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