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MYOPATHIES

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Definitions

From Braddom:

A group of muscle diseases whose most

common primary symptom is proximal limb

muscle weakness.

Differ in etiology, course, specific muscle

involvement, and associated problems.

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From DeLisa:

Any disease of muscle resulting from any

biochemical, electrical, or other pathologic

change occurring in muscle fibers or theconnective tissue of muscles.

Pathology is not a result of nervous system

dysfunction.

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From Harrison:

Disorders with structural changes or

functional impairment of muscle.

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Important points in the definitions given

are:

A GROUP OF CONDITIONS

Affects SKELETAL MUSCLE

Primary problem is MUSCLE WEAKNESS,

specifically of PROXIMAL LIMBS

Must RULE OUT NEUROLOGIC DISEASE

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Classification

Braddom:

Dystrophies

Congenital myopathies

Metabolic myopathies

Endocrine myopathies

Inflammatory myopathies

Infectious myopathies

Toxic myopathies

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ICD-9-CM Diagnosis 359: Muscular

dystrophies and other myopathies

359.0: Congenital hereditary muscular

dystrophy

359.1: Hereditary progressive muscular

dystrophy

359.2: Myotonic disorders 359.3: Periodic paralysis

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359.4: Toxic myopathy

359.5: Myopathy in endocrine diseases

classified elsewhere

359.6: Symptomatic inflammatory myopathyin diseases classified elsewhere

359.8: Other myopathies

359.9: Myopathy unspecified

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Epidemiology

Condition-specific

Most researched are the muscular

dystrophies

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Etiology

Either:

Inherited

Acquired

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Pathophysiology

Inherited:

X-linked recessive

Autosomal recessive

Autosomal dominant

Acquired

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X-linked Recessive

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Autosomal Recessive

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Autosomal Dominant

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Clinical Manifestations

Muscle weakness

Primary

Muscle pain, cramps, stiffness

Associated with involuntary muscle activity

Muscle enlargement or atrophy

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Complications

Contractures

Functional limitations and disabilities

Respiratory distress Death

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Diagnosis

History

Look for:

Family history

Exposure

Physical examination

Primarily on musculoskeletal system

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Laboratory tests

Serum enzymes

Creatinekinase (CK)

Aspartate aminotransferase (AST)

Alanine aminotransferase (ALT)

Lactic dehydrogenase (LDH)

Gamma-glutamyl transferase (GGT)

Aldolase

Electrodiagnostic studies

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Other laboratory tests

To diagnose specific types of myopathy

DNA analysis

Forearm exercise test

Muscle biopsy

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Differential Diagnosis

Through correlation of history, physical

examination, and lab tests

Then, compare to a table of possible

diagnoses

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Prognosis

Varying as to type

Some have minimal limitation

Some are progressive

Some are life-threatening/fatal

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Medical Management

Depend on:

Type of disease

Causes

Supportive and symptomatic treatment

may be the only treatment available or

necessary for some disorders

Medications:

Immunosuppresion (Steroids-Prednisone)

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Other Management

Respiratory therapy

Surgery

Occupational therapy Long-term caregiving

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PT Examination/Evaluation

History

Family history

Exposure

Progression of condition

Physical examination

Musculoskeletal system

Functional assessment

Pulmonary assessment

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PT Problem List

Muscle weakness

Focus of PT intervention

Presence of or risk of complications

Musculoskeletal

Pulmonary

Muscle pain

Not usually treated by PT

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PT Diagnosis

Depending on type, may fall under:

Impaired joint mobility, motor function, muscle

performance, and range of motion associated

with connective tissue dysfunction Impaired joint mobility, motor function, muscle

performance, and range of motion associated

with localized inflammation

Other diagnostic classification

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PT Goals

Improve muscle strength (if non-

progressive)

Prevent further muscle weakness (if

progressive)

Prevent musculoskeletal/pulmonary

complications

Address functional limitations/disabilities

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PT Intervention

ACUTE:

May be necessary to perform preparatory

intervention (ROM, stretching)

Patient education

Risk reduction (for risk of complications)

Modalities need to be justified

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SUBACUTE-CHRONIC:

Muscle strengthening

Precaution on fatigue

Use functional activities in strengthening

Functional activities

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THANK YOU

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