S3_L3: Lower Motor Neuron Flashcards
This is also known as Gehrig’s Disease
Amyotrophic Lateral Sclerosis
Enumerate the hierarchy of initial symptoms of ALS patients.
- Leg weakness
- Arm weakness
- Bulbar Involvement
- Generalized weakness
Phenomenon characterized by weight loss due to muscle atrophy and reduced caloric intake.
Cachexia
Gold standard used to Dx UMN pathology
Presence of Pathologic Reflexes
Determine if the following is an UMN or LMN sx.
- Fasciculations
- Hyperreflexia
- Spasticity
- (+) Corneomandibular reflex
- Cramps
A. LMN
B. UMN
- A
- B
- B
- B
- A
Determine if the following is an UMN or LMN sx.
- Flaccid Dysarthria
- Muscle Atrophy
- Cramps
- (+) Hoffman’s Sign
- (+) Jaw jerk
A. LMN
B. UMN
- A
- A
- A
- B
- B
Most common adult motor neuron disease
Amyotrophic Lateral Sclerosis
UMN syndrome caused by motor neuron loss in the corticobulbar tracts
Pseudobulbar Affect
This UMN condition involves the corticospinal tract and DCML.
Hereditary Spastic Paraplegia
Hereditary d/o causing degeneration of motor neurons and leading to weakness and atrophy of skeletal muscles.
Spinal Muscular Atrophy
This is also known as Acute Ascending Symmetrical Polyneuropathy
Guillain Barre Syndrome
This condition has a triad of Ataxia, Areflexia, and Ophthalmoplegia.
Miller-Fisher Syndrome (MFS)
Determine which condition is being described through the following s/sx or details.
- 2nd Wind Phenomenon
- Initially with strength, then decreases
- Decreased ACH receptors
- Pre-synaptic
- M > F
A. Myasthenia Gravis
B. Lambert-Eaton
- B
- A
- A
- B
- B
Determine which condition is being described.
- Associated with thymoma
- Associated with bronchogenic carcinoma
- Ptosis and diplopia – initial manifestations
- Sexual dysfunction
- Post-synaptic
A. Myasthenia Gravis
B. Lambert-Eaton
- A
- B
- A
- B
- A
Condition with damage to the anterior horn cell of the spinal cord caused by the poliovirus
Poliomyelitis
HSMN Ib has a defect on which chromosome?
1
The hallmark presentations of this condition are peroneal and distal leg atrophy, weakness, sensory loss and areflexia
Hereditary Sensory Motor Neuropathy
TRUE OR FALSE: ALS affects all of the major muscle groups of the mechanical respiratory system. Oxygen may be used to treat respiratory insufficiency
A. both statements are true
B. both statements are false
C. only the 1st statement is true
D. only the 2nd statement is true
C. only the 1st statement is true
TRUE OR FALSE: A a 12-week moderate resistance exercise program results in strength gains without any notable deleterious effects. If a pt cannot receive tx for 12 weeks, maximal resistance at 6-weeks should be implemented.
A. both statements are true
B. both statements are false
C. only the 1st statement is true
D. only the 2nd statement is true
C. only the 1st statement is true
TRUE OR FALSE: Pts with MND are advised not to exercise to exhaustion. Otherwise, this can produce more muscle damage and dysfunction
A. both statements are true
B. both statements are false
C. only the 1st statement is true
D. only the 2nd statement is true
A. both statements are true
This is a pure UMNL that involves primarily the corticospinal tract. Its initial Sx is LE Spasticity
Progressive Lateral Sclerosis
This condition presents with loss or chromatolysis of motor neurons of the SC and Brainstem. It affects the AHC and presents with distal leg weakness with atrophy.
Progressive Muscular Atrophy
What is the screening tool used to guide diagnosis of ALS?
El Escorial Criteria
Fill in the blanks: Poor Prognosticating Factors for ALS pts
- _____ age at time of onset, > ____ y/o
- _____ dysfunction early in the clinical course of the disease
- Short time period from _____ to _____
- Older; 40-60
- Bulbar and/or pulmonary
- symptom onset to diagnosis
Fill in the blanks: Poor Prognosticating Factors for ALS pts
- Predominance of _____ findings at the time of diagnosis
- ______
- More _______ symptoms
- LMN
- Women
- bulbar
What is the pharmacologic intervention used to slow progression of ALS?
