Paraparesis.pptx

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Paraparesis /paraplegia Dr.Abiy F . sep17,2015 Arsi University

Revision 1.which type of brain tumor is common? A.Astrocytoma B.Meningioma C.Glioblastoma multiforme D.Metastatic brain tumor

2.which one is focal( lateralie )sign of brain tumor? A.Aphasia B.personality changes C.headache D.Gait abnormality

3.one of the statement is true about brain tumors. A.The most common source of metastatic brain tumor is melanoma B.Brain metastsasis lesions are always multiple B/c they disseminate hematogenously C.the commonest risk factor for primary brain tumor is genetic factors D. Glioblastoma is the most common malignant primary brain tumor in adults.

4.one of the following statement is false about intracranial pressure. A. pathologic intracranial hypertension (ICH) is present at pressures ≥20 mmHg B.Commonest cause of ICH is pseudotumor cerebri C. Low ICP is caused by diminished CSF volume D.Postural headache is typical for low ICP

Outline of presentation Definition Causes UMN lesion/LMN lesion Diseases of spinal cord DDx Work up

Definition Weakness is a reduction in the power that can be exerted by one or more muscles. Paralysis or the suffix “- plegia ” indicates weakness so severe that a muscle cannot be contracted at all, whereas paresis refers to less severe weakness. The prefix “hemi-” refers to one-half of the body, “ para -” to both legs, “mono-” to one limb and “ quadri -” to all four limbs.

Spinal Shock When the corticospinal pathways are affected by a brain or spinal cord lesion of sudden onset, there may also be a period of flaccidity and areflexia accompanying the paralysis below the level of the lesion. This is the period of “spinal shock,” which sooner or later gives way, in most instances, to the corticospinal syndrome. During the neural shock phase, the plantar responses may be mute and the superficial reflexes absent. The pyramidal, or upper motor neuron, syndrome gradually emerges over hours to weeks with spasticity, hyperactive tendon reflexes, extensor plantar responses, and continued absence of superficial reflexes.

Pattern of weakness Distribution UMN/LMN or Others ( myopathic , psychogenic) Duration Acute, Subacute , or Chronic

Distribution of weakness EMG= electromyelography , NCS=Nerve conduction study

Causes of paraparesis Upper motor neurons An intra spinal lesion at or below the upper thoracic spinal cord level is most commonly responsible Others-especially para-sagittal intracranial lesions Lower motor neurons anterior horn cell disorders cauda equina syndromes and peripheral neuropathies

Disorders affecting the spinal cord … Course Acute – Symptoms develop rapidly & reach peak of severity with in days. Subacute – evolves over a period of 2 to 6 wks Chronic – more than 6 wks elapse b/n the onset & full devt . of the clinical Picture No sharp division between these classes

Acute paraparesis UMN Spinal cord Compressive lesions (particularly epidural tumor, abscess, and hematoma ,disc prolapse and vertebral involvement by malignancy or infection) spinal cord infarction ,an AV-fistula or other vascular anomaly, and transverse myelitis Brain-diseases of the cerebral hemispheres – anterior cerebral artery ischemia, superior sagittal sinus acute hydrocephalus Multiple sclerosis,ADEM LMN Spinal cord a rapidly evolving anterior horn cell disease (such as poliovirus or West Nile virus infection), peripheral neuropathy such as Guillain-Barré syndrome,CIDP Myopathy .

Subacute or chronic paraparesis Upper motor neuron disease Spinal cord chronic spinal cord disease Brain Para- sagittal Meningioma chronic hydrocephalus Rarely-LMN lesion or Myopathy .

