Peripheral Nervous System Disorders II Flashcards

1
Q

What is Bells palsy/facial nerve paralysis

A
  • Unilateral facial paralysis due to compression of CN VII caused by inflammation & swelling
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

Incidence of Bells palsy/facial nerve paralysis

A
  • Develops suddenly over hrs to days
  • Causes one side of face to droop, become stiff over time
  • Mostly temporary
  • Affects 20-30 per 100,000 people/year
  • Common age group 15-45 yrs
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

Etiology and pathology of Bells palsy

A
  • Suspected to be triggered by viral infections but lacks direct evidence, many cases lack signs of acute viral infection preceding onset
  • More likely caused by re-activation of a latent virus rather than direct infection
  • Most likely caused by herpes simplex/zoster, mumps, rubella
  • Inflammation of the nerve causes compression that could lead to ischemia, demyelination, & axonal degeneration
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

Risk factors of Bells palsy

A
  • DM
  • Pregnancy
  • Hypothyroidism
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

Describe the facial nerve course

A
  • Passes through long bony canal causing increased risks for compression with inflammation & swelling
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

Clinical manifestations of Bells palsy

A
  • Unilateral facial paralysis, asymmetrical facial appearance
  • Sx develop rapidly, often overnight
  • Corner of mouth droops, nasolabial fold flattens, eyelids do not close
  • CN VII also innervates stapedius muscle, receive taste sensation & provides autonomic innervation for salivation & lacrimation
  • Compression proximal to chorda tympani branch can cause loss of taste
  • If stapedius muscle innervation lost = sounds become louder
  • Autonomic involvement causes dry/red eyes (lack. of tearing), thicker saliva
  • Severe/chronic = pain, contractures, semi-facial spasms
  • Bell’s reflex: rolling up of eyeball during attempts to close eye
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

After severe injury axonal regrowth/rewiring could be misdirected & can cause

A
  • Synkinesis: involuntary & undesirable facial movements associated with voluntary facial movements
  • Voluntary activation of one muscle group can activate other muscles (blinking can activate mouth twitch & smelling can cause blink
  • Autonomic misdirection can also happen: involuntary lacrimation while eating (crocodile tears)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

Describe how to diagnose Bells palsy

A
  • Observation & physical exam
  • Ask patient to wrinkle forehead, close eyes tightly, smile, whistle and observe for symmetry
  • Electrodiagnostic tests: NVC/EMG can determine demyelination vs axonal degeneration
  • NVC/EMG: Amplitude 90% drop = less than complete recovery; loss >98% = significant residual weakness & synkinesis
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

Differential diagnosis between Bells palsy(facial nerve) and a stroke

A
  • Observe forehead wrinkle, eye closing, & voluntary smiling
  • Facial nerve/Bells palsy will effect one whole side of face while a stroke will only effect the lower half of face
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

House Brackmann grading scale for Bells palsy severity categorization

A
  • (1) Normal = normal facial function in all areas
  • (2) Mild dysfunction = slight weakness noticeable only on close inspection; no synkinesis, contracture, or hemifacial spasm
  • (3) Moderate dysfunction = pts who have obvious but no disfiguring synkinesis, contracture, and/or hemifacial spasm. are grade III. regardless of degree of motor activity
  • (4) Moderately severe dysfunction = pts with synkinesis, mass action, and/or hemifacial spasm severe enough to interfere with function are grade IV regardless of motor activity
  • (5) Severe dysfunction = only barely perceptible motion; synkinesis, contracture, and hemifacial spasm usually absent
  • (6) Total paralysis = loss of tone, no motion
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Treatment for Bells palsy

A
  • Early Tx with corticosteroids: anti-inflammatory effect can reduce the swelling of the facial nerve/compression in the facial canal
  • General care includes proper eye care using artificial tears & ophthalmic ointment to prevent corneal abrasion & ulceration
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Prognosis for bells palsy

A
  • Favorable that 85% show recovery within 3 wks
  • 71% show. full recovery
  • Degree of severity at onset is an important factor
  • EMG results: A 90% drop in amplitude predicts less than complete recovery, and loss greater than 98% predicts significant residual weakness and synkinesis
  • Absence of improvements at 3-4 mo should raise concerns regarding diagnosis & lead to search for alternative diagnoses
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Bells palsy implications for PT

A
  • Neuromuscular re-ed using EMG biofeedback to regain motor control: helpful for synkinesis Tx
  • Muscle stretches & exercises like wrinkling, puffing, closing eyes tightly, smiling, chin tuck, etc. can help maintain flexibility, decrease pain, & improve circulation in the affected muscles
  • Facial E-stim lacks much evidence
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

