LEC-10 Neuromuscular Diseases

Question 1

Lambert Eaton myasthenic syndrome is typically a paraneoplastic syndrome, with ______________________ cancer being the most commonly associated malignancy.

Answer 1

Small cell lung cancer

Question 2

How do patients with peripheral nerve disease present?

Answer 2

I. Numbness
II. Impaired vibration perception
III. Atrophy of small muscles in hands and feet
IV. Ataxia
V. Pain

Question 3

Reduced muscle tone, depressed tendon and superficial reflexes, along with weakness that is patchy in distribution may signify a problem or disorder at the _________________.

Answer 3

Neuromuscular junction

Question 4

______________________ is the loss of motor neurons in the anterior horn and corticospinal tracts. It is most commonly caused by a loss of the enzyme superoxide dismutase 1.

Answer 4

Amyotrophic Lateral Sclerosis, ALS or Lou Gehrig’s disease

Question 5

ALS may be treated by administration of the drug _____________.

Answer 5

Riluzole

Lou Gehrig disease = riLOUzole

Question 6

________________ is the autosomal recessive, most common form of an inherited motor neuron disease.

Answer 6

Spinal muscular atrophy

Question 7

Spinal muscular atrophy that onsets in infancy is known as (Werdnig Hoffman disease/Kugelberg Welander disease).

Answer 7

Werdnig Hoffman disease

Question 8

Spinal muscular atrophy that onsets in adolescence is known as (Werdnig Hoffman disease/Kugelberg Welander disease).

Answer 8

Kugelberg Welander disease

Question 9

A peripheral nerve disorder in which the pattern of weakness conforms to the distribution of a single nerve is known as a(n) __________________.

Answer 9

Mononeuropathy

E.g. Carpal tunnel - The weakness follows the distribution of the median nerve.

Question 10

A peripheral nerve disorder in which multiple nerves are affected in a seemingly random pattern, has acute onset, and is frequently painful to the patient is known as ________________.

Answer 10

Mononeuritis multiplex

Question 11

(T/F) Sensory symptoms in a polyneuropathy first start in the feet and move proximally. Hand symptoms then appear when lower extremity symptoms reach the knees.

Answer 11

True

Question 12

Guillain Barre syndrome is an example of a (mononeuropathy/polyneuropathy).

Answer 12

Polyneuropathy.

Question 13

What are the three types of Myasthenia Gravis antibody to ACh receptor?

Answer 13

I. Binding
II. Blocking
III. Modulating

Question 14

ACh receptor antibody is detected in __-__% of patients with generalized Myasthenia Gravis.

Answer 14

90-95%

Question 15

In patients with myasthenia gravis, symptoms tend to (improve/worsen) with exercise.

Answer 15

Worsen

Question 16

_____________________ drugs may be given to patients with myasthenia gravis.

Answer 16

Acetylcholinesterase inhibitor drugs

Question 17

(T/F) One of the primary distinguishing features between botulism and Myasthenia Gravis is that botulism pathology affects the pupils, while Myasthenia Gravis does not.

Answer 17

True.

Question 18

Lambert Eaton myasthenic syndrome is a (presynaptic/postsynaptic) complication, while Myasthenia Gravis is a (presynaptic/postsynaptic) complication.

Answer 18

Lambert Eaton myasthenic syndrome - Presynaptic

Myasthenia Gravis - Postsynaptic

Question 19

Antibodies in Lambert Eaton myasthenic syndrome target ___________________. This impedes the release of vesicles containing ACh.

Answer 19

voltage-gated calcium channels

Question 20

(Duchenne muscular dystrophy/Myotonic dystrophy) is the most common of the dystrophies.

Answer 20

Myotonic dystrophy

Question 21

(T/F) Myotonic dystrophy causes failure of the muscle to relax after contraction, often resulting in prolonged hand contractions or hands “getting stuck”.

Answer 21

True.

Question 22

How do patients with upper motor neuron lesions present?

Answer 22

Weakness or paralysis
Spasticity (increased muscle tone)
Increased reflexes
An extensor plantar (Babinski) response
Loss of superficial abdominal reflexes
Little, if any, muscle atrophy

Question 23

How do patients with lower motor neuron lesions present?

Answer 23

Weakness or paralysis (at level of anterior horn)
Wasting and fasciculations (not immediate after insult)
Hypotonia (flaccidity); immediately after insult
Normal abdominal and plantar reflexes
No increased tone
No pathological reflexes (no Babinski’s sign)