Last reviewed: November 2017
Last updated: September  2017

Summary

Definition

History and exam

Key diagnostic factors

  • presence of risk factors
  • limb weakness
  • dry mouth
  • weakness

Other diagnostic factors

  • dysarthria
  • areflexia
  • ptosis
  • diplopia
  • impotence
  • dysphagia
  • orthostatic hypotension
  • pupillary dilation
  • dyspnoea
  • cerebellar ataxia

Risk factors

  • underlying small cell lung cancer or other malignancy
  • co-existing autoimmune disorder
  • cigarette smoking
  • FHx of autoimmune disease

Diagnostic investigations

1st investigations to order

  • nerve conduction studies
  • low-frequency repetitive nerve stimulation
  • anti-P/Q voltage-gated calcium-channel serology
  • chest CT scan
  • anti-acetylcholine receptor (AChR) serology
Full details

Investigations to consider

  • serial PFTs
  • total-body fluoro-2-deoxyglucose positron emission tomography (FDG-PET) scan
  • bronchoscopy
  • high-frequency or tetanic repetitive nerve stimulation (RNS)
  • single-fibre electromyography
  • HLA haplotyping
  • thyroid-stimulating hormone (TSH)
  • antinuclear antibodies (ANA)
  • rheumatoid factor (RF)
  • antineutrophil cytoplasmic autoantibodies (ANCA)
  • B12 and methylmalonic acid (MMA)
Full details

Emerging tests

  • alpha-1A P/Q voltage-gated calcium-channel subunit antibodies
  • anti-SOX1 antibodies
Full details

Treatment algorithm

Contributors

Authors VIEW ALL

Staff Neurologist

University of Tennessee Erlanger Neurology

Chattanooga

TN

Disclosures

JPA declares that he has no competing interests.

Associate Professor of Neurology

Department of Neurology

Duke University Medical Center

Durham

NC

Disclosures

VCJ declares that he has no competing interests.

Peer reviewers VIEW ALL

Assistant Professor

Medical University of South Carolina

Charleston

SC

Disclosures

DS declares that he has no competing interests.

Associate Professor

Division of Neurology

University of Alberta

Edmonton

Canada

Disclosures

ZS declares that he has no competing interests.

Department of Neurology

Haga Hospital

The Hague

The Netherlands

Disclosures

PW is an author of a number of references cited in this monograph.

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