Amyotrophic Lateral Sclerosis Questions & Answers

Updated: Jun 14, 2018
  • Author: Carmel Armon, MD, MSc, MHS; Chief Editor: Nicholas Lorenzo, MD, MHA, CPE  more...
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Questions & Answers

Overview

What is amyotrophic lateral sclerosis (ALS)?

What are the initial lower limb symptoms of amyotrophic lateral sclerosis (ALS)?

What are the initial upper limb symptoms of amyotrophic lateral sclerosis (ALS)?

What are the initial bulbar symptoms of amyotrophic lateral sclerosis (ALS)?

What are the emotional and cognitive symptoms of amyotrophic lateral sclerosis (ALS)?

What are the symptoms of-advanced amyotrophic lateral sclerosis (ALS)?

What are symptoms of progression of bulbar amyotrophic lateral sclerosis (ALS)?

Which anatomy is involved by amyotrophic lateral sclerosis (ALS)?

What is required for a confirmed diagnosis of amyotrophic lateral sclerosis (ALS)?

What is the World Federation of Neurology (WFN) diagnostic algorithm for amyotrophic lateral sclerosis (ALS)?

How is amyotrophic lateral sclerosis (ALS) diagnosed?

What is the role of genetic testing in the diagnosis of amyotrophic lateral sclerosis (ALS)?

What are the American Academy of Neurology recommendations for management of amyotrophic lateral sclerosis (ALS)?

When is invasive ventilatory support indicated in the management of amyotrophic lateral sclerosis (ALS)?

What is amyotrophic lateral sclerosis (ALS)?

What is the prognosis of amyotrophic lateral sclerosis (ALS)?

When was amyotrophic lateral sclerosis (ALS) first described?

What is the cause of amyotrophic lateral sclerosis (ALS)?

What are the degenerative effects of amyotrophic lateral sclerosis (ALS)?

How does amyotrophic lateral sclerosis (ALS) affect lower motor neurons (LMNs)?

What is classic amyotrophic lateral sclerosis (ALS)?

What are progressive muscular atrophy (PMA) and flail limb syndrome in amyotrophic lateral sclerosis (ALS)?

What is primary lateral sclerosis in amyotrophic lateral sclerosis (ALS)?

What is progressive bulbar palsy in amyotrophic lateral sclerosis (ALS)?

What is familial amyotrophic lateral sclerosis (ALS)?

What is the role of genetics in the etiology of amyotrophic lateral sclerosis (ALS)?

What are the complications of amyotrophic lateral sclerosis (ALS)?

How is amyotrophic lateral sclerosis (ALS) diagnosed?

What is the goal of treatment for amyotrophic lateral sclerosis (ALS)?

What are the treatment options for amyotrophic lateral sclerosis (ALS)?

How is amyotrophic lateral sclerosis (ALS) described?

Which mechanisms have been suggested are etiologic factors in amyotrophic lateral sclerosis (ALS)?

What is the role of oxidative stress in the pathophysiology of amyotrophic lateral sclerosis (ALS)?

What is the role of cytoskeletal proteins in the pathophysiology of amyotrophic lateral sclerosis (ALS)?

What is the role of ribonucleic acid (RNA) processing in the pathophysiology of amyotrophic lateral sclerosis (ALS)?

What is the likely pathogenesis of amyotrophic lateral sclerosis (ALS)?

What is the role of misfolded proteins in the pathophysiology of amyotrophic lateral sclerosis (ALS)?

How does the motor axon degenerate in the pathogenesis of amyotrophic lateral sclerosis (ALS)?

What is the role of Wallerian degeneration in the pathogenesis of amyotrophic lateral sclerosis (ALS)?

Which motor neurons are initially spared in the pathophysiology of typical amyotrophic lateral sclerosis (ALS)?

Which factors may mediate cell death in amyotrophic lateral sclerosis (ALS)?

What is the role of the copper/zinc superoxide dismutase 1 (SOD1) gene in the pathophysiology of amyotrophic lateral sclerosis (ALS)?

What is the role of glutamate excitotoxicity in the pathophysiology of amyotrophic lateral sclerosis (ALS)?

What is the role of derangements of ribonucleic acid (RNA) metabolism in the pathophysiology of amyotrophic lateral sclerosis (ALS)?

What is the role of the TAR DNA-binding protein gene (TARDBP) in the pathophysiology of amyotrophic lateral sclerosis (ALS)?

