eMedicine Specialties > Neurology > Neuromuscular Diseases

Focal Muscular Atrophies: Treatment & Medication

Author: Ramaratnam Sridharan, MD, FRCP, FAAN, Head of the Department of Neurology, Professor, Neurology, Chennai Neurospeciality & Research Institute
Coauthor(s): Nicholas Y Lorenzo, MD, Chief Editor, eMedicine Neurology; Consulting Staff, Neurology Specialists and Consultants; Lakshmi Narasimhan Ranganathan, MD, Assistant Professor, Department of Neurology, Stanley Medical College, India
Contributor Information and Disclosures

Updated: Mar 13, 2007

Treatment

Medical Care

Treatment 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.
  • 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, 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.
    • A randomized placebo-controlled trial of 142 patients with postpolio syndrome with IV immunoglobulin revealed nonsignificant improvement in muscle strength with treatment.
    • 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.

Consultations

  • 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

Medication

The goal of pharmacotherapy is to reduce morbidity.

Blood products

These are useful in minimizing the effects of autoimmune reactions.


Intravenous immunoglobulin (IVIg)

Following features may be relevant to its efficacy: neutralization of circulating antibodies through anti-idiotypic antibodies; down-regulation of pro-inflammatory cytokines, including IFN-gamma; blockade of Fc receptors on macrophages; suppression of inducer T and B cells and augmentation of suppressor T cells; blockade of complement cascade.

Adult

2 g/kg IV over 2-5 d

Pediatric

Administer as in adults

Documented hypersensitivity; IgA deficiency; anti-IgE/IgG antibodies

Pregnancy

C - Safety for use during pregnancy has not been established.

Precautions

Consider checking serum IgA before treatment and using IgA-depleted IVIg (G-Gard-SD), if indicated; may increase serum viscosity and risk of thromboembolic events
Reported adverse effects include migraine attacks; 10% increased risk of aseptic meningitis; increased risk of urticaria, pruritus, or petechiae 2-5 d after infusion (possibly lasting up to 1 mo); increased risk of renal tubular necrosis in patient who are older, diabetic, volume depleted, or have preexisting kidney disease
IVIg can lead to elevated antiviral or antibacterial antibody titers for 1 mo; 6-fold increased ESR for 2-3 wk; apparent hyponatremia

Glucocorticoids

These agents modify the body's immune response to diverse stimuli. Likely mechanisms of action are inhibition of synthesis/secretion of TNF-alpha, IL-6, IL-2, and IFN-gamma, and modulation of serum and leukocyte-bound levels of cell adhesion molecules.


Prednisone (Deltasone, Meticorten, Sterapred)

Useful in treatment of inflammatory and immune reactions. By reversing increased capillary permeability and suppressing PMN activity, may decrease inflammation. Dosage and length of treatment vary depending on specific diagnosis.

Adult

60-100 mg PO qam

Pediatric

4-5 mg/m2/d or 1-2 mg/kg PO qd

Estrogens may decrease clearance; when used with digoxin, may increase digitalis toxicity secondary to hypokalemia; phenobarbital, phenytoin, and rifampin may increase metabolism and may necessitate dose increase; monitor for hypokalemia when given together with diuretics

Pregnancy

B - Usually safe but benefits must outweigh the risks.

Precautions

Use with caution in patients with opportunistic infections
Patients with hyperthyroidism, cirrhosis, nonspecific ulcerative colitis, osteoporosis, peptic ulcer, diabetes, and myasthenia gravis; patients may develop hyperglycemia, edema, osteonecrosis, peptic ulcer disease, hypokalemia, osteoporosis, euphoria, acne, psychosis, growth suppression, myalgia, myopathy, infections, glaucoma, hirsutism, facial plethora; patients may develop adrenal crisis, if steroids are withdrawn abruptly

More on Focal Muscular Atrophies

Overview: Focal Muscular Atrophies
Differential Diagnoses & Workup: Focal Muscular Atrophies
Treatment & Medication: Focal Muscular Atrophies
Follow-up: Focal Muscular Atrophies
Multimedia: Focal Muscular Atrophies
References
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Further Reading

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Keywords

anti-GM1 antibody, multifocal motor neuropathy with conduction block, benign focal amyotrophy, congenital absence of muscles, focal myositis, Hirayama disease, injection myopathy, monomelic amyotrophy, peripheral nerve injuries, plexopathy, poliomyelitis, postpolio progressive muscular atrophy, postradiation toxicity and disease, spinal cord tumors, toxic neuropathies, wasted leg syndrome

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Contributor Information and Disclosures

Author

Ramaratnam Sridharan, MD, FRCP, FAAN, Head of the Department of Neurology, Professor, Neurology, Chennai Neurospeciality & Research Institute
Ramaratnam Sridharan, MD, FRCP, FAAN is a member of the following medical societies: American Academy of Neurology
Disclosure: Nothing to disclose.

Coauthor(s)

Nicholas Y Lorenzo, MD, Chief Editor, eMedicine Neurology; Consulting Staff, Neurology Specialists and Consultants
Nicholas Y Lorenzo, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Neurology
Disclosure: Nothing to disclose.

Lakshmi Narasimhan Ranganathan, MD, Assistant Professor, Department of Neurology, Stanley Medical College, India
Disclosure: Nothing to disclose.

Medical Editor

Donald B Sanders, MD, EMG Laboratory Director, Professor of Medicine (Neurology), Division of Neurology, Duke University Medical Center
Donald B Sanders, MD is a member of the following medical societies: American Academy of Neurology, American Association of Neuromuscular and Electrodiagnostic Medicine, American Neurological Association, and New York Academy of Sciences
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

Agapito S Lorenzo, MD, Laboratory Director, Associate Professor, Departments of Neurology, Creighton University and University of Nebraska Medical Center
Agapito S Lorenzo, MD is a member of the following medical societies: American Academy of Neurology and American Association of Neuromuscular and Electrodiagnostic Medicine
Disclosure: Nothing to disclose.

CME Editor

Matthew J Baker, MD, Consulting Staff, Collier Neurologic Specialists, Naples Community Hospital
Matthew J Baker, MD is a member of the following medical societies: American Academy of Neurology
Disclosure: Nothing to disclose.

Chief Editor

Helmi L Lutsep, MD, Associate Professor, Department of Neurology, Oregon Health and Science University; Associate Director, Oregon Stroke Center
Helmi L Lutsep, MD is a member of the following medical societies: American Academy of Neurology and American Stroke Association
Disclosure: Co-Axia Consulting fee Review panel membership; Talecris Consulting fee Review panel membership; AGA Medical Consulting fee Review panel membership; Boehringer Ingelheim Honoraria Speaking and teaching; Boston Scientific Honoraria Speaking and teaching

 
 
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