Final: Other Diseases Flashcards
What two conditions are under the category of peripheral neuropathies?
Guillain Barre Syndrome and Diabetic polyneuropathy
What is GBS?
Acute inflammatory demyelinating polyradiculopathy with demyelination of the PNS due to macrophages attacking the myelin sheath impairing nerve conduction ability
After what timeframe will Schwann cells re-proliferate and allow for remyelination with GBS?
2-3 weeks
What are three clinical characteristics of GBS?
- Rapid, ascending, symmetrical weakness and sensory loss
- Distal to proximal, 50% with CN involvement
- Progresses for 2-4 weeks followed by recovery
Will GBS pt’s present with intact or absent DTRs?
Absent
What symptoms of autonomic dysfunction will a pt with GBS present with?
Decreased cardiac output, arrhythmias, unstable BP
How is GBS diagnosed?
Lumbar puncture or nerve conduction velocity tests
How is GBS medically managed?
Intravenous immunoglobulin, plasmapheresis, corticosteroids
What will indicated a poorer prognosis for GBS recovery?
Ventilator, older than 60, longer time to peak deficits, failure to show improvement within three weeks of plateau
What is stage 1 of GBS, and how long does it last?
Acute infection, lasts 0-3 weeks
What is stage 2 of GBS, and how long does it last?
Static phase, 10-14 days post peak. Can last for several months
What is stage 3 of GBS?
Rehab phase indicated my slow recovery over a period of months
What interventions are indicated during the acute stage of GBS?
Positioning, ROM, splinting, pressure relief, upright tolerance
What muscle should specifically be target when stretching for pt’s with GBS?
2 joint muscles
What types of interventions are indicated for GBS?
Strengthening, stretching, functional mobility, CV training, pain management, adaptive equipment
What are four considerations during GBS rehab?
- Adequate rest breaks
- Avoid overworking
- Bowel and bladder dysfunction
- High intensity in later recovery after 1 year
Why should you avoid overworking denervated muscles?
Can result in loss of functioning motor units, which can then be linked to central fatigue
What does CIDP stand for?
Chronic Inflammatory Demyelinating Polyneuropathy
What is CIDP?
Chronic inflammation caused long-term demyelination that developed over weeks/months
What does evidence recommend for treating CIDP?
Resistance and aerobic training can improve muscle strength and aerobic capacity
Practice Question: Which of the following is recommended for patients with a diagnosis of GBS?
a. Blood transfusion
b. High intensity gait training in the first week of rehab phase
c. Mechanical ventilation
d. Multi-disciplinary rehab
d. Multi-disciplinary rehab
Practice Question: Which of the following clinical symptoms can be found in patients with GBS?
a. Hyperreflexia
b. Confusion
c. Tingling
d. Clonus
c. Tingling
What is Myasthenia Gravis
Autoimmune disorder that affects NMJ and attacks Ach receptors on the motor end plate
What receptors does Myasthenia Gravis specifically affect?
Post-synaptic Ach receptors
Describe post-exercise decrement in MG
Fatigable weakness - activity causes fatigue that is worse at the end of the day due to each muscle contraction having less Ach available
What muscle groups are impacted by post-exercise decrement in MG?
Asymmetrical, neck and face muscles, bulbar, facial, axial, limb, respiratory, ocular involvement, dysphagia, dysarthria, dyspnea
What are the risk factors for developing MG?
Family history, autoimmune disease. Women < 40 and men > 60
What is myasthenic crisis?
Emergency when respiratory muscles are severely impacted
What are the three diagnostic methods for Myasthenia Gravis?
- Pharmacological
- Immunologic
- Electrophysiologic
What is the Tensilon Test?
Confirms MG diagnosis. Tensilon is given and examiner assesses movement before and one minute after injection. If strength improves, it is positive
What electrophysiologic testing is used to diagnose MG?
Repetitive nerve stimulation shows a rapid decrease in motor action potential amplitude
What are four medical interventions for Myasthenia Gravis?
- AChE inhibitor medication
- Thymectomy
- Immunosuppressive - corticosteroids
- Plasmapheresis and IVIG
What does LEMS stand for?
