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Movement Disorders
Tory Davis PA-C
UNE PA Program
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Tremor Classification Rest vs Action Body part(s) affected Frequency- how fast, measured in
hertz (cycles/second) Amplitude- fine or coarse
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Resting Tremor Body part affected is supported
against gravity, no muscle contraction (hands in lap)
Amplitude with mental stress with general mvmt (walking) with target directed mvmt (finger to
nose)
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Action Tremor Produced by voluntary muscle
contraction– 1. Postural- body part maintaining
position against gravity – 2. Isometric- muscle contraction against
stationary object (finger squeeze)– 3. Kinetic-with voluntary mvmt
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Kinetic subtypes Simple kinetic tremor- assoc with
mvmt of extremities (pronate/supinate) Intention tremor- present during
visually-guided, target-directed motion. amplitude fluctuation on approaching target (finger to nose)
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Physiologic tremor Every “normal” person has a High frequency, low amplitude postural
tremor Enhanced by hyper-adrenergic states:
hypoglycemia, thyrotoxicosis, drugs (caffeine), withdrawal, public speaking
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Benign Essential Tremor aka benign familial tremor Most common movement disorder
worldwide Prevalence reported up to 5% of
people over 60- BUT, half of people with mild essential tremor aren’t aware
FHx reports vary (20-60%)
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Essential tremor Insidious development, slow
progression 95% start w/ postural distal arm tremor
– Wrist flex/ext , 4-12 Hz frequency Bimodal onset: teens and 50s Unilateral progresses to bilateral UE, head (yes or no), palate (rare)
– Legs usually spared
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Essential tremor Amplitude
with stress, fatigue, CNS stimulants, voluntary activity
with EtOH, ß-blockade, rest
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Pt Ed Avoid stimulants Avoid fatigue Avoid stress
…and don’t self-medicate with alcohol
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Tx: Primidone Primidone 50-750 mg/day.
Anticonvulsant. Start at 25mg qhs and slowly titrate up to avoid sedation.
Contraindicated in asthma SEs: sedation, dizziness, nausea,
mood changes
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Tx: Beta blocker ß- Blockade: Propranolol 40-320
mg/day. Better tolerated, no more effective than primidone
Contraindicated in asthma, bradycardia, cardiac conduction defects
SEs: sexual side effects, fatigue, depression
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Parkinson’s Disease
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What it is
Neurodegenerative disorder resulting from dopaminergic transmission in basal ganglia
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Parkinson’s Disease-4 Cardinal Signs
Tremor
Rigidity
Bradykinesia (slowness of movement)
Postural impairment (comes later in ds)
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PD Tremor Present in 85% 4-6 Hz resting tremor Distal, unilateral “pill-rolling” by voluntary activity, by stress One limb or one side of body for
months to years Spares head
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PD Rigidity Increased resistance to passive
movements “Cogwheel rigidity” No weakness No change in DTRs
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Bradykinesia Slowness of movements Noticed in speech as well as voluntary
movements Start hesitation
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Postural impairment Difficulty with balance and gait
Occurs later in disease course.– If you see this early, question dx and refer
to neuro
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Gait Classic “festinating gait” Flexed trunk. Legs and hips stiff and flexed. Arms still (not swinging) Short fast steps- trying to keep up with the
forward center of gravity Turn “en bloc” Later in disease, freezing w/ direction
change or when entering small space (doorway)
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Other features “Mask-like” face Widened palpebral
fissures Decreased blinking Seborrhea scalp or
face Dementia 6x nl
population- AD in 40%
rapid alt mvmts “Freezing”/akinesia Sialorrhea Depression Micrographia Hypophonia Dystonia
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Epidemiology >1 million in US, 50k new cases yearly Estimates of 400% increase in coming
decades Peak onset 60 (35-85) Course 10-25 years Male > female Some genetic predisposition
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Risk factors + FHx (5-10%) Male gender Pesticide exposure Head trauma Rural living Well water
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Reduced Risk Coffee drinking Smoking NSAID use Estrogen replacement in post-
menopausal women
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Parkinson’s Dz Pathophysiology
Loss of melanin-containing, dopaminergic neurons in substantia nigra
Lewy bodies- protein lint balls. Pathological hallmark of PD when in basal ganglia, but also seen in other disease states
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Diagnosis Difficult! Clinical! No lab test No biomarker And by the time symptoms appear,
dopamine depleted by 70%
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Clinical Dx Progressive, slow unfolding of
characteristic PD s/s during the first few years after onset of sx
Can confirm dx postmortem– Not helpful
Suspect and refer
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Make the case Presenting sx: C/o difficulty with dressing,
cutting food, writing, getting in/out of car, feeling stiff. Spouse notes slowness, blank face
1st visit- usually after 1-2 years of minor changes
Check the hx: gradual worsening, fhx of neuro disorder, drug use, hx encephalitis, toxic exposure
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Office exam I Tremor- resting, not action. Test it.
