Olol Pa Neurology

Front 1

Neuromuscular diseases (NMDs) are characterized by disorders of the ___ unit and of the ___ and ___ nerves.

Back 1

motor unit and of the sensory and autonomic peripheral nerves.

Front 2

A motor unit consists of a motor neuron ___ found in the spinal cord anterior horn or cranial nerve nucleus.
The ___ of the motor neuron is found in the peripheral or cranial nerve.
What is innervated by the motor neuron?

Back 2

-cell body
-axon
-muscle fibers

Front 3

What type of nerves are comprised of a sensory neuron cell body in the dorsal root ganglion, a central axon passing to the spinal cord in the post. root, distal axon in the peripheral nerve, and terminate in skin, muscle, joint capsule, and other structures?

Back 3

Sensory peripheral nerves

Front 4

Decreased sensation (hypoesthesia, hypoalgesia), abnormal sensation (parasthesia), or painful sensations (dysesthesia) are signs and symptoms of ____ disease.

Muscle weakness, wasting, fatigue, cramps, fasiculation, pain, and stiffness are signs and Sx's of ___ disease.

Back 4

-Peripheral nerve disease

-Motor neuron disease

Front 5

Postural dizziness, abnormal cardiac, visceral, and ocular functions, and changes in sweating are signs and Sx's of ___ disease.

Back 5

-Autonomic nervous system disease

Front 6

Sx's of NMD do not always distinguish what type of disorders from each other?

Most Sx's of NMD are ___, whereas focal CNS diseases are usually ___.

Back 6

-Peripheral nervous system disorders from disorders of the CNS

-Symmetrical
-asymmetrical

Front 7

___ are located in the brain stem and ventral spinal cord.

___ are locatd in the motor cortex and terminate in the medulla or spinal cord.

The most common acquired motor neuron disease is ____.

Back 7

-Lower motor neurons (LMNs)
-Upper motor neurons (UMNs)

-Amyotrophic lateral scherosis

Front 8

Decreased strength, tone, and reflexes with fasiculations and atrophy are signs of ___ motor neuron disease.
While ___ and ___ are signs of an upper motor neuron disease.

Back 8

-Lower

-Spacticity and exaggerated reflexes

Front 9

What enzyme levels can be checked and will be elevated with NMDs?

___ causes creatine kinase (CK) to become elevated in the serum.
What disorders would CK levels be normal?

Back 9

-CK (creatine kinase)
-LDH (lactic dehydrogenase)
-AST (aspartate aminotransferase)
-ALT (alanine aminotrnsferase)

-Muscle damage

-Peripheral neuropathies and neuromuscular junction disorders

Front 10

Electromyography is a measurement of ____ arising from muscle fibers.
Normal muscle is silent at rest and spontaneous activity such as ___, ___, and ___ occurs during complete relaxation.

A spontaneous activity in a single muscle fiber is called a ____, while a ___ involves the entire motor unit.

Back 10

-electrical activity

-Myotonic disorders, inflammatory myopathies, and denervated muscles

-Fibrillation, fasiculation

Front 11

During a nerve conduction study, AP's are recorded more proximally in ___ type of nerve fibers and over the muscle distally in ___ fibers in a mixed motor sensory nerve.

Back 11

Large sensory nerve fibers

motor fibers

Front 12

When talking about diseases of the motor neuron, if only the lower motor neuron is involved, the term ____ is used.

SMAs are ___ and ___ type of disorders that begin in utero, infancy, childhood, or adulthood.
This disease is produced by a defect in ____.
The 3 types of SMA have what type of genetic inheritence?

Back 12

-Spinal muscular atrophy (SMA)

-Progressve and inherited
-Neuronal apoptosis

-Autosomal recessive

Front 13

___ is a disease of the motor neuron that alters the function of bulbar and spinal motor neurons (EX: bulbar cerebellum, medulla, and pons)

THe bulbar cerebellum refers to the ___ part of the brain.

The mean onset age is ___

Back 13

-Bulbospinal muscular atrophy

-Brain stem

-30 yo

Front 14

Pts with bulbospinal muscular atrophy experience what type of weaknesses?

50% of pts can have ___. (HINT: happens in men)

Symptoms that involve the mouth are...
Pts also experience widespread ____.

Back 14

-Facial, tongue, and proximal weakness
-Gynecomastia in 50%
-Dysphagia and dysarthria
-Widespread fasiculations

Front 15

Amyotrophic Lateral Sclerosis is also called ___ and involves the death of ___ for unknown reasons.

ALS leads to progressive loss of ___ and ___ is replaced by hardened tissue.

Back 15

-Lou Gerhig's Dz
-upper and lower motor neurons

-ability to move any muscles in the body
-healthy tissues

Front 16

T/F...

ALS can be acquired or inherited?

Which type is more common?

Back 16

-True

-Acquired

Front 17

Sporadic ALS accounts for 80% of all cases of ____ motor neuron disease.

Sporadic ALS can present with what type of weakness?

Other symptoms that sporadic ALS can present with?

Back 17

-acquired

-Painless, progressive weakness that is usually focal then spreads to contiguous muscle groups

-Difficulty swallowing, chewing, coughing, breathing
-Muscle atrophy/fasiculations
-Spacticity
-Hyperreflexia (upper motor neuron sign)
-Progressive muscle wasting and weakness (lower motor neuron sign)

Front 18

The inherited form of ALS called ___.

T/F...
FALS is clinically and pathologically identical to sporadic ALS?

Back 18

-Familial ALS, which is autosomal dominant

-True

Front 19

ALS is a relentlessly progressive disease that culminates in ____.
____ is the drug used in treatment of ALS, but may only slightly delay progression of the disease.

ALS is usually fatal within ___ yrs, especially in pts with ___ involvement b/c they have the poorest prognosis.

Back 19

-Respiratory muscle paralysis

-Riluzole

-3-5 yrs, bulbar involvement

Front 20

Cronic partial denervation with abnormal spontaneous activity in the resting muscle and decreased motor units under voluntary control can be seen in what type of study?

In SMA or ALS, a diagnosis can be made only if the above changes are found in at least __ regions OR ___ and ___.

Back 20

-EMG

-At least 3 spinal regions OR 2 spinal regions and bulbar musculature

Front 21

Other lab studies:

___ shows changes in denervation.
___ may be slightly elevated.
___ is normal.

Back 21

-Muscle biopsy
-CK (serum)
-CSF

Front 22

Treatment:

___ may slow progression of ALS, while ___ can help with drooling.

Braces and walkers for ___.
PT for ___, diazepam for ___.

What can be done to treat feeding or swallowing problems?

Back 22

-Riluzole, anticholinerinergics

-Mobility
-contractures, spasticity

-Liquid diet or NG tube