Motor Disorders - Motor Neuron Flashcards
1
Q
Movement Disorders
A
Parkinson’s
Benign Essential Tremor
Huntington’s chorea
Restless Leg Syndrome
2
Q
Drugs that can cause Parkinsonism
A
ANTIPSYCHOTICS - Haldol METOCLOPRAMIDE Amiodarone Alcohol Methylphenidate Lipitor Carbamazepine Stimulatants Corticosteroids Cocaine Albuterol
3
Q
Pathophysiology of Parkinsons
A
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
Q
Supstantia nigra is located
A
in the midbrain
5
Q
Parkinsons typically presents after age
A
50
6
Q
Incidence of Parkinsons
A
1% >60 yo
4% >80 yo
7
Q
_% with Parkinsons have some type of family history.
A
25%
8
Q
Parkinsons Disease
Men vs Women
A
Men > Women
9
Q
Parkinsons Signs and Symptoms
A
Tremor - Rest
Bradykinesia
Rigidity
10
Q
Parkinsons Tremor
A
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
Q
Parkinsons Rigidity
A
"Cogwheeling” “Lead Pipe resistance” Posture- stooped Decreased arm swing Masked facies
12
Q
Smaller handwriting
A
Micrographia
13
Q
Parkinsons Bradykinesia
A
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
Q
Parkinsons - blinking
A
infrequent
15
Q
Parkinsons - gait
A
slow and shuffling
festinating
difficulty initiating steps
16
Q
PE Parksinsons - rigidity
A
Lead Pipe Resistance
17
Q
Parkinsons Faces
A
Masked
18
Q
Parkinsons Later Symptoms
A
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
Q
Parkinsons movement
A
Slow - Bradykinesia
20
Q
Parkinsons - problems with swallowing
A
Slow! May become an aspiration risk.
21
Q
Difficult to stop walking
A
festinating gait
Seen in Parkinsons
22
Q
Parkinsons turning
A
End Block Turns
Can’t pivot
23
Q
Parkinsons Differential Diagnosis
A
Dementia with Lewy Body (visual hallucinations) Vascular Parkinsonism NPH Benign essential tremor Progressive supranuclear palsy
24
Q
Parkinsons Primary Treatment
A
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
