscles?

Clinical approach to Spinal cord syndromes Is there a history of root pains? Is it unilateral or bilateral? Does it radiate to Limbs? Does it aggravate with coughing? Any pyramidal tract involvement? Buckling of knees while walking? Slipping of foot Wear? Tipping on small Objects?

History of vaccinations? Clinical approach to Spinal cord syndromes History of vaccinations? Anti Rabies Vaccination? Polio vaccination? Others? History of increased ICT Fever and headache? Projectile vomiting? Seizures or loss of consciousness?

Clinical approach to Spinal cord syndromes What is the nature of neurological deficit? Is it a? Paraplegia? Tetraplegia? Brain stem lesion? Cerebral diplgia? Consider and exclude Gullian Barre Syndrome

Clinical approach to Spinal cord syndromes What is the mode of onset of paraplegia Acute within days Transverse myelitis Anterior spinal artery syndrome Traumatic paraplegia Sub-acute 2- 6 weeks Pott’s paraplegia Spinal epidural abscess Spinal cord tumors Chronic ˃ 6weeks Familial spastic paraplegia Amyotrophic lateral sclerosis Cranio-vertebral junction anomalies

Legend First-order neuron Lesion Second-order neuron Pain stimulus Third-order neuron Light touch stimulus Sensory impairment Function intact Function lost

Lesion of the right dorsal column at L1 produces what impairment? Damage to the right dorsal column at L1 causes the absence of light touch, vibration, and position sensation in the right leg. Only fasciculus gracilis exists below T6.

Right Dorsal Column Lesion DRG R L L1 Dorsal column lesion Ipsilateral loss of light touch, vibration, and position sense generalized below the lesion level Below T6 only the fasciculus gracilis is present. Common causes include MS, penetrating injuries, and compression from tumors.

R L Lesion of the right fasciculus cuneatus at C3 produces what impairment? Damage to the right fasciculus cuneatus at C3 causes the absence of light touch, vibration, and position sensation in the right arm and upper trunk.

Right Fasciculus Cuneatus Lesion DRG R L C3 Fasciculus cuneatus lesion Ipsilateral loss of light touch, vibration, and position sense In the right arm and upper trunk Common causes include MS, penetrating injuries, and compression from tumors.

R L Lesion of the right lateral corticospinal tract at L1 produces what impairment? Damage to the right lateral corticospinal tract at L1 causes upper motor neurons signs (weakness or paralysis, hyperreflexia, and hypertonia) in the right leg.

Right Lateral Corticospinal Tract Lesion UMN R L L1 Lateral corticospinal tract lesion Ipsilateral upper motor neurons signs generalized below the lesion level UMN signs Weakness (Spastic paralysis) Hyperreflexia (+ Babinski, clonus) Hypertonia Common causes include penetrating injuries, lateral compression from tumors, and MS.

R L Lesion of the right lateral spinothalamic tract at L1 produces what impairment? Damage to the right lateral spinothalamic tract at L1 causes the absence of pain and temperature sensation in the left leg.

Right Lateral Spinothalamic Tract Lesion DRG R L L1 Lateral spinothalamic tract lesion Contralateral loss of pain and temperature sense Common causes include MS, penetrating injuries, and compression from tumors.

Lesion of the anterior gray and white commissures (central cord syndrome) at C5-C6 produces what impairment? R L Damage to the anterior gray and white commissures at C5-C6 causes the absence of pain and temperature sensation in the C5 and C6 dermatomes in both upper extremities.

Central Cord Syndrome C5-C6 R L Impaired pain and temperature DRG DRG Lateral Spinothalamic Tract Common causes include posttraumatic contusion and syringomyelia, and intrinsic spinal cord tumors. Impaired pain and temperature sensation, C5-C6 dermatomes, bilaterally

Complete transection of the right half the spinal cord (Hemicord or Brown-Sequard syndrome) at L1 produces what impairments? R L Damage to the right dorsal columns at L1 causes the absence of light touch, vibration, and position sense in the right leg. Damage to the lateral corticospinal tract causes upper motor neuron signs in the right leg (Monoplegia), and damage to the lateral spinothalamic tract causes the absence of pain and temperature sensation in the left leg.