Riluzole
What is the half-life or riluzole?
9-15 hours, usually 12 hours
This condition is associated with bronchial carcinoma and botulism poisoning. It presents with weakness and fatigability of proximal limb muscle with sparing of ocular ms.
Lambert-Eaton Myasthenic Syndrome
This is an autoimmune disorder of peripheral nerve in which antibodies form against acetylcholine (Ach) nicotinic postsynaptic receptors at myoneural junctions
Myasthenia Gravis
What is type 1 Poliomyelitis called?
Brunhilde
What is known as the sine qua non of all adult MND?
weakness and fatigue
This is the type of HSMN that has lesser hypertrophic change in myelin with more neuronal or axonal involvement
HSMN II
This kind of SMA has an adult onset, usually at mid-30s, with normal survival.
SMA IV
Match the following respiratory dysfunction based on the muscle affectation.
- abnormal swallowing
- inadequate maintenance of ventilation
- abnormal cough
- inadequate cough
A. Inspiratory muscle
B. Expiratory muscle
C. Upper airway muscle
- C
- A
- C
- B
Ideal place for patients with ALS to do aerobic exercise training.
Pool
The following are signs of over exhaustion, EXCEPT:
A. Feeling weaker rather than stronger within 30 mins post exercise
B. Excessive muscle soreness 12 to 24 hours following exercise
C. Severe muscle cramping, heaviness in the extremities, and prolonged shortness of breath
D. All
E. None
B. Excessive muscle soreness 12 to 24 hours following exercise
Must exceed 24 hours, up until 48 hours
Which of the following must present with normal findings in ALS pts.
A. Mental Status
B. Sensation
C. Red Nucleus
D. Corticospinal Tracts
E. Cerebellar Examinations
A, B, E
The following must present with normal findings in ALS pts, EXCEPT:
A. Mental Status
B. Optic Nerve
C. Spinocerebellar Tracts
D. Onufrowicz Nucleus
E. None
E. None
Optic nerve is a sensory CN, which must be normal in ALS.
Enumerate the stages of Poliomyelitis.
- Acute Stage
- Convalescent/Recovery Stage
- Recovery of Strength
- Residual/Chronic Stage
This is a GBS Variant that is a pure motor subtype that is prevalent among pediatrics
Acute Inflammatory Demyelinating Polyneuropathy (AIDP)
Rapidly progressive neurodegenerative disease that is a combination of upper and lower motor neuron disorders
ALS
What 2 pharmacological tx is given to treat spasticity?
Balcofen or Tizanidine
Muscle weakness leads to what manifestations in the head and foot in ALS pts?
- head drop
- foot drop
TRUE OR FALSE: The corneomandibular reflex is more sensitive and is more of a specific indicator for UMN pathology in the bulbar region. Gag reflex may also be used to assess UMN pathology.
A. both statements are true
B. both statements are false
C. only the 1st statement is true
D. only the 2nd statement is true
A. both statements are true
TRUE OR FALSE: Second wind is a phenomenon in distance running, such as marathons or road running, whereby an athlete suddenly finds the strength to press on attop performance with less exertion. This is a presentation seen in MG pts.
