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Focal Muscular Atrophies Treatment & Management

  • Author: Sridharan Ramaratnam, MBBS, MD; Chief Editor: Helmi L Lutsep, MD  more...
Updated: Oct 21, 2015

Medical Care

Treatment of focal muscular atrophy (FMA) varies according to the cause.

  • The common causes (eg, monomelic amyotrophy, PPMA, SMA) have no specific treatment.
  • When patients with these conditions have disability, the treatment consists of physical and occupational therapy and rehabilitation.
  • A recent report of increase in the SMN2 messenger RNA levels in vivo among 7 of 13 patients with spinal muscular atrophy treated with valproic acid raises possibilities of in vivo activation of causative genes in inherited diseases.[54]
  • An open trial of clenbuterol among patients with spinal and bulbar muscular atrophy (SBMA) found significant and sustained increase in walking distance covered in 6 minutes and forced vital capacity between the baseline and the 12-month assessments (P < .001), suggesting class IV evidence that clenbuterol may be effective in improving motor function.[55]
  • A randomized trial found no significant effect of dutasteride on the progression of muscle weakness in SBMA.[56]
  • Counsel patients concerning the benign nature of the illness once the diagnosis is confirmed.
  • Treatment of PPMA
    • Trials with amantadine, high-dose steroids, human growth hormone, pyridostigmine[57] , modafanil[58] and bromocriptine all have been disappointing.
    • In a study of subcutaneous insulinlike growth factor-1 in 22 patients with PPMA, patients had enhanced recovery after fatiguing exercise. However, the treatment had no impact upon strength or exercise-induced fatigue.
    • Intravenous immunoglobulin probably has no beneficial effect on activity limitations but may have modest beneficial effect on muscle strength and pain.[59, 60, 61, 62, 63]
    • One trial with weak methods found that lamotrigine might be effective in reducing pain and fatigue, resulting in fewer activity limitations. Data from 2 single trials suggest that muscle strengthening of thumb muscles (very low-quality evidence) and static magnetic fields (moderate-quality evidence) are beneficial for improving muscle strength and pain, respectively, with unknown effects on activity limitations. These interventions, however, need further investigation.
    • Screening and treating patients for osteopenia or osteoporosis may be appropriate.
  • Treatment of multifocal motor neuropathy
    • IV immunoglobulins are effective and commonly used for treating patients with multifocal motor neuropathy.
    • Either high-dose cyclophosphamide or monthly plasma exchange followed by pulse IV cyclophosphamide has been found effective in patients who do not respond to IV immunoglobulins. These patients do not respond to prednisone or plasmapheresis alone.
    • Whether the presence of anti-GM1 antibody or its titer has any bearing on the response to therapy is controversial.
  • Inclusion body myositis does not respond well to immunosuppressive medication.
  • Immunosuppressive treatment with corticosteroids may benefit focal myositis and sarcoid myopathy.

Surgical Care

Surgery has no role in FMA, except in rare instances in which FMA is secondary to a surgically treatable intraspinal or extraspinal lesion.

A randomized trial of 2 surgical techniques, namely anterior cervical discectomy decompression with autologous iliac crest bone grafting and internal plate fixation (DDF) or anterior cervical corpectomy, posterior longitudinal ligament resection, autologous iliac crest bone grafting, and internal plate fixation (CDF), for patients with monomelic amyotrophy followed up for 2 years revealed subjective and electrophysiological improvement in 60-65% of patients. Because of the benign nature of the illness, cervical collar treatment is the preferred treatment, while surgery could be an exceptional second-line alternative. If surgery is chosen, DDF carries lower procedural risk compared with CDF.[64]



See the list below:

  • When the diagnosis is uncertain, referral to a tertiary care center with expertise in neuromuscular disorders may be appropriate.
  • Physical and occupational therapy
  • Vocational rehabilitation training, when appropriate
Contributor Information and Disclosures

Sridharan Ramaratnam, MBBS, MD Director and Senior Consultant, Institute of Neurological Scienes, SRM Institute for Medical Sciences, India

Sridharan Ramaratnam, MBBS, MD is a member of the following medical societies: American Academy of Neurology, American Epilepsy Society, Royal College of Physicians and Surgeons of Glasgow, Indian Medical Association, National Academy of Medical Sciences (India)

Disclosure: Nothing to disclose.


Nicholas Lorenzo, MD, MHA, CPE Founding Editor-in-Chief, eMedicine Neurology; Founder and CEO/CMO, PHLT Consultants; Chief Medical Officer, MeMD Inc

Nicholas Lorenzo, MD, MHA, CPE is a member of the following medical societies: Alpha Omega Alpha, American Association for Physician Leadership, American Academy of Neurology

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Chief Editor

Helmi L Lutsep, MD Professor and Vice Chair, Department of Neurology, Oregon Health and Science University School of Medicine; Associate Director, OHSU Stroke Center

Helmi L Lutsep, MD is a member of the following medical societies: American Academy of Neurology, American Stroke Association

Disclosure: Medscape Neurology Editorial Advisory Board for: Stroke Adjudication Committee, CREST2.


Lakshmi Narasimhan Ranganathan, MD Tutor, Institute Of Mental Health, Chennai, India; Senior Civil Assistant Surgeon, Tamil Nadu Medical Services

Disclosure: Nothing to disclose.

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A man with neuralgic amyotrophy presenting with wasting of deltoids involving the right side more than the left.
A middle-aged man with (atypical) anterior horn cell disease presenting with wasting of the right quadriceps.
EMG at rest from the right quadriceps muscle of a patient with atypical anterior horn cell disease and isolated atrophy of the right quadriceps; EMG shows spontaneous activity.
EMG on voluntary effort from the right quadriceps muscle of a patient with atypical anterior horn disease and isolated atrophy of the right quadriceps; EMG shows motor unit potentials that exhibit prolonged duration and polyphasia.
EMG on maximal effort from the right quadriceps muscle of a patient with atypical anterior horn disease and isolated atrophy of the right quadriceps; EMG shows an impaired interference pattern.
Clinical photograph of a subject with monomelic amyotrophy showing wasting of left forearm. Note the characteristic feature of oblique atrophy, where a normal brachioradialis dominates the atrophied forearm.
Wasting of right forearm and both hand muscles in a patient with Hirayama Disease. Note the oblique atrophy of right forearm.
Wasting of small muscles of the hands in a patient with Hirayama Disease.
T2-weighted cervical spine MRI of a patient with Hirayama disease showing focal cord hyperintensity at C5-C6 level.
T2-weighted cervical spine MRI of the same patient during neck flexion showing anterior displacement of the posterior dural wall with flattening and compression of the cord against the bodies of the vertebrae with prominent dorsal epidural flow voids.
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