Anterior Horn Cell and Peripheral Nerve Disorders Flashcards Preview

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Flashcards in Anterior Horn Cell and Peripheral Nerve Disorders Deck (26)
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1
Q

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

A

Upper motor neuron (UMN)

2
Q

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

A
  • spastic tone
  • hyperactive tendon reflexes
  • pathological reflexes (ie Babinski)
  • emotional lability (inappropriate laughing and crying)
3
Q

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

A

Lower motor neuron (LMN)

4
Q

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

A
  • muscle atrophy
  • fasciculations
  • diminished tone
  • reduced or absent reflexes
5
Q

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

A
  • a nerve root disorder
  • plexus disorder
  • peripheral nerve disorder
6
Q

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

A

neuromuscular junction disorders

7
Q

Name 3 distribution patterns of weakness.

A
  • proximal
  • distal
  • cranial
8
Q

How is a neuromuscular disorder different than normal atrophy?

A
  • in normal atrophy, the muscle remains strong
  • In an NMJ disorder, the muscle is strikingly weak
9
Q

Name 4 anterior horn cell disorders.

A
  1. amyotrophic lateral sclerosis (ALS)
  2. spinal muscular atrophy
  3. Poliomyelitis
  4. West Nile virus
10
Q

Name 2 radiculopathies and plexopathies.

A
  1. cervical and lumbosacral nerve root compression
  2. brachial and lumbosacral plexopathies
11
Q

Name 3 types of peripheral neuropathies.

A
  1. mononeuropathy
  2. mononeuropathy multiplex
  3. polyneuropathy
12
Q

Name 4 neuromuscular junction disorders.

A
  1. Myasthenia gravis
  2. Lambert Eaton syndrome
  3. Botulism
  4. Organophosphate poisoning
13
Q

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
A

Amyotrophic lateral sclerosis (ALS)

14
Q

What is the average survival after diagnosis of ALS?

A

3-4 years

15
Q

How is ALS treated?

A
  • primarily symptomatic
  • Ruluzole
  • braces and durable medical equipment
16
Q

How does someone get Charcot-Marie Tooth disease?

A

it’s inherited

17
Q

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

A

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

18
Q

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

A

Charcot-Marie Tooth disease type 1A (CMT1A)

19
Q

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

A
  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
20
Q

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

A

38m/sec

21
Q

What is the medication tx for CMT?

A

none, just pain mgmt

22
Q

_____% of diabetics have diabetic neuropathy.

A

50-60%

23
Q

What is the most common type of diabetic neuropathy?

A

distal sensory or sensorimotor polyneuropathy

24
Q

Name some autonomic dysfunction seen in association with diabetic neuropathy.

A
  • postural HTN
  • diarrhea
  • impotence
  • urinary retention
  • increased sweating
25
Q

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

A

Lumbosacral plexopathy

26
Q

Lumbosacral plexopathy is characterized by?

A

acute onset of asymmetrical proximal weakness and pain in the legs