SPINAL CORD, DISEASES AND DIFFERENTIAL DIAGNOSIS Prof.Dr.Ayse ALTINTAS I.U.CERRAHPASA MEDICAL SCHOOL, NEUROLOGY DEPT. 3RD GRADE, 2011 OCTOBER

MEDULLA SPINALIS The spinal cord, the grayish-white oblong cylindrical continuation of the medulla oblongata of the brain, ***begins at the foramen magnum of the skull and ***extends within the vertebral canal to terminate as the cone-shaped conus medullaris

MEDULLA SPINALIS In the adult the caudal tip of the conus medullaris is located between vertebral levels L1 and L2. Thus, the adult spinal cord is approximately 45 cm in length with an average diameter of 1–1.5 cm

MEDULLA SPINALIS The spinal cord is a two-way conduit to and from the brain. It functions as a “central relay station”, receiving incoming information from the body and the brain, and as a “central processing station”, conveying outgoing information to the body and the brain.

Each spinal nerve leaves the vertebral column via its intervertebral foramen and is named accordingly. The first eight spinal nerves, C1–C8, exit the vertebral canal above the correspondingly numbered cervical vertebrae, whereas all subsequent spinal nerves (T1–T12, L1–L5, S1–S5, and Co1) exit below the correspondingly named vertebrae Hence there are 31 pairs of spinal nerves emerging from the spinal cord. The region of the spinal cord associated with a particular pair of spinal nerves is called a spinal segment.

Lumbar puncture (LP)

VASCULAR SUPPLY OF THE SPINAL CORD ***The anterior spinal arteries,direct branches of the vertebral arteries, join with each other to form a single median vessel, the anterior spinal artery, which provides small branches that penetrate and supply the white and gray matter of the spinal cord.

VASCULAR SUPPLY OF THE SPINAL CORD ***The posterior spinal arteries also arise from the vertebral arteries directly or frequently indirectly by way of the inferior cerebellar branch of the vertebral artery. **penetrate and serve the white and gray matter of the spinal cord.

VASCULAR SUPPLY OF THE SPINAL CORD

SPINAL CORD LESION-LOCATIONS

SPINAL CORD Acute spinal cord dysfunction Can develop over seconds to week a common reason for neurology referral

SPINAL CORD-STRUCTURE The neurologist’s tasks are to determine the etiology, institute treatment to reverse the acute deficit, subsequently determine whether there is a risk of recurrence (especially for inflammatory demyelinating myelopathies) and where necessary, institute prophylactic treatment to prevent recurrence.

SPINAL CORD Acute and subacute myelopathy occurs for numerous reasons and should always be considered a medical emergency. Unfortunately, the diagnosis of myelopathy is often missed at first, leading to missed opportunities for treatment.

SPINAL CORD Any patient who reports, or is found to have a sensory level on exam should be evaluated as a potential myelopathy patient. Also, patients who present with urinary retention should be considered a spinal cord dysfunction patient until proven otherwise. Patients with either a new sensory level or urinary retention should not be discharged from an office or ER to home without complete imaging of the spinal cord with MRI.

SPINAL CORD DISEASES- DIAGNOSTIC ALGORITM

SPINAL CORD AND ROOT COMPRESSION CAUSES: -TUMOURS (PRIMARY, SECONDARY) EXTRADURAL, INTRADURAL, INTRAMEDULLARY INFECTIONS (ACUTE, CHRONIC) DISC DISEASE AND SPONDYLOSIS HAEMATOMA (AVM, SPONTANEOUS, TRAUMA) - CYSTIC LESIONS (EXTRADURAL, INTRADURAL, INTRAMEDULLARY)

SPINAL CORD AND ROOT COMPRESSION LEVEL OF THE LESION: A lesion above the L1 vertebral body may damage both the cord and its roots. Below this, only roots are damaged.

Categories of non-compressive acute myelopathies Disorders that present as acute myelopathy can be grouped as follows: 1. Vascular 2. Demyelinating 3. Infectious 4. Non-infectious inflammatory 5. Cancer related Engelhardt and Coisne Fluids and Barriers of the CNS 2011, 8:4

LOCALIZING A PROCESS AT A SPECIFIC LEVEL IN THE SPINAL CORD Foramen magnum and upper cervical cord: suboccipital pain and neck stiffness, Lhermitte’s symptom, occipital and finger tip paresthesias, syringomyelic-type sensory dissociation, spastic tetraparesis, long tract sensory signs, bladder disturbance, Engelhardt and Coisne Fluids and Barriers of the CNS 2011, 8:4

LOCALIZING A PROCESS AT A SPECIFIC LEVEL IN THE SPINAL CORD

LOCALIZING A PROCESS AT A SPECIFIC LEVEL IN THE SPINAL CORD

APPROACH TO MYELOPATHIES WITH A NORMAL MRI Has a compression been missed? (Epidural lipomatosis, Dynamic compression (flexion-extension studies) • Is it really a myelopathy? (CNS: parasagittal meningioma, venous sinus thrombosis, ACA thrombosis, hydrocephalus, vascular parkinsonism, other EP disorders/ Ganglionopathy: Sjogren’s , B6, paraneoplastic/ Plexopathy: paraneoplastic/ PNS: AIDP, NMJn: MG or LEMS, Muscle: periodic paralysis/ Motor neuronopathy: ALS/ PLS

APPROACH TO MYELOPATHIES WITH A NORMAL MRI • Is it a metabolic/ toxic/ degenerative/ infective myelopathy? • Are the images of adequate quality? (Include contrast?) • Were the images taken too early or too late? • Is the lesion too small to be seen on MRI? • Is there subtle focal or generalized cord atrophy?

