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Neurology > Lower Motor Neuron Disease > Flashcards

Lower Motor Neuron Disease Flashcards

1
Q

Two LMN disease due to lesion in the AHC

A

Spinal Muscular Atrophy and Poliomyelitis

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2
Q

Five non-traumatic PNI

A

Infectious, Diabetes, Immune-Mediated, Toxic, Hereditary Neuropathy

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3
Q

Three LMN disease due to lesion in the NMJ

A

Myasthenia Gravis, Lambert-Eaton Myasthenic Syndrome, Botulism

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4
Q

A group of autosomal RECESSIVE disorders with degeneration in the AHC

A

Spinal Muscular Atrophy

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5
Q

What is the affected chromosome in SMA?

A

Chromosome 5

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6
Q

What is the affected gene in SMA?

A

SMN 1 (Survival Motor Neuron)

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7
Q

What is the substitute of SMN 1?

A

SMN 2

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8
Q

The severity of the SMA depends on the number of what gene in the body?

A

SMN 2

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9
Q

What is the only medication for SMA?

A

Spinraza

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10
Q

What does spinraza do to help patients with SMA?

A

Strengthen SMN 2

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11
Q

What is the unique manifestation of SMA patients?

A

Increase IQ

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12
Q

SMA: Later onset

A

SMA 3

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13
Q

SMA: Adult-onset

A

SMA 4

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14
Q

SMA: 3-6 months onset

A

SMA 1

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15
Q

SMA: Near normal lifespan

A

SMA 3

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16
Q

SMA: (+) severe and progressive muscle weakness

A

SMA 1

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17
Q

SMA: Infantile onset

A

SMA 1

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18
Q

SMA: Chronic Juvenile

A

SMA 3

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19
Q

SMA: 6-18 months onset

A

SMA 2

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20
Q

SMA: (+) Gower’s sign

A

SMA 3

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21
Q

What is the name of SMA 1?

A

Acute Werdnig - Hoffman

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22
Q

What is the name of SMA 3?

A

Kugelberg-Walender

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23
Q

What is the name of SMA 2?

A

Chronic Werdnig Hoffman

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24
Q

What is the lifespan SMA 2?

A

Mid 20’s

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25
Q

What is the common cause of death of SMA 1?

A

Respiratory Failure

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26
Q

SMA: (+) sit, stand, walk

A

SMA 3

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27
Q

What is the hallmark of SMA 1?

A

Tongue Fasciculations

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28
Q

What are the 2 deformities under Arthrogryposis?

A

Scoliosis and Severe joint contracture

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29
Q

What are the 2 deformities present in SMA 1?

A

UE: Jughandle position; LE: Frog-leg position

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30
Q

What is the position of Jughandle and Frog-leg position?

A

ABER

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31
Q

SMA: after 18 months; 5-15 years onset

A

SMA 3

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32
Q

SMA: <2 years lifespan

A

SMA 1

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33
Q

What is the muscle weakness present in SMA 3?

A

Static, very, slowly, progressive muscle weakness

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34
Q

“Kennedy’s Disease”

A

Spinobulbar Muscle Atrophy

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35
Q

Who is predominately affected by Kennedy’s disease?

A

Male

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36
Q

What is type of genetic disorder is Kennedy’s disease?

A

X-linked recessive

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37
Q

What is the gene affected in Kennedy’s disease?

A

Androgen Receptor Gene

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38
Q

What are the 3 LMNL manifestations of Kennedy’s disease?

A

(+) Generalized Weakness, (+) CN involvement, (+) Endocrinopathy

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39
Q

What are the 3 manifestations of Endocrinopathy in Kennedy’s disease?

A

Gynecomastia, Decrease Fertility, Testicular Atrophy

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40
Q

What is disease due to inflammation of the gray matter in the AHC?

A

Poliomyelitis

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41
Q

What is the other name of Poliomyelitis?

A

Heine-Medin Disease

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42
Q

What is the epidemiology of Poliomyelitis?

