1 / 22

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.

chione
Download Presentation

Amyotrophic Lateral Sclerosis

An Image/Link below is provided (as is) to download presentation Download Policy: Content on the Website is provided to you AS IS for your information and personal use and may not be sold / licensed / shared on other websites without getting consent from its author. Content is provided to you AS IS for your information and personal use only. Download presentation by click this link. While downloading, if for some reason you are not able to download a presentation, the publisher may have deleted the file from their server. During download, if you can't get a presentation, the file might be deleted by the publisher.

E N D

Presentation Transcript

  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

More Related