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Amyotrophic Lateral Sclerosis (ALS)
A simple approach; an intuitive presentation José Mª García Santos, MD, PhD. Servicio de Radiología. Hospital Morales Meseguer. Murcia
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Normal ALS Charcot, 1874 Spinal cord atrophy Muscular cord atrophy
Muscular cord atrophy
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Piramidal tract degeneration (in the adult)
Secondary degeneration Primary degeneration Wallerian degeneration Motor neuron disease Amyotrophic Lateral Sclerosis (lower and upper motor neuron disease) When a nerve fiber is cut or crushed, in which the part of the axon separated from the neuron's cell body degenerates Primary Lateral Sclerosis (only upper motor neuron disease) Progressive Spinal Atrophy (only lower motor neuron disease)
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Normal condition
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Upper motor neuron Lower motor neuron Disease
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Disease
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Lateral sclerosis Disease
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Muscular atrophy (denervation) Lateral sclerosis Disease
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The usual spinal cord onset
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Spinal cord onset Lower motor neuron Muscular atrophy Fasciculations
(denervation) Fasciculations Weakness Spinal cord onset Lower motor neuron
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Hyperreflexia Spinal cord onset Upper motor neuron
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Spinal cord onset Upper motor neuron Babinsky Hoffman Clonus
Hyperreflexia Spinal cord onset Upper motor neuron
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Distal to proximal Spinal cord onset Fasciculations Weakness
Muscular atrophy Distal to proximal Spinal cord onset
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Symmetry Spinal cord onset
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Later on Dysarthria Dysphagia Dysphonia Spinal cord onset
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Other clinical presentations
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Other clinical presentations
Proximal onset Bulbar onset Other clinical presentations Patchy onset
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Other clinical presentations
Proximal onset Bulbar onset Other clinical presentations Patchy onset Asymmetric onset
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Differential diagnosis
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Cervical Myelopathy …but also Miastenia gravis
Multiple Motor Neuropathy
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