What is Charcot Marie Tooth disease

A
  • Inherited motor & sensory neuropathy (HMSN)
  • Slowly progressive disorder
  • Characterized by distal limb weakness & wasting, skeletal deformities, distal sensory loss & reduced DTRs
  • Fibular (peroneal) nerve is frequently affected 1st, aka perineal muscular atrophy (PMA)
  • Progressive muscular atrophy foot.lower legs, progressing to hands/forearms
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Etiology and pathology of Charcot Marie Tooth disease

A
  • Relatively common neuropathy
  • Mostly autosomal dominant pattern of genetic mutation (autosomal recessive is rare)
  • 2 genetic subtypes: CMT1 & 2
  • Frequent de-/re- myelination causes hypertrophic ‘onion bulb’ formation around affected axon segments that contains Schwann cells & its processes (creates palpable areas along the peripheral nerves)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

Describe CMT1 and CMT2

A
  • CMT1: causes mutations that are involved with segmental demyelination
  • CMT2: disrupt neurofilament assembly causing axonal dysfunction/degeneration
17
Q

Clinical manifestations of Charcot Marie Tooth disease

A
  • Distal symmetric muscle weakness, atrophy, diminished DTRs
  • Fibula’s muscles affected causing weakness & wasting of DF/evertors, intrinsic muscles of foot
  • As disease progresses: weakness/wasting of intrinsic hand muscles
  • Sx. progress proximally
  • Loss of proprioception/vibration followed by decrease pain/temp., parasthesia in glove & stocking distribution
  • Balance and gait problems
18
Q

Foot deformities of Charcot Marie Tooth disease

A
  • Inverted champagne bottle appearance of lower leg
  • Pes cavus (high arch): imbalance b/w TA and peroneus longus
  • Clawing of great toe
  • Hammer toes
19
Q

How to diagnose Charcot Marie Tooth disease

A
  • History: hereditary picture
  • Lab tests: gel electrophoresis to detect genetic variations
  • Physical motor & sensory exam
  • NVC/EMG
  • Nerve biopsy can demo demyelination or axonal degeneration
20
Q

Treatment for Charcot Marie Tooth disease

A
  • No treatment to alter course of disease
  • Symptomatic treatment for now
  • If left unmanaged: can further deteriorate gait pattern due to increasing deformities, can increase fall risk, can also develop problems with writing & handling objects
  • Progesterone therapy combined with ascorbic acid have been found to have positive effects in pre-clinical studies
  • Gene therapy, stem cells, pharmacologic agents targeting mutated genes: all currently under research
21
Q

Prognosis for Charcot Marie Tooth disease

A
  • Normal life expectancy, usually not life threatening and rarely affects muscles involved in vital functions like breathing
22
Q

Charcot Marie Tooth disease implications for PT

A
  • Goal is to maintain functional mobility & minimize deformities
  • Therapist should anticipate deformities due to muscle imbalance b/w Tim ant/per longus and Tim post/per brevis, which causes pes cavus & inversion of foot (tip posterior stays unaffected)
  • Long term stretching recommended to maintain ROM: need to be more than 7 mo
  • Skin care precautions to prevent sores
  • Strengthening ex will have limited long term beenfit due to progressing axonal degeneration
  • Balance/gait training: balance walking poles
  • Orthotics for foot & hand can help in long term: AFOs w/ or a/o inserts/adjustments to protect skin & provide adequate support to deformed structures for improving gait & balance
23
Q

What is diabetic neuropathy

A
  • Metabolic neuropathy occurring in the setting of DM without evidence of other causes of peripheral neuropathy
  • Heterogeneous group of progressive syndromes
  • Diverse clinical presentations: neuropathy can be diffuse or focal. (poly- or mono- neuropathy), may include autonomic and/or somatic PNs (peripheral nerves???)
24
Q

Incidence of diabetic neuropathy

A
  • 20 million people affected by DM, expected to increase by 5% every year
  • Neuropathy more in DM type 1 than type 2
25
Q

What are the different ways hyperglycemia damages the nerves

A
  • Chronic hyperglycemia causes metabolic disturbances that damages nerve cells, Schwann cells, and can effect both myelinated/unmyelinated axons, distal nerves affected more
  • Chronic hyperglycemia also causes abnormalities in microcirculation, endometrial microvascular thickening, closed capillaries, local. ischemia/hypoxia in nerves, leading to axonal degeneration
    -Elevated glucose also reduces levels of nerve growth factor causing reduced nutrition to nerves
26
Q

