w14. movement disorders for the internist dr. davide ... 12 0915 w14 martino...w14. movement...
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W14. Movement Disorders for the Internist
Dr. Davide Martino, PhD MDMovement Disorders Program, DCNS, University of
Calgary
Canadian Society of Internal MedicineAnnual Meeting 2018
Banff, AB
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LEARNING OBJECTIVES
1. Identify the type of tremor in patients with various presentations.
2. Determine which investigations are required in the evaluation of patients with new onset ataxic gait.
3. Manage restless legs syndrome and know which medications should not be used.
CSIM Annual Meeting 2018The following presentation represents the views of the speaker at the time of the
presentation. This information is meant for educational purposes, and should not replace
other sources of information or your medical judgment.
Conflict Disclosures “I have no conflicts to declare”
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LEARNING OBJECTIVE 1
IDENTIFY THE TYPE OF TREMOR IN PATIENTS WITH VARIOUS
PRESENTATIONS
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Assign to this video one of the three possible syndromic diagnoses of tremor:
A. Essential tremor B. Parkinsonian tremor C. Cerebellar tremor
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Diagnosis is clinical• Topography: Tremors can occur in
any joint or muscle that is free to oscillate. Symmetry on the two sides can be important.
• Frequency: For exact frequency measurement a signal analysis of accelerometric or EMG recordings is necessary. However, with some experience the three main frequency ranges can be separated on inspection: high (>7 Hz), medium (4-7 Hz) and low (<4 Hz).
• Additional symptoms: e.g. parkinsonian syndrome, cerebellar ataxia or dystonia
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…and activation• Resting tremor occurs when the muscles of the affected body part are not
voluntarily activated. Rest tremor must cease or be suppressed when a voluntary movement is initiated or performed.
• Action tremor is any tremor that is produced by voluntary contraction of muscles and covers:
• Postural tremor while voluntarily maintaining a position; • Kinetic tremor during voluntary movement.
• Simple kinetic tremor is seen during purposeless voluntary movements.
• Goal-directed tremor (most commonly labeled as intention tremor) occurs when a target is reached.
• Rarer forms of action tremor occur only during certain positions or tasks, e.g.
• task or position specific tremor• isometric tremor
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Assign to this video one of the three possible syndromic diagnoses of tremor:
A. Essential tremor B. Parkinsonian tremor C. Cerebellar tremor
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Assign to this video one of the three possible syndromic diagnoses of tremor:
A. Essential tremor B. Parkinsonian tremor C. Cerebellar tremor
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What is the most likely cause of this tremor?
A. Idiopathic dystonia
B. Exposure to neuroleptics (first generation antipsychotics)
C. Dystonia D. Functional movement disorder E. Lithium exposure
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What is the most likely cause of this tremor?
A. Parkinson’s disease B. Hyperthyroidism C. Dystonia
D. Functional movement disorder E. Cerebellar lesion or degeneration
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LEARNING OBJECTIVE 2
DETERMINE WHICH INVESTIGATIONS ARE REQUIRED IN THE EVALUATION OF
PATIENTS WITH NEW ONSET ATAXIC GAIT
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?
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?
Metronidazole-inducedacute ataxia
[Hari et al. 2013]
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Acute-Subacute
Onset
Persistent
Episodic
Age at onsetViral infections: VZV (children)-
EBV (adults)
MS
SREAT
Paraneoplastic
Wernicke
Stroke
Drugs/Toxins
Genetic episodic ataxias
MS
Stroke
Drug history(CT)/MRI
Thyroid antibodies (anti-TPO + anti-Tg)
If appropriateParaneoplastic panel (anti-Hu, anti-Yo, anti-Ri, anti-CMV)
CSF
With vestibular symptoms/signs?
