PATHOLOGY EXAM 4: MYOPATHY II EXAM
2025/2026 QUESTIONS WITH ANSWERS GRADED
A+
Limb-Girdle MD - Moderate form
Teens up to early adulthood
AR (calpain)/AD (adhalin)
Pseudohypertrophy and contractures uncommon
Limb-Girdle MD: Muscles first affected - proximal muscles; hip/shoulder girdle, scap winging,
lumbar lordosis, ab protrusion, poor balance, inability to raise arms, face muscles not affected
Limb Girdle MD: Progression - Slow
Starts in hips --> shoulders
Milder impairments
Walking difficult ~20 yo
Spontaneous mutation
Limb Girdle MD stance - Wide gait, protruded stomach, rounded shoulders
Emery-Dreifuss MD - childhood to early teens
x linked in males
Shoulders, upper arms, lower legs
Contractures before weakness
Slow progression with less severe muscle weakness; life threatening heart probs can occur; life
expectancy variable
Clinical triad
type I small fibers affected
Clinical triad - Joint contractures that begin in early childhood
muscle weakness
, wasting of muscles in humeroperoneal region
Fascioscapulohumeral MD - Moderate
Teens to early adulthood
AD males/females
Face, shoulders, upper arms (NOT LATS)
Hearing/vision probs, forward sloping shoulders, difficulty raising arms over head and closing
eyes, expressionless face
Slow prox to distal; variable life expectancy
Scapuloperoneal MD - Mild
Variation of FSH w/o face
Any age (later onset = better prognosis)
AD or AR, male/female
Distal muscles of LE --> prox shoulder girdle
Foot drop first then shoulder weakness
Progression varies
Severe MD - Duchenne's
Becker's
Congenital
Myotonic
Moderate MD - Limb-Girdle
Fascioscapulohumeral
Emery-Dreifuss
Oculopharyngeal
Mild MD - Scapuloperoneal
Myotonic Dystrophy (MTD) - Severe
2025/2026 QUESTIONS WITH ANSWERS GRADED
A+
Limb-Girdle MD - Moderate form
Teens up to early adulthood
AR (calpain)/AD (adhalin)
Pseudohypertrophy and contractures uncommon
Limb-Girdle MD: Muscles first affected - proximal muscles; hip/shoulder girdle, scap winging,
lumbar lordosis, ab protrusion, poor balance, inability to raise arms, face muscles not affected
Limb Girdle MD: Progression - Slow
Starts in hips --> shoulders
Milder impairments
Walking difficult ~20 yo
Spontaneous mutation
Limb Girdle MD stance - Wide gait, protruded stomach, rounded shoulders
Emery-Dreifuss MD - childhood to early teens
x linked in males
Shoulders, upper arms, lower legs
Contractures before weakness
Slow progression with less severe muscle weakness; life threatening heart probs can occur; life
expectancy variable
Clinical triad
type I small fibers affected
Clinical triad - Joint contractures that begin in early childhood
muscle weakness
, wasting of muscles in humeroperoneal region
Fascioscapulohumeral MD - Moderate
Teens to early adulthood
AD males/females
Face, shoulders, upper arms (NOT LATS)
Hearing/vision probs, forward sloping shoulders, difficulty raising arms over head and closing
eyes, expressionless face
Slow prox to distal; variable life expectancy
Scapuloperoneal MD - Mild
Variation of FSH w/o face
Any age (later onset = better prognosis)
AD or AR, male/female
Distal muscles of LE --> prox shoulder girdle
Foot drop first then shoulder weakness
Progression varies
Severe MD - Duchenne's
Becker's
Congenital
Myotonic
Moderate MD - Limb-Girdle
Fascioscapulohumeral
Emery-Dreifuss
Oculopharyngeal
Mild MD - Scapuloperoneal
Myotonic Dystrophy (MTD) - Severe