Clinical manifestations of diabetic neuropathy

A
  • Classified into various types
  • Rapidly reversible
  • Generalized symmetric polyneuropathies
  • Focal. neuropathies
27
Q

Describe rapidly reversible neuropathy

A
  • In those who have been newly diagnosed
  • Distally symmetric sensory changes: burning, paresthesia, tenderness in feet/legs
  • Symptoms disappear when blood sugar gets controlled, although NVC abnormalities may persist
28
Q

Types of generalized symmetric polyneuropathies

A
  • Acute sensory neuropathy
  • Chronic sensorimotor neuropathy
  • Autonomic neuropathy
29
Q

Describe acute sensory neuropathy

A
  • Rapid onset of severe burning pain, deep aching pain, sharp ‘electric shock-like’ sensations, hypersensitivity of feet worse at night, allodynia
  • Motor exam is mostly normal
  • Recovery can occur within 1 year if individual can maintain good blood sugar
30
Q

Describe chronic sensorimotor neuropathy (DPN)

A
  • Most common type
  • Onset mostly insidious, slowly progressive
  • Clinical features depend on selective fiber type involvement
  • Typical glove and stocking pattern
  • Small fibers cause burning pain, paresthesia, more profound at night
  • Large fibers cause painless paresthesia, impaired proprioception, vibration sense, may feel like walking on cotton
  • Loss of ankle DTR, motor weakness mostly in feet/hands, mostly milder, but can cause hammer toe/hallux valgus/pes cavus/ankle equinous with muscle imbalance/wasting in advanced cases
  • Autonomic: sweating (less or more), orthostatic hypotension (OH), resting tachycardia
  • Balance/gait problems due to sensory ataxia, weakness, foot drop, foot deformities
31
Q

Describe autonomic neuropathy

A
  • Sympathetic and parasympathetic involvement
  • With autonomic denervation: HR becomes fixed, may not feel ischemic angina, can suffer silent MI
32
Q

Manifestations of autonomic diabetic neuropathy

A
  • Tachycardia
  • Exercise intolerance
  • Orthostatic hypotension (OH)
  • Dizziness
  • Esophageal motility dysfunction
  • Diarrhea
  • Constipation
  • Neurogenic bladder
  • Bladder urgency, incontinence
  • Erectile dysfunction
  • Sweating, heat intolerance
  • Dry skin
  • Pupillary dysfunction, blurred vision
33
Q

Describe focal neuropathies (mononeuropathies)

A
  • Occurs less often than generalized symmetric patterns
  • May involve nerves in limbs or CNs: median, ulnar, peroneal nerves most commonly affected, somatic division of oculomotor nerve
34
Q

How to diagnose diabetic neuropathy

A
  • Based on history, clinical exam, electrodiagnostic. tests, quantitative sensory tests, autonomic function testing
    -Sensory testing: temp., vibration, and touch
  • Autonomic tests: BP/HR at rest, OH test (STS), HR increase pattern with exercise
  • NVC/EMG: slowing of NVC suggests demyelination, reduced amplitude suggests axonal degeneration
  • Sensory symptoms appear before motor
  • Profound motor involvement implies some other disorder instead of DN
35
Q

Treatment for diabetic neuropathy

A
  • Control hyperglycemia through extensive patient education, pharmacological agents, nutrition, & physical activity/exercise
  • Pharm: insulin or non-insulin (metformin, glimepiride, -gliptins, -glutides) drugs dependent on type of DM/response to drug/individual needs
  • Gabapentin, pregabalin to manage pain & parethesia
  • Nutrition: distribute carbs throughout the day instead of restricting it, 5-6 smaller meals, regular menu is ok, fruit instead of juice, plenty of fluids
36
Q

Diabetic neuropathy implications for PT

A
  • Focus on overall health condition
  • Regular cardio exercises per ACSM and ADA guidelines, use REP instead of HR: Precautions = impact cardio ex/high intensity ex should be done with caution with individuals having complications like abnormal HR response to ex, foot ulcerations, Charcot’s joint (degeneration of WBing joints), unstable angina
  • Resistance ex are beneficial for weakness & glucose metabolism
  • Physical modalities for pain: TENS, infrared
  • Balance & functional mobility training: Romberg, CTSIB, TUG, BBS< gait speed
  • May need appropriate footwear, orthotics and/or ADs for skin protection & better gait
  • > 50% non-traumatic amputations occur in people with diabetes: appropriate prosthetic/prosthetic training after amputations
37
Q

Slide 37

A