Myopathies- Peripheral NM Conditions

Question 1

NM disorders overview

Answer 1

OVERALL: myopathies and neuropathies

Prognosis is determined by regenerative capacity of the neuromuscular system

There are exercise precautions indicated

Question 2

name the type of autoimmune myopathy we discussed in class

Answer 2

myasthenia gravis

Question 3

Name the 3 hereditary myopathies we discussed in class

Answer 3

limb girdle muscular dystrophy

facioscapularhumeral dystrophy

duchenne muscular dystrophy

Question 4

Myasthenia Gravis definition:

Answer 4

Chronic peripheral autoimmune neuromuscular
disease of the neuromuscular junction (motor
end plate)
-unclear contribution from thymus gland

-women: <40 years and men > 60 years

-10 in 10000

-skeletal muscle weakness that worsens after periods of activity and improves after rest * PACE THEMSELVES

Question 5

MG signs and symptoms

Answer 5

• Diplopia
• Facial weakness
• Ptosis – eyelid drooping * Dysphagia, Dysarthria
• Shortness of breath
• Weakness in neck, UE, LE
• FATIGUE
  –need physical and behavioral adaptations
  –modify activity, frequent rest breaks
  –interval training
  –monitor symptoms during ex

Question 6

Exercise in patients with MG

Answer 6

aerobic training: bicycle ergometer
-70-80% max HR
-3 sets 10-12 min

progressive resistance training:
-3 sets of 12 reps performed at 15 rep max
- 3 sets of 8 reps performed at 8 rep max

** beneficial and strength gains can be made for patients with mild MG

COMMON AREAS OF WEAKNESS
-shoulder strength
-knee extension
-ankle PF
-sit to stand
-walking greater than 400 m

Question 7

Example interval training for MG

Answer 7

2 min cycling against high load

1 min cycling against low load

7 cycles

with warm up and cool down

AEROBIC EXERCISE GOAL

Question 8

Precautions in MG

Answer 8

MG CRISIS: medical emergency where breathing stops and the individual requires ventilation

911 call or code blue

-15-20% of those with MG
-not necessarily related to exercise
-the onset of symptoms is typically slow
-monitor breathing and swallowing

Question 9

Limb girdle muscular dystrophy def:

Answer 9

6/100,000 ppl
mutations in genes that provide instructions for making proteins involved in muscle maintenance and repair

** affects proximal muscles of UE/LEs

-various subtypes and presentations

-LGMD type 1: autosomal dominant inheritance

-LGMD type 2: autosomal recessive inheritance

may present with cardiomyopathy

may require mechanical ventilation

Question 10

Early stage –> late stage LGMD

Answer 10

EARLY:
-changes in walking
-hands on knees to transition from squat to stand

LATE:
-may need wheelchair
-scapular winging, increased lumbar lordosis, pseudo hypertrophy of calves

Question 11

PT intervention LGMD

Answer 11

resistance training
-both higher and lower intensity resistance training may improve strength in arms over 6 months –> don’t know about function

aerobic and endurance training
–> may improve aerobic capacity and walking

** more focus on compensation and prevention than remediation

Question 12

Fascioscapuloarhumeral muscular dystrophy (FSHD)

Answer 12

4-10/100,000

-FACIAL, SCAPULAR, HUMERAL MUSCLES AFFECTED

FSHD1 - 95%
FSHD2- 5%
–clinically identical but genetically different

AUTOSOMAL DOMINANT- recommended genetic counseling

-mask-like appearance

-symptoms before age 20 usually

-life expectancy not shortened

Question 13

Is life expectancy shortened in those with FSHD

Answer 13

no

Question 14

FSHD management

Answer 14

-pulmonary function testing
-test for retinal and hearing problems
-speech therapy

MUSCLES AFFECTED EARLY
-hamstrings and trunk mm.

-maintain muscle flexibility, minimize atrophy
-manage pain

Question 15

Compensations for those with FSHD

Answer 15

orthotics/AFO

gait aids

wheeled mobility