Hemicord Lesion (Brown-Sequard Syndrome) Common causes include penetrating injuries, lateral compression from tumors, and MS. Dorsal column lesion Ipsilateral loss of light touch, vibration, and position sense Lateral corticospinal tract lesion Ipsilateral upper motor neurons signs Lateral spinothalamic tract lesion Contralateral loss of pain and temperature sense

Hemicord Lesion (Brown-Sequard Syndrome) UMN DRG R L DRG L1 Dorsal column lesion Ipsilateral loss of light touch, vibration, and position sense Lateral corticospinal tract lesion Ipsilateral upper motor neurons signs Lateral spinothalamic tract lesion Contralateral loss of pain and temperature sense Hemicord lesion

Complete transection of the spinal cord (Transverse cord lesion) at L1 would produce what impairments? R L Damage to the dorsal columns, bilaterally, causes the absence of light touch, vibration, and position sense in the both legs. Damage to the lateral corticospinal tracts, bilaterally, cause upper motor neuron signs in the both legs (Paraplegia), and damage to the lateral spinothalamic tracts, bilaterally, cause the absence of pain and temperature sensation in the both legs.

Transverse Cord Lesion Dorsal column lesion Bilateral loss of light touch, vibration, and position sense Common causes include trauma, tumors, transverse myelitis, and MS. Lateral corticospinal tract lesion Bilateral upper motor neurons signs Lateral spinothalamic tract lesion Bilateral loss of pain and temperature sense

Transverse Cord Lesion UMN UMN DRG DRG R L Dorsal column lesion Ipsilateral loss of light touch, vibration, and position sense Lateral corticospinal tract lesion Ipsilateral upper motor neurons signs Lateral spinothalamic tract lesion Contralateral loss of pain and temperature sense Transverse cord lesion

Clinical features anterior, central, Brown-Séquard syndrome Clinical approach to Spinal cord syndromes Clinical features anterior, central, Brown-Séquard syndrome Anterior spinal cord syndrome is usually seen as a result of compression of the ASA. Sensory loss is incomplete. Sensitivity to pain and temperature are lost while sensitivity to vibration and position are preserved. Central cord syndrome is results impairment in the arms and hands and to a lesser extent in the legs. Loss of fine control of movements in the arms and hands, relatively less impairment of leg movements. Loss of bladder control may also occur, as well as painful parethesia. Brown-Séquard syndrome is a loss of sensation and motor function (paralysis and anesthesia) that is caused by the lateral hemisection (cutting) of the spinal cord.

Complete transection of the dorsal columns, bilaterally, (posterior cord syndrome) in the cervical region would produce what impairments? R L Damage to the dorsal columns (fasciculus gracilis and cuneatus), bilaterally, causes the absence of light touch, vibration, and position sense, bilaterally, from the neck down (below the lesion level).

Posterior Cord Syndrome DRG DRG R L Common causes include trauma, compression from posteriorly located tumors, and MS. Dorsal column lesion (bilateral) Bilateral loss of light touch, vibration, and position sense, generalized below lesion level

Complete transection of the lateral corticospinal and lateral spinothalamic tracts with sparing of the dorsal columns, bilaterally, (anterior cord syndrome) in the cervical region would produce what impairments? R L Damage to the lateral corticospinal tracts cause upper motor neuron signs, bilaterally, below the lesion level. Damage to lower motor neurons in the ventral horns cause lower motor neuron signs, bilaterally, at the lesion level. Damage to the lateral spinothalamic tracts cause absence of pain and temperature sensation, bilaterally, below the lesion level. Sparing of the dorsal columns leaves light touch, vibration, and position sense intact throughout.

Anterior Cord Syndrome UMN UMN DRG DRG R L Anterior cord lesion Common causes include anterior spinal artery infarct, trauma, and MS. Lateral corticospinal tract lesion Ipsilateral upper motor neurons signs Lateral spinothalamic tract lesion Contralateral loss of pain and temperature sense

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Paraplegia & Spinal cord syndromes 60 year old female presented with hand clumsiness, worsening handwriting, difficulty with grasping and holding objects, diffuse numbness in the hands, and increasing difficulty with balance and ambulation. Physical examination revealed unsteadiness, broad-based gait, increased muscle tone, weakness and wasting of the upper and lower limbs, and diminished sensation to light touch, temperature, proprioception, and vibration. What is the likely diagnosis? What are imaging features? What is recommended treatment?

