Paraparesis biplave nams

approach to paraparesis

1. Approach to paraplegia/ paraparesis Dr Biplave Karki Resident Internal Medicine KUSMS

2. Paraplegia Definition : Impairment in motor function of lower extremities with or without involvement of sensory system. Usually caused by involvement of : cerebral cortex spinal cord nerves supplying muscles of of lower limbs or due to involvement of muscles directly

3. Complete paralysis of both lower limbs is known as paraplegia whereas partial paralysis is known as paraparesis . Both upper limbs +Both lower limbs Quadriplegia Only one limb (usually upper limb) Monoplegia Paralysis of symmetrical parts on both sides of the body, typically affecting the legs more severely than the arms Diplegia

4. Paraplegia Spastic (UMN type) Flaccid (LMN type) Paraplegia in Extension Paraplegia in Flexion

5. Features Paraplegia in extension Paraplegia in flexion Mode of transection Incomplete transection (only corticospinal tract involved ) Complete transection (affects both corticospinal and extrapyramidal tracts) Evolution Early Late Hypertonia more in extensors more in flexors Position of Lower limbs Extended Flexed Deep Reflexes Exaggerated Less exaggerated Clonus Present Absent Flexor withdrawal reflex Not present Present Mass reflex Not present Present Any stimulus below the level of lesion produces : a. Flexor spasm b. Emptying of the bladder and bowel c. Seminal emission

6. Causes of Paraplegia Due to upper motor neuron lesion (spastic paralysis) Intracranial/Cerebral Spinal (myelopathy) Non compressive Compressive Due to lower motor neuron lesion (flaccid paralysis) Anterior horn cells Roots Peripheral nerves Myo -neuronal junction Muscles Functional or hysterical

7. CEREBRAL CAUSES : Causes in parsagittal Region Traumatic : depressed fracture of vault of skull, subdural hematoma Vascular : Superior sagittal sinus thrombosis Inflammatory : Encephalitis, meningocephalitis Neoplasm : Parasagital meningioma Degenerative : Cerebral palsy Causes in Brain Stem Syringobulbia and midline tumors

8. SPINAL CAUSES Compressive Non-compressive myelopathy myelopathy

9. Compressive

10. Non-compressive

11. SUDDEN ONSET CEREBRAL CAUSES SPINAL CAUSES 1. Thrombosis of unpaired anterior cerebral artery 2. Superior Sinus Thrombosis 1. Acute transverse myelitis 2. Injury to spinal cord 3. Thrombosis of anterior spinal artery 4. Haematomyelia 5. Post vaccination 6. Prolapsed intervertebral disc

12. Most Common Causes of Paraplegia 1. Trauma 2. Tumor 3. Tuberculosis 4. Thrombosis 5. Transverse myelitis

13. Lower motor neuron lesion UMN lesion in shock stage sudden onset in neuronal shock , acute transverse myelitis , spinal injury Anterior horn cells Infectious: polio, HIV, HTLV-1 Motor neuron disease

14. Lower motor neuron lesion Disease affecting nerve roots Guillain Barre Syndrome Infection: Tabes dorsalis , HZV, EBV, CMV Cauda equina syndrome Prolapsed intervertebral disc Diabetic amyotrophy Peripheral nerves Peripheral neuritis Disease affecting myoneural junction Myasthenia Gravis Periodic Paralysis due to Hypo or Hyperkalemia

15. Muscles Polymyositis Myopathy , myositis Muscular dystrophy Lower motor neuron lesion

16. APPROACH TO PATIENT WITH PARAPLEGIA History : Ask about Onset : Sudden Trauma ( fracture/dislocation of vertebra ) Infection : epidural abscess, Vascular : thrombosis of ASA, Endarteritis, Transverse myelitis ) Duration of symptom : Short : Traumatic ,Infective causes Long : Neoplastic , Heriditary , Congenital, Demyelinating causes Sensory area involve : Sacral sparing or sacral area involved Radicular (Root) pain : indicates extradural lession Progression : Symmetrical or serial Proximal or distal muscles Weakness is progressive or static