A. both statements are true
B. both statements are false
C. only the 1st statement is true
D. only the 2nd statement is true
C. only the 1st statement is true
TRUE OR FALSE: MG presents with destruction of the post-synaptic receptors for ACH. Lambert-Eaton MG affects the presynaptic membrane
A. both statements are true
B. both statements are false
C. only the 1st statement is true
D. only the 2nd statement is true
A. both statements are true
MG Clinical feature: Myasthenic crisis
A. class 1
B. class 2
C. class 3
D. class 4
E. class 5
E. class 5
MG Clinical feature: Moderate generalized weakness and mild to mod ocular-bulbar weakness
A. class 1
B. class 2
C. class 3
D. class 4
E. class 5
C. class 3
MG Clinical feature: Ocular muscles only – 20%
A. class 1
B. class 2
C. class 3
D. class 4
E. class 5
A. class 1
MG Clinical feature: Mild generalized weakness
A. class 1
B. class 2
C. class 3
D. class 4
E. class 5
B. class 2
MG Clinical feature: Severe generalized and ocular-bulbar weakness
A. class 1
B. class 2
C. class 3
D. class 4
E. class 5
D. class 4
GBS Phase: Mechanical ventilation
A. phase 1
B. phase 2
C. phase 3
D. phase 4
E. phase 5
F. phase 6
E. phase 5
GBS Phase: Respiratory Monitoring
A. phase 1
B. phase 2
C. phase 3
D. phase 4
E. phase 5
F. phase 6
D. phase 4
GBS Phase: Tingling of hands and feet
A. phase 1
B. phase 2
C. phase 3
D. phase 4
E. phase 5
F. phase 6
A. phase 1
GBS Phase: Recovery, full activity
A. phase 1
B. phase 2
C. phase 3
D. phase 4
E. phase 5
F. phase 6
F. phase 6
GBS Phase: Difficulty in arising from chair
A. phase 1
B. phase 2
C. phase 3
D. phase 4
E. phase 5
F. phase 6
B. phase 2
GBS Phase: Areflexia, weakness, distal sensory loss
A. phase 1
B. phase 2
C. phase 3
D. phase 4
E. phase 5
F. phase 6
C. phase 3
Miller-Fisher Syndrome (MFS) pts recover in a span of how many months?
1-3
TRUE OR FALSE: ALS has a better prognosis if there are more LMN findings than UMN findings
True
Disinhibition of limbic motor control that produces “emotional incontinence”
Pseudobulbar Affect
Enumerate the factors that increases the risk of acquiring ALS.
- Cigarette smoke
- Increased dietary fat intake
- Glutamate intake
Presentation: Progressive spasticity, LE weakness, hypertonic urinary bladder, impaired vibration sense.
What condition is this?
Hereditary Spastic Paraplegia
The gold standard outcome for ALS trials currently remains ____.
survival
Fill in the blanks: goals in treating ALS pts.
- To maximize _____ capacities
- Prolong or maintain independent function and ______
- Inhibit or prevent ______
- Provide access to full _______ with good quality of life.
- functional
- locomotion
- physical deformity
- community integration
Pool therapy is best done in a therapy pool with a flat, uniform depth floor that is heated to _____.
92º F to 95ºF
Risk for aspiration and pneumonia is due to problems with (1)_____ and (2)____
- upper airway function
- cough
- Chronic Juvenile
- Ambulates indep
A. SMA I
B. SMA II
C. SMA III
D. SMA IV
- C
- C
- Before 18mo. Of age
- Acute Infantile
A. SMA I
B. SMA II
C. SMA III
D. SMA IV
- B
- A
- Before 6mo. Of age
- Over 50% survive to mid-20s
A. SMA I
B. SMA II
C. SMA III
D. SMA IV
- A
- B
- Kugelberg-Welander
- Chronic Infantile
A. SMA I
B. SMA II
C. SMA III
D. SMA IV
- C
- B
TRUE OR FALSE: SMA III become apparent between 5-15 year
True
The following EDX must be normal in ALS pts, EXCEPT:
A. sensory nerve conduction studies
B. amplitudes
C. conduction velocities
D. distal motor latencies
E. None
E. None
TRUE OR FALSE: Males has a poorer prognosis in ALS. The mean age of affectation is around 58 y/o
A. both statements are true
B. both statements are false
C. only the 1st statement is true
D. only the 2nd statement is true
D. only the 2nd statement is true
TRUE OR FALSE: ALS presents with higher incidence in urban areas. Bulbar symptoms presents with worse prognosis if it occurs initially in the early stage of the disease
A. both statements are true
B. both statements are false
C. only the 1st statement is true
D. only the 2nd statement is true
A. both statements are true
TRUE OR FALSE: Progressive bulbar atrophy presents with degeneration of CN 9 and 10. Its usual cause of death is aspiration.
