Term
| This dz is chronic, progressive, and terminal |
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Definition
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Term
| This dz is chronic, progressive, but not terminal |
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Definition
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Term
| This dz is acute and largely reversible in most cases. |
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Definition
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Term
| This dz affects both UMN and LMN and variations of both: Only LMN, only UMN, or primarily cranial nerves. |
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Definition
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Term
| This dz results in weakness, increased spasticity and stiffness, hyperreflexia. Fasciculations may be the first symptom. The largest and most heavily myelinated nerves are usually the first to be affected. |
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Definition
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Term
| This dz results in destruction of UMN and LMN as well as destruction of anterior horn cells of the spinal cord causing death and replacement with scar tissue. It causes sclerosis of the lateral spinal cord and is characterized by muscle wasting and increased tone. |
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Definition
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Term
| What are the possible causes of ALS? |
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Definition
Unknown
-Environmental/toxins
-Genetic
-Excitotoxic (increased glutamate release) |
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Term
| What is the mean age of onset for ALS? |
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Definition
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Term
| Which gender does ALS affect more? |
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Definition
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Term
| What race is most commonly effected by ALS? |
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Definition
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Term
| Name 3 risk factors for the onset of ALS |
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Definition
-Occupation (heavy metal or leather)
-Exposure to heavy metals, chemicals, mechanical or electrical trauma
-Vigorous athletic activity |
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Term
| Describe the life expectancy for people with ALS |
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Definition
2-5 years after dx
5-10 = 20%
10-20 = 10%
Over 20 = 5% |
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Term
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Definition
| By the presence of LMN in 1 of 4 regions, UMN in 1 of 4 regions, or by the progression of signs within a region or to other regions |
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Term
| There are 4 categories of ALS, describe category 1. |
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Definition
Idopathic (primary ALS)
-Classic (sporadic) - 90-99%
-Familial - 5-10%
-Restricted
~PBP
~PMA
~PLS |
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Term
| Loss of brainstem motor neurons resulting is dysarthria, dysphagia, respiratory dysfunction, weakness, wasting, etc. Poor prognosis because it starts in the cranial nerves. Respiratory system affected immediately |
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Definition
PBP
Progressive Bulbar Palsy |
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Term
LMN form of ALS
-Loss of spinal cord motor neurons
-More prolonged dz process
-Respiratory complications late in dz
-May live closer to 10 years after dx |
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Definition
PMA
Progressive Muscular Atrophy |
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Term
UMN form of ALS
-Degeneration of corticospinal tracts in brain and spinal cord
-May be more prolonged dz process
-Respiratory complications later |
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Definition
PLS
Primary Lateral Sclerosis |
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Term
| Name 4 additional complications of ALS |
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Definition
1. Decreased cognition really late in dz process if at all
2. Dysphagia
3. Respiratory management
4. Referral for therapy may be at end stage when there is significant ADL and function loss |
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Term
| When a person with ALS is first diagnosed they may be in Stage 1 of rehabilitation. What does this entail? |
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Definition
Ambulatory, Independent in ADLs, mild weakness and ms cramps, fatigue of weakened muscles.
TX: ROM, strengthening, functional activities, medications, psychological support.
-Do gross and functional strengthening. Do not isolate ms groups because that will cause too much fatigue |
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Term
| What does stage 2 of ALS rehabiliation entail? |
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Definition
Slightly ambulatory, Decreased ADLs, Moderate selective weakness.
TX: ROM, orthosis, ADL training, exercise muscles 3+, avoid overuse, psychosocial support |
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Term
| What does stage 3 of ALS rehabilitaion entail? |
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Definition
Moderate decrease in ambulation, decrease in ADLs, severe selective weakness of distal ms, fatigue with long-distance ambulation, limb stiffness and spasticity, slight increase in respiratory effort.