17. Associated symptoms : Fever : Infective causes Seizures Delayed milestone: cerebral cause Various symptoms associated with vitamin deficeincy etc Bladder and Bowel involvement Specific history regarding: preceding illness, Trauma, prior vaccination , involuntary movements In Children : antenatal, natal and post-natal history (maternal infection, birth asphyxia, Hyperbilirubinemia , hospitalization etc) Significant past and family history

18. Examination Complete systemic examination including neurological examination is must It includes Higher mental function status ( affected in cerebral and degenerative disease) Cranial nerve examination ( affected in Brain stem lesion) Tone (increased in UMN and decreased in LMN lesion) DTR ( exaggerated in UMN and absent in LMN lesions and spinal shock )

19. Sensory examination : to assess particular sensory level and to what extent and which sensation is lost Proper examination of skull and spine : to look for any localized tenderness , depressed fracture , deformity etc.

20. Determining the Level of the Lesion Presence of horizontally defined level below which sensory, motor, and autonomic function is impaired is a hallmark of a lesion of the spinal cord. The sensory level is sought by asking the patient to identify a pinprick or cold stimulus applied to the proximal legs and lower trunk and successively moved up toward neck on each side .

21. Sensory loss below this level is due to damage to the spinothalamic tract on opposite side, one to two segments higher in the case of a unilateral spinal cord lesion , and at the level of bilateral lesion. Discrepancy in the level of a unilateral lesion is the result of the course of the second-order sensory fibers, which originate in the dorsal horn, and ascend for one or two levels as they cross anterior to the central canal to join the opposite spinothalamic tract.

22. Lesions that transect descending corticospinal and other motor tracts cause paraplegia or quadriplegia heightened deep tendon reflexes, Babinski signs, and eventual spasticity (the upper motor neuron syndrome). Transverse damage to cord also produces autonomic disturbances consisting of absent sweating below implicated cord level and bladder, bowel, and sexual dysfunction.

23. Uppermost level of spinal cord lesion can also be localized by attention to the segmental signs corresponding to disturbed motor or sensory innervation by an individual cord segment. A band of altered sensation ( hyperalgesia or hyperpathia ) at the upper end of the sensory disturbance, fasciculations or atrophy in muscles innervated by one or several segments, or a muted or absent deep tendon reflex may be noted at this level. These signs also can occur with focal root or peripheral nerve disorders; thus, they are most useful when they occur together with signs of long tract damage

24. With severe and acute transverse lesions, limbs initially may be flaccid rather than spastic. This state of “spinal shock” lasts for several days, rarely for weeks, and may be mistaken for extensive damage to the anterior horn cells over many segments of the cord or for an acute polyneuropathy .

25. Order of compression of the tracts 1 st Pyramidal tract, 2 nd Posterior column, lastly Spinothalamic tract 26

26. Reasons: Pyramidal tract is supplied by terminal branches of spinal arteries and hence most susceptible to compressive ischemia. Pyramidal tract lying closest to denticulate ligament subject to traction in spinal cord compression.

27. Features of transverse damage at each level Cervical Cord quadriplegia and weakness of the diaphragm. C4-C5 produce quadriplegia; C5-C6: loss of power and reflexes in the biceps; C7 weakness affects finger and wrist extensors and triceps; C8, finger and wrist flexion impaired Horner's syndrome- any level Cervical spondylosis never involves C8 so small muscle wasting rules out cervical spondylosis

28. Thoracic cord Nipples (T4) and U mbilicus (T10). Leg weakness and disturbances of bladder and bowel function Lesions at T9-T10 paralyze the lower abdominal muscles, resulting in upward movement of the umbilicus when the abdominal wall contracts ( Beevor's sign )

29. T12-L1 segments: Abdominal reflexes preserved Cremastric lost. Paraplegia Wasting of internal oblique & transverse abdominal muscle.

