Non-Compressive Myelopathy PDF

Non- compressive myelopathies Dr Kumarangie Vithanage Consultant Neurologist and Senior Lecturer Faculty of Medicine Univeristy of Colombo A 37-year old woman, a mother 2 little children, who is also a teacher by profession presents with sudden onset diffculty in walking over the past 2 days ………… ...

Non- compressive myelopathies Dr Kumarangie Vithanage Consultant Neurologist and Senior Lecturer Faculty of Medicine Univeristy of Colombo A 37-year old woman, a mother 2 little children, who is also a teacher by profession presents with sudden onset diffculty in walking over the past 2 days …………  Define non-compressive myelopathy and differentiate it from compressive spinal cord disorders. Objectives Classify the causes of non-compressive myelopathy. Describe the pathophysiology of common non-compressive myelopathies, such as multiple sclerosis, transverse myelitis, neuromyelitis optica, and vascular myelopathies. Recognize the clinical manifestations of non-compressive myelopathy, including motor weakness, sensory deficits, autonomic dysfunction, and spasticity. Discuss the diagnostic approach to non-compressive myelopathy, including MRI, cerebrospinal fluid (CSF) analysis, and serological tests for autoimmune or infectious cause Identify key differentiating factors between non-compressive myelopathy and compressive lesions based on clinical presentation and imaging findings. Explain treatment strategies for different causes of non-compressive myelopathy, including immunosuppressive therapy, antibiotics for infections, and vascular management. Prognosticate non-compressive myelopathy. Recognize complications of non-compressive myelopathy Non-compressive myelopathies Spinal cord disorders that are not caused by mechanical compression These conditions primarily affect the spinal cord's function through inflammation Ischemia Degeneration metabolic processes Rather than direct physical pressure from structures like a tumor, disc herniation, or bone abnormalities.  Non-compressive vs compressive myelopathies Compressive: External physical structures cause direct mechanical pressure on the spinal cord. Non-Compressive: Pathological changes within the spinal cord itself (e.g., inflammation, infection). Non-compressive myelopathies Inflammatory/Autoimmune Myelopathies Vascular Myelopathies Infectious Myelopathies Metabolic and Nutritional Myelopathies Degenerative Myelopathies Toxic Myelopathies  Inflammatory/ MS NMOSD Autoimmune Sarcoidosis SLE, etc Spinal cord AVM Vascular Spinal cord infarction Viral Non- Infectious compressive Bacterial Metabolic and myelopathies Nutritional B12 / Cu Fungal deficiency Classification HSP Degenerative ALS Chemotherapy Toxic Radiation Paraneoplastic Inflammatory/Autoimmune myelopathies MS NMOSD SLE Sarcoidosis Other CTD Vascular Myelopathies Spinal Cord Infarction (Ischemic Myelopathy): Ischemia or lack of blood flow to the spinal cord, often resulting from arterial occlusion, hypotension, or aortic dissection. Spinal Arteriovenous Malformation (AVM): A vascular anomaly that can lead to bleeding or ischemia within the spinal cord. Spinal Dural Arteriovenous Fistula (DAVF): Abnormal connections between arteries and veins within the spinal cord's dural layers, leading to ischemia. Infectious Myelopathies Viral Myelitis: Infections caused by viruses like herpes simplex virus (HSV), varicella-zoster virus (VZV), human T-lymphotropic virus (HTLV-1), HIV, or enteroviruses can cause spinal cord inflammation. Bacterial Infections: Infections like tuberculosis (TB) or syphilis can affect the spinal cord. Fungal and Parasitic Infections: Rarely, organisms like fungi (e.g., cryptococcus) or parasites can invade the spinal cord. Metabolic and Nutritional Myelopathies Vitamin B12 Deficiency (Subacute Combined Degeneration): Causes degeneration of the posterior and lateral columns of the spinal cord, leading to sensory ataxia and weakness. Copper Deficiency Myelopathy: A condition that mimics subacute combined degeneration but is caused by copper deficiency. Folate Deficiency: Can contribute to myelopathy, though less common than B12 deficiency. Thyroid Disorders: Severe hypothyroidism can rarely cause myelopathy. Degenerative Myelopathies Hereditary Spastic Paraplegia (HSP): A group of inherited disorders that cause progressive weakness and stiffness of the legs due to degeneration of the corticospinal tracts. Amyotrophic Lateral Sclerosis (ALS): While primarily a motor neuron disease, ALS can affect the spinal cord, leading to spasticity and weakness. Neoplastic Myelopathies (Non-compressive) Paraneoplastic Myelopathy: An autoimmune condition associated with cancer, where antibodies attack the