Midterm Flashcards
(161 cards)
1
Q
Is weakness an UMN or LMN sign?
A
Can be both.
UMN: disuse atrophy
LMN: atrophy
2
Q
UMN sensory loss pattern
A
Adjacent body regions
Spinal cord: dermatomal pattern
3
Q
LMN sensory loss pattern
A
Spinal nerve roots: dermatomal pattern
Peripheral nerve pattern
Stocking glove
4
Q
6 other systems indicating CNS involvement
A
- Vision
- Language
- Cognition
- Cerebellum
- Basal ganglia
- Corticobulbar signs
5
Q
If a diagnosis presents with only motor symptoms and no sensory symptoms what are 3 possible diagnoses?
A
- ALS
- MG
- MD
6
Q
ALS affects what part of the nervous system?
A
Motor neuron cell bodies
7
Q
Are males or females more likely to get ALS?
A
Males
8
Q
2/3 of ALS patients have a _____ onset whereas 1/3 of ALS patients have a ____ onset
A
2/3 have spinal onset
1/3 have bulbar onset
9
Q
What is the mean age of ALS onset?
A
60 years
10
Q
What process damages the motor axon in ALS?
A
Wallerian degeneration
11
Q
What 4 nuclei are typically spares with ALS?
A
- Oculomotor
- Trochlear
- Abducens
- Onuf
12
Q
5 temporal steps of the ALS disease process
A
- Subtle loss of dexterity, stiffness, cramps, fatigue
- Weakness, slurred speech, atrophy, spasticity
- Speech, swallowing, respiration involvement
- Dependent for mobility and ADLs
- Avg 3-5 year progression to death
13
Q
3 main signs of spinal onset ALS
A
- Limb onset
- Start distally or proximally
- Usually asymmetrical early on
14
Q
2 signs of bulbar onset ALS
A
- Dysarthria and dysphasia
2. Simultaneous limb symptoms
15
Q
Imaging for ALS
A
MRI to rule out cortical, brainstem, or cervical spine pathology
16
Q
What is the purpose of a LP for ALS?
A
Rule out inflammatory
17
Q
What is the purpose of blood tests for ALS?
A
Rule out toxic, metabolic, infections, inflammatory, genetic
18
Q
What are the findings in NCV for ALS
A
Normal
19
Q
What are findings in an EMG for ALS?
A
Decreased compound motor unit action potentials (CMAPs)
Fibrilations and Sharp waves
Fasciculations
Scarce or partial interference pattern
20
Q
What are 5 symptoms that may require medical management in ALS?
A
- Sialorrhea
- Muscle cramps
- Spasticity
- Dyspnea
- Depression
21
Q
What is the cause of death with ALS?
A
Respiratory failure
22
Q
What part of the nervous system does MG affect?
A
Neuromuscular junction
23
Q
What age are men and women effected by MG?
A
Women: 20-40
Men: 50-70
24
Q
Unlike ALS, MG has a definite etiology:
A
Autoimmune
25
What are common (10%) comorbid diseases with MG?
Thyroid disease
26
What 2 things occur to the neuromuscular junction with MG?
1. Post-synaptic receptor loss
| 2. Damage to membrane
27
4 temporal stages of MG
1. Subtle onset
2. Potential exacerbation with illness, pregnancy
3. Fluctuating weakness and fatigability with full body involvement within one year
4. Progression to max severity in 1st 2 years
28
3 patterns of weakness with MG
1. Proximal > distal
2. Arms > legs
3. Quads, triceps and neck extensors effected greatly
29
4 common diagnostic tests for MG
1. Tensilon
2. Serologic testing
3. Slow repetitive nerve stimulation
4. CT/MRI
30
What is one risk of Tensilon testing?
Cardiac side effects
31
What is the purpose of Imaging for MG?
Screen for thymoma
32
Side effects of cholinesterase inhibitors
```
Diarrhea
Cramping
Excessive secretions
Muscle fasciculations
Cholinergic crisis
```
33
What percent of patients with focal disease eventually develop generalized MG?
