Motor Disorders - Motor Neuron Flashcards Preview

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Flashcards in Motor Disorders - Motor Neuron Deck (155):
1

Movement Disorders

Parkinson’s
Benign Essential Tremor
Huntington’s chorea
Restless Leg Syndrome

2

Drugs that can cause Parkinsonism

ANTIPSYCHOTICS - Haldol
METOCLOPRAMIDE
Amiodarone
Alcohol
Methylphenidate
Lipitor
Carbamazepine
Stimulatants
Corticosteroids
Cocaine
Albuterol

3

Pathophysiology of Parkinsons

Degeneration of the dopaminergic neurons in the substantia nigra

Substantia nigra is located in the midbrain; part of the basal ganglia

Control/modulation of extrapyramidal systems; motor control

4

Supstantia nigra is located

in the midbrain

5

Parkinsons typically presents after age

50

6

Incidence of Parkinsons

1% >60 yo
4% >80 yo

7

_% with Parkinsons have some type of family history.

25%

8

Parkinsons Disease
Men vs Women

Men > Women

9

Parkinsons Signs and Symptoms

Tremor - Rest
Bradykinesia
Rigidity

10

Parkinsons Tremor

Rest tremor (may have action tremor too)
“Pill rolling”
Bilateral or asymmetrical
Starts in fingers, extends to elbows
Also affects head, face, tongue

May be absent in up to 20%

11

Parkinsons Rigidity

"Cogwheeling”
“Lead Pipe resistance”
Posture- stooped
Decreased arm swing
Masked facies

12

Smaller handwriting

Micrographia

13

Parkinsons Bradykinesia

Affects speech, swallowing, eating
Smaller handwriting; micrographia
Infrequent blinking
Slow, shuffling gait
Difficulty initiating steps but difficulty stopping- festinating gait
Difficulty arising from a chair

14

Parkinsons - blinking

infrequent

15

Parkinsons - gait

slow and shuffling
festinating
difficulty initiating steps

16

PE Parksinsons - rigidity

Lead Pipe Resistance

17

Parkinsons Faces

Masked

18

Parkinsons Later Symptoms

Cognitive impairment/dementia (60% by 12 y)
Depression
Fatigue (1/3)
Orthostatic hypotension
Decreased olfaction
Dysautonomia
Dysphagia
Drooling
Urinary urgency
Constipation
Sleep disturbance
Daytime somnolence

19

Parkinsons movement

Slow - Bradykinesia

20

Parkinsons - problems with swallowing

Slow! May become an aspiration risk.

21

Difficult to stop walking

festinating gait

Seen in Parkinsons

22

Parkinsons turning

End Block Turns

Can't pivot

23

Parkinsons Differential Diagnosis

Dementia with Lewy Body (visual hallucinations)
Vascular Parkinsonism
NPH
Benign essential tremor
Progressive supranuclear palsy

24

Parkinsons Primary Treatment

Sinemet - Levodopa/Carbidopa

25

Parkinsons Imaging

Spect Imaging

can see degeneration

Not routinely done.

26

Sinemet

Sinemet
25/100 mg TID
may increase by one pill every 2 days;
Max - 3 pills TID

27

Side Effects of Sinemet

dyskinesias/choreiform movements

+/- hallucinations

28

Long term Sinemet therapy issues

After 5-10 years: wearing off, “on/off” issues

29

Parkinsons Adjunctive Treatment

Amantadine (Symmetrel)
Dopamine Agonists
COMT Inhibitors
MAO-B Inhibitors
Anticholinergics
Surgery - deep brain stimulation

30

What is Amantadine (Symmetrel) used for?

