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Amyotrophic Lateral Sclerosis: Clinical Features and Diagnosis Amyotrophic Lateral Sclerosis: Clinical Features and Diagnosis
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Introduction Introduction
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Epidemiology Epidemiology
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Clinical Manifestations of ALS Clinical Manifestations of ALS
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Types Types
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Pattern of Progression of ALS Pattern of Progression of ALS
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Prognosis Prognosis
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Additional Symptoms Additional Symptoms
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ALS and Frontotemporal Dementia ALS and Frontotemporal Dementia
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Additional Genetic Subtypes of ALS Additional Genetic Subtypes of ALS
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Atypical Presentations and Manifestations Atypical Presentations and Manifestations
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Mimics of ALS Mimics of ALS
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Diagnosis of ALS Diagnosis of ALS
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Diagnostic Testing Diagnostic Testing
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Electrodiagnostic Testing Electrodiagnostic Testing
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Transcranial Magnetic Stimulation Transcranial Magnetic Stimulation
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Imaging Imaging
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Laboratory Tests Laboratory Tests
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Genetic Testing Genetic Testing
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Treatment Treatment
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References References
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23 Clinical Presentations, Diagnostic Criteria, and Lab Testing
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Published:August 2016
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Abstract
Amyotrophic lateral sclerosis (ALS) is the protypical motor neuron disease, which is characterized by the simultaneous presence of upper motor neuron (UMN) and lower motor neuron (LMN) signs in the same extremity or in the cranial-bulbar region. UMN signs at spinal levels include spasticity, slowness of motor activation, hyperactive deep tendon reflexes and extensor plantar responses, whereas UMN signs at the cranial level include spastic dysarthia (slow, labored, nasal); slowness of tongue movements, and hyperactive jaw, gag, and facial reflexes. LMN signs at the spinal level include muscle atrophy, fasciculations, and weakness and LMN signs at the cranial level include tongue atrophy and weakness, facial weakness, tongue and facial fasciculations, palatal weakness, weak cough, and dysphonia. ALA is fatal in 2 to 4 years, and the only medication known to prolong tracheostomy-free survival
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