A

Male = Female; Child > Adult

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43
Q

What is the etiology of Poliomyelitis?

A

Enteroviral (Picorna Virus)

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44
Q

What is the route of Poliomyelitis?

A

Fecal-oral

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45
Q

Polio Virus: Most Fatal

A

Leon

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46
Q

Polio Virus: Most Paralytogenic

A

Brunhilde

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47
Q

Polio Virus: Most Frequent

A

Lansing

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48
Q

What is the key prevention of Poliomyelitis?

A

Vaccine

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49
Q

What vaccine is administered intramuscularly?

A

Salk

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50
Q

What vaccine is administered orally?

A

Sabin

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51
Q

Two types of Acute Minor Polio

A

Asymptomatic and Abortive

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52
Q

Two types of Acute Major Polio

A

Non-paralytic and Paralytic

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53
Q

Acute Polio: (+) Viremia

A

Asymptomatic

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54
Q

Acute Polio: (+) Meningitis

A

Non-paralytic

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55
Q

Acute Polio: (+) Influenza-like symptoms

A

Abortive

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56
Q

Three types of Paralytic Polio

A

Spinal, Bulbar, Bulbospinal

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57
Q

Paralytic Polio: Lesion at AHC

A

Spinal Polio

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58
Q

Paralytic Polio: Phrenic Nerve Involvement

A

Bulbospinal

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59
Q

The first area to get damage in Spinal Polio

A

Lumbar Area

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60
Q

The first area to atrophy in the Spinal Polio

A

Proximal > Distal

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61
Q

The first muscle to atrophy in the Spinal Polio

A

Quadriceps

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62
Q

The cranial nerves affected in Bulbar Polio

A

CN 5, 9, 10, 11

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63
Q

The common cause of death of Bulbospiral Polio

A

Respiratory Failure

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64
Q

What extremity is more affected in Spinal Polio?

A

LE > UE

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65
Q

How long does the acute stage of polio last?

A

<2 years

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66
Q

What stage of polio rehabilitation starts due to all the s/sx subsides?

A

Recovery Stage

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67
Q

What stage where the recovery of muscle plateaus?

A

Stage of Chronicity

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68
Q

What paralysis is present in the Stage of Chronicity?

A

Residual Paralysis

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69
Q

What are the criteria used to identify if the patient is post-polio syndrome?

A

Halstead and Rossi Criteria

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70
Q

How long should the partial to complete functional recovery patient has to confirm it is post-polio syndrome?

A

15 years

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71
Q

How long should the symptoms persist a patient has to confirm it is a post-polio syndromre?

A

1 year

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72
Q

What are the three other items of Halstead and Rossi Criteria?

A

Confirmed history of paralytic polio, onset of new muscle weakness, no other medial diagnosis

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73
Q

NMJ: Post-synaptic disorder

A

Myasthenia Gravis

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74
Q

NMJ: Incrementing Muscle Response

A

LEMS

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75
Q

NMJ: Decrementing Muscle Response

A

Myasthenia Gravis

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76
Q

NMJ: Pre-synaptic disorder

A

LEMS

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77
Q

Myasthenia Gravis is common in what type of patients?

A

Thymoma

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78
Q

LEMS is common in what type of disease?

A

Lung cancer

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79
Q

What cell is present in LEMS?

A

Oat cell / Small cells

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80
Q

What is the protein that kills ACH in Myasthenia Gravis?

A

Acetylcholinesterase

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81
Q

What gender is predominantly affected with Myasthenia Gravis?

A

Females

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82
Q

What gender is predominantly affected with LEMS?

A

Male

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83
Q

What do the antibodies destroy in Myasthenia Gravis?

A

ACH receptors

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84
Q

What do the antibodies destroy in LEMS?

A

Calcium Channel

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85
Q

What CN involved in Myasthenia Gravis?

A

CN 3,4,6

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86
Q

What is the most common symptom in MG?

A

Ptosis&raquo_space; Opthalmoplegia

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87
Q

What is the extremity more affected in MG and LEMS?

