Movement Disorders Flashcards
1
Q
Benign Essential Tremors (7)
A
- Familial Tremor, FmHx common
- Autosomal dominant
- May temporarily improve with alcohol
- No other abnormal findings
- Can start at any age and is enhanced by stress
- Postural tremor of the hands, head (or both) or voice (legs are spared)
- Little disability (manual skills, handwriting)
2
Q
Tx for Benign Essential Tremors
A
- Usually unnecessary
- Propanolol is 1st line
- Primidone, alprazolam, clozapine, topiramate, gabapentin, mirtazapine
- Botox (if localized)
- If unresponsive to meds try contralateral thalamotomy or unilateral high frequency thalamic stimulation
3
Q
Causes of Parkinsonism (6)
A
- Idiopathic parkinson’s dz (onset 45-65, rarely familial)
- Postencephalitic parkinsonism
- Secondary to toxin exposure
- Secondary to head trauma
- Neuroleptic drugs (reversible)
- Malignancy (rare!)
4
Q
Idiopathic Parkinson’d Dz
A
- Dopmine depletion d/t degeneration of the dopaminergic nigrostriatal system
- Dopamine and Ach imbalance in corpus striatum
5
Q
Clinical Presentation of Parkinsonism
A
- Tremor, rigidity, bradykinesia and postural instability are hallmarks
- Bradykinesia + tremor or rigidity
- Often the tremor starts on one side
- Tremor is most noticeable at rest and worsens with rapid motion of the opposite side
- Mild decline in intellectual fxn
6
Q
Rigidity- Parkonsonism
A
- Increased resistance to passive movements
- Cogwheeling (rigidity with passive movements)
- Characteristic flexed posture
7
Q
Bradykinesia- Parkinsonism (6)
A
- Slow voluntary movements
- Reduction in automatic movements (not swinging the arms while walking)
- Difficulty getting up from a chair
- Walking is difficult to start, but once they start it quickens. looks like they are falling forward
- Shuffling gait, difficulty turning, difficulty stopping
- Effective voluntary movement can be regained in an emergent situation
8
Q
Physical Features of Parksonism (7)
A
- Mask-like facies
- immobile face
- widened palpebral fissures (opening of the eye)
- infrequent blinking
- seborrhea on the face and scalp is common
- mild blepharoclonus (muscle spasms of the eye)
- tremor about the mouth
9
Q
Signs of Parkinsonism (7)
A
- Myerson’s sign (Pt continues to blink as you tap on their forehead, glabellar reflex)
- Drooling
- Soft, monotone voice
- Dysdiadochokinesia
- Micrographia
- No muscle weakness
- No alteration in DTR’s or Babinski’s reflex
10
Q
Differential Diagnosis for Parkinsonism (7)
A
- Slight tremor
- Depression
- Wilson’s dz
- Huntington’s dz
- Multisystem atrophy (Shy-Drager syndrome)
- Progressive supranuclear palsy
- Creutzfeld-Jakob dz
11
Q
Parkinsonism Medication Goals
A
- Tx is symptomatic
- Increase dopamine
- Decrease Ach
12
Q
Amantadine
A
- Parkinsonism medication
- Mild symptoms, no disability
- Mode of action is unclear
- *Improves all the types of symptoms
13
Q
Anticholinergic Drugs (6)
A
- Tx for Parkinsonism
- Help alleviate tremor and rigidity
- Does not help bradykinesia
- Dose is titrated up to response
- Contraindications: BPH, narrow angle glaucoma, obstructive GI dz, elderly
- Side effects: dry mouth, constipation, arrhythmias, confusion, increased ocular eye movements etc…
14
Q
Levodopa (5)
A
- Medication for Parkinsonism
- Improves all features of Parkinsonism
- Does not stop or progress the disease
- Early side effects: N/V, hypotension, cardiac arrhythmias
- Later on: dyskinesia, restlessness, confusion and behavioral changes
15
Q
Levodopa-Induced Dyskinesia (4)
A
- Many forms: chorea, athetosis, dystonia tremor, tics or myoclonus
- On/off phenomenon d/t changing levodopa serum levels
- On: dyskinesias are conspicuous but mobility is improved (high meds)
- Off: dyskinesias are better but mobility is limited (meds low)
16
Q
Levodopa- Carbidopa (Sinemet)
A
- Parkinsonism medication
- Carbidopa inhibits the breakdown of levodopa to dopamine in the periphery, does not cross the BBB
- Decreases overall dose of levodopa
- Lowers systemic side effects
- Does not prevent on/off phenomenon
- Dyskinesia, psychiatric complications may be increased
17
Q
How do you treat Levodopa-Induced Dyskinesias?
