Faculty of Medicine Siriraj Hospital Mahidol University The Neurological Society of Thailand February 6th, 2020 Chiangrai Hospital Myelopathy in Systemic Diseases Naraporn Prayoonwiwat, M.D. Neurology Division, Department of Medicine Siriraj Hospital [email protected] 02 419 7101-2 Myelopathy An Approach Scope Essential neuroanatomy ascending tract descending tract vascular supply Approach: differential diagnosis clinical presentation clinical course etiology d NP AscendingMyelopathy tracts LateralAn spinothalamicApproach tract Scope Ventral spinothalamic tract Essential Dorsalneuroanatomy funiculus: Fasciculus cuneatus Fasciculus gracilis P ascendingSpinoreticulothalamic tract tract descendingSpinocerebellar tract tract vascularCuneocerebellar supply tract Approach:Rostrocerebellar differentialtract diagnosis clinical presentation clinical course etiology d NP Brazis: Localization in Clinical Neurology 2017 Myelopathy An Approach Scope Essential neuroanatomy P ascending tract descending tract Sensory vascularafferent fibers supply via dorsal root Unmyelinated fiber (pain, temperature) ascend/descend few Approach:levels to synapse differential in dorsal horn diagnosis (2nd order neurons) axons decussateclinical presentationin anterior white commissure to ventrolateral clinical quadrant course as lateral spinothalamic tract ascends etiology to ventrolateral thalamus d NP Brazis: Localization in Clinical Neurology 2017 Sensory Spinothalamic tract in Tracts dorsolateral Pain and brain stem Temperature Sensation S Pathway L T C Cross midline S via anterior L commissure T Lateral Fibers ascend Spinothalamic 2-3 segments Tract S L Patten: d NP Neurological Differential Diagnosis 1996 Sensory LTC Tracts Tactile Sensation Spinothalamic tract to VPL nucleus of thalamus Cross midline via anterior Ventral white Spinothalamic commissure Tract d NP Myelopathy An Approach Scope Essential neuroanatomy P ascending tract descending tract Myelinated vascular fibers (position, supply vibration) enter dorsal funiculus medially as fasciculus gracilis (sacral > T6) laterally Approach: as fasciculus differential cuneatus (> diagnosis T6) ascend to synapse clinical 2nd order presentation neurons (nucleus gracilis, nucleus cuneatus clinical) in medulla; course decussate and ascend as medial lemniscus etiologyto ventrolateral thalamus d NP Brazis: Localization in Clinical Neurology 2017 Sensory Nucleus gracilis S Nucleus cuneatus Tracts CT L Cross midline Cervical Dorsal Column Sensation Fasciculus cuneatus L S (mid T to C) T Thoracic Fasciculus gracilis (S, L to lower T) LS Lumbar Accurate touch, 2-point discrimination, joint position d NP Sacral Neuroanatomy of Spinal Cord Cross Section: Ascending Tracts Fasciculus gracilis Fasciculus cuneatus S L Th C Dorsal spinocerebellar Th Lateral C L S corticospinal S Ventral L Th spinocerebellar C Lateral Ventral corticospinal spinothalamic d NP Brazis 2001 Myelopathy Descending tracts An Approach Lateral corticospinal tract Scope Ventral corticospinal tract Essential Corticorubrospinalneuroanatomytract ascendingVestibulospinal tract tract Reticulospinal tract P descending tract From 1o vascularmotor, lateral supply premotor and supplementary motor Approach: cortex descend differential through corona diagnosis radiata, posterior limb ofclinical internal capsule presentation, ventral midbrain, pons; 90% decussate clinical at ventral course medulla cross midline to lateral corticospinal etiology tract, 10% continue as ventral cortico- spinal tract to cross at anterior commissure (C, upper T ) d NP Brazis: Localization in Clinical Neurology 2017 CTL Medullary decussation Lateral CS Motor tract 90% Pathway L TC Cervical Ventral CS tract 10% Corticospinal Tract L Cross midline T via anterior commissure Thoracic L d NP Lumbar Neuroanatomy of Spinal Cord Cross Section: Descending Tracts Fasciculus gracilis Fasciculus cuneatus S L Th C Dorsal spinocerebellar Th Lateral C L S corticospinal S Ventral L Th spinocerebellar C Lateral Ventral corticospinal spinothalamic d NP Brazis 2001 Myelopathy Arterial supply An Approach PAnterior spinal artery Scope Anterior sulcal artery Essential neuroanatomyCircumflex artery ascendingPPosterior tract spinal artery descendingRadicular tract artery Radiculomedullary artery P arterial supply Approach: differential diagnosis clinical presentation clinical course etiology d NP Brazis: Localization in Clinical Neurology 2001 Neuroanatomy of Spinal Cord Arterial Supply Posterior spinal arteries Anterior spinal Radiculo arteries medullary artery of Adamkiewicz Radicular artery Aorta d NP Prasad S, Price RS, Karnick SM, et al. Neurology 2007;69:E41 Neuroanatomy of Spinal Cord Arterial Supply Level Feeder C1-C4 Anterior spinal artery, no radiculo- medullary branch C5-T2 