What is the role of the fused in sarcoma/translated in liposarcoma (FUS/TLS) gene in the pathophysiology of amyotrophic lateral sclerosis (ALS)?

Where is the support for a fused in sarcoma/translated in liposarcoma (FUS/TLS) gene etiology of amyotrophic lateral sclerosis (ALS)?

What is the role of a large hexanucleotide repeat expansion in the pathophysiology of amyotrophic lateral sclerosis (ALS)?

What is the distinction between the pathogenesis and pathophysiology of amyotrophic lateral sclerosis (ALS)?

What is the disease progression of amyotrophic lateral sclerosis (ALS)?

What are characteristics of the early stage of amyotrophic lateral sclerosis (ALS) pathophysiology?

Which factors trigger the onset of amyotrophic lateral sclerosis (ALS)?

What is the role of aging on the pathogenesis of amyotrophic lateral sclerosis (ALS)?

What role does misfolded protein play in the pathogenesis of amyotrophic lateral sclerosis (ALS)?

What is the role of non-coding C9ORF72 hexanucleotide expansion in the pathogenesis of amyotrophic lateral sclerosis (ALS)?

What is a focal trigger for the onset of amyotrophic lateral sclerosis (ALS)?

What is the most common cause of amyotrophic lateral sclerosis (ALS)?

What causes familial amyotrophic lateral sclerosis (ALS)?

How many SOD1 mutations have been identified in amyotrophic lateral sclerosis (ALS)?

Which SOD1 mutation causing amyotrophic lateral sclerosis (ALS) is the most common in the US?

What is the role of SOD1 mutations in the etiology of amyotrophic lateral sclerosis (ALS)?

What are the roles of the TAR DNA-binding protein (TARDBP) and the fused in sarcoma (FUS) genes in the etiology of amyotrophic lateral sclerosis (ALS)?

What is the role of the C9orf72 mutation in the etiology of amyotrophic lateral sclerosis (ALS)?

What are less common genetic etiologies of familial amyotrophic lateral sclerosis (ALS)?

What causes sporadic amyotrophic lateral sclerosis (ALS)?

What evidence support the hypothesis that genetic risk factors are present in sporadic amyotrophic lateral sclerosis (ALS)?

What is the role of smoking in the etiology of amyotrophic lateral sclerosis (ALS)?

What is the significance of smoking as an established risk factor for amyotrophic lateral sclerosis (ALS)?

How does focusing on the processes of initiation of amyotrophic lateral sclerosis (ALS) provide new avenues for treatment?

Which factors are unlikely to increase the risk for amyotrophic lateral sclerosis (ALS)?

Which suggested risk factors for amyotrophic lateral sclerosis (ALS) are uncertain?

What is the risk of amyotrophic lateral sclerosis (ALS) in soccer players?

What is the risk of amyotrophic lateral sclerosis (ALS) in US National Football League (NFL) players?

What is the association between head injury and amyotrophic lateral sclerosis (ALS)?

What are possible non-sport-related risk factors for amyotrophic lateral sclerosis (ALS) in American football players?

What causes amyotrophic lateral sclerosis (ALS) in Guam?

What is the annual incidence of amyotrophic lateral sclerosis (ALS) in the US?

What is the lifetime risk for developing amyotrophic lateral sclerosis (ALS)?

What is the global incidence for amyotrophic lateral sclerosis (ALS)?

What are the racial predilections for amyotrophic lateral sclerosis (ALS)?

How does the incidence of amyotrophic lateral sclerosis (ALS) vary by sex?

How does the incidence of amyotrophic lateral sclerosis (ALS) vary by age?

What is the prognosis of amyotrophic lateral sclerosis (ALS)?

How does the progression of amyotrophic lateral sclerosis (ALS) vary?

How is the progression of amyotrophic lateral sclerosis (ALS) measured?

What are the milestone stages and corresponding times of occurrence in amyotrophic lateral sclerosis (ALS)?

At what disease stage are most patients diagnosed with amyotrophic lateral sclerosis (ALS)?

How is progressive muscular atrophy (PMA) distinguished from classic amyotrophic lateral sclerosis (ALS)?

When is individualized prognostic information of amyotrophic lateral sclerosis (ALS) discussed with a patient?

Which education resources are available to patients with amyotrophic lateral sclerosis (ALS)?

When can online patient information about amyotrophic lateral sclerosis (ALS) be found?

Presentation

How is amyotrophic lateral sclerosis (ALS) diagnosed?

