Focal Muscular Atrophies

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Focal atrophy of an individual muscle or group of muscles, often encountered clinically, may create diagnostic and therapeutic challenges.

A wide variety of neurologic disorders may present with focal muscular atrophy (FMA). FMA also may be secondary to nonneurologic conditions, leading to disuse of part of a limb.

The organ ultimately affected is the muscle, although the pathology may be anywhere along the lower motor neuron (LMN) or, at times, secondary to nonneurologic disorders.

Etiologic factors include the following:

Infection

Trauma

Inflammation

Spinal cord disorders

Vasculitis

Entrapment

Altered immune mechanisms

Toxins

Physical agents, such as electrical or radiation injury

Genetic and enzyme defects

United States

FMA is a heterogenous disorder with diverse etiologies, so overall prevalence rates are not available.

An estimated 1.63 million polio survivors reside in the US; 28-50% of them will develop postpolio progressive muscular atrophy (PPMA). ^{[1, 2]}

International

For the same reasons outlined for the US, incidence and prevalence data are not available. Even for the individual diseases, considerable geographic variation exists. A population-based study revealed a PPMA prevalence of 92 per 100,000 population in a Swedish county. ^[3] The prevalence of post-polio syndrome in Kitakyushu, Japan was 18 per 100,000 population. ^[4] The frequencies of PPMA among survivors of polio in other countries (not community-based studies) are 60% in the Netherlands ^[5] , 58% in Norway ^[6] , 68% in Germany, and 22% in India ^[7] . An estimated 3000-5000 persons with PPMA reside in New Zealand. ^[8] These figures are likely to be overestimated. ^[9]

Many of the infectious causes of FMA (eg, polio, leprous neuropathy) are more frequent in developing countries.

Monomelic amyotrophy has been reported more often in India ^[10] , Korea ^[11] , and Japan than in other countries. In a hospital-based study from India, among 110 patients with anterior horn cell disease, 10.9% had progressive muscular atrophy; 1.8%, PPMA; and 22.7%, monomelic amyotrophy. ^[12]

Most disorders that cause FMA are benign and do not lead to higher-than-normal mortality rates. Most patients do not suffer significant disability, except when the FMA involves an entire limb, becomes generalized, or has an acute onset.

Most conditions that cause FMA do not have any racial predilection. The geographic variations of some of these disorders probably reflect environmental conditions rather than genetic predisposition.

Bulbospinal muscular atrophy (an X-linked disorder) involves only males. Monomelic amyotrophy is more common in men. PPMA is more frequent in women.

Disorders such as polio and monomelic amyotrophy are more common in younger people.

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Sridharan Ramaratnam, MD, MBBS Director and Senior Consultant, The Nerve Centre, Chennai, India

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

Disclosure: Nothing to disclose.

Nicholas Lorenzo, MD, MHA, CPE Co-Founder and Former Chief Publishing Officer, eMedicine and eMedicine Health, Founding Editor-in-Chief, eMedicine Neurology; Founder and Former Chairman and CEO, Pearlsreview; 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 Academy of Neurology, American Association for Physician Leadership

Disclosure: Nothing to disclose.

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.

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; Executive Committee for the NINDS-funded DEFUSE3 Trial; Physician Advisory Board for Coherex Medical.

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

Disclosure: Nothing to disclose.

Focal Muscular Atrophies

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