spastic paraplegia

Introduction

SPINAL CORD DISORDERS• AETIO-PATHOGENESIS & CLINICAL FEATURES

PRESENTED BY DR. AGHO E. JMBBS (AAU)

Outline• Epidemiology• Classification of spinal cord disorders• Pathophysiology

Epidemiology• Epidemiology of spinal cord disorders is perculiar to

specific diseases entity, for example Low back Pain of spinal origin is a common presentation in young Americans below the age of 45yr.

ctn• Cervical spondylotic changes occur naturally with aging, it

appears radiologically in about 90% of the population aged 65 or older.

• Cauda equina syndrome acct for 1-2% of patients with intervertebral disc herniation and occur in 7/100000 per/yr

classification

• Compressive Vs Non Compressive• Acute Vs sub-acute and chronic • Etiology

Classification

Spinal cord disordersMyelopathy due to degenerative and Structural spine Diseases

a) Cervical spondylotic myelopathiesb) Thoracic and lumbar spondylosisc) Syringomyeliad) Hirayama Disease

ctn Vascular myelopathiesa. Spinal cord infarctionb. Spinal dural arteriovenous fistulac. Intramedullary arteriovenous malformationd. Cavernous angiomae. Vasculitisf. Epidural hematomas

ctn

Metabolic and Toxic causes of myelopathy

a. Nutrient deficiency myelopathies•Vitamin B12 •Folic acid•Copper•Vitamin E

ctnb. Toxic myelopathies• Nitrous oxide• Heroin• Konzo (cassava)• Neurolathyrism• Radiation

Myelopathy associated with micro-organismsa. Viruses• HIV 1• Herpes 1&2• VZV• EBV• CMV• Rubella• Mump

a. Bacteria• M. tuberculosis• Treponema pallidum• Listeria monocytogenes• Brucella species

a. Fungi• Aspergillus fumigatum• Cryptococcus neoforma• Candida species• Coccidioides immitis• Blastomyces dermatitidis

a. Parasites• Taenia solium• Toxoplasma gondii• Trypanosoma cruzi• Echinococcus granulosis

Immune-mediated myelopathiesa. Transverse myelitis• Due toMultiple sclerosis (MS)Neuromyelitis optica

Mixed connective tissue disordersSarcoidosisAcute demyelinating encephalomyelitisparaneoplastic

Neoplastic myelopathiesa. Direct involvement of the spinal cord by neoplasm• Intradural intramedullary (parenchymal)i. Primary spinal cord tumorsEpendymomaAstrocytomahemagioblastoma

ctn

ii. Metastatic • Intradural extramedullaryPeripheral nerve sheath tumorMeningiomaSchwannomaneurofibromasLeptomeningeal metastasis

Extradural Extramedullary• metastasis Breast CA, Lung CA, MM• hematoma• TB spine

ctnb. Indirect involvementRadiation injuryChemotherapy injuryParaneoplastic

ctn

Disorders of cauda Equina.Diskogenic Cauda Equina Compression

Cauda equina syndrome

CTN

Nondiskogenic Cauda Equina Disorders

Traumatic Neoplastic

Ctn

Infectious Iatrogenic.Other structural etiologya. Dural arteriovenous fistulab. Dutal ectasiac. Epidural lipomatosis

Pathophysiology• Cervical spondylotic myelopathy; • The development of cervical spondylotic myelopathy is

due to a combination of factors which include external compression from spondylotic canal stenosis, biomechanical stretch and vascular factors.

• Spondylosis refers to age related degenerative changes of the spine

ctn• It begins with dessication of the intervertebral discs,

bulging or herniation of the disc material, • Osteophyte formation along the vertebral endplates, this

combine with the degenerative disc to form osteophytic bars which impinge on the spinal cord.

• Calcification of the posterior longitudinal ligament may also compress the cord ventrally, while ligamentum flava pathology may compromise cord .

Clinical features of spinal cord disorders

• Cervical spondylotic myelopathy Presents with;Progressive gait dysfunctionNeck stiffnessVague sensory changes in lower extremetiesDifficulty performing fine motor functionsProximal limb weakness

ctnParaesthesia of the hands, shoulder, subscapular regionsBladder disturbance; incontinence,retention, Spastic gaitIncreased tone, ankle clonus, hoffman and babinski sign

positive, hyperreflexiaLarge fibre sensory loss if posterior column is affected

with reduced stability

Anterior horn cell involvement manifest as segmental lower motor n. finding

Location Sign and symptoms

Cervical spine

Headache or neck, shoulder, or arm painBreathing difficultiesLoss of sensation in the armsMuscle weakness in the neck, trunk, arms, and handsParalysis involving the neck, trunk, arms, and hands.Lhermites sign

Thoracicspine

Pain in the chest and/or backLoss of sensation below the level of the tumorIncreased sensation above the level of the tumorMuscle weaknessParalysisPositive Babinski reflexBladder and bowel problemsSexual dysfunction

Lumbosacralspine

Low back pain that may radiate down the legs and/or perineal areaWeakness in the legs and feetParalysis in the legs and feetLoss of sensation in the legs and feetBladder and bowel problemsSexual dysfunctionFoot dropDecreased or absent reflexes in the legs

C/FCONUS MEDULLARIS CAUDA EQUINA

Sudden and bilateral onset Gradual and unilateral onset

Radicular pain less prominent

Radicular pain more prominent

More low back pain Less low back pain

Symmetric, distal, hyperreflexicparesis

Asymmetric, areflexic paraplegia

Symmetric, bilateral, typicallyperianal area sensory loss,sensory dissociation occurs

Asymmetric, typically saddlearea, no sensory dissociation

Early sphincteric signs Late sphincteric signs

Management• Investigations• Diagnosis• Treatment.