30. Lumbar and sacral cord L2-L4: Paralyze flexion and adduction of thigh Weakens leg extension at knee Loss of patellar reflex L5-S1: Paralyze movements of foot and ankle Weakens Flexion at knee Weakens extension of thigh Loss of ankle reflex

31. Sacral cord / conus medullaris Tapered termination of spinal cord is conus medullaris and comprises of sacral and single coccygeal segments Syndrome Prominent bladder and bowel dysfunction (urinary retention and incontinence with lax anal tone) and Impotence Bulbocavernous (S2-S4) and anal reflexes (S4-S5) are absent Muscle strength preserved Sensory abnormality precede motor and reflex changes by many months

32. Cauda equina lesion Low back and radicular pain Asymmetric leg weakness and sensory loss Variable areflexia in lower extremities and relative sparing of bowel and bladder function

33. Conus medullaris Cauda equina syndrome Onset Sudden and bilateral Gradual and unilateral Aetiology Intramedullary SOL; glioma PID, metastases Root pain Usually absent Severe low back Motor involvement Not marked Asymmetric limb weakness Sensory loss Bilateral saddle anesthesia Asymmetric sensory loss Bladder and bowel function Early and marked Late and less marked Deep reflexes Knee jerk normal, ankle jerk lost Ankle and knee jerks absent Trophic changes Prominent Less Planter response Extensor Flexor or no response

34. Special Patterns of Spinal Cord Disease 36 Brown- Sequard Hemicord Syndrome Segmental signs, such as radicular pain, muscle atrophy, or loss of a deep tendon reflex, are unilateral. Partial forms are more common than the fully developed syndrome.

35. Central Cord Syndrome arm weakness out of proportion to leg weakness “dissociated” sensory loss loss of pain and temperature sensations over the shoulders, lower neck, and upper trunk (cape distribution), in contrast to preservation of light touch, joint position, and vibration sense in these regions Selective damage to gray matter nerve cells and crossing spinothalamic tracts surrounding central canal

36. Foramen magnum syndrome Lesions interrupt decussating pyramidal tract fibers for legs Classically produces “around the clock” progression of weakness Elsberg’s phenomenon Sub-occipital pain spreading to neck and shoulders Marked imbalance Cerebellar and cranial nerve involvement if they spread intracranially

37. Foramen Magnum Syndrome Elsberg’s phenomenon -U pattern weakness. Ipsilateral upper limb Ipsilateral lower limb Contralateral lower limb Contrallateral upper limb 39

38. Intramedullary and Extramedullary Syndromes Intramedullary processes: Arising within the substance of the cord Extramedullary : that lie outside the cord and compress the spinal cord or its vascular supply. The differentiating features are only relative and serve as clinical guides.

39. Extramedullary lesions: Radicular pain is often prominent early sacral sensory loss and spastic weakness in the legs with incontinence due to the superficial location of the corresponding sensory and motor fibers in the spinothalamic and corticospinal tracts. Intramedullary lesions: Produce poorly localized burning pain rather than radicular pain spare sensation in the perineal and sacral areas (“sacral sparing”), reflecting the laminated configuration of the spinothalamic tract with sacral fibers outermost; corticospinal tract signs appear later.

40. Regarding extra- medullary lesions Extradural lesions are generally malignant and intradural benign ( neurofibroma being a common cause) Consequently , a long duration of symptoms favors an intradural origin.

41. ....

42. Extra- medullary lesions

43. Intradural Extradural (3P) Dissociative anesthesia Bladder involvement early Not so high Symmetrical involvement Trophic ulcers common Pain present Root pain and spinal tenderness Pyramidal involvement early Protein in CSF high Froins syndrome Assymetrical

44. Points, which help in determining level of lesion in spinal cord compression: 1 . Sensory level 2.Motor level 3. Reflex level 4. Root pain-shows dermatome involved. 5. Type of bladder involvement. 6. Autonomic disturbances .