A

Proximal > Distal

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88
Q

What are the symptoms present in MG that is also presenting MS?

A

Heat Sensitivity

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89
Q

What nerve affectation present in LEMS?

A

Autonomic Nerves

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90
Q

What is the diagnostic tool of MG?

A

Tensilon Test / Endophonium

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91
Q

What is the area tested in Tensilon Test?

A

EOM

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92
Q

What are the three medications of MG?

A

Stigmine = Neostigmine, Pyridostigmine, Physostigmine

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93
Q

How long is the half-life of Stigmine medications?

A

2-4 hours

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94
Q

What is the medications of LEMS?

A

Guanidine

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95
Q

What is the mechanism Guanidine used to help LEMS patients?

A

Helps ACH to flow out from NMJ

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96
Q

What is the crisis due to the over-accumulation of ACH in the synaptic cleft?

A

Cholinergic Crisis

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97
Q

NMJ disease due to clostridium botulism toxin?

A

Botulism

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98
Q

What type of synaptic disorder botulism is?

A

Pre-synaptic

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99
Q

What botulism causes in the pre-synaptic NMJ?

A

Total nerve blockage

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100
Q

Three sources of botulism?

A

Infantile, Wound, Food

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101
Q

What is the nerve affected is botulism?

A

Phrenic Nerve Affectation

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102
Q

What pattern does the weakness present in botulism?

A

Descending pattern

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103
Q

What are the 2 weaknesses present in botulism?

A

Generalized and Oculobulbar muscle weakness

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104
Q

True or False: botulism has autonomic dysfunction?

A

True

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105
Q

What is the common cause of death in botulism?

A

Respiratory Failure

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106
Q

What is the medications for botulism?

A

HBAT: Heptavalent Botulinum Anti-Toxin

107
Q

What disease is an example of Immune-mediated Non-traumatic PNI?

A

GBS

108
Q

Two other names of GBS

A

Acute Inflammatory Demyelinating Polyneuropathy, Landry’s Paralysis

109
Q

What is the demyelinating disease of CNS?

A

Multiple Sclerosis

110
Q

What is the demyelinating disease of PNS?

A

GBS

111
Q

What most common paralysis GBS results in?

A

Acute Flaccid Paralysis

112
Q

6 most common clinical presentation of GBS?

A

API-SAD: Autoimmune, Post-infectious, Idiopathic, Self-limiting, Ascending, Demyelinating

113
Q

What protein causes GBS?

A

IgG

114
Q

What is the 3 intervention to combat GBS IgG?

A

Plasma Pheresis, IV Ig, Rehabilitation

115
Q

What is the common virus that causes GBS?

A

Campylobacter jejuni

116
Q

What are the other two viruses that cause GBS?

A

Cytomegalovirus, Epstein Barr Virus

117
Q

When does recovery start in GBS?

A

3-18 months

118
Q

True or False: There is a full recovery in GBS

A

True

119
Q

What are the 5 poor prognosticating factors of GBS?

A

MADCA: Male, Advance Age, Diarrhea, Cytomegalovirus, Axonal Form

120
Q

What is the hallmark paralysis of GBS?

A

ABS: Ascending, Bilateral, Symmetrical

121
Q

What are the two diagnostic tools of GBS?

A

NCV and Cytoalbuminologic dissociations

122
Q

True or False: GBS has increase NCV?

A

False

123
Q

True or False: There is an increase in albumin and decrease cells in the GBS

A

True

124
Q

What is the common cause of death in GBS?

A

Respiratory Failure

125
Q

What are four types of GBS?

A

AIDP, Axonal Forms, Miller Fisher Variant, Acute Pandysautonomia

126
Q

Type of GBS: South America and Asia

A

Axonal Forms

127
Q

Type of GBS: North America and Europe

A

AIDP

128
Q

Type of GBS: classic and most common

A

AIDP

129
Q

Type of GBS: descending form

A

Miller Filler Variant

130
Q

Type of GBS: Most Fatal

A

Acute Pandysautonomia

131
Q

What is the CN affected in AIDP?

