Exam 3 Flashcards
1
Q
Normal Muscle Biopsy
A
- mosaic pattern
- muscles may have proportionately more fast-glycolytic (FG) muscles fibers as compared to slow, oxidative (SO) secondary to its function
2
Q
Neurogenic Diseases
A
- involves nerve cell bodies, axons and/or dendrites
- needle EMG
3
Q
Needle EMG for Neurogenic Diseases
A
- denervated muscle is spontaneously active at rest (fibrillation potentials)
- short duration (<3 ms) and low amplitude (<300 uv), fibrillation potentials occur in semi-rhythmical runs (<30/sec)
4
Q
Muscle biopsy of neurogenic disease
A
- neuron atrophy causes muscle atrophy of the same muscle fiber type
- regernation occurs through collateral sprouting
- since motor neuron type determines muscle fiber type, re-innervated muscle fibers assume histochemical properties of neuron (change fiber type)
- results in muscle fiber type grouping due to re-innervation (as opposed to the mosaic pattern of normal muscle)
5
Q
Myogenic diseases
A
- myogenic or myopathies involve muscle degeneration
- little/less muscle activity at rest with a needle EMG
6
Q
Muscle biopsy of Myogenic diseases
A
- muscle fiber atrophy in a random fashion
- all muscle fiber types are affected
- retains the mosaic pattern but fewer overall number and size of all types of muscle fibers
7
Q
Examples of neurogenic diseases
A
- compression of nerve roots or periopheral nerves
- bell’s palsy
- diabetic plyneuropathy
- alcoholic poluneuropathy
- polio and post-polio syndrome
- amyotrophic lateral sclerosis (ALS)
- Guillian-Barre syndrome (GB)
8
Q
Demylinating diseases
A
- multiple sclerosis
9
Q
Myogenic diseases
A
- Muscular Dystrophy
10
Q
Bell’s Palsy
A
- lesion of CN VII
- demylination in mild cases
- demyelination and axonal damage in more severe cases
- innervation to upper face is bilateral
- “a” is Bell’s Palsy
- “b” is stroke
- person is asked to smile and close eyes
- Bell’s Palsy typically presents as flaccid paralysis on the ipsilateral side of face, affecting the upper and lower quadrants of the face
11
Q
Bell’s Palsy Etiology
A
- Unknown in most cases
- may be secondary to viral infection causing swelling in auditory canal (remember that CN VII goes through the auditory canal)
- in a small number of cases, secondary to acoustic neuroma impinging on nerve
12
Q
Motor S&S of Bell’s Palsy
A
- mouth droops
- nasolabial fold is flattened
- eyelid does not close
13
Q
Sensory S&S of Bell’s Palsy
A
- decreased taste on ipsilateral tongue
14
Q
ANS S&S of Bell’s Palsy
A
- decreased tearing (dry eye)
15
Q
Medical Treatment for Bell’s Palsy
A
- high-dose corticosteroids for 5 days followed by a tapered dose for another 5 days
- antiviral medications (e.g. acyclovir)
- eye patch, artificial tears (eye drops every 4 hrs)
- gentle massage and gentle heat occasionally used
- PT for muscle retraining only if problems persist (E-stim for facial muscles)
- 70% of people completely recover within 2-3 weeks (in simple cases ) or 3-6 months (in severe cases)
16
Q
Diabetic Polyneuropathy sensory loss
A
- sensory loss is not usually in dermatome or periphery pattern
- usually in sock or glove-like patterns
17
Q
Diabetic Polyneuropathy
A
- affects PNS axons primarily (some demyelination)
18
Q
Diabetic Polyneuropathy Etiology
A
- disrupted microcirculation
19
Q
Diabetic Polyneuropathy Onset
A
- after long duration diabetes
- in diabetics who have diabetes for 25+ years, 50% have this condition
- occurs in insulin-dependent and non-insulin dependent diabetes
- some regeneration with control of diabetes
- common in those who are not routinely caring for their diabetes
20
Q
Diabetic Polyneuropathy characteristics
A
- large nerve fiber sensory involvement of MANY NERVES IN LOWER LEGS AND FEET
- Ia, Ib, II from mm spindle and GTOS
- most common
- painless paresthesias in BILATERAL feet and lower legs
- decreased vibration and proprioception sense
- small nerve fiber sensory involvement (A delta and C afferent fibers)
- deep aching pain in legs and burning feeling in feet
- decreased touch, pain, and temp sensations
- nocturnal pain and paresthesias
21
Q
Screening/Examination Tests for Diabetic Polyneuropathy
A
- NCVs
- Monofilament screening with 5.07/10 gm. filament
