What are the causes of distal muscle weakness (myasthenia)?

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From the Guidelines

Distal muscle weakness is primarily caused by peripheral neuropathies, neuromuscular junction disorders, and certain myopathies, with the most recent and highest quality evidence supporting this multifactorial etiology 1.

Causes of Distal Muscle Weakness

The causes of distal muscle weakness can be broadly categorized into:

  • Peripheral neuropathies, such as diabetic neuropathy, Guillain-Barré syndrome, and chronic inflammatory demyelinating polyneuropathy (CIDP) 1
  • Neuromuscular junction disorders, including myasthenia gravis and Lambert-Eaton myasthenic syndrome, which affect signal transmission between nerves and muscles 1
  • Myopathies, such as inclusion body myositis, distal muscular dystrophies, and myotonic dystrophy, that directly affect muscle strength and function 1

Evaluation and Diagnosis

Evaluation of distal muscle weakness typically involves a thorough neurological examination, electromyography, nerve conduction studies, blood tests, and sometimes genetic testing or muscle biopsy 1.

Treatment

Treatment depends on the underlying cause and may include managing primary conditions, immunosuppressive therapy, physical therapy, or supportive care to maintain function and prevent complications 1.

Additional Considerations

It is essential to consider other potential causes of muscle symptoms, such as statin-associated muscle symptoms, and to evaluate patients for predisposing factors and non-statin etiologies 1. In summary, the management of distal muscle weakness requires a comprehensive approach, considering the various potential causes and underlying conditions, with a focus on maintaining function and preventing complications, as supported by the most recent and highest quality evidence 1.

From the FDA Drug Label

Neurological Increased intracranial pressure with papilledema (pseudo-tumor cerebri) usually after treatment Convulsions Vertigo Headache

The FDA drug label does not answer the question.

From the Research

Causes of Distal Muscle Weakness (Myasthenia)

The causes of distal muscle weakness, also known as myasthenia, can be attributed to various myopathic and genetic conditions. Some of the common causes include:

  • Facioscapulohumeral muscular dystrophy (FSHD) 2
  • Myotonic dystrophy 2
  • Inclusion body myositis 2
  • Myofibrillar myopathy (MFM) 2
  • Distal muscular dystrophies 2, 3
  • Metabolic myopathies 2
  • Congenital myopathies 2

Inherited and Acquired Causes

Distal muscle weakness can be caused by both inherited and acquired conditions. Inherited causes are mostly due to mutations in genes coding for myopathic or neurogenic diseases, while acquired causes occur due to inflammatory, endocrine, or toxic etiologies 4.

Genetic Aspects

Advances in molecular genetics have led to the identification of gene lesions responsible for several distal myopathies, and have expanded our understanding of the genetic relationships of distal myopathies to other inherited disorders of muscle 5, 3.

Clinical Features

The clinical features of distal myopathies can be challenging to diagnose due to the unusual phenotype and significant overlap in clinical features of many of these entities. However, characteristic clinical and histological features can aid in their identification 5, 6.

References

Guideline

Guideline Directed Topic Overview

Dr.Oracle Medical Advisory Board & Editors, 2025

Research

Distal Myopathies: Case Studies.

Neurologic clinics, 2016

Research

Genetics and pathogenesis of distal muscular dystrophies.

Advances in experimental medicine and biology, 2009

Research

Distal myopathies.

Seminars in neurology, 1999

Professional Medical Disclaimer

This information is intended for healthcare professionals. Any medical decision-making should rely on clinical judgment and independently verified information. The content provided herein does not replace professional discretion and should be considered supplementary to established clinical guidelines. Healthcare providers should verify all information against primary literature and current practice standards before application in patient care. Dr.Oracle assumes no liability for clinical decisions based on this content.

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