A

CN 7, 9, 10, 11

132
Q

What is the CN spared in AIDP?

A

CN 3, 4, 6

133
Q

What the most common CN affected in AIDP?

A

CN 7

134
Q

What are three manifestations of AIDP motor paralysis?

A

LE>UE; Symmetrical, Proximal > Distal

135
Q

What is the most common presenting symptom?

A

Paresthesia (Gloves and stocking sensation)

136
Q

What LMN lesion seen in AIDP?

A

Areflexia

137
Q

What type of pain presents in AIDP?

A

Aching pain on the affected muscle

138
Q

What are the spared structures in AIDP?

A

Ocular Muscle, Sensory System, Onufruwic’s nucleus

139
Q

Two axonal forms of GBS?

A

AMAN and AMSAN

140
Q

What age group predominantly affected in the AMSAN?

A

Pedia

141
Q

What age group predominantly affected in the AMAN?

A

Geria

142
Q

What is the triad of Miller-Fisher Variant?

A

AOA: Ataxia, Areflexia, Ophtalmoplegia

143
Q

What is the CN affected in Miller-Fisher Variant?

A

CN 3, 4, 6

144
Q

What nerve affectation is present in Acute Pandysautonomia?

A

Autonomic nerve affectation

145
Q

What are the three causes of death of Acute Pandysautonomia?

A

Cardiac failure, respiratory failure, infection

146
Q

“Tic borne disorder”

A

Lyme’s disease

147
Q

What bacteria causes Lyme’s disease?

A

Spirochaetes Borella Burgdorferi

148
Q

What is the medication used for Lyme’s disease?

A

Ceftriaxone

149
Q

Most common neurologic abnormality of Lyme’s disease?

A

Meningitis

150
Q

Most common CN affected in Lyme’s disease?

A

CN 7

151
Q

What extremity is more affected in Lyme’s disease?

A

LE > UE

152
Q

Stage of Lyme: (+) distal symmetric affectation

A

Late infection

153
Q

Stage of Lyme: (+) erythematous lesion

A

Early infection

154
Q

Stage of Lyme: (+) CN affectation

A

Disseminated Infection

155
Q

“Harsen’s disease”

A

Leprosy

156
Q

What bacteria causes leprosy?

A

Myobacterium Leprae

157
Q

What is the medication used for Leprosy?

A

Dapsone

158
Q

Two types of leprosy

A

Tuberculoid and Lepromatous

159
Q

Type of Leprosy: Severe

A

Lepromatous

160
Q

Type of Leprosy: (+) Lepromin test

A

Tuberculoid

161
Q

Type of Leprosy: Mild

A

Tuberculoid

162
Q

Type of Leprosy: Multibacillus

A

Lepromatous

163
Q

Type of Leprosy: (+) granulomas

A

Tuberculoid

164
Q

Type of Leprosy: (+) direct nerve involvement

A

Lepromatous

165
Q

Type of Leprosy: (+) skin lesion

A

Tuberculoid

166
Q

Type of Leprosy: Paucibacillus

A

Tuberculoid

167
Q

The cranial nerve affected in Leprosy

A

CN 7 (facial) and 5 (posterior auricular)

168
Q

The UE nerve affected in Leprosy

A

Ulnar, Median, Superficial Radial Nerve

169
Q

The LE nerve affected in Leprosy

A

Common Peroneal, Sural, Interdigital Nerve

170
Q

The most common cause of non-traumatic PNI

A

Diabetic Neuropathy

171
Q

What does high blood sugar inhibit in the body?

A

Nitric oxide (vasodilator)

172
Q

Three types of diabetic neuropathy

A

Symmetric, Asymmetric, Focal

173
Q

The 3 cranial nerve affected in Focal diabetic neuropathy

A

CN 3, 6, 7

174
Q

The 3 peripheral nerve affected in Focal diabetic neuropathy

A

Median, Ulnar, Peroneal

175
Q

What is the most common diabetic neuropathy

A

Symmetric

176
Q

“Diabetic Amyotrophy”

A

Asymmetric

177
Q

The 2 peripheral nerve affected in asymmetric diabetic neuropathy

A

Femoral and Obturator nerve

178
Q

What the extremity affected in symmetric and asymmetric diabetic neuropathy?

