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ALS: Clinical Features of Respiratory Muscle Weakness Initially complain of fatigue/shortness of breath triggered by decreasing levels of activity or by lying flat. Often develop disturbed nocturnal sleep with frequent awakenings and excessive daytime sleepiness. ALS: Clinical Features of Axial Muscle Weakness Patients with axial neck weakness complain of posterior neck pain or strain with a gradually worsening tendency for head drop. Patients with axial truncal weakness complain of difficulty maintaining an erect posture when standing and of stooping when walking. Some will support their trunk by placing their hands in their front pants pockets or on their upper thighs. ALS: Other Clinical Features Front temporal executive dysfunction may precede or follow the onset of UMN and LMN dysfunction. Symptoms include changes in personality, impairment of judgment, and development of obsessional behaviors. ALS: Diagnosis The clinical standard for diagnosis is the Revised El Escorial World Federation of Neurology criteria which requires: 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. ALS: Diagnosis ALS is primarily a clinical diagnosis, sensory and motor nerve conduction studies and electromyography (EMG) are a standard part of the evaluation of motor neuron disease. EMG findings in ALS combine features of acute and chronic denervation. Sensory and motor NCS are most often normal in ALS. ALS: Differential Diagnosis Other Motor Neuron Diseases Primary lateral sclerosis (UMN only) Progressive muscular atrophy (LMN only) Progressive bulbar palsy Neuropathies GB, CIDP Myopathies PM, inclusion body myositis