Approach to the Patient The first priority is to exclude a treatable compression of the spinal cord Compressive myelopathy : The common causes are tumor, epidural abscess or hematoma, herniated disc, or vertebral pathology Causes warning signs that precede the dev’t of paralysis : - neck or back pain - bladder disturbances - sensory symptoms Spinal subluxation , hemorrhage may not have warning sxs Non compressive myelopathy : - produce myelopathy without antecedent symptoms

Compressive Myelopathies 1- NEOPLASMS Less frequent than tumors of the brain Majority are benign & effect is mainly by compression than by invasion Early recognition is utmost important Relative frequency about: 5% intramedullary , 40 % intradural -extra- medullary , & 55% extra- dural

A- Epidural Neoplasms : Result from metastases to the adjacent spinal bones Any malignant tumor can metastasize to the spinal column Most frequent: breast, lung, prostate, kidney, lymphoma, and plasma cell dyscrasia 60 % occur in the thoracic, 30 % in the lumbosacral , & 10 % in the cervical spine Metastases from prostate & ovarian ca occur disproportionately in the sacral and lumbar vertebrae

Epidural Neoplasm Presentation : Pain is usually the initial Sx in 83- 95 % of pts at the time of dx may be aching and localized or sharp and radiating Typically worsens with movement, coughing, or sneezing and characteristically awakens pts at night. Pain is often worse with recumbency, a feature attributed to distension of the epidural venous plexus. Over time, the pain may develop a radicular quality

Epidural Tumors Motor findings   Weakness in 60 - 85 % of pts at the time of dx Weakness is from CST dysfunction when the lesion is at or above the conus medullaris UMN lesion signs Sensory findings  Are less common than motor findings Ascending numbness and paresthesias

Bladder and bowel dysfunction   A late finding , may be present in as many as one-half of pts Most commonly presents as urinary retention and is rarely the sole symptom of ESCC ( autonomic neuropathy + opiate use) Ataxia  : Spinocerebellar tract dysfunction Investigation: Plain radiographs and radionuclide bone scans : - have only a limited role in dx - they do not identify 15–20% of metastatic vertebral lesions - fail to detect paravertebral masses that reach the epidural space through the intervertebral foramina

MRI is the preferred modality for the initial evaluation of a patient provides excellent anatomic resolution & extent of tumors able to distinguish b/n malignant lesions and other masses—epidural abscess, tuberculoma , or epidural hemorrhage Myelography + CT Roughly equivalent in sensitivity & specificity to MRI

Treatment: - Glucocorticoids to reduce cord edema - Local RXT (initiated as early as possible) to the symptomatic lesion - Specific therapy for the underlying tumor type Surgery - decompression by laminectomy or vertebral body resection - Indications:

B- Intradural mass lesions: Are slow-growing and benign - Intramedullary = 10% ( Glial tumors or 2 ) - Extramedullary = 90% Commonest : Meningiomas and neurofibromas Occasional cases : chordoma , lipoma , dermoid , or sarcoma Spinal cord gliomas are rare, probably because of the relative paucity of glial tissue in the spinal cord

Intradural tumors MRI of a thoracic meningioma MRI of an IM astrocytoma Symptoms: EM: compression of nerve roots, spinal cord or vessels IM: - presents as central or hemicord syndrome - usually in cervical cord - pain is poorly localized &burning - sacral sparing of sensation Management: Surgery (complete resection or debulking ) - The value of adjunctive RXT & CXT is uncertain

Compressive Myelopathy… contd 2- Spinal Epidural Abscess: Abscesses that begin at one level extend to multiple levels Presents as a clinical triad of midline dorsal pain, fever, and progressive limb weakness Aching pain is either over the spine or in a radicular pattern The duration of pain prior to presentation is generally 2 wks but may on occasion be several months or longer Risk factors : - an impaired immune status (DM, RF, alcoholism, HIV, Ca) - intravenous drug abuse, Tatooing , acupancture - infections of the skin or other tissues, trauma

2/3 rd are due to hematogenous spread form the skin, soft tissue, or deep viscera The other arise from direct extension of a local infection to the subdural space… Vertebral OM, decubitus ulcers, Or direct inoculation… LP, epidural anesthesia, or spinal surgery Causative org : S.aureus ( 63%) , GN-bacilli( 16%), Strept ( 9%) , anaerobes(2%), and fungi ( 1%) TBC from an adjacent vertebral source, in the underdeveloped world Mechanism of damage to the spinal cord: • Direct compression • Thrombosis and thrombophlebitis of nearby veins • Interruption of the arterial blood supply • Bacterial toxins and mediators of inflammation

Invest - CBC, ESR , MRI, LP ( if signs of meningoencephalitis ) MRI scans localize the abscess & exclude other causes of myelopathy Treatment : Decompressive laminectomy with debridement + long-term antibiotic Rx ( > 6 wks) Empiric Rx: Vancomycine + 3 rd /4 th cephalosporins + metronidazole Surgical evacuation prevents devt . of paralysis and may improve or reverse paralysis in evolution. it is unlikely to improve deficits of more than several days duration.

