Movement disorders Flashcards
(84 cards)
1
Q
What two parts of the brain is thought to be responsible for movement?
A
- Basal Ganglia - function is not fully known - thought to be linked to initiation and execution of movements
- Thalamus - transmits signals from the cerebral cortex to the spinal cord
2
Q
MOA of dopamine
A
- NT involved in interneuronal communication
- Inhibits release of Ach at nerve terminal
- Helps to make smooth motor movements and motor control
3
Q
inhibitory neurotransmitter that relaxes muscle and allows motor control
A
GABA
Gamma-aminobutyric acid
4
Q
a NT found at neuromuscular junction and in the autonomic ganglia. Increased neuronal excitability in the periphery and functions as as neuromodulator in the CNS
A
Acetylcholine
5
Q
An involuntary, rhythmic, and oscillatory movement of a body part
A
Tremor
Can affect hands, arms, head, face, voice, trunk, and legs
6
Q
how are tremors caused?
A
either alternating or synchronous contractions of antagonistic muscles
Neural dysfunction or lesions that result from injury, ischemia, metabolic abnormalities, or a neurodegenerative disorder.
7
Q
MC movement disorder
A
tremor
8
Q
how are tremors categorized?
A
resting or action
9
Q
this tremor occurs in a body part that is fully supported, relaxed, and not voluntarily activated
A
resting tremor
10
Q
Occurs in an attempt to maintain a specific posture or position against the force of gravity
what type of tremor?
A
Action
Postural
11
Q
Occurs during any voluntary movement
what type of tremor
A
action kinetic
12
Q
Occurs during a muscle contraction against a rigid, stationary object
what type of tremor
A
action isometric
13
Q
what are the 3 subcategories of action kinetic tremor?
A
- Simple Kinetic: during voluntary movements that are not target directed (e.g. pronation-supination)
- Intentional: worsens as the body part approaches its target (e.g. eating)
- Task-specific: occurs during a specific task (e.g. writing)
14
Q
descriptors of tremors
A
- Frequency Hz / sec
- < 4 (slow)
- 4-8
- 8-12
- >12 (fast) - Amplitude - ROM
- low (smaller movement)
- high (larger movement)
15
Q
A very low-amplitude, high-frequency (8 to 12 Hz) physiologic action tremor in the upper limbs.
MC type of action tremor
A
Enhanced physiologic tremor
16
Q
Enhanced physiologic tremor is present only when there is ?
A
sympathetic activation
- stress, anxiety, excitement, muscle fatigue
- F, hypoglycemia, thyrotoxicosis, pheochromocytoma
- alc or opioid withdrawal
- meds, drugs, and substances
17
Q
management for enhanced physiologic tremor?
A
- Tremor resolves when precipitating factor is removed
- Treat/remove underlying causes
18
Q
Most common adult onset “neurological” movement disorder
A
essential tremor (ET)
19
Q
pathophys of ET
A
not fully understood - altered cellular activity in ventral intermediate (VIM) nucleus of thalamus
20
Q
epidemiology of ET
A
- Incidence increases with age
- Mean age - 35-45 years
- Usually manifests by 65 y/o
- FHx present 30-70 % of pts
21
Q
presentation of ET
A
- MC bilaterally affects hands and arms
- > the head, voice, face and trunk - Both postural & kinetic properties
- arms postured against gravity or during goal-directed movements (e.g. drinking from a glass or F-N testing)
- Resolves w/ rest or if extremity is fully supported - trouble w/ fine motor activity - eating, drinking, pouring, writing, typing, texting, and applying makeup
- Exacerbated by emotion, hunger, fatigue, temperature extremes - NOT by caffeine
- Improves w/ alcohol (small)
- Progressive over time
- F-N testing - mild or absent tremor until after the target is reached
22
Q
You suspect an essential tremor in your patient but they have more of a isolated head or voice tremor, what is your next step?
A
Find other cause! - Dystonic head tremor, Spasmodic dysphonia
isolated head or voice tremor excludes ET
23
Q
How to DDX of ET
A
- Enhanced physiologic tremor - worsened by caffeine, resolves when precipitating factor is removed
- Parkinson disease - at onset unilateral resting tremor, rigidity, bradykinesia
- Dystonic head tremor - isolated head tremor
- Spasmodic dysphonia - isolated voice tremor
- Cerebellar tremor - intention related qualities and other signs cerebellar dysfunction (ataxia)
- Wilson’s disease - asymmetric tremor, other neurologic sx (dysarthria, dystonia, chorea etc.)
24
Q
How to DX ET?