45. Noncompressive acute transverse myelopathy (ATM ) Spinal cord infarction Systemic inflammatory disorders, including SLE and sarcoidosis Demyelinating diseases, including multiple sclerosis (MS) Neuromyelitis optica (NMO) Postinfectious or idiopathic transverse myelitis

46. Transverse myelitis It is of acute onset with total transection of cord. At times it may evolve over a period of several days to weeks Although non-compressive, behaves like compressive Post-infectious occur in recovery phase of disease Post- vaccinal Mid-thoracic region is most common site Fever may present before paralysis develops Early bladder involvement Band like sensation

47. Level of hyperaesthesia Spinal shock stage- acute Flaccid paralysis Loss of sensory ,autonomic feature below the lesion UMN feature develop later 70 % recover within 12 wks ( 3 month ) Other causes Multiple sclerosis HIV Sarcoidosis ACA occlusion (unpaired) Syphilis Radiation myelopathy Transverse myelitis contd..

48. Spinal Cord Infarction paired posterior spinal arteries single anterior spinal artery

49. Acute infarction of the anterior spinal artery produces paraplegia or quadriplegia, dissociated sensory loss affecting pain and temperature sense but sparing vibration and position sense, and loss of sphincter control ("anterior cord syndrome”).

50. Posterior spinal arteries ischaemia ( loss of posterior column function) Loss of tendon reflexes/motor weakness Loss of joint position sense.

51. Inflammatory and Immune Myelopathies ( Myelitis ) Demyelinating conditions MS NMO Post-infectious myelitis Sarcoidosis Systemic autoimmune disease

52. Multiple Sclerosis MS may present with acute myelitis MRI Mild swelling of the cord and diffuse or multifocal “shoddy” areas of abnormal signal on T2-weighted sequences. Contrast enhancement, indicating disruption in the blood-brain barrier associated with inflammation, is present in many acute cases CSF may be normal, but more often mild mononuclear cell pleocytosis , with normal or mildly elevated CSF protein levels; oligoclonal bands. IV methylprednisolone (500 mg qd for 3 days) followed by oral prednisone. Plasma exchange : severe case if glucocorticoids are ineffective

53. Neuromyelitis Optica Immune-mediated demyelinating disorder consisting of a severe myelopathy typically longitudinally extend-three or more vertebral segments . Associated with optic neuritis often bilateral may precede or follow myelitis by weeks or months

54. CSF variable mononuclear pleocytosis ; unlike MS, oligoclonal bands are generally absent. Diagnostic serum auto-antibodies against the water channel protein aquaporin-4 present in 60–70% of patients with NMO. Associated with SLE and antiphospholipid antibodies Rx: Glucocorticoids and, for refractory cases, plasma exchange Neuromyelitis Optica contd..

55. Systemic Immune-Mediated Disorders SLE Sjögren's syndrome Mixed connective tissue disease Behçet's syndrome Vasculitis with perinuclear antineutrophilic cytoplasmic antibodies (p-ANCA), a nd Primary CNS vasculitis

56. Sarcoid Myelopathy Slowly progressive or relapsing disorder Typical CSF profile variable lymphocytic pleocytosis and mildly elevated protein level; in a minority of cases reduced glucose and oligoclonal bands are found Initial treatment is with oral glucocorticoids; immunosuppressant drugs are used for resistant cases.

57. Post-infectious / post- vaccinal Myelitis Postinfectious or postvaccinal , follow an infection or vaccination Epstein-Barr virus (EBV ), Cytomegalovirus (CMV) Mycoplasma Influenza Measles , Mumps, Varicella An autoimmune disorder triggered by infection and is not due to direct infection of the spinal cord Rx: Glucocorticoids or, in fulminant cases, plasma exchange

58. Acute Infectious Myelitis Virus Bacteria Parasites

59. Acute Infectious Myelitis Herpes zoster is the best characterized cause of viral myelitis HSV types 1 and type 2 ,EBV, CMV, and Rabies virus are other well-described causes HSV-2 (and less commonly HSV-1) Causes a distinctive syndrome of recurrent sacral cauda equina neuritis in association with outbreaks of genital herpes ( Elsberg’s syndrome).

60. Bacterial and mycobacterial myelitis (most are essentially abscesses) are less common than viral causes, and much less frequent than cerebral bacterial abscess . Borrelia burgdorferi (Lyme disease), Listeria monocytogenes , Mycobacterium tuberculosis, and Treponema pallidum (syphilis ) Mycoplasma pneumoniae may be a cause of myelitis , but its status is uncertain because many cases are more properly classified as post-infectious .