- vibration
22
Q
Complications with Diabetic Polyneuropathy
A
- diabetic ulcers
- 50% of non-traumatic amputations in US are performed on individuals with diabetes
23
Q
Diabetic PERIPHERAL Neuropathy S&S
A
- numbness or reduced ability to feel pain or changes in temp, esp in feet/toes
- serious foot problems, such as ulcers, infections, deformities, and bone and joint pain
- a tingling or burning feeling
- sharp, jabbing pain that may be worse at night
- pain when walking
- extreme sensitivity to the lightest touch
- muscle weakness and difficulty walking
24
Q
ALS
A
- amyotrophic lateral sclerosis
- lou gehrig’s diseases
25
Q
LMN Signs for ALS
A
- anterior horn cells (alpha and gamma motor neurons (LMN SIGNS))
- lateral corticospinal tract
- motor nuclei of brainstem
- motor area of frontal lobe
- loss of function in hands in feet starts DISTAL and progresses PROXIMAL
26
Q
UMN Signs for ALS
A
- anterior horn cells
- lateral corticospinal tract
- motor nuclei of brainstem (red nucleus)
- motor area of frontal lobe
27
Q
ALS is caused by what
A
- atrophy/degeneration of cell bodies in ventral grey horn
- therefore degenerating the grey matter in the SC
28
Q
ALS S&S
A
- motor signs and symptoms only
- asymmetric, distal weakness (first sign)
- by time patient complain of weakness, often have lost 80% of their ALPHA MOTOR NEURONS in area of weakness
- EMG: spontan fibrillations, fasciculations with giant unit spikes upon voluntary activity
29
Q
ALS
A
- primary pathologic defect is in the motor neuron cell body (specific to anterior horn cell in SC)
- motor cells in the brainstem motor nuclei (cranial nerves)
- pyramidal cells in the primary motor cortex thus loss of the upper motor neurons in corticospinal tracts (Betz cells)
30
Q
Theory as to why ALS occurs
A
- technically unknown
- could be due to mitochondria
- SOD1 gene mutation causing the superoxide disputes (SOD) enzyme to function improperly
31
Q
Other possible causes for ALS
A
- glutamate
- premature aging
- exogenous toxins
- viral diseases
- immunologic distrubances
- premature aging
32
Q
How many ALS cases are familal
A
- 5-10% of cases
33
Q
ALS stats
A
- 0.1% of deaths in US
- ALS disease of late middle life and rarely seen prior to age 40
- 90% of all cases begin between the ages of 40 and 70 years
- slight predominance in men
34
Q
What is the first sign of ALS usually?
A
- asymmetric, distal weakness
35
Q
What are the early signs of ALS?
A
- severe muscle cramps
- fasciculations at rest
36
Q
ALS signs and symptoms progression
A
- progress to muscle atrophy and severe muscle weakness
- decrease in number of muscle fibers, type II fibers tend to atrophy earlier and more rapidly than type 1 fibers
- cranial nerve cell atrophy leads to dysarthria, dysphagia, difficulty in chewing, tongue weakness, and fasciculations may be prominent
- control of bladder, bowel, and autonomic function is largely unimpaired, however, some studies report sub-clinical involvement
37
Q
LMN signs of ALS
A
- Progressive muscle atrophy
- Weakness (asymmetric weakness is often a presenting sign)
- Fasciculations (especially evident in tongue, deltoid)
- Muscle cramps (painful
38
Q
UMN signs of ALS
A
- Spasticity
- Hyperreflexia
- Positive Babinski
39
Q
ALS Stats
A
- Death in 2-5 years commonly from respiratory compromise
- Life expectancy of a patient with ALS can vary from less than 1 year to more than a decade with ventilator support
- Average survival rate is 4.1 years
- 20% survive more than 5 years
- Those who have ALS before age 50 generally live longer
- Those that have early CN involvement usually live less years post-Dx
- Stephen Hawkins is the exception
40
Q
ALS Brief Intervention
A
- General care revolves around the relief of discomfort and minimization of musculoskeletal, integumentary, and systemic effects
- Problems with swallowing, speech, postural control, and respiration are common so that equipment/therapy must be targeted to these needs (IPE – team approach)
- Therapy is directed at preventing contractures, skeletal deformity and/or respiratory complications, maintaining activity level, and recommending and issuing equipment as needed.