80%
34
How long does it take for MG to progress to max severity?
2 years
35
5 common signs and symptoms to identify in MG
1. Fluctuating and fatigable weakness
2. Proximal>distal weakness
3. UE>LE weakness
4. Bilateral asymmetric ocular muscle weakness
5. Nasal quality in voice
36
Muscular dystrophy is a form of what type of neurological dysfunction
Myopathy
37
Myopathies primarily produce what 4 problems with muscle weakness and fatigability
1. Affects proximal > distal
2. Low tone
3. Atrophy
4. Pseudohypertrophy
38
9 routine diagnostic tests for MD
1. Creatine kinase level
2. Urinalysis and urine myoglobin
3. Liver function tests
4. Thyroid function tests
5. Chemistry panel
6. Erythrocytes sedimentation rate
7. Electrocardiography
8. Electromyography
9. Muscle biopsy* gold standard
39
Most common and severe form of childhood muscular dystrophy
Duchesse muscular dystrophy
40
5 steps of temporal course of MD
1. Normal at birth
2. Delayed motor milestones after 1 year
3. 3-7 years old: toe walking, gowers, lordosis, scoliosis
4. 7-12 years old: loose ability to walk
5. 20s: death from fatty infiltrations to heart and respiratory infections
41
7 complications to look out for as MD progresses
1. Contractures
2. Scoliosis / lordosis
3. Obesity
4. Decreased respiratory capacity
5. Cognitive deficits
6. Cardiac dysfunction
7. GI dysfunction
42
What medication has been shown to prolong ambulation for up to 3 years in patients with MD?
Prednisone
43
What age are children with MD typically wheelchair dependent?
12 y.o.
44
3 structures involving UMN
1. Cortex
2. Brainstem
3. Spinal cord
45
4 structures involving LMN
1. Anterior horn cells / cranial nerve nuclei
2. Spinal nerve roots / peripheral nerves (includes cranial nerves and autonomic)
3. Neuromuscular Junction (NMJ)
4. Muscle
46
4 PNS motor paterns
1. Weakness
2. Fasciculations
3. Atrophy
4. Areflexia
47
3 PNS sensory paterns
1. Peripheral nerve: cutaneous loss
2. Polyneuropathy: stocking-glove pattern of loss
3. Spinal nerve root: dermatomal pattern of loss
48
4 PNS Autonomic patterns
1. Flushing (redness)
2. Change in HR, SOB, BP
3. Incontinence, Constipation, Diarrhea
4. Dry eyes / mouth
49
4 categories of nerve injury
1. Neuronal degeneration
2. Wallerian degeneration
3. Segmental demyelination
4. Axonal degeneration
50
Cell body damage with degeneration of axons
Neuronal degeneration
51
Damage to axon at a specific point with distal degeneration
Wallerian degeneration
52
Injury to myelin sheath without injury to axon
Segmental demyelination
53
Diffuse axonal damage. Distal portion furthest from the cell body undergoes earliest and most severe change. Causes initial symptoms in hands and feet with gradual, proximal scent and continued injury
Axonal degeneration
54
3 types of mononeuropathy
1. Nerve entrapment
2. Repetitive motion injury
3. Trauma
55
5 types of polyneuropathy
1. Infectious diseases (HIV)
2. Inflammatory diseases (AIDP/GBS, CIDP)
3. Other systemic diseases (DM, Critical illness, Vitamin deficiency)
4. Genetic disorders (Charcot Marie Tooth)
5. Toxins (Therapeutic drugs i.e. Chemo, alcohol)
56
7 steps to the general diagnostic approach for polyneuropathy
1. Comprehensive history
2. General physical exam
3. Neurologic exam focusing on diagnostic possibilities
4. Blood studies
5. LP (rule out GBS/AIDP and CIDP)
6. EMG/NCV** Gold standard
7. Possibly nerve biopsy
57
What is the most common cause f acute neuromuscular paralysis in developed countries?
GBS
58
GBS male:female ratio
2:1
59
2 facts about etiology of GBS
1. Immune mediated disorder - Inflammatory
| 2. Precedes infection in about 60% of cases
60
5 steps of the temporal course of GBS
1. Inciting infection
2. 1-3 weeks until symptom onset
3. 1-3 weeks to reach nadir (worst point)
4. Plateau for 2-4 weeks
5. Recovery for 1-2 years
61
6 signs and symptoms of GBS
1. Initial paresthesias, usually hands and feet
2. Symmetric weakness with usually normal sensation
3. Ascending weakness distal to proximal and possibly autonomic
4. Flaccid paralysis with diminished or absent reflexes
5. CN involvement, especially weakness of facial muscles
6. May require ventilation due to respiratory failure
62
5 differential Dx's for GBS
1. Acute spinal cord disease
2. Brain stem ischemia
3. Myasthenia gravis
4. Botulinum intoxication
5. CIDP
63
Medical management of GBS
Either IVIG or Plasmapheresis
64
4 indicators of poor prognosis with GBS
1. Older age
2. Requiring vent support
3. Rapid progression (<7days)
4. Axonal injury
65
What is the key distinction between CIDP and GBS?
Progression time course
66
4 steps to the temporal course of CIDP
1. Insidious onset of symptoms >2months
2. Gradual progressive weakness may include: LMN, Sensory, Cranial, Autonomic
3. Treatment: stabilization with some improvement
4. May be relapsing
67
5. Signs and symptoms of CIDP
1. Gradual progressive weakness over at least 2 months
2. Areflexia and flaccid paralysis
3. Can have involvement of motor, sensory, and autonomic
4. Cranial neuropath is and respiratory muscle weakness are rare, but possible
5. Weakness may begin vocally becoming bilateral or multifocal within a few months
68
3 diagnostic studies for CIDP
1. EMG: fibrillations and + sharp waves (signs of denervation)
2. NCV: slowed velocity both motor and sensory
3. LP - Electrophoresis: elevated protein in CSF without increased cell count
69
8 Differential Dx's for CIDP
1. Multifocal stroke
2. Motor neuron disease
3. Polyradiculopathy
4. Inflammatory myopathy
5. NMJ disease
6. DM, B12 deficiency
7. MS
8. Tabes dorsalis
70
Medical management of CIDP
Prednisone, IVIG, Plasmapheresis, Azathioprine, Mycophenolate
71
Prognosis
Prolonged neurological disability, high relapse rate
72
4 Patterns of loss in Diabetic neuropathy
1. Distal symmetric sensorimotor neuropathy
2. Typical presentation of stocking-glove distribution
3. Paresthesias, dysesthesias
4. Later weakness
73
5 steps in the temporal course of diabetic neuropathy
1. DM
2. Sensory loss distal hands and feet in months-years
3. Weakness distal to proximal
4. Possible ulceration due to sensory loss
5. Possible limp amputation
74
6 methods of medical management for diabetic neuropathy
1. Optimal glucose control prevents and limits progression
2. Medications for neuropathic pain
3. Diabetic foot care
4. If autonomic symptoms present, refer to specialist
5. Exercise
6. Diet modification
75
Spinal cord ends at what level?
L1/L2
76
End of spinal cord called:
Conus medularis
77
Cauda equina considered:
LMN (L2 and below)
78
Injury to L2 vertebra affects:
conus medularis
79
What 2 systems make up the UMN?
1. Corticobulbar
| 2. LCST
80
8 spinal cord syndromes
1. Lateral cord (Brown-Sequard)
2. Complete cord
3. Central Cord
4. Anterior cord
5. Posterior cord
6. Pure motor
7. Conus
8. Cauda Equina
81
4 different MOI for SCI
1. Impact with persistent compression (burst fracture)
2. Impact with transient compression post hyper-injuries
3. Distraction: forcible stretching of spinal cord or blood supply
4. Laceration from missile injury, sharp bone fragment dislocation or severe distraction
82
Inflammation may occur up to ____ segments away
10
83
Inflammation (specifically in the L/S) can produce:
1. Maladaptive neuroplasticity
| 2. No motor learning
84
5 steps in the temporal course of SCI
1. Initial traumatic event
2. Spinal shock with flaccid paralysis and loss of sensation and motor function
3. Emergency response: immobilization and stabilization with diagnosis via MRI
4. Stable: possible UMN signs like spasticity and hyperreflexia
5. Neurologic return: most recovery within 1st year, especially first 6 months.
85
6 diagnostic studies for SCI
1. MRI
2. X-rays
3. CT
4. Somatosensory - evoked potentials
5. EMG
6. NCV
86
7 sues in rehab of SCI
1. Bladder dysfunction
2. Sexual dysfunction
3. Bowel dysfunction
4, Wheelchair seating
5. Decubitus ulcers
6. Spasticity
7. Autonomic dysfunction
87
Exaggerated, massive autonomic response to noxious stimuli
Autonomic dysreflexia
88
Autonomic dysreflexia is most common at what SCI level?
T6 and above
89
4 common causes of autonomic dysreflexia
1. Bowel/bladder distension
2. Ulcers
3. Constrictive clothing
4. Ingrown toenails
90
Common signs and symptoms of autonomic dysreflexia
```
Pounding headache
Hypertension
Diaphoresis
Red blotches
Goose bumps
Flushed face
Nausea
Slow pulse
Cold/clammy skin
Restlessness
Nasal congestion
```
91
How to determine sensory level in SCI
The most caudal level normally innervated dermatome for both pin prick and light touch (grade 2)
92
How to determine motor level in SCI
The most caudal level, normal or intact innervated spinal level (must be a grade greater than or equal to 3, provided the next most roster all key muscle tests as 5/5
93
```
Asia impairment scale
A:
B:
C:
D:
E:
```
A: complete - no motor or sensory function preserved in S4-5
B: incomplete - Sensory but not motor function is preserved below the neurological level and includes the sacral segments S4-5
C: incomplete - motor function is preserved below neurological level, and more than half of key muscles below the neurological level have a muscle grade less than 3
D: incomplete - motor function is preserved below the neurological level, and at lease half of the key muscles below the neurological level have a muscle grade of 3 or more
E: normal - motor and sensory function are normal
94
6 non-traumatic disorders of the spinal cord
1. Syringomyelia
2. Myelitis
3. SC infarction
4. Spinal epidural abscess
5. Spinal epi/subdural hematoma
6. Spina Bifida
95
Fluid-filled gliosis-lined, irregular cavity
Syringomyelia
96
A syrinx commonly disrupts what structures in the spinal cord?
Anterior horns of gray matter
97
4 signs of syringomyelia
1. Segmental atrophy
2. Loss of pain and temp
3. Areflexia
4. Proprioception intact
98
4 guidelines to conservative management of syringomyelia
1. Avoid high-force isometric contractions
2. Avoid valsalva expiration
3. Head elevation at night
4. Maintain neutral neck
99
3 surgical interventions for syringomyelia
1. Decompression
2. Shunt replacement
3. Tumor resection and radiation
100
Aperta:
Visible or open
101
Occulta:
Hidden or not visible
102
Spina bifida aperta:
Myelomeningocle
103
What is the most common form of spina bifida?
MMC
104
3 clinical finding with MMC
1. Degree of motor and/or sensor loss varies
2. Extrusion of neural tissue
3. Musculoskeletal deformities
105
Sensation of movement in the absence of stimuli: spinning, rocking tilting
Vertigo
106
Unsteadiness or imbalance, occuring mainly when standing up or walking and better when sitting or lying down
Disequilibrium
107
Presyncope, light-headed Ess., foggy head, special disorientation
Dizziness hypotension
108
MRI for vestibular dysfunction
Brain and internal auditory canals with or without dye to identify infarction or tumor
109
CT for vestibular dysfunction
Temporal bones, brain, and internal auditory canals to identify hemorrhage, infarction, or tumor
110
Audiometric exam for vestibular dysfunction
Distinguishes conductive or sensorineural loss
111
6 assessments of inner ear responses
1. Vestibular evoked myogenic potentials (VEMP)
2. Spontaneous eye movements
3. Position testing
4. Visual tracking
5. Caloric
6. Rotary chair testing
112
Nystagmus at rest is usually a _____ sign but could indicate accute ______ dysfunction
Usually central, could be acute peripheral dysfunction
113
4 signs of peripheral nystagmus
1. Direction fixed
2. May decrease with visual fixation
3. Exaggerated when looking in the direction of the fast beat
4. Characteristic mixed pattern of torsion with vertically, e.g., right torsion, upbeating
114
5 signs of central nystagmus
1. Direction changing nystagmus with changes in gaze
2. Direction changing nystagmus while remaining in one position
3. May increase With visual fixation
4. Pure vertical or pure torsional
5. Intense without subjective complaints of vertigo
115
Most common cause of vertigo
BPPV
116
2 special tests for BPPV
Dix hallpike: ant/post canals
| Roll test: horizontal canals
117
2 forms of BPPV
Canlithiasis
| Cupulolithiasis
118
4 signs/symptoms of BPPV
1. Vertigo + Nystagmus with positional testing
2. Brief latency
3. Lasting < 60 seconds canalithiasis
4. Lasting > 60 seconds cupulolithiasis
119
3 steps in temporal course for BPPV
1. Sudden onset of vertigo and nystagmus with positional change
2. Lasts seconds (possibly minutes)
3. Episodic vertigo and nystagmus with positional changes
120
Etiology of UVH
Neuritis:
Labryinthitis:
UVH: probably viral
Neuritis: CN VIII
Labryinthitis: Endolymph fluids
121
In UVH, symptoms are typically exacerbated by 1.______ due to inaccurate 2._____
1. Head movements
| 2. VOR
122
4 stages of temporal course with UVH
1. Sudden onset of vertigo, may experience nausea, vomiting, nystagmus
2. Lasting 1-3 days
3. After 3 days, decreased or no vertigo, but continued dizziness/disequilibrium
4. Symptoms improving but may still be worse with quick movements
123
Diagnostic study for UVH
VNG/ENG: identifies reduced unilateral response to caloric stimulation
124
Medical treatment of UVH
Meclizine
125
6 stages of temporal course with meniere's disease
1. Sudden onset of vertigo, tinnitus, fullness, hearing loss
2. Lasting minutes to hours
3. Resolution of symptoms
4. Sudden onset of vertigo, tinnitus, fullness, hearing loss
5. Fluctuating, progressive in nature
6. Eventual permanent hearing loss, disequilibrium
126
2 diagnostic tests for Meniere's disease
1. Audiogram
| 2. VNG/ENG - Calorics
127
Type of diet for meneir's disease
Low sodium, alcohol, nicotine, and caffeine
128
8 types of central vestibular dysfunction
1. Vestibular migraine
2. MS
3. TIA / stroke
4. TBI / concussion
5. Vertebrobasilar ischemia
6. Cerebellum disorders
7. Tumors
8. Drug intoxication
129
9 non-vestibular causes of dizziness
1. Multi-factorial faller
2. Orthostatic hypotension
3. Arrhythmia
4. Diabetes
5. Hypoglycemia
6. Infection
7. Medications
8. Panic attacks
9. Anxiety
130
5 types of movement disorders
1. Parkinson's disease
2. Atypical parkinsonian syndromes
3. Essential tremor
4. Huntington's disease
5. Dystonia
131
5 Main components of the basal ganglia
1. Caudate nucleus
2. Putamen
3. Globes pallidus
4. Subthalamic nucleus
5. Substantia nigra
132
3 hypokinesias
1. Akinesia
2. Bradykinesia
3. Rigidity
133
6 hyperkinesias
1. Resting tremor
2. Chorea
3. Athetosis
4. Tics
5. Hemiballism
6. Dystonia
134
Slowness of movement =
Bradykinesia
135
Loss or absense of movement =
Akinesia
136
Stiffness of muscle tone with passive movement =
Rigidity
137
Oscillatory, usually rhythmical and regular movement affecting one or more body parts
Tremor
138
Random, quick, unsustained, purposeless movements that have an unpredictable, flowing pattern
Chorea
139
Torsional movements that are partially sustained and produce twisting postures =
Dystonia
140
Sudden, brief, shock-like, involuntary movements usually caused by muscular contractions
Myoclonus
141
In PD, accumulation of 1.______ spreads through brain areas forming 2._____
1. Mis-folded protein alpha-synuclien
| 2. Lewy bodies
142
PD typical age of onset:
Young onset:
Late onset:
Typical: 55-60
Young: <40
Late: >78
143
5 stages of the temporal course for PD
1. Death of dopamine cells
2. Nonmotor symptoms (loss of smell, constipation) then motor symptoms
3. Dx
4. Progression of all symptoms leading to problems with mobility
5. Bed bound: dementia
144
Hoehn and Yahr stages 1-5
1. Symptoms on one side
2. Bi/Axial: no balance impairment
3. Balance impairment, physical independent
4. Severe disability, able to stand or walk
5. W/C bound or bed ridden
145
2 symptoms of which one is required to make a PD diagnosis
1. Bradykinesia
| 2. Resting tremor
146
6 most common nonmotor PD symptoms
1. Neuro-pychiatric and cognitive
2. Sleep disorders
3. Sensory awareness
4. Fatigue
5. Bradyphrenia
6. Autonomic dysfunction
147
5 common motor features of PD
1. Hypophonia
2. Hypomimia (masked face)
3. Microgrphia
4. Gait abnormality: shuffling, freezing, en bloc turns, small stride
5. Stooped/flexed posture
148
Symptomatic improvement with what drug helps to confirm idiopathic PD dx
Levadopa
149
2 out of the following 6 symptoms dx's PD
1. Rest tremor
2. Bradykinesia
3. Freezing
4. Flexed posture
5. Rigidity
6. Loss of postural responses
150
7 forms of secondary Parkinsonism
1. drug induced
2. Vascular
3. Hydrocephalus
4. Trauma
5. Metabolic
6. Tumors
7. Toxins
151
4 forms of atypical parkinsonian syndromes
1. MSA
2. PSP
3. Dementia with Lewy bodies
4. CBD
152
5 ways to help prevent PD
1. Diet
2. Exercise
3. Environmental agents
4. Neuroprotection
5. Early detection and tx
153
Trade name for carbidopa/levodopa
Sinemet
154
Typical progression
Mild PD:
Moderate PD:
Advanced PD:
Mild: 5-7 years
Moderate: 7-15 years
Advanced: 15-20 years
155
What is the most frequent atypical parkinsonian syndrome
PSP
156
PSP affects not only the astrocytes in the basal ganglia but also in the
Brainstem
157
How does PSP compare to PD?
Progresses more rapidly than PD and death often occurs in 5-10 years due to aspiration
158
6 Key features of PSP
1. Progressive parkinsonism
2. Vertical Supra nuclear ocular palsy or slow vertical saccades
3. Early onset of falling
4. Axial rigidity
5. Facial dystonia
6. Usually no tremor
159
Key features of MSA
1. Parkinsonism
2. Symptomatic orthostatic hypotension
3. Cerebellum ataxia
4. Poor therapeutic response to levadopa
160
Key features of CBD
1. Parkinsonism
2. Unilateral arm rigidity and dystonia
3. Cortical sensory deficits
161
What is the cause of essential tremor?
Neurodegeneration of the cerebellum or abnormal GABA function