A
made clinically based upon hx and PE
25
Labs for ET
**USED TO R/O SECONDARY CAUSES OF TREMOR**
CMP - electrolyte imbalance
Thyroid function tests - hyperthyroidism
Serum ceruloplasmin - Wilson disease¹
26
Diagnostic Criteria for ET
International Parkinson and Movement Disorder Society (IPMDS) task force
* Isolated tremor consisting of bilateral upper limb action (kinetic and postural) tremor, w/o other motor abnormalities
* At least 3 years in duration
* w/ or w/o tremor in other locations
* Absence of other neurologic signs (dystonia, ataxia, or parkinsonism)
27
management for ET
Tx recommended for **intermittent /persistent disability** d/t tremor
_Intermittent:_
* 1st line - **_Propranolol_** (30 min prior to activity); (Alt) **Primidone** qhs
* 2nd line - _BZD - alprazolam (Xanax), clonazepam (Klonopin)_ - reduces anxiety that worsens ET - not recommended for chronic use
_Continuous_
1. 1st line - **_propranolol BID; primidone; Combo_**
1. 2nd line - _Anticonvulsants: gabapentin (Neurontin), pregabalin (Lyrica), topiramate (Topamax)_
1. 2nd line - refer to neurologist
- _Botox_ into affected muscle - reserved for head tremors unresponsive to pharm
- Surgical intervention: _Deep brain stimulation vs thalamotomy_
28
indication for surgical therapy for ET
disabling tremor with **failure of 2 oral regimens**
29
CI for surgery in ET
dementia or significant cognitive impairment¹, uncontrolled anxiety/depression
30
What is VIM nucleus deep brain stimulation
Implantation of an electrode into the VIM nucleus connected to a pulse generator implanted in the chest wall below the clavicle which delivers **unilateral high-frequency electrical stimulation when activated**
| For ET
31
what is MRI-guided focused ultrasound thalamotomy
Uses **high-energy US beams to create a permanent lesion in the VIM nucleus of the thalamus** - performed on the _opposite side of the most severely affected arm_
| for ET
32
A target-directed coarse tremor that worsens when closest to the terminal target
**Intention (kinetic) tremor**
Frequency - 3 to 4 Hz
33
How would an Intention (kinetic) tremor present on a finger-to-nose test?
**Abnormal** finger-to-nose testing
* No tremor at start but become more severe as directed movement advances to target
* may continue for several beats after the target has been reached
34
neurological presentations of intention tremor
* **Nystagmus**
* **Dysarthria** - poor articulation and slurred speech
* **Dysmetria** - overshoots or undershoots their target
* **Dysdiadochokinesia** - abnormal RAM’s
* **_Holmes-Stewart maneuver_**: abnormal finding - inability to control rebound from a release of flexion against resistance (about to punch yourself)
* **Heel shin ataxia**
* **Ataxic gait**
35
Etiologies and associated conditions of Intention (kinetic) tremor
| 6
* Damage to midbrain, thalamus, cerebellum, or pons
* MS
* Stroke
* Severe forms of ET
* Wilson dz
* Mercury poisoning
36
work up and management for inention tremor
1. Work-up - Same as ET to r/o underlying etiologies
2. Management
- No successful medical treatment exists
- Refer to neurology - surgical intervention as in ET
37
Sustained or repetitive involuntary muscle contractions, frequently causing patterned twisting movements and/or abnormal posture
Dystonia
involuntary contraction of both agonistic and antagonistic muscles
38
what worsens and improves Dystonia
Worsened by voluntary movement, stress and fatigue
Improved with active relaxation and sensory tricks
39
Dystonia related conditions are classified based upon what factors?
| 5
1. age
2. body distributrion
3. Anatomical location
4. Inherited or Acquired
5. Primary or Secondary
- Primary: no other neuro s/s
- Secondary: d/t underlying disease
other neuro s/s
40
ages of dystonia
| "Dystonia related conditions classified based upon various factors" 5
* Infancy: birth to 2 years
* Childhood: 3 to 12 years
* Adolescence: 13 to 20 years
* Early adulthood: 21 to 40 years
* Late adulthood: >40 years
41
Generalized dystonia involves **multiple areas of the body** often including the ___ and ____
torso and limbs
42
cause of generalized dystonia
* Genetic: Childhood onset; Adolescent onset dystonia
* Acquired - birth injury, exposure to certain drugs, head injury, infection
43
MC focal dystonia
Cervical dystonia (aka. torticollis)
* Contraction of neck muscles causing the head to deviate to one side - SCM, traps, and posterior cervical muscles
* MC onset 30-50 years of age
44
subtypes of Cervical dystonia (aka. torticollis)
torticollis, retrocollis, anterocollis, laterocollis
more than one may occur simultaneously
45
* Contraction of muscles in the **UE** brought out by specific activities
* involuntary flexion, extension, and/or rotation of the fingers, wrist, elbow and/or shoulder
* **triggered by repetitive activities**
Upper Extremity Dystonias
Onset is usually 10-50 years of age
46
Task-specific UE dystonias and which is MC?