– How? Rigidity- Check passive ROM. Feel
for mechanical, ratchet-like sensation Bradykinesia- watch her get out of
chair, write*. – *(BET- large, shaky scrawl; PD-
micrographia)
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Office Exam II Impaired postural reflexes- gait
testing- walk away, pivot and return. PD will take extra turning steps.
Pull test. Stand behind and (with warning) pull back on pt. Nl- stops potential fall in 1-2 steps. Be braced to help.
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NOT PD? No response to levodopa Symmetrical, bilateral at onset Rapid progression, including early falls Dysautonomia: incontinence,
orthostatic hypotension, urinary retention
Early cognitive defects Abnormal eye movements
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Differential Dx Drug induced
parkinsonism Progressive
supranuclear palsy Alzheimer’s disease Normal pressure
hydrocephaly Wilson’s Depression
Multiple system atrophy
Dementia with diffuse Lewy body disease
Multi-infarct parkinsonism
Huntington’s Essential tremor
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PD Treatment No proven clinically neuroprotective
drug. But that’s the goal… Start tx when functional disability
starts. Varies based on multiple factors.
Goals: maintain function and QOL, avoid drug-induced complications
(Do no harm.)
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PD Tx- Dopamine
Levodopa- Gold Standard. Converted to dopamine in brain. (Dopamine itself can’t cross blood/brain barrier.)
Improves all features of PD, but wears off over time– Think “Awakenings”
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Dopamine
First line for years, but now primarily second line due to– Side effects (see next slide)– Wearing off– Hypothetical (?) concern that free radicals
generated by the oxidative metabolism of dopamine contribute further to the degeneration of dopaminergic neurons
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Dopamine Side Effects Nausea Wearing off-when effects of single dose
don’t last as long Dyskinesias- sudden, uncontrollable, jerky
movements of arms, legs, head, trunk On/off response- due to fluctuating levels of
dopa– On- uncontrolled movements– Off- motion, freezing
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Add-on meds Dopamine plus…. Carbidopa- (decarboxylase inhibitor) Entacapone (COMT inhibitor, inhibits
break down of catecholamines) Purpose: decreased levodopa
breakdown/conversion in bloodstream, maximizes delivery to brain, minimizes nausea
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Dopamine agonists Maybe some neuroprotection Behave like dopamine by stimulating
dopamine receptor directly Can be used as initial monotherapy
(first line) to preserve use of dopamine for later in disease course
Add-on to dopamine when levodopa alone no longer effective (or SEs intolerable)
Side effect of sudden-onset sleepiness
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Dopamine agonists Bromocriptine (Parlodel) Pergolide (Permax) Pramipexole (Mirapex) Ropinirole (Requip) Apomorphine- (Apokyn) injectable,
rapid-acting, “rescue” med for acute freezing episodes. SE: severe n/v
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Anticholinergics Primarily to alleviate tremor. (Balances
acetylcholine and dopamine.) Trihexylphenidyl (Artane), benztropine
(Cogentin) SE- dry mouth, nausea, constipation,
palpitations, arrhythmias, urine retention Contraindications- BPH, narrow angle
glaucoma, obstructive GI disease Poorly tolerated by elderly
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Amantidine Antiviral flu drug, also anti-dyskinetic
for mild symptoms ? MOA SEs: restlessness, confusion, rash,
edema, nausea, cardiac arrhythmias
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MAO-B inhibitor Monoamine Oxidase type B Inhibitor
Selegiline, rasagiline (also used in Alzheimer’s)
dopa breakdown, may dopamine reuptake
Modest effect for mild sx, reduces “off” time May be neuroprotective SE- confusion, nausea, headache,
insomnia
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Antioxidants Depleted in PD patients. May be
neuroprotective. Glutathione Coenzyme Q Ongoing studies for these relatively
new treatments
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DBS Deep Brain Stimulation
– Surgically implanted neurostimulator in subthalamic nucleus
– Blocks abnormal signals that cause PD sx
– Only for pts whose sx are uncontrolled by medications
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Future/Research Research into causation
– Toxic– Environmental– Genetic
Research into treatment– Neuroprotection– Meds to delay, prevent, or reverse effects
of disease
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Huntington’s Disease
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Definition Autosomal dominant
neurodegenerative disorder. Triad of motor, cognitive and
psychiatric symptoms Insidious onset, no cure Age of onset of sx 30-50, usually after
people have reproduced Fatal in 15-20 years
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Movement disorder Presence of involuntary movements Impairment of voluntary movements Catch 22: Tx of involuntary can
worsen impairment of voluntary, and impairment of voluntary movements is correlated with functional disability
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Involuntary movements Chorea- “the dance” Primary invol
mvmt in HD
Athetosis- proximal limb writhing
Hemiballismus- violent, proximal limb flinging
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Chorea Involuntary, irregular, rapid, uncontrolled,
excessive movement Stark contrast to paucity of movement in
Parkinson’s Seem to move randomly from one body part
to another Appears to be almost playful, fidgety Often not noticed by (nor disturbing to) the
pt
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Impaired movement Abnl eye movements Slow or uncoordinated fine motor
control Dysarthria Dysphagia Gait disturbance Bradykinesia and rigidity late in course
as chorea peaks and wanes
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Psych disorder Under recognized and under treated Depression- no diff tx from “nl” pt Mania-tx with mood stabilizers OCD Irritability Perseveration- esp on focus of irritation Apathy- Can, but won’t. Frontal lobe dysfn Anxiety- rigid thinking develops, and
departure from routine very upsetting
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Cognitive disorder Dysfunction of executive functions:
organization, regulation, perception Problems with planning, judgment, emotion
regulation, attention, learning, cognitive speed, decision making
What it looks like: can’t follow recipe, plan and run errands, work. Temper outbursts. Difficulty with spatial perception.
