Amyotrophic Lateral Sclerosis (ALS) A simple approach; an intuitive presentation José Mª García Santos, MD, PhD. Servicio de Radiología. Hospital Morales Meseguer. Murcia

Normal ALS Charcot, 1874 Spinal cord atrophy Muscular cord atrophy http://upload.wikimedia.org/wikipedia/commons/6/6a/Jean-Martin_Charcot.jpg Muscular cord atrophy

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)

Normal condition

Upper motor neuron Lower motor neuron Disease

Disease

Lateral sclerosis Disease

Muscular atrophy (denervation) Lateral sclerosis Disease

The usual spinal cord onset

Spinal cord onset Lower motor neuron Muscular atrophy Fasciculations (denervation) Fasciculations Weakness Spinal cord onset Lower motor neuron

Hyperreflexia Spinal cord onset Upper motor neuron

Spinal cord onset Upper motor neuron Babinsky Hoffman Clonus Hyperreflexia Spinal cord onset Upper motor neuron

Distal to proximal Spinal cord onset Fasciculations Weakness Muscular atrophy Distal to proximal Spinal cord onset

Symmetry Spinal cord onset

Later on Dysarthria Dysphagia Dysphonia Spinal cord onset

Other clinical presentations

Other clinical presentations Proximal onset Bulbar onset Other clinical presentations Patchy onset

Other clinical presentations Proximal onset Bulbar onset Other clinical presentations Patchy onset Asymmetric onset

Differential diagnosis

Cervical Myelopathy …but also Miastenia gravis Multiple Motor Neuropathy