Mild symptoms, may help with dyskinesias associated with Levodopa

31

Dopamine Agonists

Pramipexole (Mirapex)
Ropinirole (Requip)
Apomorphine (Apokyn)

32

COMT Inhibitors

Entacapone (Comtan) (decreases metabolism of levodopa and extends half-life)

33

MAO-B Inhibitors

Selegiline (Eldepryl)
Rasagiline (Azilect)

34

Anticholinergics

may alleviate tremor/rigidity but not bradykinesia

Trihexyphenidyl (Artane)
Benztropine mesylate (Cogentin)

35

Parkinsons Disease
What other things may you have to treat?

Constipation- Miralax
Depression
Fatigue- methylphenidate (Ritalin)
Daytime somnolence- modafinil (Provigil)

36

Parkinsons constipation treatment

Miralax

37

Parkinsons fatigue treatment

methylphenidate - Ritalin

38

Parkinsons daytime somnolence treatment

modafinil - Provigil

39

Onset of benign essential tremor

20-60 yo

40

Benign Essential Tremor is _______ inheritance

autosomal dominant

41

Benign essential tremor is a ________ tremor.

Action

42

Benign essential tremor affects what?

Hands (wrists)
Head (flex/extension- “yes” or lateral “no”)

+/- voice, LEs

43

Where does benign essential tremor increase in amplitude?

at end point

44

BET exacerbating factors

Stress/fatigue
Stimulants

45

BET alleviating factors

Alcohol
Rest

46

Benign Essential Tremor Treatment

Beta-blockers (Propranalol - nonselective; Atenalol, Metoprolol)

Anticonvulsant - Primidone (Mysoline)

Other: topirimate, gabapentin, botulinum toxin

47

BET and occupation

Up to 25% need to change careers or retire early due to tremor

48

Huntington's Chorea onset

Mid-life

49

Huntington's Chorea inheritance

autosomal dominant

50

Huntington's Chorea onset

Gradual

Failing memory, restlessness, lack of initiative

51

Huntington's Chorea classic symptoms

Choreiform movements
mental decline/dementia

52

Huntington's Chorea duration

20 years

53

4th leading cause of insomnia

restless leg syndrome

54

Family history of RLS

50-60%

55

Women vs Men RLS

Women > Men

56

RLS ages

18 or younger to >65

57

Uncomfortable sensation in the legs that can range from mild to severe

Restless Leg Syndrome

58

Restless Leg Syndrome is described as

Creeping, crawling
Tingling
Pulling or drawing sensation

59

What temporarily relieves RLS?

movement

60

What worsens RLS?

Prolonged sitting/laying down

61

RLS and sleep

Movements continue early in sleep phase.

62

RLS Pathophysiology

Decreased dopamine brainstem
Decreased endogenous opiates
Small fiber neuropathy

63

RLS Contributing Factors/Causes

Idiopathic
Iron deficiency anemia
Pregnancy
Hypothyroidism
DM
Medications: SSRIs, others
Caffeine
smoking

64

RLS Treatment

Pramipexole (Mirapex)
Ropinirole (Requip)
+/- Sinemet, gabapentin, others

65

Polyneuropathy DDx

Diabetes
Alcoholism induced
HIV/AIDs
Malignancies
Toxins
Metals - arsenics, lead
Medications
Vitamin Deficiencies
Protenemia
Infections
Sarcoid

66

Degenerative Motor Neuron Diseases

Progressive Bulbar Palsy
Pseudobulbar palsy
Progressive Spinal Muscular Atrophy
Primary Lateral Sclerosis
Amyotrophic Lateral Sclerosis

67

Voluntary Movement of the Limbs

One of the largest descending tracts. Arises in the motor cortex. The majority (90%) of fibers cross over at the pyramids and descend to the lower spinal levels. The function is voluntary movements of the limbs.