A. both statements are true
B. both statements are false
C. only the 1st statement is true
D. only the 2nd statement is true
C. only the 1st statement is true
Pneumonia → usual cause of death
The lack of LMNL is a key feature in this condition. It presents with bowel and bladder problems. It mostly spinal, rarely bulbar.
Progressive (or Primary) Lateral Sclerosis
TRUE OR FALSE: In ALS, bowel & bladder are okay because the Onufrowicz nucleus if not affected
True
Parameters of diaphragmatic breathing exercises is?
3-5 reps only
Use of trapezius in breathing is called _____?
labored breathing
Polyneuritis with progressive muscular weakness that develops rapidly
Etiology: Unknown associated with an autoimmune attack, usually occurs after recovery from an infectious illness (respiratory or gastrointestinal)
What is this condition?
Guillain Barre Syndrome
What is the gender predominance in MG pts?
Female
What is a red flag that may urge the clinician to consider a Dx of ALS?
Abdominal Cramps
TRUE OR FALSE: Bulbar onset in ALS is typically the last to emerge. If not, prognosis becomes poor.
A. both statements are true
B. both statements are false
C. only the 1st statement is true
D. only the 2nd statement is true
A. both statements are true
Nuclei found on AHC on sacral part of spinal cord responsible for micturition, defecation, and sexual reproduction functions
Onufrowicz Nucleus
This condition presents with optic atrophy, cerebellar ataxia, and (+) Serology in blood or CSF.
Tropical Spastic Paraparesis
This is a progressive condition that presents with dysphagia, decreased gag reflex, weakness of palatal movements, and tongue fasciculations
Progressive Bulbar Atrophy
What will be the dose for riluzole?
100 mg qd
NOTE: Standard dose of riluzole is one 50 mg tablet two times a day (Sullivan)
This drug is more effective in the earlier stages of the disease, with less severe signs and symptoms and a greater forced vital capacity (FVC). It is the second FDA approved drug for ALS
Radicava/Radicut
TRUE OR FALSE: Frequent turning must be done in full-time mechanical ventilation patients.
True
Overuse weakness does not occur in muscles with an MMT grade of (1)_____ or greater out of (2)_____.
- 3 (fair)
- 5 (normal)
TRUE OR FALSE: Heavy eccentric exercise is indicated for ALS pts.
False
TRUE OR FALSE: Moderate resistance exercises can increase strength in muscles with an MMT grade of 3 or greater out of 5.
True
TRUE OR FALSE: Exercise may not improve the strength of muscles already weakened by ALS.
True
TRUE OR FALSE: Cold can temporarily decrease spasticity. Postural and positioning techniques can be incorporated to decrease spasticity
A. both statements are true
B. both statements are false
C. only the 1st statement is true
D. only the 2nd statement is true
A. both statements are true
TRUE OR FALSE: Engaging in a 15-min, twice-daily, maximum intensity exercise decreases spasticity. Mild pain or pain associated with joint discomfort is usually addressed with analgesics.
A. both statements are true
B. both statements are false
C. only the 1st statement is true
D. only the 2nd statement is true
D. only the 2nd statement is true
TRUE OR FALSE: Pain is a secondary impairment in ALS. Restorative interventions in the late stage of ALS are done to remediate impairments that result from other systems pathology
A. both statements are true
B. both statements are false
C. only the 1st statement is true
D. only the 2nd statement is true
A. both statements are true
TRUE OR FALSE: SMA presents with a more distal affectation. It has abnormal sensory nerve conduction studies.
A. both statements are true
B. both statements are false
C. only the 1st statement is true
D. only the 2nd statement is true
B. both statements are false
HSMN IV is also known as?
Refsum’s Disease
HSMN III is also known as?
Dejerine Sottas Disease
- With retinitis pigmentosa
- Presents with prominent spinocerebellar degeneration
- With optic atrophy
A. HSMN VII
B. HSMN V
C. HSMN VI
- A
- B
- C
Asymmetric weakness and atrophy affecting the legs>arms or bulbar muscles progress to flaccid paralysis
Poliomyelitis
Type 3 poliomyelitis is termed as?
