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Amyotrophic Lateral Sclerosis

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Presentation on theme: "Amyotrophic Lateral Sclerosis"— 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 First described in publication by Dr. Jean-Martin Charcot, in Paris. 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 % 5600 cases per year in the US, 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 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 Curved MPR: Corticospinal Tract extension
ALS: Imaging Findings Curved MPR: Corticospinal Tract extension

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

12 Bilateral high T2 signal in corticospinal tracts.
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 cases / 100,000 (nml 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 : Kalra, S. et al, Gabapentin Therapy for Amyotrophic Lateral Sclerosis: Lack of Improvement in Neuronal Integrity Shown by MR Spectroscopy AJNR, Mar 2003; 24: Bowen, B. , MR Imaging and Localized Proton Spectroscopy of the Precentral Gyrus in Amyotrophic Lateral Sclerosis AJNR, Apr 2000; 21:

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