(nystagmus, vertigo, others)
LithiumPhenytoin
5-fluorouracilCapecitabine
Cytosine arabinosideMetronidazole
Amiodarone
Hg-Pb-MnToluene-benzene
derivativesAlcohol-malnutrition
CEREBELLAR
Look for oculomotor abnormalities
Cognitive changes –nystagmus/abducenspalsy/papilloedema
Cognitive/speech changes – tremor –
myoclonus – seizures –sleep probl - psychosis
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Episodic ataxias (aut dom)Attacks precipitated by stress or emotions
EA1 (KCNA1)Delayed rectifierpotassium channel, Kv1.1
Onset: late childhoodto adolescence
Attacks: secs-mins
Interictal myokimia or neuromyotonia with stiffness and weakness duringattacks
Responds to acetazolamide
EA2 (CACNA1A)P/Q-type calcium channel alpha 1A subunit
Onset: infancy to early adulthood
Attacks: hours to days, with vertigo, nausea, ± headacheInterictal downbeat nystagmus, ataxia and rarely dystonia
Responds to acetazolamide or 4-aminopyridine
Allelic to SCA6, IGE, FHM type 1, congenital ataxia and hemiplegic migraine with cerebral edema
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Acute-Subacute
Onset
Persistent
Vitamin deficiency (B12, E, folic acid)
Neurosarcoidosis
Vertebrobasilarinsufficiency
Syphilis
Drugs/Toxins
Sensory polyneuropathies
Associated with proprioceptive sensory loss de-afferentation
ataxia
CDDP, cisplatin, carboplatin, oxaliplatin
DoxorubicinBortezomib
Suramin sodiumThalliumPenicillin
Subacute combined
degeneration
• Drug history• Vitamin B12 (MMA-Hcy)
and E• MRI spine• CXR/CT chest + serum
markers of sarcoidosis (SAA, sIL-2R, ACE, KL-6)
• Syphilis serology• NCS/CSF, anti-GQ1b
Cognitive – depression - neuropathy – spastic
para/tetraparesis
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Rapidprogression
Sporadic
Inherited
Age at onset
SREAT
Paraneoplastic
CJD
GSS(PRNP gene: P102L most
frequently)
MRIThyroid
antibodiesCSF/EEG
Cognitive/speech changes – tremor –
myoclonus – seizures –sleep probl - psychosis
Cognitive – psychosis and agitation -
depression
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What is the most likely diet that has improved this patient’s ataxia?
A. Low-protein diet B. Ketogenic diet C. Feingold diet
D. Atkins diet E. Gluten-free diet
[Hernàndez-Lahoz et al. 2014]
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What is the most likely diet that has improved this patient’s ataxia?
A. Low-protein diet B. Ketogenic diet C. Feingold diet
D. Atkins diet E. Gluten-free diet
[Hernàndez-Lahoz et al. 2014]
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66-yr old woman
4-year hx gait and
hearing problems
Forgetfulness – mood
No hx of trauma or
intradural surgery
Babinski on the right
Serum iron mildly
decreased
Serum ferritin mildly
increased
What is the most likely cause for this patient’s ataxia?
A. Folic acid deficiency B. Neuroferritinopathy
C. Superficial siderosis of the CNS D. Spontaneous intracranial hypotension
E. Vitamin E deficiency
[Bae et al. J Mov Disord 2014]
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66-yr old woman
4-year hx gait and
hearing problems
Forgetfulness – mood
No hx of trauma or
intradural surgery
Babinski on the right
Serum iron mildly
decreased
Serum ferritin mildly
increased
What is the most likely cause for this patient’s ataxia?
A. Folic acid deficiency B. Neuroferritinopathy
C. Superficial siderosis of the CNS D. Spontaneous intracranial hypotension
E. Vitamin E deficiency
[Bae et al. J Mov Disord 2014]
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SLOW PROGRESSION ATAXIAS
Sporadic
Alcohol-related* chronic thiamine deficiency; 11-27% of chronic alcohol users
// gait and LL>UL and speech // vermal atrophy
Gluten ataxia* anti-TG6 IgA (73%) // most have cerebellar atrophy // responds
to gluten-free diet // PNpathy in 40%
Metabolic subacute combined degeneration* // vit.E, vit.B1 // hypothyroidism
and hypoparathyroidism
Toxic lithium, phenytoin, 5-FU, capecitabine, citarabine, metronidazole and
other azoles, amiodarone, heavy metals and solvents (Hg, Pb, Mn,
toluene/benzene derivatives)
Infections neurosyphilis*, Lyme*, Whipple’s
Superficial siderosis +hearing loss, pyramidal, cognitive, seizures, visual loss,
hyposmia
MSA-C and heredodegenerative ataxias
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LEARNING OBJECTIVE 3
MANAGE RESTLESS LEGS SYNDROME AND KNOW WHICH MEDICATIONS
SHOULD NOT BE USED
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CASE #1
34-yr old nurse referred for early insomnia (on bad days latency of up to 2-3 hrs),
non-refreshing sleep (frequent awakenings, 3-5 times per night, with achy legs;
partner prefers to sleep in different bed), and malaise throughout the day
Onset in late adolescence, but as a child she suffered from «growing pains» in her
legs and feeling that her legs were only loosely attached to her body and at times
hard to control
Aches and irritating discomfort in her legs during the evening, initially attributed to
stress and shiftwork – urgency to move her legs to alleviate the discomfort,
especially when lying or sitting down (typically not occurring around a daytime
nap)
Relaxation training, sleep health habits, prescription hypnotics (temazepam 30mg
hs) unsuccessful
PSG observation of PLMS, with short arousals in 70% of them
5 cups of strong coffee throughout the day
Serum ferritin = 24 ng/mL
[from sleepdisorders.sleepfoundation.orgNational Sleep Foundation]
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How do we approach this patient with RLS?