A

LE > UE (Proximal)

179
Q

“Chronic sensorimotor distal polyneuropathy”

A

Symmetric

180
Q

“Proximal motor neuropathy”

A

Asymmetric

181
Q

Two types of toxic neuropathy

A

Alcohol and Lead

182
Q

What causes toxic neuropathy?

A

Decrease Vitamin B1 (thiamine)

183
Q

What condition is caused by a decrease in Vit B1?

A

Beri-beri

184
Q

Other names of Alcohol Toxic Neuropathy

A

Wernicke’s - korsakoff disease

185
Q

What is the triad of Alcohol Toxic Neuropathy?

A

ADO: Ataxia, Dementia, Opthalmoplegia

186
Q

What is the nerve affected in mild lead toxic neuropathy?

A

Radial nerve

187
Q

What is deformity present in lead toxic neuropathy?

A

(B) wrist drop

188
Q

What condition results from severe lead toxic neuropathy?

A

ALS

189
Q

What is the most common neuropathy seen in lead toxic neuropathy?

A

Motor > Sensory

190
Q

Hereditary motor sensory neuropathy

A

Charcot-Marie-Tooth Disease

191
Q

What is the muscle atrophies in CMTD?

A

Peroneal muscle

192
Q

What is the chromosome affected in CMTD?

A

Chromosome 17

193
Q

What is the genetic course of CMTD?

A

Autosomal Dominant

194
Q

What is the gene affected in CMTD?

A

CMT

195
Q

What are the two-foot deformities seen in CMTD?

A

Pes Cavus and Stork Leg deformity

196
Q

What sign seen in stork leg deformity?

A

Inverted Champagne Bottle Sign

197
Q

Type of CMTD: Refsum’s Disease

A

CMTD 4

198
Q

Type of CMTD: (+) Spinocerebellar Ataxia

A

CMTD 5

199
Q

Type of CMTD: Hypertrophic myelin sheath

A

CMTD 1

200
Q

Type of CMTD: Axonal

A

CMTD 2

201
Q

Type of CMTD: (+) Optic neuropathy

A

CMTD 6

202
Q

Type of CMTD: Dejerine-Sotta

A

CMTD 3

203
Q

What is the hallmark of CMTD 1?

A

Onion-bulb formation

204
Q

What causes the onion-bulb formation?

A

Frequent demyelination and remyelination

205
Q

True or False: There is an increase in NCV in CMTD 1

A

FALSE (always decrease in demyelinating disease)

206
Q

What acid over accumulates in the Schwann cells in CMTD 4?

A

Phytanic Acid

207
Q

Type of CMTD: (+) Refinitis Pigmentosa

A

CMTD 7

208
Q

Type of CMTD: Severe

A

CMTD 3

209
Q

Type of CMTD: Autosomal Dominant

A

CMTD 1, 2, 3

210
Q

Type of CMTD: Autosomal Recessive

A

CMTD 3, 4

211
Q

Type of CMTD: (+) Hypertrophic neuropathic on infancy

A

CMTD 3

212
Q

Type of CMTD: 1st decade

A

CMTD 1

213
Q

Type of CMTD: 2nd decade

A

CMTD 2

214
Q

What is the cranial nerve affected in CMTD 4?

A

CMTD 1 and 8

215
Q

What NCV results present in CMTD 2?

A

Normal NCV

216
Q

What is refinits pigmentosa?

A

Blindess of both eyes

217
Q

What amyotrophic means?

A

Muscle atrophy

218
Q

What lateral sclerosis means?

A

Hardening of lateral SC

219
Q

3 other names of ALS

A

Adult motor neuron disease, Charcot’s disease, Lou Gehrig’s disease

220
Q

Epidemiology of ALS

A

M>F; 40-60 years old

221
Q

What is the etiologic of ALS?