68

Cortex to brainstem/spinal cord
Cortex to spinal cord

Upper Neurons

69

Spinal motor neurons
Cranial nerve motor neurons

Lower Neurons

70

Upper Motor Neuron Injury casues

MS
CVA
TBI
Cerebral palsy
ALS

71

Lower Motor Neuron Injury causes

Progressive Bulbar Palsy
Bell's Palsy
ALS

72

Characteristics of upper motor neuron diseases

Muscle weakness
Decreased motor control
Spasticity
Hyperreflexia
Babinksi’s (upgoing plantar)

73

Characteristics of lower motor neuron diseases

Paralysis
Atrophy
Fasciculations
Loss of all reflex

74

Degenerative Motor Neuron Disease

Progressive Bulbar Palsy
Pseudobulbar palsy
Progressive Spinal Muscular Atrophy
Primary Lateral Sclerosis
Amytrophic Lateral Sclerosis

75

Progressive Bulbar Palsy affects

lower motor neurons
cranial nerves

76

Progressive Bulbar Palsy affects CN

V
VII
IX
X
XII

77

Effects of progressive bulbar palsy

drooling
chewing
dysphagia
dysarthria

78

Progression of progressive bulbar palsy

aspiration pneumonia 1-3 years

79

Pseudobulbar Palsy

Similar to Progressive Bulbar Palsy
Crying or laughing outbursts.

80

Progressive Spinal Muscular Atrophy affects

lower motor neurons - spinal level

81

Primary Lateral Sclerosis affects

Upper motor neurons only

82

Amytrophic Lateral Sclerosis affects

Upper and motor neurons

83

Amytrophic Lateral Sclerosis etiology

Idiopathic
+/- inheritance?

84

"Lou Gehrigs Disease"

Amyotrophic Lateral Sclerosis

85

ALS presentation - age

30-60

86

S/S ALS

Muscle aches/cramps

Weakness- distal upper limbs and progresses inferiorly

Dysarthria, dysphagia, spasticity, hyperreflexia, muscle fasiculations

87

Diagnosis of ALS

EMG

88

Treatment of ALS

No Treatment

Riluzole 50mg BID (extends life 3m) $$$$$
10% alive at 1-3 years

89

Who does Multiple Sclerosis affect?

White>Black
Female>Male
Higher socioeconomic classes
Northern latitudes

90

MS age of onset

15-60

91

MS pathophysiology

Demyelination of white matter
Autoimmune
Repeated inflammation causes formation of plaques

92

MS Causes

Environmental/toxin exposures?
Familial patterns?
HLA?
Vitamin D deficiency?

93

MS Types

Relapsing remitting

Secondary progressive (will occur in 65% of patients with relapsing remitting)

Primary progressive

Progressive relapsing

94

MS Facts

Remission after first episode (90%)

Progressive course after onset (10%)

Benign course: 1-2 relapses then recovery (20%)

Progressive course after 5 years (60-90%)

Rapidly progressive course after onset (rare- Marburg)

95

Optic Neuritis presents

Usually unilateral

Acuity 20/100 (rather than total blindness)

Onset: hours – days

+/- pain

96

Optic Neuritis Exam

Optic nerve pallor
+/- pain with EOM
NORMAL PUPILLARY REFLEX

97

What are patients with optic neuritis at higher risk for?

Patients with optic neuritis have a 50% chance of developing MS within 15 years

98

MS Presentation

Sensory loss (37%)

Optic neuritis (36%)

Weakness (35%)

Paresthesias (24%)

Other:
Ataxia, diplopia, Lhermitte sign (electrical shock sensation down spine when head is flexed), telegraphic speech, dementia, facial palsies, impotence

99

MS Exam may show

Dysarthria

Decreased pain, vibration, position sense

Ataxia

Horizontal nystagmus

Increased DTRs, spasticity, Babinski (up), ankle clonus

100

MS Diagnosis

MRI
Spotty, irregular demyelination
Commonly affected areas: brainstem, cerebellum, corpus callosum, white/grey junctions

101

MS Lumbar Puncture will show

Oligoclonal bands >75 – 90% patients
Bands of immunoglobulin

102

MS Serum Tests

+/- anti-myelin oligodendrate glycoprotein (ant-MOG)

+/- anti-myelin base protein (anti-MBP)

103

MS Acute Exacerbation Treatment

Methylprednisone IV
1000mg daily x 3 days

Then…

After the first three days,
Oral prednisone taper 1 – 3 weeks

104

MS Management to slow progression

First line:
Betaseron SC QOD
Avonex SC Q week
Rebif SC 3x weekly
Glatiramer (Copaxone) SC daily

Know treatments are SC so pts will have to give themselves shots.

Second line:
Mitoxantrone (Novantrone) IV Q 3 months

Third Line:
Natalizumab (Antegren, Tysabri) IV once monthly $$$

105

MS Other Management

Spasticity: Baclofen 10 – 40 mg TID
Others: tizanidine, gabapentin
Pain: Amitriptyline, gabapentin, carbamazepine
Urinary
-Oxybutynin (Ditropan)
-Tolterodine (Detrol)
Constipation
Fatigue - Modafinil (Provigil)
Depression - SSRIs
Temperature control - heat makes it worse
Education and support

106

MS Prognosis

Most live 30 years from onset
-5-10 years less than expected
90 % lose independent walking by 10 yrs
Almost all lose the inability to walk by death
Death is often secondary to sequelae

107

What will an MS MRI show?

Spotty, irregular demyelination
Commonly affected areas: brainstem, cerebellum, corpus callosum, white/grey junctions.

108

Cause of Guillain Barre

Idiopathic
Inflammatory Neuropathy

109

Progression of Guillain Barre

Progressive symmetrical weakness
Maximum weakness within 4 weeks
Mild to severe (respiratory failure)

110

Guillain Barre affects...

Males > Females

111

What is affected in Guillain Barre?

Sensory and Motor Neurons

112

Guillian Barre Pathophysiology

Usually follows an infection/exposure- ‘antigen mimicry

113

Types of Guillain Barre

Acute Inflammatory Demyelinating Polyradiculoneuropathy (AIDP) 90%
Acute Motor Axonal Neuropathy (AMAN)- Campylobacter jejuni
Other rare forms

114

Guillain Barre is Associated with which infections?

C jejuni
EBV
Mycoplasma
H influenzae
CMV
VZV

115

Guillain Barre is associated with which vaccinations?

H1N1 influenza
+/- tetanus
hepatitis

116

Guillain Barre usually follows

acute illness
1-3 weeks

117

Guillain Barre Presentation

Proximal muscle weakness (+/- CNs)
Legs then arms
Myalgias: shoulder, back, thighs
Paresthesias
Decreased DTRs

118

Guillain Barre Diagnosis

CSF – elevated protein (NL WBC)
EMG
+/- PFTs, ABGs (if respiratory sxs)

119

Guillain Barre Treatment

Supportive
IVIg or plasmapheresis

120

Guillain Barre Prognosis

85% will make full recovery
Long term: foot drop, hand weakness
Relapse: 3-5%

121

CIDP

Chronic Idiopathic Demyelinating Polyneuropathy

122

What is CIDP?

Chronic form of Guillain Barre.
Symptoms >8 weeks.

123

Diagnosis of CIDP

CSF, EMG
Nerve biopsy

124

Treatment of CIDP

Corticosteroids or immunosuppressants
Plasmapheresis, IVIg
PT

125

What is Myasthenia Gravis?

Neuromuscular autoimmune disease where antibodies form to nicotinic acetylcholine receptors.

126

Symptoms of Myasthenia Gravis

Proximaly asymmetric limb weakness (85%)

CN weakness:
Lid lag, ptosis, diplopia
Facial weakness, slurred speech
Easy fatiguability

127

Most common form of Guillain Barre

Acute Inflammatory Demyelinating Polyradiculoneuropathy

128

Diagnosis of Myasthenia Gravis

Blood
Ach receptor antibodies (will not be present in those with isolated eye symptoms)
Anti-MuSK (present in 30-40% of those with neg Ach rec)

EMG

Edrophonium test (Tensilon)- blocks acetylcholinesterase and improves symptoms

129

What autoimmune diseases is Myasthenia Gravis associated with?