When is amyotrophic lateral sclerosis (ALS) suspected?

What are the motor neuron symptoms of amyotrophic lateral sclerosis (ALS)?

What are the initial symptoms of amyotrophic lateral sclerosis (ALS)?

What are the clinical differences between upper and lower limb motor neuron symptom onset in amyotrophic lateral sclerosis (ALS)?

What are the clinical manifestations of amyotrophic lateral sclerosis (ALS) progression?

What are bulbar symptoms of amyotrophic lateral sclerosis (ALS)?

What are the pseudobulbar symptoms of amyotrophic lateral sclerosis (ALS)?

Which motor functions are preserved in amyotrophic lateral sclerosis (ALS)?

What is the focus of family history in suspected amyotrophic lateral sclerosis (ALS)?

What are the signs and symptoms of amyotrophic lateral sclerosis (ALS)?

Which physical findings are characteristic of upper or lower motor neuron (UMN or LMN) dysfunction in amyotrophic lateral sclerosis (ALS)?

Which physical findings reflect upper motor neuron (UMN) dysfunction in amyotrophic lateral sclerosis (ALS)?

Which physical findings reflect lower motor neuron (LMN) dysfunction in amyotrophic lateral sclerosis (ALS)?

What is the key physical finding characteristic of amyotrophic lateral sclerosis (ALS)?

What are the emotional and cognitive symptoms of amyotrophic lateral sclerosis (ALS)?

What are the bulbar physical findings characteristic of amyotrophic lateral sclerosis (ALS)?

How are axial truncal weakness characterized in amyotrophic lateral sclerosis (ALS)?

What is the significance of a suspected amyotrophic lateral sclerosis (ALS) diagnosis?

What are the El Escorial World Federation of Neurology diagnostic criteria for amyotrophic lateral sclerosis (ALS)?

What are the Awaji diagnostic criteria for amyotrophic lateral sclerosis (ALS)?

What are the World Federation of Neurology (WFN) diagnostic criteria for amyotrophic lateral sclerosis (ALS)?

How are the regions of the body defined in the World Federation of Neurology (WFN) criteria for amyotrophic lateral sclerosis (ALS)?

What are the World Federation of Neurology (WFN) categories for the diagnosis of amyotrophic lateral sclerosis (ALS)?

How is the degree of certainty for the diagnosis of amyotrophic lateral sclerosis (ALS) increased?

Why have qualifiers lost their meaning in the diagnosis of amyotrophic lateral sclerosis (ALS)?

How are qualifiers used in the diagnosis of amyotrophic lateral sclerosis (ALS)?

What revisions have been proposed to the WFN diagnostic criteria for amyotrophic lateral sclerosis (ALS)?

DDX

At what disease stage does the presentation of other neurologic conditions overlap with amyotrophic lateral sclerosis (ALS)?

What are the differential diagnoses for amyotrophic lateral sclerosis (ALS) in its early stages?

What are the differential diagnoses for more advanced amyotrophic lateral sclerosis (ALS)?

What are the differential diagnoses for a rapid onset of amyotrophic lateral sclerosis (ALS)?

What conditions may be consider in the differential diagnoses of amyotrophic lateral sclerosis (ALS)?

What are the differential diagnoses for Amyotrophic Lateral Sclerosis?

Workup

What is needed to make a definitive diagnosis of amyotrophic lateral sclerosis (ALS)?

How is the diagnosis of amyotrophic lateral sclerosis (ALS) confirmed?

What is the role of lab tests in the diagnosis of amyotrophic lateral sclerosis (ALS)?

What is the role of biochemical markers in the diagnosis of amyotrophic lateral sclerosis (ALS)?

What is the role of genetic testing in the diagnosis of amyotrophic lateral sclerosis (ALS)?

What is the role of imaging studies in the diagnosis of amyotrophic lateral sclerosis (ALS)?

When is muscle or nerve biopsy indicated in the diagnosis of amyotrophic lateral sclerosis (ALS)?

How are needle electromyography and nerve conduction studies performed in the diagnosis of amyotrophic lateral sclerosis (ALS)?

What is the role of electromyography (EMG) in the diagnosis of amyotrophic lateral sclerosis (ALS)?

Which motor unit recruitment findings indicate amyotrophic lateral sclerosis (ALS)?

Which muscle innervation findings indicate amyotrophic lateral sclerosis (ALS)?