A

Idiopathic

222
Q

What is the medication for ALS?

A

Riluzole / Rilutek

223
Q

What is the NT that increases the death of motor neurons?

A

Glutamate

224
Q

What are the diagnostic criteria used to diagnose ALS?

A

El Escorial Criteria

225
Q

What is the 4 region used in El Escorial Criteria

A

Head, trunk, UE, LE

226
Q

El Escorial: (+) UMN of 2 regions

A

Clinically Possible

227
Q

El Escorial: (+) UMN and LMN in 3-4 regions

A

Clinically Definite

228
Q

El Escorial: (+) UMN and LMN in 2 regions

A

Clinical Probable

229
Q

El Escorial: (+) UMN and LMN in 1 region

A

Clinically Possible

230
Q

What are the 3 variants of ALS?

A

Pure UMN, Pure LMN, Pure CN

231
Q

Variant: Pure UMN

A

Primary Lateral Sclerosis

232
Q

Variant: Pure CN

A

Progressive Bulbar Palsy

233
Q

Variant: Pure LMN

A

Progressive Muscular Atrophy

234
Q

What is the cardinal sign of LMN-ALS

A

Cervical extensor weakness (Head drop)

235
Q

What is the weight loss seen in ALS?

A

ALS cachexia

236
Q

What causes ALS cachexia?

A

Muscle atrophy and decrease intake

237
Q

What deformity seen in the hands of ALS patients?

A

Cadaveric or Skeletal hand

238
Q

What 6 cranial nerves affected by ALS?

A

CN 5, 7, 9, 10, 11, 12

239
Q

What 6 structures spared in ALS?

A

COMONS: Cerebellar system, Ocular muscle (CN 3,4,6), Mental status, Onufruwic’s Nucleus, Non-motor nerves (CN 1,2,8), Sensory

240
Q

AHC in cervical nerve 3-4

A

Phrenic

241
Q

AHC in spinal nerve 2

A

Onufruwic

242
Q

What is the function of Onufruwic’s nucleus?

A

For bowel and bladder

243
Q

What are the 5 poor prognosticating factors of ALS?

A

BPOLS: Bulbar dysfunction, Pulmonary Dysfunction, Old Age, LMN at onset, Short-time period from onset to diagnosis

244
Q

What are the 5 types of ALS according to etiology?

A

Sporadic, Familial, Juvenile, Guamanian, Secondary

245
Q

Type of ALS: Most common and classic form

A

Sporadic ALS

246
Q

Type of ALS: Autosomal Dominant

A

Familial ALS

247
Q

Type of ALS: <25 years old

A

Juvenile ALS

248
Q

Type of ALS: secondary to lead toxicity and cancer

A

Secondary ALS

249
Q

Type of ALS: western pacific

A

Guamanian ALS

250
Q

What chromosome is affected in Familial ALS?

A

Chromosome 21

251
Q

What gene is affected in Familial ALS?

A

SOD 1 (superoxide dismutase)

252
Q

What toxicity of the Guamanian ALS?

A

Cycadseed Toxicity

253
Q

Juvenile ALS: teenager

A

ALS 5

254
Q

Juvenile ALS: <10 years

A

ALS 2

255
Q

Juvenile ALS: <25 years

A

ALS 4

256
Q

Juvenile ALS: Autosomal Dominant

A

ALS 4

257
Q

Juvenile ALS: Autosomal Recessive

A

ALS 5, 2

258
Q

Type of ALS: (+) frontotemporal dementia

A

Guamanian ALS

259
Q

What is the chromosome affected in ALS 5?

A

Chromosome 15

260
Q

What is the chromosome affected in ALS 2?

A

Chromosome 2

261
Q

What is the chromosome affected in ALS 4?

A

Chromosome 9

262
Q

Most inheritable neuropathy?

A

CMTD

263
Q

Benchmark of motor neuron disease?

A

ALS

Neurology (14 decks)

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