Paraplegia & Spinal cord syndromes 72-year-old man NIDDM, dyslipidemia and psoriasis. He was referred for left- side weakness that had been worsening since 6/12 O/E The left pupil was smaller, 3 millimeters reactive to 2. Mild left eye ptosis. Hyperreflexia more on the left than the right. Bilateral planter up going Left hemisensory syndrome. Mildly spastic gait What is diagnosis? What is cause of ptosis? What is treatment of choice?

Paraplegia & Spinal cord syndromes 27-year (F) had a 2-week history of back and lower extremity pain with gradual weakness and paresthesias of the legs, accompanied by urinary retention. This progressed to involve all extremities and inability to stand. No antecedent neurological/respiratory or gastrointestinal illness. Four days prior to the onset of symptoms, she received the attenuated-virus nasal novel influenza A(H1N1) vaccine. Examination revealed Mild proximal muscle weakness UL Mild diminished sensation of the UL Moderate proximal weakness LL Profound sensory loss in the LL. Sensations moderately diminished on the trunk. T4 sensory level. Diffuse hyperreflexia Plantar response was silent and no clonus. What is the diagnosis? List 3 major investigations of choice? What is treatment of choice?

Paraplegia & Spinal cord syndromes 48 year old male presented with sudden onset of: Complete motor paralysis of all 4 limbs Loss of temperature and pain sensation below T2 Retained proprioception & vibratory sensation. Areflexia Flaccid anal sphincter, urinary retention. What is the diagnosis? List 3 causes for this condition? What treatment can be offered?

Paraplegia & Spinal cord syndromes 60 year old lady had H/O recurrent lower limb paralysis. Now presented with quadreparesis. She had for many years suffered from Rheumatoid arthritis and has been on NSAID on and off for years. She had been using artificial tears for grittiness and dryness of eyes. Not known to have DM or HTN. O/E Joint deformities of RA Quadreparesis of UMN below C2 Dysesthesia of both lower extremities and the left trunk, weakness of the left leg, and urinary disturbance. Decreased sensations for touch and pin prick glove and stocking type Bilateral up-going planter response Reflexes present symmetrical in all four limbs What is diagnosis? What is cause for recurrent Paraparesis?

Paraplegia & Spinal cord syndromes 68-year-old woman with a 9-month history of back pain and sensory loss, progressing to the point of loss of bowel and bladder function along with a sudden onset of paraparesis. The pain occurred both at rest and on exercise and at times radiated to lower limbs. O/E Grade IV weakness both LL Sensory deficit to pin prick to D4 Hyper-reflexia knee & ankle jerks Bladder incontinence Bilateral extensor planter response What are imaging abnormalities? What is the cause of paraplegia? What investigations are pertinent?

Paraplegia & Spinal cord syndromes 31 year old man presented history of dorsal pain that had progressed for 4/12. He complained of numbness of both lower limbs and had developed urinary incontinence and constipation. He had bilateral facial weakness a year earlier that had partially improved with steroid therapy. 6/12 ago he had painful redness of eye O/E Decreased sensation to pin prick, light touch, and impaired JPS & vibration to T7 – T8 bilaterally. LL weakness grade IV. Hyperactive tendon reflexes Bilateral up going planters Absent superficial abdominal reflexes CSF: Cells 42 lymphocytes. Protein 1Gm/L Case for discussion

Paraplegia & Spinal cord syndromes A 56-year-old right-handed man with recurrent bilateral uveitis had memory disturbance, constructional and dressing apraxia at age 53. He presented with subacute spastic paraparesis and urinary retention age 56. O/E Hyperreflexia in the lower limbs with bilateral extensor response. Memory disturbance, dressing and constructional apraxia. CSF mild lymphocytic pleocytosis. After intravenous methylprednisolone treatment, clinical symptoms resolved and abnormal intensities with enhancement of the cord disappeared. What is the likely cause of his symptoms? What is the likely cause of his MRI lesion? What is long-term treatment modality?

Paraplegia & Spinal cord syndromes 30 year old lady presented with bilateral loss of vision and weakness of LL in 2010 that resolved in few weeks. 8/12 later she presented with LL weakness and urine incontinence improved with I/V prednisolone significantly. One year later she developed quadrepresis that showed some improvement with steroid + Azothioprin What is the imaging feature in MRI? What is the likely diagnosis? Name two investigations of relevance?