2-4 Radicular arteries from vertebral, cervical arteries T3-T8 One radiculomedullary artery, few intercostal arteries T9-S5 Radiculomedullary branches, Adamkewicz artery d NP Brazis: Localization in Clinical Neurology 2017 Vertebral artery From costo-cervical Watershed trunk area Aortic From T5 or T6 arch intercostal artery Adamkewicz artery Spinal cord from left lower arterial intercostal arteries T10, T11 or T12 d NP supply Posterior spinal arteries Anterior sulcal artery Spinal Cord Segmental Arterial Supply Circumflex artery Radiculo- medullary arteries Anterior spinal d NP artery Brazis: Localization in Clinical Neurology 2017 Neuroanatomy of Spinal Cord Arterial Supply Fasciculus gracilis Posterior spinal artery Fasciculus cuneatus Lateral corticospinal Lateral Anterior sulcal artery spinothalamic Anterior spinal artery d NP Brazis: Localization in Clinical Neurology 2017 Neuroanatomy of Spinal Cord Cord-Vertebra Levels C Difference: spinal and vertebral segmentsT SpinalEssentialNo. neuroanatomySpinous process segment Versus Cord segment Cervical 8 0 (matched) Thoracic 12 T6 cord ~ T4 vertebra L Lumbar 5 L3 cord ~ T11 vertebra S J Emer Med Service Sacral 5 S1 cord ~ T12 vertebra 11 Nov2014 Coccygeal 1 conus medullaris ~ L1-2 d NP Brazis: Localization in Clinical Neurology 2001 Myelopathy Differential Diagnosis Differential diagnosis: acute myelopathy versus non myelopathy SignsMyelopathy strongly indicating myelopathy versus non myelopathy : Sensory level on torso : Spinal tract crossed findings (e.g., unilateral pyramidal signs with contralateral spinothalamic findings) : Spinal tract-specific sensory findings (e.g., selective spinothalamic findings with preserved dorsal column findings; suspended band of spinothalamic sensory loss) : Urinary retention Signs consistent with but not diagnostic of myelopathy : Glove-and-stocking sensory loss (consider peripheral neuropathy) : Hyporeflexia/hypotonia (consider peripheral neuropathy) : Unilateral or bilateral upper motor neuron signs (consider brain or brainstem disorders) Signs suggesting alternative diagnosis : Spasms, rather than spasticity (consider stiff-person syndrome) : Paratonic rigidity (consider frontal lobe disorder) : Cognitive impairment (consider frontal lobe or diffuse brain disorder) : Dysarthria and dysphagia (consider brainstem disorder, such as motor neuron disease) d NP Schmalstieg WF, Weinshenker BG. Neurology 2010;75;S2 Myelopathy Differential Diagnosis Extramedullary versus intramedullary Symptoms & Signs Extramedullary Intramedullary Spontaneous pain Radicular pain: lancinating Funicular (central) pain: Dermatome distribution painful dysesthesia Common, early Ill-defined Vertebral pain Common Unusual LMN signs Unusual Widespread with Segmental, if present atrophy, fasciculation UMN signs Early, present Late, present Sensory deficits Ascending progression Descending progression Dissociative sensory loss Perineal sensation Common, marked ‘Sacral sparing’ (saddle area) Sphinctor Late Early with caudal lesion involvement (conus/cauda equina) Trophic changes Unusual Common d NP Brazis PW, Masdeu JC, Biller J. Ed. Localization Clinical Neurology 2017.pp105-32 Spinal Cord Syndromes Complete transection Brown Sequard Central cord Anterior spinal artery d NP Brazis: Localization in Clinical Neurology 2017 Inflammatory:Spinal Post vaccine, Cord Trauma:Syndromes spinal injury (stab) post infectious, MS, NMOSD Neoplasm: intramedullary Trauma: spinal injury, disc tumor (primary/metastatic) herniation Inflammatory: MS, Post Vascular: epidural vaccine, post infectious hematoma (anticoagulant) Neoplasm: tumor, paraneoplastic ✔ ✔ Trauma:Complete hyperextension transection Vascular:Brown Aortic Sequarddissection, Neoplasm: intramedullary vasculitis, aortic tumor atherosclerosis Degenerative/hereditary: Iatrogenic: Post-op spine, syringomyelia, hydromyeliaaorta, thoracic surgery; post-op spinal AVM; decompression injury ✔ Central cord ✔Anterior spinal artery d NP Brazis: Localization in Clinical Neurology 2017 Spinal Cord Syndromes Posterolateral column Posterior column Anterior horn Anterior horn-pyramidal tract d NP Brazis: Localization in Clinical Neurology 2017 Inflammatory:Spinal HIV vacuolar Cord Infection:Syndromes neurosyphilis myelopathy, HTLV-1 (tabes dorsalis) associated (HAM/TSP) Degenerative/hereditary: Toxic: Nitrous oxide myelo- cervical spondylosis neuropathy Vascular: posterior spinal Metabolic: B12, copper artery infarction deficiency-related myeloneuropathy ✔Degenerative/hereditary:Posterolateral column ✔Posterior column Infection:Cervical spondylosis poliomyelitis Iatrogenic: post radiation Degenerative: amyotrophic Infection: postpolio synd lateral sclerosis (ALS) Metabolic: hexosaminidase deficiency Degenerative/hereditary: