Lower Motor Neuron Disease Flashcards
(263 cards)
1
Q
Two LMN disease due to lesion in the AHC
A
Spinal Muscular Atrophy and Poliomyelitis
2
Q
Five non-traumatic PNI
A
Infectious, Diabetes, Immune-Mediated, Toxic, Hereditary Neuropathy
3
Q
Three LMN disease due to lesion in the NMJ
A
Myasthenia Gravis, Lambert-Eaton Myasthenic Syndrome, Botulism
4
Q
A group of autosomal RECESSIVE disorders with degeneration in the AHC
A
Spinal Muscular Atrophy
5
Q
What is the affected chromosome in SMA?
A
Chromosome 5
6
Q
What is the affected gene in SMA?
A
SMN 1 (Survival Motor Neuron)
7
Q
What is the substitute of SMN 1?
A
SMN 2
8
Q
The severity of the SMA depends on the number of what gene in the body?
A
SMN 2
9
Q
What is the only medication for SMA?
A
Spinraza
10
Q
What does spinraza do to help patients with SMA?
A
Strengthen SMN 2
11
Q
What is the unique manifestation of SMA patients?
A
Increase IQ
12
Q
SMA: Later onset
A
SMA 3
13
Q
SMA: Adult-onset
A
SMA 4
14
Q
SMA: 3-6 months onset
A
SMA 1
15
Q
SMA: Near normal lifespan
A
SMA 3
16
Q
SMA: (+) severe and progressive muscle weakness
A
SMA 1
17
Q
SMA: Infantile onset
A
SMA 1
18
Q
SMA: Chronic Juvenile
A
SMA 3
19
Q
SMA: 6-18 months onset
A
SMA 2
20
Q
SMA: (+) Gower’s sign
A
SMA 3
21
Q
What is the name of SMA 1?
A
Acute Werdnig - Hoffman
22
Q
What is the name of SMA 3?
A
Kugelberg-Walender
23
Q
What is the name of SMA 2?
A
Chronic Werdnig Hoffman
24
Q
What is the lifespan SMA 2?
A
Mid 20’s
25
What is the common cause of death of SMA 1?
Respiratory Failure
26
SMA: (+) sit, stand, walk
SMA 3
27
What is the hallmark of SMA 1?
Tongue Fasciculations
28
What are the 2 deformities under Arthrogryposis?
Scoliosis and Severe joint contracture
29
What are the 2 deformities present in SMA 1?
UE: Jughandle position; LE: Frog-leg position
30
What is the position of Jughandle and Frog-leg position?
ABER
31
SMA: after 18 months; 5-15 years onset
SMA 3
32
SMA: <2 years lifespan
SMA 1
33
What is the muscle weakness present in SMA 3?
Static, very, slowly, progressive muscle weakness
34
"Kennedy's Disease"
Spinobulbar Muscle Atrophy
35
Who is predominately affected by Kennedy's disease?
Male
36
What is type of genetic disorder is Kennedy's disease?
X-linked recessive
37
What is the gene affected in Kennedy's disease?
Androgen Receptor Gene
38
What are the 3 LMNL manifestations of Kennedy's disease?
(+) Generalized Weakness, (+) CN involvement, (+) Endocrinopathy
39
What are the 3 manifestations of Endocrinopathy in Kennedy's disease?
Gynecomastia, Decrease Fertility, Testicular Atrophy
40
What is disease due to inflammation of the gray matter in the AHC?
Poliomyelitis
41
What is the other name of Poliomyelitis?
Heine-Medin Disease
42
What is the epidemiology of Poliomyelitis?
Male = Female; Child > Adult
43
What is the etiology of Poliomyelitis?
Enteroviral (Picorna Virus)
44
What is the route of Poliomyelitis?
Fecal-oral
45
Polio Virus: Most Fatal
Leon
46
Polio Virus: Most Paralytogenic
Brunhilde
47
Polio Virus: Most Frequent
Lansing
48
What is the key prevention of Poliomyelitis?
Vaccine
49
What vaccine is administered intramuscularly?
Salk
50
What vaccine is administered orally?
Sabin
51
Two types of Acute Minor Polio
Asymptomatic and Abortive
52
Two types of Acute Major Polio
Non-paralytic and Paralytic
53
Acute Polio: (+) Viremia
Asymptomatic
54
Acute Polio: (+) Meningitis
Non-paralytic
55
Acute Polio: (+) Influenza-like symptoms
Abortive
56
Three types of Paralytic Polio
Spinal, Bulbar, Bulbospinal
57
Paralytic Polio: Lesion at AHC
Spinal Polio
58
Paralytic Polio: Phrenic Nerve Involvement
Bulbospinal
59
The first area to get damage in Spinal Polio
Lumbar Area
60
The first area to atrophy in the Spinal Polio
Proximal > Distal
61
The first muscle to atrophy in the Spinal Polio
Quadriceps
62
The cranial nerves affected in Bulbar Polio
CN 5, 9, 10, 11
63
The common cause of death of Bulbospiral Polio
Respiratory Failure
64
What extremity is more affected in Spinal Polio?
LE > UE
65
How long does the acute stage of polio last?
<2 years
66
What stage of polio rehabilitation starts due to all the s/sx subsides?
Recovery Stage
67
What stage where the recovery of muscle plateaus?
Stage of Chronicity
68
What paralysis is present in the Stage of Chronicity?
Residual Paralysis
69
What are the criteria used to identify if the patient is post-polio syndrome?
Halstead and Rossi Criteria
70
How long should the partial to complete functional recovery patient has to confirm it is post-polio syndrome?
15 years
71
How long should the symptoms persist a patient has to confirm it is a post-polio syndromre?
1 year
72
What are the three other items of Halstead and Rossi Criteria?
Confirmed history of paralytic polio, onset of new muscle weakness, no other medial diagnosis
73
NMJ: Post-synaptic disorder
Myasthenia Gravis
74
NMJ: Incrementing Muscle Response
LEMS
75
NMJ: Decrementing Muscle Response
Myasthenia Gravis
76
NMJ: Pre-synaptic disorder
LEMS
77
Myasthenia Gravis is common in what type of patients?
Thymoma
78
LEMS is common in what type of disease?
Lung cancer
79
What cell is present in LEMS?
Oat cell / Small cells
80
What is the protein that kills ACH in Myasthenia Gravis?
Acetylcholinesterase
81
What gender is predominantly affected with Myasthenia Gravis?
Females
82
What gender is predominantly affected with LEMS?
Male
83
What do the antibodies destroy in Myasthenia Gravis?
ACH receptors
84
What do the antibodies destroy in LEMS?
Calcium Channel
85
What CN involved in Myasthenia Gravis?
CN 3,4,6
86
What is the most common symptom in MG?
Ptosis >> Opthalmoplegia
87
What is the extremity more affected in MG and LEMS?
Proximal > Distal
88
What are the symptoms present in MG that is also presenting MS?
Heat Sensitivity
89
What nerve affectation present in LEMS?
Autonomic Nerves
90
What is the diagnostic tool of MG?
Tensilon Test / Endophonium
91
What is the area tested in Tensilon Test?
EOM
92
What are the three medications of MG?
Stigmine = Neostigmine, Pyridostigmine, Physostigmine
93
How long is the half-life of Stigmine medications?
2-4 hours
94
What is the medications of LEMS?
Guanidine
95
What is the mechanism Guanidine used to help LEMS patients?
Helps ACH to flow out from NMJ
96
What is the crisis due to the over-accumulation of ACH in the synaptic cleft?
Cholinergic Crisis
97
NMJ disease due to clostridium botulism toxin?
Botulism
98
What type of synaptic disorder botulism is?
Pre-synaptic
99
What botulism causes in the pre-synaptic NMJ?
Total nerve blockage
100
Three sources of botulism?
Infantile, Wound, Food
101
What is the nerve affected is botulism?
Phrenic Nerve Affectation
102
What pattern does the weakness present in botulism?
Descending pattern
103
What are the 2 weaknesses present in botulism?
Generalized and Oculobulbar muscle weakness
104
True or False: botulism has autonomic dysfunction?
True
105
What is the common cause of death in botulism?
Respiratory Failure
106
What is the medications for botulism?
HBAT: Heptavalent Botulinum Anti-Toxin
107
What disease is an example of Immune-mediated Non-traumatic PNI?
GBS
108
Two other names of GBS
Acute Inflammatory Demyelinating Polyneuropathy, Landry's Paralysis
109
What is the demyelinating disease of CNS?
Multiple Sclerosis
110
What is the demyelinating disease of PNS?
GBS
111
What most common paralysis GBS results in?
Acute Flaccid Paralysis
112
6 most common clinical presentation of GBS?
API-SAD: Autoimmune, Post-infectious, Idiopathic, Self-limiting, Ascending, Demyelinating
113
What protein causes GBS?
IgG
114
What is the 3 intervention to combat GBS IgG?
Plasma Pheresis, IV Ig, Rehabilitation
115
What is the common virus that causes GBS?
Campylobacter jejuni
116
What are the other two viruses that cause GBS?
Cytomegalovirus, Epstein Barr Virus
117
When does recovery start in GBS?
3-18 months
118
True or False: There is a full recovery in GBS
True
119
What are the 5 poor prognosticating factors of GBS?
MADCA: Male, Advance Age, Diarrhea, Cytomegalovirus, Axonal Form
120
What is the hallmark paralysis of GBS?
ABS: Ascending, Bilateral, Symmetrical
121
What are the two diagnostic tools of GBS?
NCV and Cytoalbuminologic dissociations
122
True or False: GBS has increase NCV?
False
123
True or False: There is an increase in albumin and decrease cells in the GBS
True
124
What is the common cause of death in GBS?
Respiratory Failure
125
What are four types of GBS?
AIDP, Axonal Forms, Miller Fisher Variant, Acute Pandysautonomia
126
Type of GBS: South America and Asia
Axonal Forms
127
Type of GBS: North America and Europe
AIDP
128
Type of GBS: classic and most common
AIDP
129
Type of GBS: descending form
Miller Filler Variant
130
Type of GBS: Most Fatal
Acute Pandysautonomia
131
What is the CN affected in AIDP?
CN 7, 9, 10, 11
132
What is the CN spared in AIDP?
CN 3, 4, 6
133
What the most common CN affected in AIDP?
CN 7
134
What are three manifestations of AIDP motor paralysis?
LE>UE; Symmetrical, Proximal > Distal
135
What is the most common presenting symptom?
Paresthesia (Gloves and stocking sensation)
136
What LMN lesion seen in AIDP?
Areflexia
137
What type of pain presents in AIDP?
Aching pain on the affected muscle
138
What are the spared structures in AIDP?
Ocular Muscle, Sensory System, Onufruwic's nucleus
139
Two axonal forms of GBS?
AMAN and AMSAN
140
What age group predominantly affected in the AMSAN?
Pedia
141
What age group predominantly affected in the AMAN?
Geria
142
What is the triad of Miller-Fisher Variant?
AOA: Ataxia, Areflexia, Ophtalmoplegia
143
What is the CN affected in Miller-Fisher Variant?
CN 3, 4, 6
144
What nerve affectation is present in Acute Pandysautonomia?
Autonomic nerve affectation
145
What are the three causes of death of Acute Pandysautonomia?
Cardiac failure, respiratory failure, infection
146
"Tic borne disorder"
Lyme's disease
147
What bacteria causes Lyme's disease?
Spirochaetes Borella Burgdorferi
148
What is the medication used for Lyme's disease?
Ceftriaxone
149
Most common neurologic abnormality of Lyme's disease?
Meningitis
150
Most common CN affected in Lyme's disease?
CN 7
151
What extremity is more affected in Lyme's disease?
LE > UE
152
Stage of Lyme: (+) distal symmetric affectation
Late infection
153
Stage of Lyme: (+) erythematous lesion
Early infection
154
Stage of Lyme: (+) CN affectation
Disseminated Infection
155
"Harsen's disease"
Leprosy
156
What bacteria causes leprosy?
Myobacterium Leprae
157
What is the medication used for Leprosy?
Dapsone
158
Two types of leprosy
Tuberculoid and Lepromatous
159
Type of Leprosy: Severe
Lepromatous
160
Type of Leprosy: (+) Lepromin test
Tuberculoid
161
Type of Leprosy: Mild
Tuberculoid
162
Type of Leprosy: Multibacillus
Lepromatous
163
Type of Leprosy: (+) granulomas
Tuberculoid
164
Type of Leprosy: (+) direct nerve involvement
Lepromatous
165
Type of Leprosy: (+) skin lesion
Tuberculoid
166
Type of Leprosy: Paucibacillus
Tuberculoid
167
The cranial nerve affected in Leprosy
CN 7 (facial) and 5 (posterior auricular)
168
The UE nerve affected in Leprosy
Ulnar, Median, Superficial Radial Nerve
169
The LE nerve affected in Leprosy
Common Peroneal, Sural, Interdigital Nerve
170
The most common cause of non-traumatic PNI
Diabetic Neuropathy
171
What does high blood sugar inhibit in the body?
Nitric oxide (vasodilator)
172
Three types of diabetic neuropathy
Symmetric, Asymmetric, Focal
173
The 3 cranial nerve affected in Focal diabetic neuropathy
CN 3, 6, 7
174
The 3 peripheral nerve affected in Focal diabetic neuropathy
Median, Ulnar, Peroneal
175
What is the most common diabetic neuropathy
Symmetric
176
"Diabetic Amyotrophy"
Asymmetric
177
The 2 peripheral nerve affected in asymmetric diabetic neuropathy
Femoral and Obturator nerve
178
What the extremity affected in symmetric and asymmetric diabetic neuropathy?
LE > UE (Proximal)
179
"Chronic sensorimotor distal polyneuropathy"
Symmetric
180
"Proximal motor neuropathy"
Asymmetric
181
Two types of toxic neuropathy
Alcohol and Lead
182
What causes toxic neuropathy?
Decrease Vitamin B1 (thiamine)
183
What condition is caused by a decrease in Vit B1?
Beri-beri
184
Other names of Alcohol Toxic Neuropathy
Wernicke's - korsakoff disease
185
What is the triad of Alcohol Toxic Neuropathy?
ADO: Ataxia, Dementia, Opthalmoplegia
186
What is the nerve affected in mild lead toxic neuropathy?
Radial nerve
187
What is deformity present in lead toxic neuropathy?
(B) wrist drop
188
What condition results from severe lead toxic neuropathy?
ALS
189
What is the most common neuropathy seen in lead toxic neuropathy?
Motor > Sensory
190
Hereditary motor sensory neuropathy
Charcot-Marie-Tooth Disease
191
What is the muscle atrophies in CMTD?
Peroneal muscle
192
What is the chromosome affected in CMTD?
Chromosome 17
193
What is the genetic course of CMTD?
Autosomal Dominant
194
What is the gene affected in CMTD?
CMT
195
What are the two-foot deformities seen in CMTD?
Pes Cavus and Stork Leg deformity
196
What sign seen in stork leg deformity?
Inverted Champagne Bottle Sign
197
Type of CMTD: Refsum's Disease
CMTD 4
198
Type of CMTD: (+) Spinocerebellar Ataxia
CMTD 5
199
Type of CMTD: Hypertrophic myelin sheath
CMTD 1
200
Type of CMTD: Axonal
CMTD 2
201
Type of CMTD: (+) Optic neuropathy
CMTD 6
202
Type of CMTD: Dejerine-Sotta
CMTD 3
203
What is the hallmark of CMTD 1?
Onion-bulb formation
204
What causes the onion-bulb formation?
Frequent demyelination and remyelination
205
True or False: There is an increase in NCV in CMTD 1
FALSE (always decrease in demyelinating disease)
206
What acid over accumulates in the Schwann cells in CMTD 4?