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Cognitive symptoms usually result in placement outside home before psychiatric or movement symptoms do
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Etiology GABA acetylcholine dopamine Opposite of PD, so instead of too little,
you have too much dopamine.
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Autosomal Dominant Autosomal (not sex-linked) Dominant (expresses itself more strongly
than the normal gene it’s paired with) Each child of a parent with HD has 50%
chance of inheriting gene. All or nothing.
– If you do not inherit the abnormal gene, no chance of passing it on
– A person who inherits the gene defect will eventually develop the disease
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Genetics Huntington gene on 4th autosomal
chromosome (IT-15) has excess repeats of glutamine “CAG” sequence
Normal gene has 10-35 repeats 27-35 repeats may result in nl individual
who transmits increased risk of HD to offspring
36-39 repeats- abnl but may not have symptoms in nl lifespan
40+ repeats will have HD 70+ repeats will have juvenile HD
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No FHx? 2-5% occur with no known FHx Possible causes: early parental death,
adoption, mistaken paternity, or rare “new mutation” caused by expansion of high-normal repeats to cause offspring to be in affected range
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Treatment of HD Directed at symptoms
No cure
No slowing of disease progression
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Treatment- Chorea This isn’t family practice stuff! REFER Neuroleptics: haloperidol,
risperdone, fluphenazine– SEs: sedation, parkinsonism, dystonia,
tardive dyskinesia, dry mouth, weight gain, akathesia (uncomfortable internal sense of restlessness, causes pacing, etc.) **If misinterpret as agitation/anxiety, you may mistakenly medication, and get stuck in a …..
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HD Tx Benzodiazepines clonazepam,
diazepam– SEs: sedation, ataxia, apathy, withdrawal
seizures Dopamine depleting agents
reserpine, tetrabenzine– SEs: hypotension, sedation, depression,
parkinsonism
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Your Role You are a member of an
interprofessional team Who else is on your team?
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Your Role Be aware of meds used, possible side
effects…vicious cycles and all Treat comorbid conditions, such as
depression, insomnia, in consultation with neurologist and others
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Genetic Counseling Half of the offspring will be affected Refer to specialized center
Testing options:– Diagnostic– Predictive– Prenatal
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Future/Research Into how defective gene affects brain
structures and body chemistry and metabolism
Into symptoms and progression of disease Fetal tissue implanted into rodents/primate
brains to try to understand/restore/replace lost function
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Tourette’s
a.k.a.