TX: May require w/c use (power is best but manual is more practical money wise), baclofen, may need home modifications such as ramps or bathroom equipment |
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Term
| At wha rehabilitation stage does a PT usually see the ALS pt? |
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Definition
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Term
| What does stage 4 of ALS rehabiliation look like? |
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Definition
W/c dependent, ADLs from w/c, severe LE weakness, +/- spasticity, moderate UE weakness.
TX: Home mods, transportation issues, power w/c, ROM, strengthening as able, modalities for pain, family training |
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Term
| What does stage 5 of ALS rehabiliation look like? |
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Definition
W/c dependent (may not have any more head control), increased dependence in ADLs, severe weakness, increased risk of skin breakdown, increased respiratory complications.
TX: may need head support in w/c, trunk supports, positioning in bed and w/c, hosp bed for care, adapt w/c for vent |
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Term
| Does ALS affect sensation? |
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Definition
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Term
| Describe what stage 6 of ALS rehabilitation would look like. |
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Definition
Continued w/c or bed dependence, vent dependent, severe weakness, increased risk of skin breakdown.
TX: power w/c with tilt/recline, hospital bed, EADLs |
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Term
| Name the only reason an ALS patient might be bed dependent? |
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Definition
| If they do not have a power w/c |
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Term
| One of the most common acquired neuropathies. Also called Acute Idiopathic Polyneuritis, or Landry's Ascending Paralysis. It is the most common acute dz causing paralysis in developed countries. |
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Definition
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Term
| This is sometimes confused with Chronic Inflammatory Demyelinating Polyradical Neuropathy where pt may experience periods of worsening and improvement with relapses. |
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Definition
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Term
| This dz progresses rapidly or may cause paralysis and/or respiratory arrest in hours. May continue to progress over 1-2 weeks then plateu. Full recover may occur although many have some remaining disability. 10% experience chronic dz. This is NOT hereditary. |
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Definition
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Term
| The cause is unknown for this dz and it has no preference to young, old, male, or female. 60% have a history of UTI, mumps, flu, mono, etc. 85% are ambulatory within 6 months. 5% mortality rate due to rapid onset of respiratory problems. |
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Definition
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Term
| Name some common diagnostic features of GBS. |
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Definition
-Motor weakness
-Mild sensory deficits
-Autonomic dysfunction such as tachycardia, arrythmias, fluctuating hypo/hyper tension
-Absence of fever at onset
-May have increased CSF
-Nerve conduction velocities are abnl
-Recovery begins 2-4 weeks after plateu |
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Term
| May see abnormal sweating. Need to monitor vitals closely. First symptom may be tingling or numbness in fingers and toes then progress proximally. May be uni or bilateral. |
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Definition
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Term
20-30% require assisted ventilation
5% die due to secondary complications |
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Definition
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Term
| What are the sensory symptoms of GBS? |
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Definition
Tingling
Burning
Numbness
Decreased vibratory or position sense |
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Term
| What are the pain symptoms for GBS? |
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Definition
Aching
Stiffness
May experience severe hypersensitivity or severe burning to touch or air
May not can tolerate WB on hands or feet |
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Term
-80-85% are ambulatory within 6 months
-65-75% return to clinically normal motor function
-50% may have minor neuro deficits
-15% have residual deficits in function
-Most common long-term deficits are in the anterior tib, weakness in feet, hand intrinsics or quad and gluts
-2-5% have a recurrence of symptoms similar in onset and pattern to original onset. |
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Definition
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Term
| Name 3 factors that affect prognosis in GBS. |
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Definition
1. Rapid onset and progression to quadriplegia
2. Respiratory dependence
3. Failure to show improvement within 3 weeks of plateau |
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Term
| How do you medically manage GBS? |
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Definition
| Respiratory compromise should be closely monitored as well as cardiac complications. Drug Rx: plasma exchange, IVIG (intravenous immunoglobin) |
