Neuromuscular disorders : weakness

Question 1

when do we suspect a NM disorder?

Answer 1

Baby ‘floppy’ from birth
Slips from hands
Paucity of limb movements
Alert, but less motor activity
Delayed motor milestones
Able to walk but frequent falls

Question 2

Gower’s sign indicates?

Answer 2

pelvic muscle weakness

Question 3

Most common muscular dystrophy?

Answer 3

Duchenne

• male infants
• dystrophin gene

Question 4

Presentation of Duchenne?

Answer 4

Delayed gross motor skills

• walk late
• Symmetrical proximal weakness
• Waddling gait, calf hypertrophy
• Gower’s sign positive

Question 5

Duchenne test?

Answer 5

Elevated Creatinine Kinase levels
>1000 in DMD
- need monitored for cardiomyopathy and resp involvement

Question 6

Clinical signs of NM weakness in Duchenne’s

Answer 6

waddling gait
hold shoulders back
prominent abdomen 
hyperextension at the knees
walk in toes - achilles tightening

Question 7

Muscle weakness - facial

Answer 7

myopathic facies

• open mouth
• poppy eyelids

Question 8

what is myotonia?

Answer 8

sustained contraction of the muscle

Question 9

Muscle - problems affecting?

Answer 9

muscular dystrophies, myopathies- congenital and inflammatory, myotonic syndromes

Question 10

Neuromuscular junction - what syndromes?

Answer 10

myasthenic syndromes

- congenital or acquired

Question 11

Anterior horn cell disease can cause?

Answer 11

Spinal muscular atrophy

Question 12

Nerve

Answer 12

Hereditary or acquired neuropathies

Question 13

MYOPATHY features

Answer 13

proximal 
pure motor
reflexes preserved till late 
contractures 
cardiac dysfunction

Question 14

NEUROPATHY features

Answer 14

distal weakness
reflex lost early
sensory symptoms and signs
fasciculation’s