Neuro: Test 1 : 4. Cordingley : Neuromuscular Disease

Front 1

Clumsiness in greater proportion than loss of power
Babinski sign present (immediate)
Spasticity (delayed)
Increased tendon reflexes (delayed)
Little if any atrophy

what type of lesion?

(o)

Back 1

Upper motor neuron lesion

Front 2

what is Spasticity...what is it associated with (upper or lower mn)

(o)

Back 2

A unidirectional increase in muscular tone

Involves increased tone in “anti-gravity” muscles

In the upper extremities, resistance to stretching out biceps, but not triceps
In the lower extremities, resistance to stretching out quadriceps but not hamstrings

seen in upper motor neuron

Front 3

What is Rigidity? what is it seen in?

(o)

Back 3

Seen in Parkinson’s disease and other extrapyramidal disorders

A bi-directional increase in tone to passive range of motion

The resistance to passive range of motion is relatively independent of degree of force or rate of speed

Front 4

What is Paratonia? commonly seen in?

(o)

Back 4

feels like the pt is trying to stop you from moving the limb

Commonly seen in dementia

Also known as gegenhalten (to hold against)

A bidirectional resistance to passive range of motion that increases with force and rate of movement, as if voluntary

Front 5

Loss of power in greater proportion than clumsiness
Hypotonia
Muscle atrophy
Muscle fasciculations

what type of lesion?

(o)

Back 5

Lower Motoneuron

Front 6

Compare Duchenne, Facioscapulohumeral, and Limb girdle Muscular dystrophies for the following characteristics:
Age of onset
Sex
Pseudohypertorphy
Initial distribution
Involvement of the face
Rate of progression
Contractures and deformity
Inheritance

(o)

Back 6

Front 7

what is the genetic defect in duchenne muscular dystrophy?

Back 7

Dystrophin gene on short arm of X chromosome codes for dystrophin protein, which is a cytoskeletal structural protein.

this is damaged

Front 8

a boy presents to your office and on physical exam you note that their calves are pretty toned. Mom says that when the boy is on the ground he does this strange thing where he arches his back and climbs his way off the ground...what is this called? sign of?

Back 8

Gower's sign

in Duchenne

Front 9

you are in your neuro office and examining a young lady patient who has issues with smiling. You ask her to smile and she physically can't. Her forehead is unwrinkeld and the corners of her mouth do not curl. On physical exam, you note winging of the scapula. What does this pt have?

Back 9

Facioscapulohumeral Muscular Dystrophy

One key gene is on the long arm of chromosome 4. The function of the gene product is as yet unknown.

Front 10

a man presents to you with a seemingly triangular face. On a hunch, you hit their hypothenar eminence with your reflex hammer and it causes their thumb to move towards their pinky. To confirm what you think, you have the pt squeeze your hand and tell them to open it as soon as you tell them. When you say go, their hand stays in a grip...what do they have? What is the genetics going on?

Back 10

Myotonic Dystrophy
A gene near the centromere of chromosome 19 codes for myotonic dystrophy protein kinase. In M.D. dozens to hundreds of “CTG” repeats occur in the gene. The transcribed mRNA gets trapped within the cell’s nucleus.

Front 11

what happens in Myasthenia gravis physiologically?

(o)

Back 11

antibodies to the AChR

Front 12

pt presents to your office with muscle weakness. They say they are fine in the morning, but by the end of the day they have drooping eyelids, jaw drop, and drooling...what do they likely have?

Back 12

MG

Front 13

what are some of the associated dz with MG? what is the most important one?

Back 13

THYMOMA (malignant mediastinal tumor)

thyroid disease

widespread immune complex disease

Front 14

what is a polyneuropathy

(o)

Back 14

Bilaterally symmetric
Distinct from mononeuropathy and multiple mononeuropathies

so the key is that you have to have the deficit on the same spot on both sides

mulitple mononeuorpathies would be like radial on one side, ulnar on the other

poly is like whole left and right hand (stocking and glove is a common characteristic of polyneuopathy)

Front 15

Distal-greater-than-proximal weakness
Distal-greater-than-proximal numbness
A “stocking-glove” pattern of weakness, numbness or both
“Length-dependent” neuropathy
Disrupts DTRs more than other forms of lower-motoneuron weakness

this describes?

(o)

Back 15

Polyneuropathy

the longer the axon the worse it is affected

Front 16

Werdnig-Hoffman is a motoneuron disease affecting what population?

*

Back 16

NEONATES

you will see characteristic FROG LEG position and floppy baby
(other thing that causes floppy baby? Botulin toxin)

BABIES WILL NOT HAVE REFLEXES

Front 17

a 60 year old man presents to your office and says that he cannot do routine tasks such as going up steps, getting out of a chair, or swallowing. You note widespread atrophy. What do you suspect...

Back 17

Amyotrophic Lateral Sclerosis

ALS

Front 18

Back 18

Front 19

of the following, which will have a MARKEDLY increase CK level?

Myopathy
MG
Polyneuropathy
Motorneuron disease

Back 19

Myopathy

(think duchenne's muscular dystrophy)