Anterior Horn and Periph Nerve Disorders

Question 1

UMN injury produces what symptoms

Answer 1

1) spasticity
2) hyeprreflexia
3) Babinski
4) emotional lability (inappropriate laughing/crying)

Question 2

LMN injury produces what symptoms

Answer 2

1) muscle atrophy
2) fasciculations
3) decr tone
4) decr or absent reflexes

Question 3

all motor neuron, NMJ and muscle diseases have no ___

Answer 3

sensory changes accompanying the weakness

Question 4

sensory changes imply ___ disorders

Answer 4

nerve root
plexus
or peripheral nerve

Question 5

ex of rapdily developing weakness

Answer 5

NMJ disorders like
MG
botulism
organophosphate poisoning

Question 6

disease caused by acute remissions and relapses

Answer 6

myasthenia gravis

Question 7

proximal weakness most characteristic of

Answer 7

muscle disorders

difficulty climb stairs
get up from low chairs
raise arms over head 
broad gait
lordotic posture, wide gait

Question 8

distal weakness of hands and feet + distal sensory loss most characteristic of

Answer 8

neuropathies

with slapping, footdrop gait
difficulty with fine hand movements

Question 9

cranial weakness involving extraocular, facial, oropharyngeal muscles seen in

+ ptosis, double vision, dysphagia

Answer 9

Myasthenia graivs

Question 10

disuse of a limb such as bedridden or fracture produces ___

Answer 10

atrophy but retained strength

old people can still be strong even with muscle atrophy

Question 11

myaglia and tenderness with muscle inflamm seen in

Answer 11

polymyositis

Question 12

myotonia involves

Answer 12

stiffness due to delayed relaxation of muscle after voluntary contractions

Question 13

muscle cramps due to

Answer 13

involuntary contraction of muscle and benign

Question 14

diseases assoc with skeletal muscle deformitis such as socoliosis, club foot, contratures (loss of ROM)

Answer 14

neuromuscular disorders

Question 15

rhabdomyolysis and myoglobulia seen when?

Answer 15

inability to generate ATP d/t glycogen or lipid metabolic defects

Question 16

best lab value for muscle necrosis

highest in what dz
mod in what dz

Answer 16

incr serum creatine kinase

highest with myoglobinuria

moderate with muscular dystrophies and inflamm myopathies

Question 17

nerve condition studies used to differentiate btwn

Answer 17

1) primary demyelinating (very slow)

2) axonal neuropathies (fast)

Question 18

needle EMG perfoermed when___

Answer 18

complement to NCS for differentiating myopathic from neuropathic disorders

neuropathic disorders (anterior horn cell and peripheral nerve) = loss of whole motor units

myopathic disorders = drop out of fibers within motor unit

Question 19

repetitive stim of nerve in MG produces

Answer 19

decr in amplitude of compound muscle AP

Question 20

ALS due to ?

majority are sporadic or genetic?

Answer 20

degeneration of brainstem and spinal cord LMN
+ spasticity, hyperreflexia from UMN injury

2) sporadic

Question 21

symptoms of ALS

Answer 21

1) asymmetric limb weakness + fasciculations
2) footdrop
3) contracture from inteorsseous wasting
4) UMN signs
5) sensory normal
6) slurred speech
7) spared facial muscles
8) diaphragm weakness

Question 22

Treatment of ALS

Answer 22

1) Rilouzole

2) braces for mobility

Question 23

Charcot Marie Tooth

2 catgories

Answer 23

1) demyelinating neuropathy = CMT 1

2) axonal degeneration = CMT2

Question 24

Difference btwn CMT1 and CMT2

Answer 24

1) slow NCV

2) normal NCV

Question 25

inheritance of CMT1 and CMT2 CMT4

Answer 25

autosomal dominant recessive

Question 26

CMT1A due to what molecularly?

Answer 26

duplication of PMP22

Question 27

deletion of PMP22 causes what?

Answer 27

HNPP

Question 28

3 phenotypes of CMT

Answer 28

1) most common = onset walking normal but distal weakness/sensory loss in 20's 2) impaired as infants with delayed walking 3) adult onset at 40 y/o

Question 29

Diabetic neuropathies what kind of neuropathies patients with asymptomatic diabetes may show ___ NCVs that normalize with ___

Answer 29

most common neuropthy can be poly, mono, plexo, radiculo may show decr NCVs normalized with improved blood sugar control

Question 30

most common subtype of diabetic neuropathy

Answer 30

distal sensory or sensorimotor polyneuroapthy - numbness + burning dysesthesias in feet --> spread up legs and into hands - slapping foot drop gait - decr grip str, decr fine dexterity

Question 31

examination of paitent with diabetic neuroapthy

Answer 31

pin sensation loss in stocking globe distrib but asymm loss of position, vibr, light touch, decr reflex in 'LARGE FIBER' pain and temp loss + pain in 'SMALL FIBER'

Question 32

autonomic symptoms of neuroapthy

Answer 32

1) postural hypotension 2) diarrhea 3) impotence 4) urinary retention 5) incr sweating

Question 33

lumbosacral plexopathy is characterized by onset when?

Answer 33

1) acute onset asymm prox weakness and leg pain occurs at onset of diabetes and assoc with weight loss

Question 34

mononeuropathies can affect what?

Answer 34

carpal tunnel ulnar peroneal + CN (3rd and 6th nerve palsies

Question 35

pathogenesis of diabetic neuroapathies

Answer 35

1) metabolic derangements and change in endoneural vessels + ischemia 2) occlusion of small nutrient blood vessels supplying nerves

Question 36

tx for autonomic symptoms of diabetic neuropathy

Answer 36

1) codeine + diphenoxylate for diarrhea 2) stockings 3) fluorocortisone or midodrine for postural hypotenison 4) voiding assisted by pressure for bladder atony

Question 37

tx for cramps/spasticity in ALS

Answer 37

hydration benzodiazepans phenytoin quinine lioresal dantrolene gabapentin helps jaw quiver/clench; incr weakness; decr FVC

Question 38

tx for pseudobulbar affect in ALS

Answer 38

TCA and SSRI | dextromethorphan + quinidine

Question 39

tx for drooling, thick phlegm, and laryngospasms

Answer 39

decr saliva metoprolol and propranolol for thick saliva decongestants

Question 40

tx for sleep distrubance in ALS

Answer 40

multiple causes avoid short acting sedatives test for desaturation = nocturnal NIPPV

Question 41

sx of sensory or sensorimotor distal polyneuropathy

Answer 41

stocking/glove numbness and burning pain foot drop and weak hand muscles

Question 42

sx of autonomic neuropathy in diabetic neuropathy

Answer 42

1) hypotension 2) diarrhea 3) impotence 4) urinary retention

Question 43

sx of mononeuropathy in diabetic neuropathy

Answer 43

``` CN 3, 6, 7 or peripheral (median, ulnar, peroneal) ```

Question 44

sx of lumbosacral plexopathy

Answer 44

pelvic girdle pain | asymm hip flexor weakness