- Muscle spasms – stretching, increased movement, quinine or baclofen
- Diet – anti-oxidants – vitamins E and C
- In general, encourage a balance between activity and rest. “Do no harm” philosophy.
- If more than one third of the motor units are intact, exercise led to hypertrophy. If less than one third of the motor units are intact, vigorous exercise damaged the muscles
- If the patient shows evidence of significant, persistent weakness following institution of an exercise program or persistent morning fatigue, the therapist must redesign the HEP and monitor the patient’s activity level and response to HEP carefully
41
Q
PALS
A
- organization with equipment closet
42
Q
Guillain-Barre Syndrome (GBS) History
A
- named after the french physicians Georges Guillain and Jean Alexandre Barre, 1916
- an acute polyneuropathy affecting the peripheral nervous system
- may be autoimmune attack on both the schwann cells and peripheral and cranial nerves by circulating antibodies
43
Q
GBS
A
- acute: develops rapidly
- often follows the flu or respiratory infection
- may be idiopathic
- is rare (1-2 cases per 100,000 people annually), but is most common cause of acute non-trauma paralysis
44
Q
GBS
A
- characterized by symmetrical weakness that usually affects the lower limbs first, and rapidly progresses in an ascending fashion.
- Individuals generally notice weakness in their legs, manifesting as “rubbery legs” or legs that tend to buckle, with or without numbness or tingling (“Jeff”)
- As the weakness progresses upward, usually over periods of hours to days, the arms and facial muscles also become affected.
45
Q
GBS S&S
A
- Frequently, the cranial nerves may be affected, leading to; Oropharyngeal dysphagia (drooling, or difficulty swallowing and/or maintaining an open airway)
- Respiratory complications: Can cause life-threatening complications, in particular if the respiratory muscles are affected or if the autonomic nervous system is involved
- Most patients require hospitalization and about 30% require ventilator assistance
- Facial weakness is also common
- Sensory loss, if present, usually takes the form of loss of proprioception
- Pain is a common symptom in GBS, presenting as deep aching pain, usually in the weakened muscles
- In severe cases of GBS, loss of autonomic function is common, manifesting as wide fluctuations in blood pressure, orthostatic hypotension, cardiac arrhythmias
46
Q
GBS Prevalence
A
- can occur at any age
- mostly 5-8th decade
- more common in men than woman
47
Q
GBS Disease Course
A
- maximal onset in less than 4 weeks (often in a few days)
- static phase (plateau of 2-4 weeks)
- recovery takes months to years
- recurs in 10% of cases
48
Q
GBS Mortality rate
A
- 5% relatively low mortality rate
49
Q
GBS Has poorer prognosis with what
A
- onset at older age
- extended time before recovery begins
- need for artificial respiration
50
Q
Time frame with recovery in GBS
A
- at 6 months, 85% are ambulatory
- at 1 year, 20% remain significantly handicapped by weakness
- at 2 years, 8% have NOT achieved full recovery
51
Q
GBS Diagnosis
A
- nerve conduction studies
- studies of the cerebrospinal fluid
52
Q
GBS Intervention
A
- supportive medical care
- plasmaphoresis: filtering blood plasma to remove circulating antibodies responsible for destruction of Schwann cells and peripheral and cranial nerves
- Intravenous Immunoglobins
53
Q
MS Prevalence
A
- more than 2.3 million people worldwide
- female > male (2-3X more)
- predominantly caucasians
- high frequency: northern US, scandinavian and European countries
54
Q
Age with MS
A
- Most people are diagnosed between the ages of 20 and 50
- Although MS can occur in young children and significantly older adults