1. **handwriting/typing (writer’s cramp) - MC**
1. playing a musical instrument (musician’s cramp)
1. putting in golf (the yips)
47
Involuntary movements involving masticatory, lingual, and pharyngeal muscles.
**Oromandibular Dystonia (OMD)**
* Jaw clenching, jaw opening, jaw deviation, and tongue protrusion
* Difficulty speaking and swallowing
* Cosmetically disfiguring
48
OMD is often associted with what other movements/conditions?
blepharospasm & torticollis
49
Contraction of the eyelids causing increased blinking and involuntary eye closure that may interfere with daily functions
**Blepharospasm**
bilateral, synchronous, and symmetric, can be asymmetric
50
Task-specific focal dystonia involving the laryngeal muscles causing contraction of the vocal cords during speech
Spasmodic dysphonia
51
Characterized by irregular and involuntary voice breaks that interrupt normal speech
Pt may feel they have to “yell” or use more energy than normal to talk
Spasmodic dysphonia
52
general management for dystonia
Refer to a movement disorder specialist (neurologist with additional training)
No cure, management is symptomatic only
53
Focal Dystonia Management
**Botox injection** - for focal dystonias ONLY
Injection into the affected muscle(s) weakens the muscle
54
MOA of botoxin
blocks the release of Ach at the NMJ
Indicated for focal dystonias only
55
management for generalized dystonia
1. for ALL: Trial of **levodopa/carbidopa**
2. Adult
- 1st line: **Clonazepam** (Klonapin) - works on GABA receptor
- 2nd: _baclofen_ (GABA agonist)
- 2nd: Ach - _trihexyphenidyl_ (Artane)
3. Children
- 1st-line: **trihexyphenidyl**
- 2nd: _baclofen & BZD’s_
4. 2nd line: _VMAT2 inhibitor: tetrabenazine (Xenazine), deutetrabenazine (Austedo)_
5. _Deep brain stimulation (DBS)_ - last resort for those who fail more conservative therapies
| -abenazine = VMAT2i
56
MOA of levodopa/carbidopa
**levodopa is converted into dopamine** in the CNS; carbidopa prevents levadopa from being broken down too early
57
trihexyphenidyl - MOA
is not well understood - muscarinic receptor antagonist which **increases striatal dopamine release** and efflux
| for Generalized Dystonia Management:
58
MOA of VMAT2 inhibitor
depletes dopamine (and other monoamines) at the neuron synapse reducing the excitability of the neurons
59
cause of infantile torticollis
1. pain, torticollis, and decreased range of motion
2. MC d/t damage or inflammation to the SCM or trapezius
- must R/O infectious etiologies - strep pharyngitis, cervical adenitis, retropharyngeal abscess, parapharyngeal abscess, and viral URI
60
management for infantile torticollis
Treat underlying disorder if appropriate
Tx with NSAIDS, soft cervical collar physical therapy
61
Idiopathic neurological movement disorder of the limbs often associated with a sleep complaint
Restless Leg Syndrome (RLS)
Onset - may begin at any age, but **MC middle aged or older**
62
epidemiology of RLS
* 5-15% of the general population in the US
* Familial in 25 – 75%
* Women > Men (2:1)
* Caucasian > Black American
* Increases with pregnancy
63
pathophys of RLS
* Unknown
* Theories - genetic component, lack of dopamine uptake and impaired iron homeostasis
64
possible causes of RLS
1. _MC idiopathic_
1. May be secondary to:
* Nutrient deficiency: Iron, Mg, B12, Folate
* Renal failure (uremia)
* Neuropathy
* Hyperthyroidism
* Spinal cord pathology
* Traumatic spinal cord lesions, demyelinating or postinfectious lesions, tumors
* Pregnancy
* Parkinson disease (PD)
* Multiple sclerosis
65
risk factors for RLS
* Frequent blood donation
* DM
* Chronic excessive alcohol use
* Amyloidosis
* Motor neuron disease
66
presentation of RLS
1. often have difficulty describing sx - "need to move," "crawling," "tingling," "restless," "cramping," "creeping," "pulling," "electric," "tension," "discomfort," "soreness," and "itching"
1. **Not painful;** bothersome
1. **Irresistible urge to move the legs w/ or w/o uncomfortable sensations** - UE can also be affected
1. **Temporarily relief after movement** (walking or stretching)
1. **Worse in the evenings or during times of inactivity**
1. Not associated with other chronic disease sx - myalgia, venous stasis, leg edema, arthritis, leg cramps, positional discomfort, medication side effect, drug abuse or habitual foot tapping
2. **Majority - periodic movements of sleep (PLMS)**: involuntary, forceful dorsiflexion of foot lasting up to 5 sec and occurring every 20-40 sec throughout sleep
1. Daytime fatigue
1. Insomnia