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Term
| Describe the PT evaluation in GBS pts. |
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Definition
-Motor Eval - only ms specific to function, do not fatigue pt
-Use of pain scale - pts will tend to be hypersensitive
-Respiratory and dysphagia eval - speech therapy but we may need to help via positioning
-Functional assessment
-Sensory assessment - specific areas
-Autonomic system monitor (BP, HR, bowel and bladder)
-EMG testing |
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Term
| Name some treatments done on GBS pts |
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Definition
-ROM
-Massage to help with pain and hypersensitivity
-Strengthening/sitting program - recline w/c with jay/roho cusion. Do this ASAP b/c of respiratory fxn
-Active exercise - gross motor only
-Functional activities -concentrate on proximal ms because recover is proximal to distal
-Equipment assessment, transportation
-Family training |
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Term
| How should you progress ROM in GBS pts |
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Definition
| PROM initially then AAROM and AROM if the pt can perform full ROM without pain or fatigue |
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Term
| Strength returns proximal to distal in GBS pts, what is the therapy goal related to this? |
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Definition
| Maintain the ms in a "ready" state, prevent overwork and pace recovery process to obtain max function. Exercise should be directed to functional strength. Reps should be low and rest periods high |
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Term
| Describe the sitting program for GBS pts |
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Definition
| Gradual sitting program should be established as the axial musculature may be fatigued even with supportive sitting |
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Term
| What are some long term exercise precautions for GBS pts? |
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Definition
| May need to limit fatiguing exercises for up to 1 year. Some pt's continue to show deficits during strenuous exercise that requires max endurance and may have difficulty returning to work or activities that require max effort. Pt fatiguing could cause relapse |
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Term
| The most common hereditary neuropathy. Also called Peroneal Muscular Atrophy. Set of inherited peripheral neuropathies that severely range widely in the same family. Onset is usually ~ 20 years old. |
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Definition
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Term
| Effects both motor and sensory. Death may be due to respiratory or secondary complications. Does not occur any more in men or women |
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Definition
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Term
| Ppl with this dz may unknowingly carry the recessive genes. They may only discover their true gene history too late and have already passed it onto their children. It affects 60-70% of family members. |
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Definition
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Term
| It is rare, but it may be caused by a spontaneous gene mutation. It is linked to defective genes on chromosome 1, 17 and X. 60-70% are inherited in a dominant pattern. Female = male. Rare recessive genes tends to be the most severe cases. Diagnosed by blood, genetic testing. |
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Definition
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Term
| Name some common characteristics of CMT. |
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Definition
-Muscle wasting
-Pt may not be able to distinguish hot from cold
-Loss of DTR's
-Bone and joint deformities (pt may trip a lot) (high arches - pes cavus, hammer toes, foot drop)
-Can progress to distal UEs (loss of fine motor, may effect quads but more rare, may need hand splints, inability to hold grasp things)
-Initially below the knee ms wasting then progresses to below elbow |
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Term
| This dz can cause arrhythmias so be careful when exercising. It can also cause speech deficits, respiratory complications in severe cases, and the initial symptoms may appear as falls, inability to pick up toes, ankle instability and deficits in static stance, visible atrophy of ms, fatigue, and the inability to stoop, knee, climb, or run due to decreased LE sensation. |
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Definition
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Term
| Medications are available for this disease but they may cause regression or make symptoms worse. They are scientifically documented as being toxic and in some cases dangerous for these pts. Common meds are zoloft, stantins, dilantin, etc. |
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Definition
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Term
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Definition
Type 1 = most common; onset ~20y/o
Type 2 = usually appears after age 20 |
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Term
| Name some treatments for CMT |
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Definition
-Strengthen muscles to compensate
-Maintain LE ROM
-Optimize level of function (transfer/gait training) (put AFOs on)
-W/C needed in severe cases
-EADLs especially if UE are involved
-Balance
-Pool therapy
-UE orthosis
-Fall prevention
-Surgery if foot deformity is severe |
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