A
- Amantadine
- contraindicated in psychotic illness and narrow angle glaucoma
18
Q
Dopamine Agonists
A
- Parkinsonism medication
- Given before or in junction with levodopa
- Bromocriptine
- Pramipexole
- Ropinirole
19
Q
Dopamine Agonist Side Effects (9)
A
- Anorexia (bromocriptine)
- N/V/C
- postural hypotension
- digital vasospasm
- cardiac arrhythmias
- nasal congestion
- erythromelalgia
- pulmonary infiltrates
- pericardial/pleural/pulmonary fibrosis
20
Q
Selective Monoamine Oxidase Inhibitors (MAOI)
A
- Parkinsonism medication
- Rasagiline/Selegiline
- Adjunctive therapy in patients with response fluctuations to levodopa
- Reduce metabolism of L-dopa, more sustained plasma levels, more constant dopaminergic stimulation to the brain
21
Q
MAOI side effects
A
- Tyramine rich foods may cause hypertension
- May slow progression?
22
Q
COMT inhibitors
A
- tolcapone/entacapone
- reduce the metabolism of levodopa
- more sustained plasma levels
23
Q
COMT side effects
A
- fulminant hepatic failure (tolcapone)
- Entacapone does not have hepatotoxicity
24
Q
Atypical Antipsychotics
A
- Used for Parkinsonism
- Used for confusion and psychotic symptoms
- olanzapine, quetiapine, risperdone, clozapine
- Improves iatrogenic dyskinesias
25
Q
Parkinsonism Management (5)
A
- PT
- Speech therapy
- Aids to daily living
- Devices to amplify voice
- High frequency thalamic stimulation
26
Q
Huntington’s Dz Characteristics
A
- Characterized by chorea and dementia
- Inherited, autosomal dominant dz
- huntington gene is at 4p16.3
- Onset 30-50
- Fatal within 15-20yrs
27
Q
Initial S/S of Huntington’s
A
- Abnormal movement or intellectual changes
- Early mental changes are behavioral: irritability, antisocial behavior, moody
- progress to more obvious dementia
28
Q
Huntington’s Dyskinesia
A
- Starts as fidgeting or restlessness
- Progresses to choreiform (occasional jerking or writhering) movements and dystonic posturing
- Then progressive rigidity and akinesia rather than chorea may occur, especially in childhood cases
29
Q
Imaging for Huntington’s Dz
A
- CT/MRI show cerebral and caudate nucleus atrophy
- PET shows decreased metabolic rate in corpus striatum
30
Q
Huntington’s Dz Differential Diagnosis (8)
A
- Stroke
- SLE
- Paraneoplastic Syndrome
- HIV
- Various medications
- Sydenham chorea (group A strep)
- Huntington’s dz like disorder
- Dentatorubral-pallidoluysian atrophy
31
Q
Huntington’s tx
A
- Symptomatic, no cure
- Underactivity of GABA and Ach neurons
- Overactivity of dopaminergic neurons
32
Q
Huntington’s Medications (5)
A
- Tetrabenazine
- Phenothiazines
- Haloperidol
- Quetiapine
- Amantadine
33
Q
Childhood Onset Idiopathic Torsion Dystonia
A
- FmHx
- Symptoms start in the legs
- Progress to disabling severe dystonia
34
Q
Adult Onset Idiopathic Torsion Dystonia
A
- No FmHx
- Symptoms start in the arms or trunk
- Severe disability is not usual
35
Q
Diagnosis of Idiopathic Torsion Dystonia
A
- Diagnosis of exclusion
- R/o birth trauma, anoxia, kernicterus
- No mental delay with ITD
- Other thing to consider: wilson’s dz, huntington’s dz, parkinsonism, sequela of encephalitis, neuroleptic drug therapy
36
Q
Tx of Idiopathic Torsion Dystonia (5)
A
- Responds poorly to meds
- 1st line therapy: levodopa, diazepam, baclofen, carbamazepine, amantidine, anticholinergics
- 2nd line: tetrabenazine, phenothiazine, haloperidol
- pallidal deep brain stimulation
- thalamotomy if unilateral
37
Q
Focal Torsion Dystonia
A
- Atypical or attenuated form of ITD if there is FmHx
- Focal manifestation of adult on set if no FmHx of ITD
38
Q
Focal Torsion Dystonia (4)
A
- Spastic torticolis
- Blepharospasms
- Oromandibular dystonia
- Writer’s cramp
39
Q
Spastic torticolis
A
- Twisting the head to one side
- Onset 25-50
- Some resolve in a year, if not it persists for life
40
Q
Blepharospasms
A
- Spontaneous, forced closure of the eyelids
41
Q
Oromandibular Dystonia (4)
A
- Involuntary contraction of muscles about the mouth
- Opening and closing of the mouth
- Roving or protrusion of the tongue
- Retraction of the platysma
42
Q
Writer’s Cramp
A
- Dystonic contraction of the hand and forearm when the hand is used
43
Q
