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Page 1: Movement Disorders - WordPress.com · Movement Disorders Charles Braun, MD IHA Neurology Consultants . Case 1: •73 year old right handed woman presents for initial neurological

Movement

Disorders Charles Braun, MD

IHA Neurology Consultants

Page 2: Movement Disorders - WordPress.com · Movement Disorders Charles Braun, MD IHA Neurology Consultants . Case 1: •73 year old right handed woman presents for initial neurological

Case 1:

• 73 year old right handed woman presents for initial neurological evaluation for complaint of “right sided weakness”. She describes progressive difficulty over the last 2 years with fine movements in the right hand, problems writing and change in walking.

• On review of systems she endorses chronic constipation and decreased sense of smell/taste.

• Her PCP obtained an MRI C spine which showed moderate cervical stenosis with spondylosis with multilevel neuroforaminal narrowing but no cord compression.

• Her exam shows moderate to severe bradykinesia in the right hand, decreased blink rate, asymmetric right>left rigidity, mild postural instability on pull test. She has no tremor.

Page 3: Movement Disorders - WordPress.com · Movement Disorders Charles Braun, MD IHA Neurology Consultants . Case 1: •73 year old right handed woman presents for initial neurological

Case 2:

• 23 year old woman transferred to MICU from psychiatry service after prolonged GTC seizure. She was originally admitted to psychiatry service 2 weeks ago with paranoid delusions and auditory hallucinations for 1 week. She originally endorsed rare cocaine use in college and 1 episode of MDMA use 3 months ago. She was treated with olanzapine and prn haldol. On day 5 of admit she became catatonic for 2 days and was given ativan 1 mg q6h.

• Her basic labs and head CT were unremarkable when she presented originally.

• Her exam shows T: 38C, BP: 144/94, HR: 94, RR: 8, cessation of convulsive activity, no response to verbal commands, diffuse rigidity, occasional bizarre tongue protruding movements and brisk reflexes throughout. Her eyes are open but she does not acknowledge anyone or blink to threat.

Page 4: Movement Disorders - WordPress.com · Movement Disorders Charles Braun, MD IHA Neurology Consultants . Case 1: •73 year old right handed woman presents for initial neurological

Case 3:

• 64 year old man with recent falls. The family describes

cognitive changes with more impulsive behavior and a

change in gait. He fell down stairs and sustained a

subdural hematoma that was evacuated 1 year ago.

• On review of systems, the patient describes difficulty

with aspirating both liquids and solids.

• Exam shows symmetric rigidity, severe postural

instability with exaggerated findings on pull test and

limitation in down gaze.

• MRI brain shows atrophy in midbrain described as

hummingbird sign.

Page 5: Movement Disorders - WordPress.com · Movement Disorders Charles Braun, MD IHA Neurology Consultants . Case 1: •73 year old right handed woman presents for initial neurological

Case 4:

• 42 year old obese woman with poorly controlled type II

diabetes x 10 years with gastroparesis and migraine

headaches. She presents to ED with 5 days of intractable

migraine. She is given migraine cocktail that she

typically has responded to in the past which includes IV

toradol, reglan, and valium. 3 hours later she wakes up

from sleep with agitation and tightness in the right side of

her neck.

• Exam shows her agitated, pacing with rigid right sided

cervical paraspinal muscles and trapezius.

Page 6: Movement Disorders - WordPress.com · Movement Disorders Charles Braun, MD IHA Neurology Consultants . Case 1: •73 year old right handed woman presents for initial neurological

Case 5:

• 64 year old veteran male with hepatitis C, alcoholism, cirrhosis, HTN, DM type II and CKD stage 3 admitted for worsening volume overload and abdominal pain for which he has been getting breakthrough oxycodone. For concern of SBP he was started on levaquin. Over the last 12 hours he reports multifocal brief jerks involving hands, arms, and legs, with retained awareness.

• Medication list includes: aspirin, gabapentin, citalopram, insulin, lactulose, amlodipine, lisinopril, morphine 30 mg

• Exam shows ascites, slowed responses to questions but appropriate, bilateral ptosis, normal strength, bulk and tone, without asterixis.

Page 7: Movement Disorders - WordPress.com · Movement Disorders Charles Braun, MD IHA Neurology Consultants . Case 1: •73 year old right handed woman presents for initial neurological

Step 1: Observe • Are the movements normal or

abnormal?

• Are they paroxysmal (sudden on/off), continual (repeated again and again), or continuous (non-stop)?

• How does voluntary movement affect abnormal movements?

• Are movements present at rest, posture, action, intention, or combination?

