Anterior Horn Cell and Peripheral Nerve Disorders

Question 1

_____ diseases produce spastic tone, hyperactive tendon reflexes, pathological reflexes (ie Babinski), and emotional lability (inappropriate laughing and crying).

Answer 1

Upper motor neuron (UMN)

Question 2

Upper motor neuron (UMN) diseases produce what signs/symptoms?

Answer 2

• spastic tone
• hyperactive tendon reflexes
• pathological reflexes (ie Babinski)
• emotional lability (inappropriate laughing and crying)

Question 3

______ diseases produce muscle atrophy, fasciculations, diminished tone, and reduced or absent reflexes.

Answer 3

Lower motor neuron (LMN)

Question 4

Lower motor neuron (LMN) diseases produce what signs/symptoms?

Answer 4

• muscle atrophy
• fasciculations
• diminished tone
• reduced or absent reflexes

Question 5

If the pt complains about sensory changes with accompanied weakness, this suggest problems with?

Answer 5

• a nerve root disorder
• plexus disorder
• peripheral nerve disorder

Question 6

Rapidly developing weakness (hours to days) is characteristic of _______.

Answer 6

neuromuscular junction disorders

Question 7

Name 3 distribution patterns of weakness.

Answer 7

• proximal
• distal
• cranial

Question 8

How is a neuromuscular disorder different than normal atrophy?

Answer 8

• in normal atrophy, the muscle remains strong
• In an NMJ disorder, the muscle is strikingly weak

Question 9

Name 4 anterior horn cell disorders.

Answer 9

1. amyotrophic lateral sclerosis (ALS)
2. spinal muscular atrophy
3. Poliomyelitis
4. West Nile virus

Question 10

Name 2 radiculopathies and plexopathies.

Answer 10

1. cervical and lumbosacral nerve root compression
2. brachial and lumbosacral plexopathies

Question 11

Name 3 types of peripheral neuropathies.

Answer 11

1. mononeuropathy
2. mononeuropathy multiplex
3. polyneuropathy

Question 12

Name 4 neuromuscular junction disorders.

Answer 12

1. Myasthenia gravis
2. Lambert Eaton syndrome
3. Botulism
4. Organophosphate poisoning

Question 13

Dx:

• progressive weakness and wasting degeneration of brainstem and spinal cord neurons
• asymmetric limb weakness
• fasciculations
• foot drop or hand deformity
• pathological reflexes present
• normal sensory exam

Answer 13

Amyotrophic lateral sclerosis (ALS)

Question 14

What is the average survival after diagnosis of ALS?

Answer 14

3-4 years

Question 15

How is ALS treated?

Answer 15

• primarily symptomatic
• Ruluzole
• braces and durable medical equipment

Question 16

How does someone get Charcot-Marie Tooth disease?

Answer 16

it’s inherited

Question 17

What is the most common form of Charcot-Marie Tooth disease type 1? What causes it?

Answer 17

CMT1A; duplication of the PMP22 gene (peripheral myelin protein gene)

Question 18

What does a duplication of the PMP22 gene (peripheral myelin protein gene) cause?

Answer 18

Charcot-Marie Tooth disease type 1A (CMT1A)

Question 19

Most pts with CMT present with 3 phenotypes. What are they, and what is the most common?

Answer 19

1. normal onset of walking, distal weakness and sensory loss in first 2 decades** most common
2. impaired as infant, delayed walking
3. adult onset appearing around age 40

Question 20

What is the cutoff between demyelinating and axonal forms of CMT?

Answer 20

38m/sec

Question 21

What is the medication tx for CMT?

Answer 21

none, just pain mgmt

Question 22

_____% of diabetics have diabetic neuropathy.

Answer 22

50-60%

Question 23

What is the most common type of diabetic neuropathy?

Answer 23

distal sensory or sensorimotor polyneuropathy

Question 24

Name some autonomic dysfunction seen in association with diabetic neuropathy.

Answer 24

• postural HTN
• diarrhea
• impotence
• urinary retention
• increased sweating

Question 25

_____ is characterized by acute onset of asymmetrical proximal weakness and pain in the legs.

Answer 25

Lumbosacral plexopathy

Question 26

Lumbosacral plexopathy is characterized by?

Answer 26

acute onset of asymmetrical proximal weakness and pain in the legs