Leon
What is the cardinal sign of Poliomyelitis?
Cardinal Signs: Asymmetrical muscle weakness esp LE
The following are correct management parameters for Poliomyelitis, EXCEPT:
A. Every other day
B. RPE = 13
C. 6 SH
D. 3 sets of 5-10 reps
E. 5 minutes rest between sets
C. 6 SH
Only 4-5 SH with 10 second rest between each bout.
TRUE OR FALSE: In MG, smooth and cardiac muscle are spared. Muscular strength gets worse with continuing contraction, improved with rest or application of ice.
A. both statements are true
B. both statements are false
C. only the 1st statement is true
D. only the 2nd statement is true
A. both statements are true
When the facial ms presents with weakness in MG pts, what characteristic smile is produced?
myasthenic snarl
A PT is examining the face of his MG pt. What may be seen in the tongue of the pt using OI?
triple grooved appearance/ trident tongue
Fill in the blanks: PTs goal in treating MG and LEMS pts
- generate adequate _____ and to clear bronchial secretions
- Maintain or improve ______ without exacerbating fatigue
- Promote _____ in functional mob skills and ADLs ECT and pacing
- ventilation
- CV endurance
- independence
This condition presents with reactive symmetrical distribution of weakness; ascending progression (LE→UE). It produce full tetraplegia with respiratory failure
Guillain Barre Syndrome
YES OR NO: Can myalgia be seen in GBS?
Yes
What is the common cause of death in pts with LMN affectation?
Pulmonary problems
YES OR NO: AROM will increase muscle strength in muscles graded 2/5
Yes
Among the huff and cough exercise, what must be taught first?
Huff
In teaching a pt how to huff, what must be noted upon performing it?
On 3rd exhalation in diaphragmatic breathing exercise, incorporate huffing
What does WF mean in terms of coughing?
able to cough but without any phlegm
When a PT writes “productive coughing”, what grading does it correlate to?
F (Functional)
The following is a criteria for hospice care for ALS pts, EXCEPT:
A. Rapid progression in the past 12 months
B. Recurrent Aspiration Pneumonia
C. Dehydration
D. Vital capacity less than 30% of predicted
E. None
E. None
In which condition is the onuf nucleus affected?
Progressive Lateral Sclerosis
UMN sx of spasticity is often together with what?
Hyperreflexia
LMN SX of weakness is often seen with what?
Fasciculation
What is the function of Riluzole?
Inhibits pre-synapic release of glutamate
TRUE OR FALSE: ROM & stretching exercises should be given to all MND pts.
True
This is according to Braddom.
Enumerate the muscles paralyzed in Poliomyelitis
- tibialis anterior
- peroneals
- quadriceps
- gluteals
- deltoids
TRUE OR FALSE: A back knee gait, anterior trunk lean and foot drop is seen in patients with Poliomyelitis
True
TRUE OR FALSE: Poliomyelitis pts have cold intolerance
True
Which MNDs manifests with head dropped syndrome
MG, ALS, POLIO
What is the recovery period of GBS?
6 months - 2 years
GBS is also known as?
glove and stocking paralysis
TRUE OR FALSE: atrophy only occurs in LMN conditions
False
LMN: denervation atrophy
UMN: disuse atrophy
Determine the intervention scenario for MNDs.
early stage of disease
A. Compensatory - Preventive
B. Preventive - Restorative
C. Restorative - Compensatory
D. Preventive - Compensatory
E. Compensatory - Restorative
B. Preventive - Restorative
Determine the intervention scenario for MNDs.
middle or late stage of disease
A. Compensatory - Preventive
B. Preventive - Restorative
C. Restorative - Compensatory
D. Preventive - Compensatory
E. Compensatory - Restorative
A. Compensatory - Preventive
TRUE OR FALSE: The smaller the object held, the more energy consumption needed. Therefore, big objects are recommended for pts c MND.
A. both statements are true
B. both statements are false
C. only the 1st statement is true
D. only the 2nd statement is true
A. both statements are true