• Do environmental stimuli/emotion precipitate/exacerbate/alleviate?

• Is patient aware of movements?

• Do movements stop with sleep?

Step 2: Describe • Onset

• Location

• Amplitude

• Frequency

• Duration

Page 8: Movement Disorders - WordPress.com · Movement Disorders Charles Braun, MD IHA Neurology Consultants . Case 1: •73 year old right handed woman presents for initial neurological

Step 3: Classify

• Hyperkinetic • Akithisia

• Ataxia

• Athetosis

• Ballismus

• Chorea

• Dyskinesia

• Dystonia

• Myoclonus

• Myokymia

• Psychogenic

• Tics

• Tremor

• Hypokinetic • Parkinsonism

• Rigidity

• Brady/akinesia

• Freezing

Page 9: Movement Disorders - WordPress.com · Movement Disorders Charles Braun, MD IHA Neurology Consultants . Case 1: •73 year old right handed woman presents for initial neurological

Step 4: diagnose

Step 5: treat

• Acute onset Ischemia, Trauma, Toxin/Medication

• Subacute onset Inflammatory, Infectious, Degenerative

• Slowly progressive Hereditary, Degenerative

• Episodic Hereditary, Toxic/Metabolic

Page 10: Movement Disorders - WordPress.com · Movement Disorders Charles Braun, MD IHA Neurology Consultants . Case 1: •73 year old right handed woman presents for initial neurological

Definitions

• Akinesia - absence of movement.

• Akithisia - restlessness, which is reduced or relieved by moving

• Asterixis – form of negative myoclonus, brief loss of postural tone

• Ataxia – “lack of order,” uncoordinated voluntary movements, can involve trunk or limbs

• Athetosis - writhing, continuous, involuntary movements.

• Ballismus – explosive large amplitude, jerking/flinging movement inv proximal musculature

• Bradykinesia - slowness of movement.

• Chorea – flowing, “dance-like”, irregular.

• Dysdiadochokinesia – impaired ability to perform rapid alternating movements

• Dyskinesia - excessive abnormal involuntary movement.

• Dystonia – sustained posture often with twisting.

• Dysmetria – misjudging the distance to a target.

• Myoclonus - sudden, brief, shock like movements caused by muscle contractions.

• Myokymia - fine persistent quivering or rippling of muscles.

• Parkinsonism - any combination of six cardinal features: tremor at rest, bradykinesia, rigidity, flexed posture, freezing, and loss of postural reflexes.

• Parkinson’s Disease – asymmetric onset, progressive, hallmark is bradykinesia.

• Rigidity – resistance to movement at a joint, not velocity dependent

• Spasticity – velocity dependent resistance at a joint, depends on direction of movement.

• Tics – compulsion to perform action, can be subdued, motor vs phonic, simple vs complex.

Page 11: Movement Disorders - WordPress.com · Movement Disorders Charles Braun, MD IHA Neurology Consultants . Case 1: •73 year old right handed woman presents for initial neurological

Anatomy

Basal Ganglia Striatum-caudate/putamen

Globus Pallidus-interna externa

Substantia Nigra

Ventral Tegmental area

Subth. Nuc.-pars comp/pars retic

Thalamus

Page 12: Movement Disorders - WordPress.com · Movement Disorders Charles Braun, MD IHA Neurology Consultants . Case 1: •73 year old right handed woman presents for initial neurological

Circuitry

Page 13: Movement Disorders - WordPress.com · Movement Disorders Charles Braun, MD IHA Neurology Consultants . Case 1: •73 year old right handed woman presents for initial neurological

Tremor

Page 14: Movement Disorders - WordPress.com · Movement Disorders Charles Braun, MD IHA Neurology Consultants . Case 1: •73 year old right handed woman presents for initial neurological

Tremor: Definitions

• Rhythmic oscillation of a body part, produced by either alternating or synchronous contractions of reciprocally innervated antagonist muscles.

• Rest tremor = present when body part is in complete repose, although totally relaxed or asleep usually disappears. Dampens with movements

• Postural Tremor = worse with maintenance of posture, eg extending arms parallel to floor

• Kinetic Tremor = present throughout a goal directed movement

• Intention Tremor = moving body part to and from a target brings it out

• Action Tremor = used for both postural and kinetic tremor

• Terminal Tremor = kinetic tremor that is worse near target

• Ataxic Tremor = combo of kinetic tremor plus limb ataxia

• Re-emergent Tremor = with severe rest tremors, with outstretched arms tremor occurs after latency of several seconds, can look postural