Phytanic Acid
207
Type of CMTD: (+) Refinitis Pigmentosa
CMTD 7
208
Type of CMTD: Severe
CMTD 3
209
Type of CMTD: Autosomal Dominant
CMTD 1, 2, 3
210
Type of CMTD: Autosomal Recessive
CMTD 3, 4
211
Type of CMTD: (+) Hypertrophic neuropathic on infancy
CMTD 3
212
Type of CMTD: 1st decade
CMTD 1
213
Type of CMTD: 2nd decade
CMTD 2
214
What is the cranial nerve affected in CMTD 4?
CMTD 1 and 8
215
What NCV results present in CMTD 2?
Normal NCV
216
What is refinits pigmentosa?
Blindess of both eyes
217
What amyotrophic means?
Muscle atrophy
218
What lateral sclerosis means?
Hardening of lateral SC
219
3 other names of ALS
Adult motor neuron disease, Charcot's disease, Lou Gehrig's disease
220
Epidemiology of ALS
M>F; 40-60 years old
221
What is the etiologic of ALS?
Idiopathic
222
What is the medication for ALS?
Riluzole / Rilutek
223
What is the NT that increases the death of motor neurons?
Glutamate
224
What are the diagnostic criteria used to diagnose ALS?
El Escorial Criteria
225
What is the 4 region used in El Escorial Criteria
Head, trunk, UE, LE
226
El Escorial: (+) UMN of 2 regions
Clinically Possible
227
El Escorial: (+) UMN and LMN in 3-4 regions
Clinically Definite
228
El Escorial: (+) UMN and LMN in 2 regions
Clinical Probable
229
El Escorial: (+) UMN and LMN in 1 region
Clinically Possible
230
What are the 3 variants of ALS?
Pure UMN, Pure LMN, Pure CN
231
Variant: Pure UMN
Primary Lateral Sclerosis
232
Variant: Pure CN
Progressive Bulbar Palsy
233
Variant: Pure LMN
Progressive Muscular Atrophy
234
What is the cardinal sign of LMN-ALS
Cervical extensor weakness (Head drop)
235
What is the weight loss seen in ALS?
ALS cachexia
236
What causes ALS cachexia?
Muscle atrophy and decrease intake
237
What deformity seen in the hands of ALS patients?
Cadaveric or Skeletal hand
238
What 6 cranial nerves affected by ALS?
CN 5, 7, 9, 10, 11, 12
239
What 6 structures spared in ALS?
COMONS: Cerebellar system, Ocular muscle (CN 3,4,6), Mental status, Onufruwic's Nucleus, Non-motor nerves (CN 1,2,8), Sensory
240
AHC in cervical nerve 3-4
Phrenic
241
AHC in spinal nerve 2
Onufruwic
242
What is the function of Onufruwic's nucleus?
For bowel and bladder
243
What are the 5 poor prognosticating factors of ALS?
BPOLS: Bulbar dysfunction, Pulmonary Dysfunction, Old Age, LMN at onset, Short-time period from onset to diagnosis
244
What are the 5 types of ALS according to etiology?
Sporadic, Familial, Juvenile, Guamanian, Secondary
245
Type of ALS: Most common and classic form
Sporadic ALS
246
Type of ALS: Autosomal Dominant
Familial ALS
247
Type of ALS: <25 years old
Juvenile ALS
248
Type of ALS: secondary to lead toxicity and cancer
Secondary ALS
249
Type of ALS: western pacific
Guamanian ALS
250
What chromosome is affected in Familial ALS?
Chromosome 21
251
What gene is affected in Familial ALS?
SOD 1 (superoxide dismutase)
252
What toxicity of the Guamanian ALS?
Cycadseed Toxicity
253
Juvenile ALS: teenager
ALS 5
254
Juvenile ALS: <10 years
ALS 2
255
Juvenile ALS: <25 years
ALS 4
256
Juvenile ALS: Autosomal Dominant
ALS 4
257
Juvenile ALS: Autosomal Recessive
ALS 5, 2
258
Type of ALS: (+) frontotemporal dementia
Guamanian ALS
259
What is the chromosome affected in ALS 5?
Chromosome 15
260
What is the chromosome affected in ALS 2?
Chromosome 2
261
What is the chromosome affected in ALS 4?
Chromosome 9
262
Most inheritable neuropathy?
CMTD
263
Benchmark of motor neuron disease?
ALS
