Neuromuscular Disorders

Question 1

Dyskinesias

Answer 1

Involuntary, non-repetitive but occasionally stereotyped movements affecting distal, proximal, and axial musculature
Most common in basal ganglia disorders

Question 1

Hemiballismus

Answer 1

involuntary flinging motions of the extremities
often violent and wide amplitudes of motion
can occur distal or proximal on one side of the body

Question 2

Dystonia

Answer 2

sustained abnormal postures and disruptions of ongoing movement due to alteration of muscle tone

Question 3

Cardinal Signs of Parkinsons

Answer 3

T= tremor
R= rigidity
A= akinesia
P= postural instability

Question 4

Stage 1 of Parkinsons

Answer 4

unilateral tremor, akinesia, minimal or no functional impairment

Question 5

Stage 2 of Parkinsons

Answer 5

bilateral tremor, rigidity, or akinesia, with or without axial signs, independent with ADL, no balance impairment

Question 6

Stage 3 of Parkinsons

Answer 6

worsening of symptoms, impaired righting reflexes, difficulties with ADL performance, can lead independent life

Question 7

Stage 4 of Parkinsons

Answer 7

requires help with ADL, unable to live alone, able to walk and stand unaided

Question 8

Stage 5 Parkinsons

Answer 8

unable to stand, walk, or perform ADL, max assist required

Question 9

Medications for Parkinsons and Side effects

Answer 9

Levodopa compensates for the loss of dopamine
As the disease progresses drug works less evenly and predictably
on-off effect of long-term use may cause symptoms to appear and disappear suddenly

Question 10

Spina bifida occulta

Answer 10

Bony malformation with separation of vertebral arches of one or more vertebrae
may not be discovered until late childhood
occasionally slight instability and neuromuscular impairments but no huge symptoms

Question 11

Occult spinal dysraphism

Answer 11

red birthmark, patch of hair, dermal sinus, tumor
may result in spinal cord being split or being tethered
neurological damage and developmental abnormalities as child grows

Question 12

Spina bifida with meningocele

Answer 12

Protrusion of sac through spine, containing CSF and meninges, does not include SC
usually does not present with symptoms impacting function on SC
Slight instability and some bowel and bladder issues may occur

Question 13

Spina bifida with myelomeningocele

Answer 13

protrusion of sac through spine containing CSF and meninges as well as SC or nerve roots
results in sensory and motor deficits occurring below the level of the lesion
may result in LE paralysis and DVT
Lesions of S2-S4 result in bowel and bladder problems

Question 14

Tethered cord syndrome

Answer 14

visible signs include: hairy patch of skin, hemangioma, dimple of lower spine
difficulties with bowel/bladder control, gait, scoliosis
Symptoms may be exacerbated by growth spurts or when pregnant

Question 15

Signs of shunt malfunction

Answer 15

CRITICAL EMERGENCY
soft spot on forehead (infants)
severe headache, vomitting, irritability
change in UE function
impaired vision
onset of seizure disorder or increased activity

Question 16

Average age diagnosis of muscular dystrophy

Answer 16

5 years unless known family history or early detection

Question 17

Duchennes muscular dystrophy

Answer 17

most common form
detected between 3-5
enlargement of calf muscles and at times forearm and thighs
weakness of proximal joints
ambulates with trendelenburg gait , uses hands to crawl up thighs to get to standing
weakness occurs in all voluntary muscles
behavioral and learning difficults
rarely survive past early 20s

Question 18

Beckers muscular dystrophy

Answer 18

slower to progress and less severe than Duchenne
loss of motor function of hips, thighs, pelvic area and shoulders
survival can be into late adulthood

Question 19

Specific Symptoms of muscular dystrophy and atrophy

Answer 19

low muscle tone and weakness
difficulties with oral motor feeding
deformities of extremities and spine
difficulties breathing

Question 20

Symptoms of ALS

Answer 20

muscle weakness and atrophy beginning dismally and asymmetrical
cramps and fasiculations
signs usually begin in hands
LMN signs (hyperactive reflexes, hypertonia)

Question 21

how is ALS symptoms documented?

Answer 21

revised ALS Functional Rating Scale
speech, salvation, swallowing, handwriting, cutting food, dressing, turning in bed etc.
Lower scores indicate greater symptoms

Question 22

ALS treatment

Answer 22

medications
treating secondary complications

Question 23

Erbs Palsy

Answer 23

paralysis of upper brachial plexus
5th and 6th cervical nerves
Arm cannot be raised, elbow flexion is weakened
positioning and ROM exercises are necessary to decrease contractures

Question 24

Klumpkes Palsy

Answer 24

paralysis of lower brachial plexus more rare compared to erbs paralysis of hand and wrist

Question 25

guillain-Barré Syndrome

Answer 25

acute and rapidly progressing demyelintion of the peripheral nerves and spinal roots

Question 26

Guillain-Barre Longterm Symptoms

Answer 26

acute, rapidly progressive form of polyneuropathy symmetric muscle weakness and mild distal sensory loss weakness if more prominent dismally first and then progresses proximally deep tendon reflexes are lost

Question 27

Medical Managment for GBS

Answer 27

medical emergency respiratory support may be needed

Question 28

Myasthenia Gravis

Answer 28

occurs at any age but often affects younger women and older men attach on ACh receptors of postsynaptic NMJ

Question 29

MS symptoms

Answer 29

weakness in legs or hands multiple and varied neurological symptoms and signs of visual and emotional disturbances cognitive deficits bowel and bladder dysfunction spasticity

Question 30

Four patterns of MS

Answer 30

relapsing remitting secondary progressive primary progressive Progressive relapsing

Question 31

symptoms of myasthenia gravis

Answer 31

episodic muscle weakness diplopia muscle fatigue after exercise dysarthria dysphasia proximal limb weakness symptoms fluctuate throughout the day