Hashimoto’s, Grave’s
DM-1
Lupus
RA

130

Drug Induced Myasthenia caused by

Tetracycline
Aminoglycosides
Propanolol
Lithium +++++

131

Autoimmune- voltage gated calcium channels at neuromuscular junction

Lambert-Eaton Syndrome

Almost the same presentation of MG but not the same. Do not respond to the Tensilon test.

132

60% of pts with Lambert-Eaton Syndrome also have...

underlying SCC of the lung.

133

Causes of Cervical Spondylotic Myelopathy

Disc herniation
Nerve roots- upper extremity radiculopathy
Cord- LE , ataxia

134

Tumors that can cause Myelopathies

Malignant: sarcoma, multiple myeloma

Mets: 25x more common than a primary
Breast, lung, renal, prostate, lymphoma, thyroid

HIV related myelopathies (varicella zoster, lymphoma, T. gondii, CMV)

135

Patients with malignancies to the spine often complain of

Pain awakens at night; worse at night laying flat

136

Myesthenia Gravis is worse when...

the patient is tired.

137

A rare thymus tumor that occurs in 15% of patients with MG.

Thymoma

CT/MRI the neck

138

MG Treatment

Anticholisterase inhibitors - Mestinon

Immunosuppression - Prednisone, azathioprine

+/- plasmapheresis, IVIg

+/- thymectomy (if thymoma present)

139

Complications of Myasthenia Gravis

“myasthenia crisis”
Paralysis of the respiratory muscles
Aspiration

140

Persistent pain in dermatomal distribution after zoster infection

Postherpetic Neuralgia

141

Pathology of postherpetic neuralgia

(?) damage to dorsal root ganlion

142

Prevalence of Postherpetic Neuralgia

Age > 80 = 30%
Age 60-65 = 20%
Age < 50 = rare

143

Risk Factors of Postherpetic Neuralgia

Age
Female
Severe rash, pain at outbreak
Zoster ophthalmicus

144

Postherpetic Neuralgia Treatment

Lidocaine 5% ointment/patches
Capsaicin crm
TCA: amitriptyline, nortriptyline
SNRI- Cymbalta, Effexor
Anticonvulsants: gabapentin, pregablin
Pain Mgmt?- nerve blocks, TENS units

Course: 30 d – 6 months

145

Complex Regional Pain Syndrome is often linked to

a previous injury

Needlestick
Sprain/strain
Immobilization- cast

146

Age where complex regional pain syndrome presents.

~40
Seen in kids

147

Regional Pain Syndrome is seen in __________ limbs.

One or Multiple

148

Complex Regional Pain Syndrome AKA

Reflex Sympathetic Dystrophy

149

Symptoms of Complex Regional Pain Syndrome

Nerves/Vascular

Skin color changes
Temperature
Swelling
Tremor/movement disorder
Pain out of proportion to anything seen on exam.
Changes in nail/hair growth

Diagnosis of exlcusion.

150

Two types of Complex Regional Pain Syndrome

CRPS1
CRPS2 - nerve

151

Treatment of CRPS

Symptomatic
Rehab!!!!!!!!!

152

Narcolepsy

Uncommon <0.2%

Secondary: head trauma, encephalitis, tumor

Daytime sleepiness- sleep attacks

Cataplexy: (30%)

Sudden loss of voluntary muscle control usually following an emotional trigger

Hallucinations (upon awakening, falling asleep)

Sleep paralysis

153

Diagnosis of Narcolepsy

PSG
REM Cycle dysfunction

154

Treatment of Narcolepsy

Stimulants
SSRI
SNRI - cataplexy, hallucinations, paralysis

155

Cause of Narcolepsy

Idiopathic
HLA-DR2