Focal Torsion Dystonia Tx
A
- Medical tx usually doesn’t work
- Trial of drugs used for ITD
- Blepharospasms and torticolis can be treated with botox injections or nerve section
- Writer’s cramp can be treated with botox or learn to use their other hand
44
Q
Myoclonus
A
- General or focal jerks, seen in:
- idiopathic epilepsy
- Lipid storage dz
- Ramsay-Hunt Syndrome
- subacute sclerosing pan-encephalitis
- Creutzfeld-Jakob Dz
- Metabolic encephalopathies
- Medication related
- Anoxic brain injury
45
Q
Myoclonus Tx (3)
A
- Responds well to valproic acid, benzodiazepines, piracetam
- Anoxic brain injury: oxitriptan
- Segmental myoclonus: could be d/t spinal cord lesion, get an MRI
46
Q
Wilson’s Dz Characteristics (5)
A
- Metabolic disorder, excessive deposition of copper in the liver and brain
- autosomal recessive
- Presents before age 40
- Abnormal movement and posture with or w/out signs of liver involvement
- Psychiatric and neuropsychological symptoms are common
47
Q
Wilson’s Dz Physical Signs
A
- Serum ceruloplasmin (plasma copper carrying capacity) is low 40mg/dL
- liver biopsy may show hepatitis or cirrhosis
- Kayser-Fleischer ring is pathognomonic
48
Q
Wilson’s Dz Neurological Presentation (5)
A
- Related to basal ganglia fxn
- Presents similarly to parkinson’s, pseudosclerosis w/tremor, ataxia, dystonic syndrome
- dysarthria, dysphagia, incoordination and spasticity
- Migraines, insomnia, seizures
- behavioral/personality changes, emotional lability
49
Q
Wilson’s Dz Tx (4)
A
- Chelating agents, penicillamine, trientine
- Oral Zinc acetate or Zinc gluconate
- Prognosis is good if there is no liver or brain damage
- Family members should be screened
50
Q
What drugs can cause chorea? (9)
A
- Levodopa
- Bromocriptine
- Anticholinergics
- Phenytoin
- Carbamazepine
- Lithium
- Amphetamines
- OC
- Chorea resolves with withdrawal of medication
51
Q
What drugs cause dystonia? (4)
A
- Levodopa
- Bromocriptine
- Lithium
- Carbamazepine
52
Q
What drugs cause Parkinsonism? (2)
A
- Reserpine
- Tetrabenaine
53
Q
What drugs can cause a postural tremor? (8)
A
- Epinephrine
- Isoproterenol
- Theophylline
- *Caffeine
- Lithium
- *Thyroid
- TCAs
- Valproic acid
54
Q
Restless Leg Syndrome
A
- Restlessness, curious sensory disturbances lead to an irresistible urge to move the legs
- Disturbed nocturnal sleep and daytime sleepiness can result
- FmHx, genetic loci have been found
55
Q
What can cause restless leg syndrome?
A
- Idiopathic
- Pregnancy
- Iron deficiency anemia
- Peripheral neuropathy (esp. uremic or diabetic)
56
Q
Restless Leg Syndrome Tx (6)
A
- Pramipexole
- Ropinirole
- Benzos
- Gabapentin
- Levodopa
- Opioids
57
Q
Tourette’s Syndrome Characteristics (4)
A
- Multiple motor and phonic tics
- Symptoms start before 21
- Tics occur frequently for at least 1 yr
- Tics vary in nature, number and frequency over time
58
Q
Initial presentation of Tourette’s
A
- 80% motor
- 20% phonic
- Motor tic usually happen around the head and shoulders (sniffing, blinking, frowning, shoulder shrugging, head thrusting)
59
Q
Phonic Tics
A
- Grunts
- Barks
- Hisses
- Throat clearing
- Coughs
60
Q
Coprolalia Tourette’s
A
Swearing
61
Q
Echolalia Tourette’s
A
Imitates everything you say
62
Q
Echopraxia Tourette’s
A
Imitates everything you do
63
Q
Other Types of Tourette’s
A
- Self mutilating: nail biting, hair pulling, lip biting
- OCD is commonly associated
64
Q
Tourette’s PE (5)
A
- No neurologic deficits other than the tics
- Psychiatric disturbances may occur
- EEG may show minor non-specific abnormalities
- FmHx
- Autosomal dominant with variable penetrance
65
Q
Tourette’s Diagnosis
A
- Often delayed
- Tic like character of the movements
- absence of other neurological signs
- Can present like Wilson’s dz so you need to rule this out
66
Q
Tourette’s Tx (7)
A
- Haloperidol
- Clonazepam
- Clonidine
- Fluphenazine
- Pimozide
- Bilateral high frequency deep brain stimulation
- Botox
67
Q
If someone presents with a movement disorder and there is a concern for seizure, what test is indicated? (2)
A
- MRI
- EEG
68
Q
If someone presents with myoclonus in a focal pattern, what test is indicated?
A
MRI of the spine