Lamber-Eaton Myasthenic Syndrome
What is LEMS?
Autoimmune disease impacting pre-synaptic NMJ by attacking calcium channels impacting the release of Ach
60% of LEMS pt’s also have what condition?
Cancer
What are the three primary symptoms of LEMS?
- Proximal weakness
- Hyporeflexia/Areflexia
- ANS dysfunction
Describe the presentation of weakness with LEMS
Proximal, symmetrical, weakness will get better with use, repeated contractions enhances reflexes and strength due to increased influx of calcium in presynaptic membrane
Describe reflexed with LEMS
Reflexed absent prior to exercises, but will occur immediately post exercise
What ANS symptoms are associated with LEMS?
Orthostatic hypotension, dry mouth, excess sweating, ptosis
How is LEMS managed?
- Treat cancer
- Plasmapheresis
What are similarities between MS and LEMS?
- Proximal muscles
- Ptosis
- Bulbar signs
- Ach
- Autoimmune
- LMN
- Sensation not affected
What are the primary differences between MG and LEMS?
- pre vs post synaptic receptors
- CN vs ANS involvement
- Etiology
- Worse vs better with exercise
What should PT intervention look like for NMJ dysfunction conditons?
Pt education for energy conservation, time of day, strengthen large muscle group, mod-low intensity aerobic and strengthening
Practice Question: 38 y/o female presents with new onset of dyspnea at rest, 3 week history of speaking and swallowing difficulty, and feeling worse by the end of the day. What is the likely diagnosis?
a. ALS
b. GBS
c. MG
d. Lambert Eaton
c. MG
Practice Question: Pt presents with reports of hip weakness and facial sweating. DTRs are absent on evaluation, and when retested at the end of the session they are a 2+. What underlying condition should you be worried about?
a. Cancer
b. MG
c. ALS
d. MS
a. Cancer
What type of disease is Huntington’s?
Neurodegenerative
What is the etiology of Huntington’s?
Autosomal dominant with genetic marker on chromosome 4. Results in brain degeneration
What movement disorder characteristics are associated with Huntington’s?
Chorea, gaze fixation abnormalities, dysarthria, dysphagia, sleep disorders, urinary incontinence, gait and balance
What psychiatric symptoms are associated with Huntington’s?
Personality changes and depression
What cognitive symptoms are associated with Huntington’s?
Memory deficits and executive dysfunction
What is the life expectancy of Huntington’s?
15-20 years after the onset
What medication is used to manage chorea symptoms with Huntington’s?
Tetrabenazine
What STOM is used for Huntington’s?
Unified Huntington Disease Rating Scale
What 4 domains of function are included in the UHDRS?
- Motor function
- Cognitive function
- Behavioral abnormalities
- Functional capacity
What other outcome measures can be used with Huntington’s?
10MWT, TUG, 6MWT, Berg, SF36, 4 square step test
What are possible benefits of exercise in pt’s with Huntington’s?
Improved motor function, gait speed and endurance, balance, VO2 max, BMI, pulmonary function
What should PT focus on in the early-mid stages of Huntington’s?
- Aerobic
- Resistance exercises
- Balance and gait
- Compensatory training
- External cues
- Caregiver education
What should PT focus on in mid-late stage Huntington’s?
- Fall risk assessment
- Wheelchair assessment
- Functional exercise
- Aerobic capacity, pulmonary training
- ROM
Practice Question: Which of the following intensities is appropriate for people with HD in the early to mid stage?
a. low intensity
b. low-mod intensity
c. mod intensity
d. mod-high intensity
d. mod-high intensity
What diagnoses cause damage to the basal ganglia?
HD and PD
What diagnoses cause damage to the pyramidal cells?
ALS
What diagnoses cause damage to the corticospinal tract?
ALS
What diagnoses cause damage to the anterior horn cells?
ALS and Post-Polio
What diagnoses cause damage to peripheral nerves?
GBS
What diagnoses cause damage to the NMJ?
MG and Lambert Eaton