spinal cord, causing myelopathy without direct compression by the tumor Radiation Myelopathy: Damage to the spinal cord following radiation therapy for tumors near the spine, leading to delayed radiation-induced myelopathy. Toxic Myelopathies Toxicity from Substances: Certain medications (e.g., chemotherapeutic agents like cisplatin), heavy metals (e.g., lead or mercury), or other toxins  Inflammatory/ MS NMOSD Autoimmune Sarcoidosis SLE, etc Spinal cord AVM Vascular Spinal cord infarction Viral Non- Infectious compressive Bacterial Metabolic and myelopathies Nutritional B12 / Cu Fungal deficiency Classification HSP Degenerative ALS Chemotherapy Toxic Radiation Paraneoplastic Pathophysiology of Common Non-Compressive Myelopathies  An immune-mediated inflammatory disease Attacks myelinated axons in CNS destroying the myelin and the axon Multiple sclerosis Hallmark of MS is symptomatic episodes that occur months or years apart and affect different anatomic locations. Microscopy: myelin loss, destruction of oligodendrocytes, and reactive astrogliosis, often with relative sparing of the axon cylinder. (In some axon is also aggressively destroyed)  Location of CNS lesions dictate the type of clinical deficit Course: As neural inflammation resolves in MS, some remyelination occurs, ?plasticity. Multiple sclerosis MS is also characterized by perivenular infiltration of lymphocytes and macrophages, Infiltration of inflammatory cells occurs in the parenchyma of the brain, brainstem, optic nerves, and spinal cord. Multiple sclerosis In pathologic specimens, the demyelinating lesions, called plaques , appear as indurated areas—hence the term sclerosis. Demyelination in multiple sclerosis. Luxol fast blue (LFB)/stain confers an intense blue to myelin. Loss of myelin is demonstrated in this chronic plaque. Multiple sclerosis In pathologic specimens, the demyelinating lesions, called plaques , appear as indurated areas—hence the term sclerosis. Inflammation in multiple sclerosis. Hematoxylin and eosin (H&E) stain shows perivascular infiltration of inflammatory cells. These infiltrates are composed of activated T cells, B cells, and macrophages. Multiple sclerosis - Pathophysiology Multiple sclerosis Multifocal inflammatory demyelination in the CNS - disseminated in time and space Relative contribution of pathological processes across 3 phases of the clinical course of MS  Neuromyelitis optica spectrum disorders (NMOSD) Autuimmunity is IgG autoantibodies to aquaporin 4 (anti-AQP4), the most abundant water channel protein in CNS AQP4 is found in astrocytes NMOSD involving AQP4-IgG can be considered an astrocytopathy or autoimmune astrocytic channelopathy, since the astrocytes are semi-selectively destroyed Neuromyelitis optica An astrocytopathy caused by autoantibodies directed against aquaporin-4 (AQP4) spectrum Typically causes recurrent optic neuritis and disorders longitudinally extensive transverse myelitis (LETM) (NMOSD) Disability is related to relapses Neuromyelitis Brain MRI usually non-diagnostic for MS AQP4-IgG detected in serum and CSF optica Treatment used in MS (IFN, fingolimod) may spectrum worsen NMOSD, hence, differentiation is important disorders Treatment (NMOSD) Acute relapse: IVMP and PLEX Relapse prevention: immunosuppressant drugs Neuroimaging Vascular myelopathy Vitamin B12 deficiency Subacute combined degeneration of the cord (SACD) Exaggerated knee jerks Absent ankle jerks Extensor plantar responses Stocking sensory loss Loss of proprioception (JPS) Which structures of the nervous system are involved? Vitamin B12 deficiency  Define non-compressive myelopathy and differentiate it from compressive spinal cord disorders. Classify the causes of non-compressive myelopathy. Describe the pathophysiology of common non-compressive Objectives myelopathies, such as multiple sclerosis, transverse myelitis, neuromyelitis optica, and vascular myelopathies. Recognize the clinical manifestations of non-compressive myelopathy, including motor weakness, sensory deficits, autonomic dysfunction, and spasticity. Discuss the diagnostic approach to non-compressive myelopathy, including MRI, cerebrospinal fluid (CSF) analysis, and serological tests for autoimmune or infectious cause Identify key differentiating factors between non-compressive myelopathy and compressive lesions based on clinical presentation and imaging findings. Explain treatment strategies for different causes of non-compressive myelopathy, including immunosuppressive therapy, antibiotics for infections, and vascular management. Prognosticate non-compressive myelopathy. Recognize complications of non-compressive myelopathy Clinical manifestations of non-compressive myelopathy Motor dysfunction - Sensory dysfucntion - weakness, mobilty, dysaesthesia, mobililty balance and balance Autonomic dysfunction – bladder/ bowel Spasticity dysfunction Diagnostic Approach History Examination Investigations MRI of the Spine: Key to identifying non-compressive myelopathies. It reveals inflammatory changes or demyelination without mechanical compression. Cerebrospinal Fluid (CSF) Analysis: Look for oligoclonal bands in MS, increased white cells in infections, or specific markers in NMO. Serological Testing: NMO-IgG antibody testing (for NMO). Autoimmune panels, infectious serologies (e.g., HTLV, HIV), and vasculitic markers. Diagnostic Approach MRI of the Spine: Key to identifying non-compressive myelopathies. It reveals inflammatory changes or demyelination without mechanical compression. Cerebrospinal Fluid (CSF) Analysis: Look for oligoclonal bands in MS, increased white cells in infections, or specific markers in NMO. Serological Testing: NMO-IgG antibody testing (for NMO). Autoimmune panels, infectious serologies (e.g., HTLV, HIV), and vasculitic markers. HIV, VDRL Vitamin B12 levels, FBC, Blood picture Neurophysiology  CSF examination Virus screen for TM MRI brain if MS suspected AQP4-IgG if NMOSD suspected Vasculitis screen: ESR, CRP, ANA HIV, VDRL Vitamin B12 levels, FBC, blood picture Neurophysiology Differentiating Non-Compressive from Compressive Myelopathy Clinical Features: Compressive: Typically progressive with focal findings (e.g., radiculopathy). Non-compressive: More acute or subacute onset with diffuse motor and sensory deficits. Imaging: Compressive: Evidence of mass effect or direct spinal cord compression on MRI. Non-compressive: Inflammation or demyelination without any structural compression. Treatment Strategies Inflammatory Causes: Immunosuppressive therapy (e.g., corticosteroids, IVIG, plasma exchange for MS and NMO) Infectious Causes: Appropriate antimicrobial treatment (e.g., antituberculous treatment for TB, antibiotics for bacterial causes) Vascular Causes: Managing the underlying vascular risk factors (e.g., anticoagulation for embolic causes or addressing hypotension in spinal cord ischemia). Prognosis of Non-Compressive Myelopathy Depends on the etiology: MS and NMO: Chronic but manageable with disease- modifying therapies. Transverse Myelitis: Variable recovery depending on severity and promptness of treatment. Spinal Cord Infarction: Often poor prognosis due to the extensive nature of infarction. Complications Chronic Spasticity Pressure sores (due to immobility in severe cases) Autonomic Dysreflexia (in patients with high spinal cord lesions) Neurogenic bladder or bowel dysfunction leading to recurrent infections or renal complications. DVT Psychological/ Financial A 37-yrar old woman, a mother of 2 little children, who is also a teacher by profession presnts with sudden onset diffculty in walking over the past 2 days ………… Diagnostic approach…….. History Onset and duration Disability Bladder/ bowel involvement ? Likely aetiology Inflamamtory/ AI Infective Vascular Toxic Metabolic Paraneiplastic Complications Degree of disability / falls.. Psychologcal/ financial status Diagnostic General examination approach…….. Examination  Neurological examination Diagnostic CN…. Specially ……. approach…….. UL Examination LL A 37-year old woman, a mother of 2 little children, who is also a teacher by profession presents with sudden onset diffculty in walking over the past 2 days. She denied any preceding history of an infection, no known neurological ailmets previously O/E patient is in spinal shock with upward plantars and AROU. She is hemodynamically stable.  FBC, blood picture, CRP, … etc etc…… Normal Vasculitic screen – normal CSF……. L 30, P 87 mg/dl, Increased CSF/Serum IgG index OCB - negative Anto AQP4 ab - negative Anti MOG ab - negative VEP – normal Management Acute treatment - MPP 1g/d for 5 days Oral steroid taper over 6 months Medical rehabilitation Bladder/ bowel care DVT prophylaxis Nutrition/ hydration Limb physiotherapy Psychological care In summary …………………………. Always first: ??? a compressive lesion Identify the cause for the non-compressive myelopathy Specific treatment is aimed at the underlying cause Needs long-term rehabilitation MCQ - SBA A 76-year-old man presented with difficulty in walking for 7 months. On examination he had spastic paraparesis with extensor plantar responses. No sensory loss. His proximal upper limb muscles were wasted and weak with absent biceps and triceps jerks. He had diffused tongue fasciculations. What is the most likely cause for his gait difficulty? A. Amyotrophic lateral sclerosis B. Cervical myelopathy C. Transverse myelitis D. Vitamin B12 deficiency E. Multiple sclerosis