Gilles de la Tourette Syndrome
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What it is
Inherited neurobehavioral disorder characterized by sudden involuntary, repetitive muscle movements and vocalizations
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Diagnostic Criteria Multiple motor and one or more vocal
tics at some time during the disorder that are NOT explained by another medical condition and are directly observed or recorded (ie: video)
Tic episodes several times a day, almost every day, or periodically during period > 1 year
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Dx Criteria Continued Change in type, severity, complexity,
frequency, and anatomical location during the course of the disorder
Symptoms before age 18
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Epidemiology Symptom onset age 2-15 50% have symptoms by age 7 Four times more common in boys Prevalence 0.1-1.0% of population
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Motor tics Initially simple, located in head and face Blinking, face twitch, head jerk, shrug, neck
stretch, sniffing Over time, change in anatomical location,
and get more complex: squatting, jumping, repetitive touching, deep knee bends, smelling things, spinning, more complex hand gestures
Echopraxia- repeating another person’s actions
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Vocal tics Sudden, involuntary, recurrent, and
often loud Onset: Simple tics- grunt, throat
clearing, sigh, bark, hiss, snort, sniff More advanced: Repeating word(s) or
phrase(s) out of context
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Vocal tics Palilalia- repeating one’s own words Echolalia- repeating heard words- last
words spoken by someone else Coprolalia (rarer, but common in
mainstream depictions) -Involuntary, explosive cursing or compulsive utterance of obscene word/phrase
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More about tics Often assoc with premonitory “urge to
tic” during absorbing activities during times of stress and fatigue Can be voluntarily suppressed for only brief periods
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Course/Prognosis Waxing and waning course Usually combination of motor and
verbal tics Few to many times a day, often in
clusters Generally considered lifelong, but
symptoms can or resolve in adolescence or adulthood
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Associated conditions Obsessive/compulsive behaviors in
25% (touching, counting, washing) Attention deficit in 50-80% Rage/poor impulse control 30% Anxiety 25%
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Pathophys Genetic predisposition Unknown basic underlying defect ?Excessive dopamine in basal ganglia ?Serotonin abnormality ?Other neurotransmitter involvement
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Treatment- Purpose To decrease tics if they present a
problem To decrease associated behavioral
problems To increase academic, occupational,
social performance
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Meds Dopamine antagonists/antipsychotics
(haloperidol, fluphenazine, risperdone) Antianxiety medications
(benzodiazepines, buspirone) Antidepressants (SSRIs)
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Other Tx Considerations Botox injections in small muscle
groups involved in tics- can alleviate tics and also the premonition of tic
HRT (habit reversal training) Biofeedback and relaxation training Vocational, academic, social services
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Trigeminal Neuralgiaa.k.a.
“Tic Douloureaux”
NB: This is part movement disorder, part peripheral
neuropathy.
Classify it how you will, it’s still neuro.
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Qu’est ce que c’est? Intermittent, progressive chronic
disorder involving aberrant firing of trigeminal nerve- usually 2nd and 3rd branches
Unilateral lancing paroxysmal facial pain
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Epidemiology Incidence 5 per 100,000 Prevalence 100-200 per 100,000 3:2 (female:male) Age > 50….younger pts should
make you think MS, other cause
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Tell me how you feel… Stabbing, shocking, “live wire” Pain can be quite severe- can be
disabling. Pain causes muscle spasm (thus a tic) Episodes last a day to several weeks
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Triggers Light touch and vibration
– Breeze, kiss, shaving, chewing, washing, talking
– but NOT firm pressure During an exacerbation, pt will hold
face VERY still to avoid triggering
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Por Que? Increase afferent firing of CN V may
be caused by peripheral injury or disease of nerve.
Nerve root irritation by meningeal inflammation, compression by aberrant vasculature (ie: cerebellar artery)
Possible failure of central inhibitory mechanisms
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Physical Exam NORMAL..
– Except for eliciting pain with soft touch
Do full CN testing, incl corneal reflex
If abnl findings, suspect pain syndrome due to another process…
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Differential Multiple Sclerosis TMJ dysfunction Dental disturbance Giant cell arteritis Sinusitis Glaucoma Acute otitis
Mass effect (tumor) Angina (jaw pain) Atypical face pain Glossopharyngeal
neuralgia Herpes Zoster Postherpetic
neuralgia
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Work-up Classic hx + nl PE = TN
But…not unreasonable to offer elective MRI to r/o uncommon mass lesion or correctable aberrant vessel
If atypical features on hx or PE, proceed to MRI
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Treatment- Drugs Anticonvulsants- hyperactivity of
trigeminal nerve nucleus– Carbamazepine (Tegretol)– Phenytoin (Dilantin)– Oxcarbazepine (Trileptal)– Gabapentin (Neurontin)
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Carbamazepine Usual effective dose 600-1600
mg/day, divided TID or QID Effectiveness over time- incr dose
during exac, decr during remission SE: dizzy, sleepy, nausea, confusion,
leukopenia, liver damage Monitor WBC, LFT serially
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Oxcarbazepine Start 300 mg BID, incr to max of 2400
mg/day Just Carbamazepine with an oxygen
tacked on, solves that pesky leukopenia problem
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Phenytoin Dose 300-500 mg/day, div TID
SE: Gum hypertrophy, dizzy, drowsy
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Gabapentin Start 300 mg TID, titrate up SEs compared to carbamazepine No interaction with phenytoin or
carbamazepine, so good for combination tx
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Surgical treatment For pts who fail or cannot tolerate
medications Subcutaneous alcohol- temporary only Percutaneous glycerol- thru foramen
ovale, inject glycerol into trigeminal cistern to cause nerve damage
Balloon compression of CN V
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More surgery Percutaneous radiofrequency thermal
rhizotomy Microvascular decompression- put
Teflon pad between nerve and offending structures
Gamma knife radiosurgery