Myopathy.

How to approve?

Mudjiani Basuki,dr. SpS

Division of Peripheral Nerve,
Dr. Sutomo Hospital-Airlangga University
Surabaya
 Myopathy:

Are the disorders affecting the channel,

structure or metabolism of skeletal muscle
Myopathy
• Congenital Myopathy and Congenital Muscular Dystrophy
• Muscular Dystrophy
• Metabolic Myopathy
• Muscle Channelopathy
• Inflammatory Myopathy
• Toxic Myopathy
Myopathy :
How to Approach Diagnosis?
Clinical Evaluation
• Which “negative” and/ or “positive” symptoms does the Px.
Experience?
• What is the temporal evolution?
• Does the patient have a family history of myopathic disorder?
• Are the presipitating factors that trigger episodic weakness or
stiffness?
• Are any associated systemic symptoms and signs present?
• What is the distribution of weakness?
Positive and Negative
symptoms:
Muscle Disease Associated With Myalgia:
Myopathies Associated With Muscle Contractures:
Myopahies Associated With Muscle Stiffness:
Causes of Myoglobinuria:
What is the the temporal Evolution?
What is the the temporal Evolution?
What is the the temporal Evolution?
Does the Patient Have a Family History of
Myopathic Disorder:
Are the precipitating Factors that trigger Episodic
Weakness of Stiffness?
Are any associated Systemic Symptoms or Sign
Present?
Are any associated Systemic Symptoms or Sign
Present?
Myopathies associated with Respiratory Insufficiency:
What is distribution of Weakness?
What is distribution of Weakness?
• Pattern 1: proximal Limb- Girdle Weakness
• Pattern 2: Distal weakness.
• Pattern 3: Proximal Arm/ Distal Leg (Scapuloperoneal) weakness
• Pattern 4: Distal Arm/ proximal Leg Weakness
• Pattern 5: Ptosis with/ without Ophthalmoparesis.
• Pattern 6: prominent Neck extensor Weakness
• Pattern 7: bulbar weakness
• Pattern 8: episodic Pain, weakness, and Myoglobinuria
• Pattern 9: episodic Weakness delayed or unrelated to exercise
• Pattern 10: stiffness and decreased Ability to relax
Pattern 2: Predominantly distal weakness.
Pattern 2: Predominantly distal weakness.
Pattern 3: Proximal Arm/ Distal Leg
(Scapuloperoneal) weakness
Pattern 5: Ptosis with/ without Ophthalmoparesis.
Pattern 6: prominent Neck extensor
Weakness
Pattern 8: episodic Pain, weakness, and Myoglobinuria
Pattern 9: episodic Weakness delayed or unrelated
to exercise
Pattern 10: stiffness and decreased Ability to relax
Laboratory Approach:
• Creatine kinase
• Electrophysiologyc examination
• Muscle Biopsy
DD/ of CK elevation
Summary:
• The diagnosis of myopathy have a lot of limitation
• The symptoms of myopathy are similar with the other disease
• Careful consideration of the distribution of muscle weakness and
good neurological examination can help us to get a best diagnosis.