Compressive Myelopathies… contd 3- Spinal Epidural Hematoma : Causes acute local/ radicular pain ffed by variable loss of sensory, motor, and bladder and bowel function The source of bleeding is usually venous and Sxs typically present over days, although more abrupt presentations are also described Predisposing conditions: -Therapeutic anticoagulation, trauma, tumor, or blood dyscrasia Rare cases complicate LP or epidural anesthesia, use of LMWH Investigation : - MRI confirm the dx and delineate the extent of the bleeding Treatment : Prompt reversal of clotting disorder and surgical decompression Surgery may be followed by substantial recovery, in pts with some preservation of motor function preoperatively

Noncompressive Myelopathies Acute transverse myelopathies (ATM) - Rapidly progressive - Max deficit in hrs to days The most frequent causes : - Spinal cord infarction - Systemic inflammatory disorders: SLE, Sarcoidosis , Sjogren’s syndrome, Scleroderma, - Demyelinating diseases (MS and NMO) - Postinfectious or idiopathic …presumed to be immune condition - Infectious myelitis (primarily viral)

Transverse Myelitis… contd The inflammation is generally restricted to one or two segments, usually in the thoracic cord Sxs develop rapidly over hrs; occasionally over several weeks Typically the inflammation is bilateral, producing weakness and multimodality sensory disturbance below the level of the lesion Unilateral syndromes have been described as well Leg weakness of varying severity, arm weakness if the lesion is in the cervical cord Diminished sensation, pain and tingling are common Back and radicular pain are also common Bowel and bladder dysfunction also occur

Transverse Myelitis… contd MRI shows gadolinium-enhancing signal abnormality, usually extending over one or more cord segments The cord often appears swollen at these levels. CSF is abnormal in half of pts …↑protein level, moderate lymphocytosis , nl glucose, Other studies can help delineate the underlying cause

Transverse M yelitis… contd Most have at least a partial recovery, which usually begins within 1-3 months Persistent disability occur in about 40 % Significant recovery is unlikely if there is no improve by 3 rd month Rapid onset complete paraplegia & spinal shock are associated with poorer outcomes TM is generally a monophasic illness Parenteral steroid Rx … limited evidence that it alters outcomes

Spinal Cord Infarction: is rare compared with cerebral infarction Spinal cord ischemia can occur at any level Greatest ischemic risk, usually T3-T4, & also at boundary zones between the anterior and posterior spinal artery territories Most frequently caused by pathologies affecting the aorta, particularly aortic dissection Other include any etiology that also produces brain infarction - atherosclerosis, embolism, and hypercoagulable & vasculitic d/o - severe systemic hypotension or cardiac arrest. The onset of sxs is sudden & is frequently associated with back pain Sxs are consistent with the functional loss within the ASA territory Noncompressive Myelopathies

Paralysis, loss of bladder function, & loss of pain & temp sensation Position and vibratory sensation are spared Investigation: MRI demonstrate a T2-signal change consistent with cord ischemia, but may be normal in the first 24 hours Treatment : Supportive and focused on the underlying aortic pathology &/or 2 stroke prevention < ½ of pts show substantial motor recovery Cont…

Immune mediated: Non-compressive myelopathies A- SLE Myelitis : Occurs in a small number of patients with SLE Symptoms of TM may be the initial feature of SLE, the onset usually coincides with other signs of active lupus B- Sarcoid myelopathy Sarcoidosis can affect the SC & produce an acute or subacute segmental myelopathy The lesions can be EM or IM, and can involve the cauda equina as well as the cord MRI of spinal cord C-Other immune-mediated myelitides include: cases associated with - Sjögren's syndrome , - Mixed connective tissue disease , - Behçet's syndrome , - Vasculitis with p-ANCA antibodies