Key questions
1. Have we ruled out the main causes of secondary RLS? What are these?
o Medical conditions: iron deficiency ( Ferrous sulfate + vit.C), end-stage
renal disease/on hemodialysis (vit.C + E suppl – ropinirole, L-dopa, exercise), obesity, COPD, DM, IBS
o Neurological diseases: small fibre neuropathies, PD
o Drugs: DR blockers, NSRIs, AEDs (e.g. zonisamide)
o Pregnancy
2. Is RLS sufficiently severe to warrant specific treatment?
o Strong evidence (AAN Level A) pramipexole [0.125, 0.25-0.5mg],
rotigotine patch [1, 1-3mg], gabapentin enacarbil [600, 600mg]
o Moderate evidence ropinirole [0.25, 0.25-4mg], pregabalin*, IV ferric
carboxymaltase* [500mg given twice 5 days apart]
o Weak evidence L-dopa*
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How do we approach this patient with RLS?
Key questions
3. Should we target sleep disruption?
o PLMS [PLMI index on PSG] causing frequent awakenings: ropinirole,
pramipexole, rotigotine, pregabalin
o Improving TST, sleep efficiency, sleep latency, wake after sleep onset:
ropinirole, gabapentin enacarbil, pregabalin
o Subjective sleep measures: gabapentin enacarbil, ropinirole,
pregabalin>pramipexole, rotigotine, L-dopa
4. Risk of augmentation?
o Pregabalin / Gabapentin enacarbil
o Pramipexole / Ropinirole / Rotigotine
o L-dopa
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How do we approach this patient with RLS?
Key questions
Is the patient not responding to treatments mentioned so far?
o Prolonged-release oxycodone/naloxone (Level C evidence for RLS symptoms,
subjective sleep symptoms, and QoL) short courses, very close monitoring!
Cannabis? Insufficient evidence
Non-pharmacologic approaches?
o Pneumatic compression (inflatable garments and electrical pneumatic pump)
before usual symptom onset likely effective
o NIBS near-infrared stimulation and rTMS (SMA,M1) possibly effective
o tDCS probably ineffective
o Vibrating pads possibly ineffective
o Acupuncture insufficient evidence
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CASE #2 72-yr old lady suffering from RLS symptoms since age 30, hospitalized for severe
mood disorder and aggravation of RLS and insomnia
Ropinirole ineffective up to 5.5mg daily (recent rapid increases)
Described a «burst of heat radiating from legs to head», toes’ pain and warm
feeling in both feet, relieved by movement; sometimes restless feeling also in
upper limbs and trunk
Both sister and mother had RLS
No psychoactive substances (previous course of sertraline worsened RLS)
Comorbidities: glaucoma, spondylodegenerative changes (C-L), LLEE varicose
veins, joint pain, stress incontinence
Bloodwork ok (HGB 13.0g/dL, ferritin 100ng/ml, EGFR >60ml/min/1.73) apart from
fasting glucose 5.6 mmol/L
PLMI left leg 47.7/h, right leg 49.9/h
NCS advanced axonal neuropathy of both sural nerves (L>R)
HOW SHALL WE APPROACH THIS CASE?
[from Narowska et al., 2015]
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CASE #2
1. Have we ruled out the main causes of secondary RLS?
2. Is RLS sufficiently severe to warrant specific treatment?
3. Should we target sleep disruption?
4. Risk of augmentation?
[from Narowska et al., 2015]
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CASE #3
35-yr old lady diagnosed with RLS in
adolescence, very intermittent in severity
Third trimester of pregnancy: sudden
worsening of symptoms (at least 3 days a
week), circadian occurrence related to
fatigue and evening exercise
No other significant complications in
pregnancy or delivery
HOW SHALL WE APPROACH THIS
CASE?
[from Policiano et al., 2014; Garbazza & Manconi, 2018]
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