1. Involuntary, repetitive, periodic, jerking limb movements - both awake and at rest
1. Anxiety/depressive sx related to chronic sleep deprivation - Associated with a 2-3 fold higher risk of suicide and self-harm
67
Aggravating factors of RLS
1. Caffeine
1. Sleep deprivation
1. Certain drugs - inhibits dopamine
* Antihistamines (benadryl and other OTC sleep aids)
* Dopamine antagonist - antipsychotics; anti-nausea meds - metoclopramide (Reglan)
* Antidepressants - TCA’s, SSRIs SNRIs
68
whcih antidepressant is less likely to exacerbate RLS
bupropion
69
DDX for RLS
* Volitional movements: foot tapping/bouncing, leg rocking; no circadian rhythm, no “urge”, easily suppressed
* Akathisia: generalized restlessness, no subjective “sensation” complaints; less relief from movement, less likely to have circadian pattern
* Nocturnal leg cramps: muscle spasms of the feet/calf; sudden onset, short duration
* Positional discomfort: no subjective “sensation” complaints
* Leg Pain: no “urge” to move; no relief with position change
70
labs for RLS
1. Iron, Ferritin, transferrin saturation - all patients
1. If secondary RLS is suspected, look for underlying etiology
* CBC
* Blood urea nitrogen (BUN)/Creatinine
* Fasting blood glucose
* Magnesium
* TSH
* Vitamin B-12
* Folate
**The diagnosis is clinical**
71
Indication for pharmacotherapy for RLS
sx for ≥ 3 nights/wk
72
pharm options for RLS
1. **Iron replacement** if the fasting serum ferritin level ≤75 mcg/L
- Ferrous sulfate 325 mg orally every other day or daily - w/ Vit C or a glass of OJ²
- Repeat iron panel after 3-6 mo
- **DC therapy** when ferritin level is >75 mcg/L and Fe saturation > 20%
2. **Alpha-2-delta calcium channel ligand**: gabapentin (IR) (Neurontin), pregabalin (IR) (Lyrica), gabapentin enacarbil (ER) (Horizant)
3. **Dopamine agonist**: pramipexole (Mirapex), ropinirole (Requip)
- oral therapy, 2 hours before bed
4. **Rotigotine (Neupro) transdermal patch** - 1 mg patch per day
73
nonpharm options for RLS
Keep the mind busy - working on a computer or doing crossword puzzles, at times of rest
Removal of exacerbating medications (if applicable)
Moderate regular exercise
Avoid caffeine
Good sleep hygiene
74
SE of Alpha-2-delta calcium channel ligand
**gabapentin** (IR) (Neurontin), **pregabalin** (IR) (Lyrica), **gabapentin enacarbil** (ER) (Horizant)
* SI/behaviors, depression, mental fog, dizziness, weight gain, dependence, addiction
* Excretion by the kidneys
* DEA: Controlled substance - risk for diversion
75
SE of Dopamine agonist
* nausea, lightheaded, fatigue usually resolve 10-14 d
* risk of impulse control disorder
* risk of augmentation
taper therapy if DC
76
an overall increase in RLS symptom severity with increasing doses of oral dopamine agonists
Augmentation
77
Pt on dopamine agonist is now experiencing **earlier onset** and **increased intensity** of their RLS
**topographic spread** of sx to other body parts, like their trunk and arms
**shorter duration of drug action**
what is your dx and management?
**Augmentation**
assess iron stores and replace if necessary
switch to alpha-2-delta calcium channel ligand
Refractory disease - refer to neurology
78
risks of augmentation
higher doses of therapy
longer duration of use
lower iron stores
79
delivers peroneal nerve stimulation via bilateral therapy units worn externally just below the knee, over the head of the fibula.
Tonic motor activation (TOMAC)
| RLS
80
A common genetic neurological disorder characterized by chronic motor and vocal tics beginning before adulthood
Tourette Syndrome (TS)
repetitive, stereotyped movements or vocalizations, such as blinking, sniffing, facial movements, or tensing of the abdominal musculature
81
epidemiology of TS
* Onset in childhood – 2-15 y/o; **Average age 6 – 11 y/o** (96% of dx)
* **Males** > Females (4:1)
* 3 out of 1,000
* New research suggests genetic inheritance through a dominant gene
82
possible RF for TS
Smoking during pregnancy
Complications during pregnancy
Low birth weight
83
pathophys of TS
unknown - hypothesis: a **disorder of neurotransmission** in the cortico-striatal- thalamic-cortical (mesolimbic) circuit, which leads to disinhibition of the motor and limbic system
84
clinical presentation of TS
Tics - sudden, brief, intermittent movements (motor tics), utterances (vocal or phonic tics) or sensations (sensory tics)
- Exacerbated by fear or anxiety
- Known to be involuntary, but can temporarily be voluntarily suppressed
- May be preceded by urges, sensations, or thoughts