APPROACH TO MYELOPATHIES WITH A NORMAL MRI • Is there a history of radiation?, Is there evidence of CSF hypovolemia?, Is there evidence of superficial siderosis? • Is it functional?

SPINAL CORD-SYNDROMES Clinical presentation of spinal cord disorders: The differential diagnosis of an acute myelopathy is dictated by the clinical setting in which the myelopathy occurs, by the specific spinal syndrome, the occurrence of systemic/non-neurological symptoms, Radiographic appearance, Clinical and radiological response to cortico-steroid therapy

SPINAL CORD-SYNDROMES Spinal cord disorders are conventionally classified as ‘syndromes’ due to the typical signs and symptoms produced as a result of the location of lesions and tract involvement.

SPINAL CORD-SYNDROMES For example, Brown-Séquard hemi cord syndrome indicates a structural myelopathy, either compressive or inflammatory and less often neoplastic disorders; it would be rarely seen in vascular, paraneoplastic and metabolic disorders. In contrast, myelopathies with selective tract involvement are characteristic of metabolic, paraneoplastic and degenerative myelopathies, rather than inflammatory or infectious; for example, corticospinal and posterior columns involvement is typical of B12 and

SPINAL CORD-SYNDROMES In contrast, myelopathies with selective tract involvement are characteristic of metabolic, paraneoplastic and degenerative myelopathies, rather than inflammatory or infectious; for example, corticospinal and posterior columns involvement is typical of B12 and copper deficiency, adrenomyeloneuropathy, Friedreich’s ataxia.

SPINAL CORD-SYNDROMES Complete spinal cord syndrome: Description: Bilateral involvement of all tracts Causes: Myelitis (parainfectious/ postvaccinal/ “transverse”), NMO, compression (trauma, tumor, hematoma, abscess), vascular, (rare with MS)

SPINAL CORD-SYNDROMES Brown-Sequard syndrome (often incomplete): Description: Pain and temperature loss contralateral to weakness, ipsilateral spastic weakness and loss of proprioceptive function Causes: compression or intrinsic cord lesion like demyelination

BROWN-SEQUARD SYNDROME

SPINAL CORD-SYNDROMES Central cord syndrome: Description: suspended analgesia, sacral sparing, dissociation of sensory loss, ipsilateral Horner, anterior horn dysfunction Causes: syrinx, intramedullary tumors, hyperextension neck injuries (“man-in-a-barrel”)

CENTRAL CORD LESION

SPINAL CORD-SYNDROMES Posterolateral column disease: Description: foot > hand paresthesias, dorsal column dysfunction, intact pain and temperature, corticospinal dysfunction, associated PN Causes: Cbl/ copper deficiency, HTLV or HIV, extrinsic compression as in spondylotic myelopathy

SPINAL CORD-SYNDROMES Posterior column disease: Description: sensory ataxia, absent reflexes, hypotonic but not weak, hyperextensible joints, trophic changes,crises, decreased deep pain, Lhermitte’s Causes: Tabes

SPINAL CORD-SYNDROMES Anterior horn cell syndrome: Description: weakness, atrophy, fasciculations, reduced tone and reflexes, intact sensations, possible involvement of cranial motor nuclei Causes: spinal muscular atrophy (infantile/ Werdnig-Hoffman, intermediate, juvenile-Kugelberg-Welander, motor neuron disease)

SPINAL CORD-SYNDROMES Combined anterior horn cell and pyramidal tract disease: Description: anterior horn cells and pyramidal tract, sphincteric sparing, asymmetric, cramping, bulbar or pseudobulbar involvement, sensory sparing, preserved superficial abdominals, reflexes variable, rare for extraocular muscles to be involved Causes: ALS

SPINAL CORD-SYNDROMES Anterior spinal artery syndrome: Description: Bilateral corticospinal and spinothalamic involvement without dorsal column, bladder dysfunction Causes: Anterior spinal artery infarct or MS

SPINAL CORD-SYNDROMES Posterior spinal artery syndrome; Description: posterior column dysfunction with loss of segmental reflexes, infarction of unilateral posterior horn and lateral column with sparing of posterior columns may also occur Causes: posterior spinal artery infarct

SPINAL CORD-SYNDROMES

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