Postinfectious or postvaccinal Myelitis: Follow an infection or vaccination Causes: Numerous organisms EBV,CMV, mycoplasma , influenza, measles, VZV,rubeola & mumps Often begins as the pt appears to be recovering from an AFI, or in the subsequent days or weeks Is an autoimmune disorder and not due to direct infection of the spinal cord An infectious agent cannot be isolated from the NS or spinal fluid Rx - Glucocorticoids - Plasma exchange in fulminant cases Noncompressive Myelopathies… contd

Acute Infectious Myelitis: Infectious in nature rather than postinfectious Causes Viruses most common - HZ, HSV1 & 2, EBV, CMV, and rabies virus - HSV-2 produces recurrent sacral myelitis mimicking MS - Poliomyelitis is the prototypic viral myelitis, restricted to the gray matter of the cord Present as acute viral illness, usually with fever, headache, & meningismus Produces flaccid weakness with reduced or absent DTR & few sensory sxs & sns Noncompressive Myelopathies… contd

Investigation: - MRI often shows hyperintensities in the anterior horns of the SC on T2-weighted imaging - MRI of brain to dx MS - LP: mild protein elevation, modest lymphocytosis - ELISA, VDRL, PCR Treatment: - Begin therapy pending lab confirmation - HZ, HSV, and EBV myelitis Rx with IV Acyclovir (10 mg/kg q8h) or oral Valacyclovir (2 gm tid ) for 10–14 days - CMV with ganciclovir (5 mg/kg IV bid) plus foscarnet (60 mg/kg IV tid ), or cidofovir (5 mg/kg per week for 2 weeks) Acute Infectious Myelitis… contd

Bacterial & Mycobacterial myelitis (most are essentially abscesses) - far less common than viral causes - Any pathogenic species may be responsible - L.monocytogenes , B.burgdorferi (Lyme disease), & TP (syphilis) Parasites : Schistosomiasis ( S.mansoni & haematobium ) .. - Toxoplasmosis cause a focal myelopathy …. considered in patients with AIDS - Acute Infectious Myelitis … contd

Demyelinating Myelopathies MS may present with myelitis NMO consist of a severe myelopathy associated with optic neuritis ON is often bilateral & may precede or follow myelitis by wks or mths NMO is also associated with SLE, APL Ab, and CTD If brain MRI shows inflammatory lesion, it is dxic of MS CSF may be normal - Mild pleocytosis , normal or mildly elevated CSF protein,OCB Rx: Steroids can be tried -Plasma exchange for severe cases Noncompressive Myelopathies… contd

Idiopathic transverse myelitis: No underlying cause can be identified in 1/4 of cases Some will later manifest with SLE or demyelinating disorder Treatment: - Glucocorticoids & plasma exchange Noncompressive Myelopathies… contd

Chronic Myelopathies I - Compressive: 1- Spondylotic myelopathy  Degenerative changes of vertebral bodies, discs, and connecting ligaments encroach on the cervical canal, producing a progressive myelopathy Symptoms begin insidiously, usually with a spastic gait Sensory loss, muscle weakness, and atrophy in the hands cause functional impairment over time Some pts may present with an acute myelopathy cxized by a central cord syndrome, often in the setting of mild neck trauma

Spondylotic myelopathy … contd Pain in the neck, subscapular region, or shoulder, radiating to the arms Numbness or paresthesias in the arms. … The sensory loss may follow a dermatomal pattern Gait disturbance, usually characterized by a spastic, scissoring Reduced joint position & vibratory sense and loss of pain sensation, can be elicited in the lower extremities … contribute to the gait impairment • Bladder dysfunction, urgency, frequency, and/or retention

2- Vascular Malformations of the Cord and Dura AVMs are located posteriorly along the surface of the cord or within the dura Most are at or below the mid-thoracic level Presentation is a middle-aged man with a progressive myelopathy that worsens slowly or intermittently Investigation: - High-resolution MRI with Contrast - Selective spinal angiography(Definitive diagnosis ) Chronic Compressive Myelopathies… contd