Which denervation findings indicate amyotrophic lateral sclerosis (ALS)?

Which nonspecific EMG findings are common in amyotrophic lateral sclerosis (ALS)?

What is the role of motor and sensory nerve conduction studies in the diagnosis of amyotrophic lateral sclerosis (ALS)?

What are the electrophysiologic findings characteristic of UMN involvement in amyotrophic lateral sclerosis (ALS)?

When are voluntary motor unit action potentials findings suggestive of amyotrophic lateral sclerosis (ALS)?

What is the role of the motor unit number estimate (MUNE) in the diagnosis of amyotrophic lateral sclerosis (ALS)?

Which EMG findings suggest multifocal motor mononeuropathy in amyotrophic lateral sclerosis (ALS)?

Which EMG findings suggest chronic inflammatory demyelinating polyradiculoneuropathy in amyotrophic lateral sclerosis (ALS)?

What EMG findings suggests generalized axonal sensorimotor peripheral neuropathy in amyotrophic lateral sclerosis (ALS)?

Which EMG findings suggest inclusion body myositis in amyotrophic lateral sclerosis (ALS)?

Which EMG findings suggest primary lateral sclerosis or monomelic amyotrophy in amyotrophic lateral sclerosis (ALS)?

What is the role of lab studies in the diagnosis of amyotrophic lateral sclerosis (ALS)?

Which lab tests may be useful in the diagnosis of amyotrophic lateral sclerosis (ALS)?

What is the role of antiacetylcholine receptor antibody and anti-muscle specific kinase (MuSK) antibody assays in the diagnosis of amyotrophic lateral sclerosis (ALS)?

What is the role of urinary 24-hour collections in the diagnosis of amyotrophic lateral sclerosis (ALS)?

When is Lyme disease serology performed in the evaluation of amyotrophic lateral sclerosis (ALS)?

What is the role of genetic testing in the diagnosis of amyotrophic lateral sclerosis (ALS)?

What is the role of MRI in the diagnosis of amyotrophic lateral sclerosis (ALS)?

What is the role of magnetic resonance spectroscopy in the diagnosis of amyotrophic lateral sclerosis (ALS)?

What is the role of CT scanning in the diagnosis of amyotrophic lateral sclerosis (ALS)?

What is the role of PET scanning and functional magnetic resonance imaging (fMRI) in the diagnosis of amyotrophic lateral sclerosis (ALS)?

How does amyotrophic lateral sclerosis (ALS) progress?

How does the pace of amyotrophic lateral sclerosis (ALS) progression vary?

What is the ALS Functional Rating Scale and how is it used in the assessment of amyotrophic lateral sclerosis (ALS)?

How are ALS Functional Rating Scale (ALSFRS) scores interpreted in the assessment of amyotrophic lateral sclerosis (ALS)?

How are pulmonary function tests used to monitor amyotrophic lateral sclerosis (ALS) progression?

Treatment

What is the focus of treatment for amyotrophic lateral sclerosis (ALS)?

What is the role of patient education in the management of amyotrophic lateral sclerosis (ALS)?

What is included in the outpatient care of amyotrophic lateral sclerosis (ALS)?

What is included in the inpatient care of amyotrophic lateral sclerosis (ALS)?

What is the role of riluzole in the management of amyotrophic lateral sclerosis (ALS)?

What is the efficacy of riluzole in the treatment of amyotrophic lateral sclerosis (ALS)?

How does treatment with riluzole affect the prognosis of amyotrophic lateral sclerosis (ALS)?

What are the adverse effects of riluzole in the treatment of amyotrophic lateral sclerosis (ALS)?

What monitoring is needed for patients with amyotrophic lateral sclerosis (ALS) taking riluzole?

What is the role of edaravone (Radicava) in the treatment of amyotrophic lateral sclerosis (ALS)?

Which organization has published clinical treatment guidelines for amyotrophic lateral sclerosis (ALS)?

What are the AAN guidelines for extending life or slowing disease progression in amyotrophic lateral sclerosis (ALS)?

What are the benefits of referral to a multidisciplinary clinic for the treatment of amyotrophic lateral sclerosis (ALS)?

What are the AAN guidelines for use of botulinum toxin in amyotrophic lateral sclerosis (ALS)?

What are the AAN treatment guidelines for pseudobulbar affect in amyotrophic lateral sclerosis (ALS)?

Where can AAN guidelines clinician and patient summaries be found for amyotrophic lateral sclerosis (ALS)?

