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Amyotrophic Lateral Sclerosis

Amyotrophic Lateral Sclerosis. Ali Nasim MD Fellow, Neuroradiology Division at UNC. What is ALS?. A-myo-trophic = no-muscle-nourishment Lateral Sclerosis refers to involvement of the lateral corticospinal tracts.

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Amyotrophic Lateral Sclerosis

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  1. Amyotrophic Lateral Sclerosis • Ali Nasim MD • Fellow, Neuroradiology Division at UNC

  2. What is ALS? • A-myo-trophic = no-muscle-nourishment • Lateral Sclerosis refers to involvement of the lateral corticospinal tracts. • ALS is a degeneration of somatic motor neurons extending from upper motor cortical pyramidal neurons to lower motor neurons of the brainstem and cord.

  3. History of ALS • 1869 - First described in publication by Dr. Jean-Martin Charcot, in Paris. • 1881 - Lectures translated into English

  4. ALS: Clinical Findings • Symptoms: • Upper motor - Babinski, spasticity, hyperreflexia. • Lower motor - asymmetric muscle weakness, atrophy, fasciculations • Bulbar signs - dysphagia, slurred speech

  5. ALS: Clinical Findings • Types: • Classic - UMN and LMN • Only UMN or only LMN • Predominantly bulbar form - worse prognosis • Familial - 15-20% • 5600 cases per year in the US, 40-70 y/o, M:F 2:1

  6. ALS: Clinical Findings • Progresses distal to proximal, with complete disability within 10 yrs • 20% of patients survive >5 yrs • Familial and juvenile onset survive 20-30 yrs after diagnosis

  7. ALS: Clinical Findings • Revised El Escorial World Federation of Neurology criteria: • Evidence of LMN degeneration by clinical, electrophysiological, or neuropathological examination • Evidence of UMN degeneration by clinical examination • Progressive spread of symptoms or signs within a region or to other regions (The body is divided into four regions: cranial, cervical, thoracic and lumbosacral) • Absence of electrophysiological, pathological or neuroimaging evidence of other disease processes.

  8. ALS: Imaging Findings • Can have normal imaging • Focal atrophy in chronic cases • T2/FLAIR hyperintensity extending along the corticospinal tract from corona radiata to the brainstem • Contrast enhancement - ? • Deposition of iron in affected cortex

  9. ALS: Imaging Findings Early unilateral (left) ALS involvement in a patient with associated callosal agenesis.

  10. ALS: Imaging Findings Curved MPR: Corticospinal Tract extension

  11. ALS: Imaging Findings Enhancement is atypical but occasionally seen.

  12. ALS: Imaging Findings Bilateral high T2 signal in corticospinal tracts.

  13. ALS: Imaging Findings Increased iron (low T2 signal) deposition in the gray matter of the peri-Rolandic regions which underlying high signal in the white matte and dilatation of the adjacent cortical sulci.

  14. ALS: Imaging Findings FLAIR images shows high signal in the cortico-spinal tracts due to Wallerian degeneration.

  15. ALS: Imaging Findings • MR Spectroscopy: • Decreased NAA/Cr ratio • Increased choline and myoinositol • Decreased glutamate in the precentral gyrus and peri-rolandi white matter

  16. ALS: Pathology • Loss of cortical pyramidal motor neurons and gliosis • Corticospinal tracts with variable patterns of degeneration • Precentral gyrus atrophy

  17. ALS: Pathophyiology • Cause of Spontaneous ALS unknown • Single gene mutations can lead to selective motor neuron loss • Glutamate excitotoxicity (etiology unknown)

  18. ALS: Pathophyiology • Familial ALS: • Copper/Zinc Superoxide dismutase (SOD1)gene mutation found to be associated with 20% of familial ALS • Gain of function mutation

  19. ALS: Interesting Info • Reports of populations with increased incidence, most notably the Chamarro people of Guam (ALS-PCD) • Incidence ranging 140-400 cases / 100,000 (nml 0.5-2 cases/ 100,00) • Recent theory is that this was due to bat consumption and exposure to BMMA excitotoxins.

  20. ALS: Notable People Affected Lou Gehrig Stephen Hawking Jon Stone

  21. ALS: Treatment • Riluzole - glutamate release inhibitor • -Has been shown to increase NAA/Cr ratio • Symptom treatment - ventilation, anti-spastic medications

  22. References • Kalra, S. et al, Neuroimaging in Amyotrophic Lateral Sclerosis. ALS and Other Motor Neuron Disorders. 2003:4 243-248. • Kalra, S. et al, Gabapentin Therapy for Amyotrophic Lateral Sclerosis: Lack of Improvement in Neuronal Integrity Shown by MR Spectroscopy AJNR, Mar 2003; 24: 476 - 480. • Bowen, B. , MR Imaging and Localized Proton Spectroscopy of the Precentral Gyrus in Amyotrophic Lateral Sclerosis AJNR, Apr 2000; 21: 647 - 658. • statDx.com • archives.org • athiestnexus.org • sabine.k12.la.us • muppet.wikia.com

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