3- Syringomyelia is a fluid-filled, gliosis -lined cavity within the spinal cord Most lesions are between C2 and T9 Can descend further down or extend upward into the brainstem Most commonly occurs in Arnold Chiari malformation Type 1 Other causes: Other congenital malformations, Postinfectious , Postinflammatory (TM & MS), neoplasms , Posttraumatic Sxs begin usually at adolescence Mostly in cervical cord Chronic Compressive Myelopathies … contd

Presentation: - CCS with Cape like distribution of pain & temp loss on the back, shoulder and upper limbs - Areflexic weakness in the upper limbs Syringobulbia - palatal or vocal cord paralysis, dysarthria , nystagmus and episodic dizziness. Dx: - MRI identify developmental & acquired syrinx cavities Mx : Surgical …. decompression with fenestration &/or shunt placement …. Recommended for pts with neurologic deterioration or intractable central pain …. Neurologic deficits usually stabilize after intervention and sometimes improve

4- Retrovirus-Associated Myelopathies, HAM Associated with HTLV-I, formerly called tropical spastic paralysis Primarily involves the thoracic cord Inflammation of the lateral CST, SCT, and STT, with relative sparing of the posterior columns Slowly progressive spastic syndrome with variable sensory & bladder disturbance Asymmetric, often lacking a well-defined sensory level Most pts are unable to walk within 10 yrs of onset Chronic Myelopathies… contd

HAM… contd MRI of the SC may show atrophy of the cervical or thoracic cord A brain MRI often shows subcortical , periventricular white matter lesions CSF… mild lymphocytosis &/or elevated protein … Anti-HTLV-I Ab in the CSF with a high CSF/serum ratio … virus culture, PCR Rx - No effective treatment - Symptomatic therapy for spasticity and bladder dysfunction

- 1.Subacute Combined Degeneration (Vitamin B 12 Deficiency) Degeneration of dorsal cord due to defect in myelin formation related to Vit . B12 deficiency Myelopathy tends to be diffuse & signs are generally symmetric Predominant involvement of the posterior and lateral tracts Subacute paresthesias in the hands & feet, loss of vibration & position sensation, and a progressive spastic & ataxic weakness Optic atrophy & irritability or other mental changes in advanced cases Not all pts with neurologic abnormalities will have anemia or macrocytosis Noncompressive chronic myelopathy… contd

Nitrous oxide abuse can also lead to SCD by inactivation of Vit B12 A similar syndrome seen with copper deficiency = HYPOCUPRIC MYELOPATHY Dx: - Macrocytic RBC, ↓ B 12 Conc., ↑serum levels of homocysteine & MMA & in uncertain cases a positive Schilling test Rx - Replacement therapy If untreated, it is progressive Complete recovery if dxed and Rxed early (< 2mth of onset) In established cases only progression is halted Subacute Combined Degeneration … contd

2- Familial Spastic Paraplegia group of inherited neurologic ds , in which the prominent feature is a progressive spastic paraparesis > 28 different causative loci have been identified, including AD, AR and X-linked forms & classified according to the mode of inheritance … the final common pathway for these disorders is a degeneration of the corticospinal tracts Pure Vs complicated Usually but not always symmetrical HSP is a clinical diagnosis, based in large part on the family history Noncompressive chronic myelopathy… contd

3.Tabes dorsalis 4.Multiple sclerosis

Thank you !

Causes of paraplegia-UMN Spinal cord Compressive myelopathy Non compressive myelopathy Hereditary Neurolathyrism Trauma Brain Prasaggital tumors Anterior cerebral artery infarction Saggital sinus thrombosis Hydrocephalus Demylenitang -MS,ADEM,PML

Causes of paraplegia-LMN Peripheral neuropathy GBS,CIDP DSPN,Radiculopathy WNV,poliomyelitis Mononeuritis multiplex Cauda equina syndrome Myopathy & psychogenic NMJ disorders

Investigations for paraplegia patient If there is UMN features Conscious, mental status is normal MRI of spine after localizing the lesion area If depressed mental status MRI of brain LMN features If UMN features with spinal shock Exclude compressive myelopathy by MRI of spine EMG & NCS Lumbar puncture
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