What are the treatment options for limb stiffness in amyotrophic lateral sclerosis (ALS)?

What are the treatment options for antisialorrhea in amyotrophic lateral sclerosis (ALS)?

What is the efficacy of botulinum toxin type B for the treatment of sialorrhea in amyotrophic lateral sclerosis (ALS)?

What are the options for managing thickened secretions in amyotrophic lateral sclerosis (ALS)?

How is depression treated in amyotrophic lateral sclerosis (ALS)?

How is anxiety treated in amyotrophic lateral sclerosis (ALS)?

What are the causes of pain in amyotrophic lateral sclerosis (ALS) and how can pain be prevented?

How is pain treated in amyotrophic lateral sclerosis (ALS)?

What are the treatment options for cramps in amyotrophic lateral sclerosis (ALS)?

How is incontinence treated in amyotrophic lateral sclerosis (ALS)?

What are the treatment options for sleep difficulties in amyotrophic lateral sclerosis (ALS)?

What causes loss of appetite in amyotrophic lateral sclerosis (ALS)?

How is loss of appetite managed in amyotrophic lateral sclerosis (ALS)?

What are the benefits of noninvasive ventilatory support in the treatment of amyotrophic lateral sclerosis (ALS)?

What is the role of overnight polysomnography in the management of amyotrophic lateral sclerosis (ALS)?

What is the role of invasive ventilatory support in the treatment of amyotrophic lateral sclerosis (ALS)?

What dietary modifications are needed for patients with amyotrophic lateral sclerosis (ALS)?

What is the role of exercise in the management of amyotrophic lateral sclerosis (ALS)?

What are the chief goals of exercise in amyotrophic lateral sclerosis (ALS)?

How do activity restrictions change as amyotrophic lateral sclerosis (ALS) progresses?

What is the role of alternative therapies in the treatment of amyotrophic lateral sclerosis (ALS)?

How is amyotrophic lateral sclerosis (ALS) prevented?

What are the American Academy of Neurology (AAN) recommendations for informing patients of a diagnosis of amyotrophic lateral sclerosis (ALS)?

Where should patients and families be directed for information about amyotrophic lateral sclerosis (ALS)?

What is the SPIKES protocol for delivering the news of an amyotrophic lateral sclerosis (ALS) diagnosis?

What are the advantages to the SPIKES protocol for delivering the news of an amyotrophic lateral sclerosis (ALS) diagnosis?

What is the role of a nonphysician staff member immediately after the patient has been given a diagnosis of amyotrophic lateral sclerosis (ALS)?

What is the role of support groups in the management of amyotrophic lateral sclerosis (ALS)?

What is the role of clinical trials in the management of amyotrophic lateral sclerosis (ALS)?

What is the role of off-label pharmaceuticals in the treatment of amyotrophic lateral sclerosis (ALS)?

What are the US government benefits available for patients with amyotrophic lateral sclerosis (ALS)?

When should end-of-life considerations be addressed in amyotrophic lateral sclerosis (ALS)?

Where can patients with amyotrophic lateral sclerosis (ALS) find social services resources?

What are the benefits of legal consultation for patients with amyotrophic lateral sclerosis (ALS)?

How is the risk of falls reduced in amyotrophic lateral sclerosis (ALS)?

When should patients with amyotrophic lateral sclerosis (ALS) cease driving?

Which specialist consultations may be helpful in the management of amyotrophic lateral sclerosis (ALS)?

What factor may increase the likelihood of consultation with a psychiatrist, psychologist, or counselor for patients with amyotrophic lateral sclerosis (ALS)?

What are the advantages of care at a designated ALS center?

What are the AAN recommendations to maximize the care and quality of life of patients with amyotrophic lateral sclerosis (ALS)?

Medications

Which medications are used to slow decline and extend life in patients with amyotrophic lateral sclerosis (ALS)?

Which medications are used for symptom relief in amyotrophic lateral sclerosis (ALS)?

Which medications in the drug class NMDA Antagonists are used in the treatment of Amyotrophic Lateral Sclerosis?

Which medications in the drug class Alpha2 Adrenergic Agonists are used in the treatment of Amyotrophic Lateral Sclerosis?

Which medications in the drug class Skeletal Muscle Relaxants are used in the treatment of Amyotrophic Lateral Sclerosis?

Which medications in the drug class Neurologics, Other are used in the treatment of Amyotrophic Lateral Sclerosis?