61. 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, typically Schistosoma mansoni . Toxoplasmosis can occasionally cause a focal myelopathy , and this diagnosis should especially be considered in patients with AIDS

62. In cases of suspected viral myelitis it may be appropriate to begin specific therapy pending laboratory confirmation. Herpes zoster, HSV, and EBV myelitis : I.V Acyclovir (10 mg/kg q8h) or Oral valacyclovir (2 g tid ) for 10–14 days CMV : Ganciclovir (5 mg/kg IV bid) plus F oscarnet (60 mg/kg IV tid ) or cidofovir (5 mg/kg per week for 2 weeks).

63. CHRONIC MYELOPATHIES

64. Spondylitic Myelopathy One of the most common causes of chronic cord compression and of gait difficulty in the elderly Neck and shoulder pain with stiffness, tingling and numbness initially progressed to radicular arm pain and weakness as well as atrophy of muscles

65. Dural arterio -venous (AV) fistulas typical presentation is a middle-aged man with a progressive myelopathy that worsens slowly or intermittently and may have periods of remission, resembling MS Incomplete sensory, motor, and bladder disturbances Dx : Spinal angiography Rx: Endovascular embolization Vascular Malformations of the Cord and Dura

66. Formerly called T ropical spastic paraparesis Insidious onset , slowly progressive spastic syndrome. Approximately half of patients have mild back or leg pain . Vacuolar degeneration of the posterior and lateral tracts, resembling SCD . Dx : ELISA , Western blot analysis Rx: No effective treatment, but symptomatic therapy Retrovirus-Associated Myelopathy

67. Developmental cavity of the cervical cord that is prone to enlarge and produce progressive myelopathy Insidious onset in adolescence or early adulthood, progress irregularly, and may undergo spontaneous arrest for several years Associated with Chiari type 1 malformations Acquired: trauma , myelitis , necrotic spinal cord tumors , and chronic arachnoiditis due to tuberculosis Syringomyelia

68. D issociated sensory loss S ensory deficit-cape distribution. Begin asymmetrically with unilateral sensory loss in the hands. Muscle wasting in the lower neck, shoulders, arms, and hands with asymmetric or absent reflexes in the arms. Spasticity and weakness of the legs, bladder and bowel dysfunction, and a Horner's syndrome appear Syringomyelia

69. Hypo-cupric Myelopathy Virtually identical to sub-acute combined degeneration Low levels of serum copper Low level of serum ceruloplasmin High level of zinc

70. Sub-acute Combined Degeneration Treatable Predominant involvement of the posterior and pyramidal tracts, symmetrical Loss of ankle reflex and Babinski signs important diagnostic clue. Optic atrophy and irritability or other mental changes Macrocytic red blood cells, Low serum B12 concentration, and Elevated serum levels of homocysteine and methylmalonic acid

71. Tabes Dorsalis Loss of reflexes in the legs; Impaired position and vibratory sense; Romberg's sign is present; and, In almost all cases , Bilateral Argyll Robertson pupils

72. Familial Spastic Paraplegia Genetic Progressive spasticity and weakness in the legs, usually but not always, symmetric . Pure motor type no sensory symptoms But sphincter disturbances may be present

73. GBS H/O Preceding Infections Ascending LMN type paralysis Bulbar weakness B/L facial palsy Hypo or areflexia . Sensory intact Bladder involvement rare

74. Hereditary spastic paraplegia Guillain Barre syndrome Lathyrism Amyotropic lateral sclerosis Flurosis Pure motor paraplegia without sensory loss

75. Others Nitrous oxide inhalation NMJ disease-Myasthenia gravis Para- neoplastic myelopathy Other Myopathies

76. Complications of paraplegia Bed sores Contractures Urinary tract infection Pneumonia Deep venous thrombosis

77. References Harrison's Principles of Internal Medicine, 19E Neurology and Neurosurgery Illustrated, 5E Uptodate Thank you