Page 15: Movement Disorders - WordPress.com · Movement Disorders Charles Braun, MD IHA Neurology Consultants . Case 1: •73 year old right handed woman presents for initial neurological
Page 16: Movement Disorders - WordPress.com · Movement Disorders Charles Braun, MD IHA Neurology Consultants . Case 1: •73 year old right handed woman presents for initial neurological

Tremor

• What body parts are affected – limbs, trunk, head/face, jaw,

tongue, voice

• Onset and time course

• Worse or better at rest, with maintained postures or with goal

directed movements

• Specific movements that bring it out

• Drugs/Toxins: neuroleptics, TCAs, AEDs, beta agonists,

caffeine, EtOH, lithium, amphetamines, theophylline, mercury,

lead, arsenic

• PMH: thyroid, hypo/hyperglycemia, pheochromocytoma

• Family history

• Amplitude, Frequency, Symmetry, Distractibility/Variations

Page 17: Movement Disorders - WordPress.com · Movement Disorders Charles Braun, MD IHA Neurology Consultants . Case 1: •73 year old right handed woman presents for initial neurological

Essential Tremor

Diagnosis:

• 10 million in US

• 50-60% familial

• Freq is fixed, amplitude is highly variable

• Usually symmetric, postural + kinetic

• No rigidity, postural inst, gait abn, etc.

• Nodding head

• Exclusion criteria: dystonia, isolated tongue/chin tremor

Treatment

• Propranolol 20-240 mg daily

• Primidone 25- 250 mg daily

• Topiramate

• Gabapentin

• ETOH prn

• Stereotactic thalamotomy

• DBS – Thalamic ventralis intermedius nucleus.

Page 18: Movement Disorders - WordPress.com · Movement Disorders Charles Braun, MD IHA Neurology Consultants . Case 1: •73 year old right handed woman presents for initial neurological

Restless Legs Syndrome

Page 19: Movement Disorders - WordPress.com · Movement Disorders Charles Braun, MD IHA Neurology Consultants . Case 1: •73 year old right handed woman presents for initial neurological

Restless Legs Syndrome:

• Sensory disturbances in the legs, including pins and needles, creeping or crawling, aching, itching, stabbing, heaviness, tension, burning, and coldness.

• Occasionally in the arms

• During recumbent position, often associated with insomnia

• 85% have periodic limb movements of sleep, which is periodic slow, sustained (1- to 2-second) movements range from synchronous or asynchronous dorsiflexion of the big toes and feet to triple flexion of one or both legs.

Page 20: Movement Disorders - WordPress.com · Movement Disorders Charles Braun, MD IHA Neurology Consultants . Case 1: •73 year old right handed woman presents for initial neurological

RLS: Diagnosis

• DSM V: An urge to move the legs that is usually accompanied by or occurs in response to uncomfortable and unpleasant sensations in the legs, characterized by all of the following: (1) the urge to move the legs begins or worsens during periods of rest or inactivity; (2) the urge is partially or totally relieved by movement; and (3) the urge to move legs is worse in the evening or at night than during the day or occurs only in the evening or at night

• Symptoms occur at least 3 times per week and have persisted for at least 3 months

• Symptoms cause significant distress or impairment in social, occupational, educational, academic, behavioral or other areas of functioning

• NEED TO RULE OUT IRON DEFICIENCY!!! Even if iron studies are normal, low ferritin up to 45 mg/dL should be corrected with ferrous sulfate 325 mg TID.

• Associated with peripheral neuropathy, so check Fasting BS, hgbA1C, Vit B12, TSH.

• No imaging necessary.

• PSG may be necessary if atypical or in children.

• DDx: Akathisia, Neuropathy, Nocturnal leg cramps, Painful legs and moving toes, Vascular disease, Radiculopathy.

Page 21: Movement Disorders - WordPress.com · Movement Disorders Charles Braun, MD IHA Neurology Consultants . Case 1: •73 year old right handed woman presents for initial neurological

RLS: Treatment

• Treat underlying cause if secondary to neuropathy or Iron Def

• Dopamine drugs: pramipexole, ropinirole, levodopa

• Gabapentin

• Benzos

• Opiates

• Discontinuation, when possible, of medications that cause or exacerbate RLS, such as selective serotonin reuptake inhibitors (SSRIs), serotonin-norepinepherine reuptake inhibitors (SNRIs), diphenhydramine, and dopamine antagonists.

• Sleep Hygiene

• Beware of Restless Genital Syndrome!!

Page 22: Movement Disorders - WordPress.com · Movement Disorders Charles Braun, MD IHA Neurology Consultants . Case 1: •73 year old right handed woman presents for initial neurological

ATAXIA

Page 23: Movement Disorders - WordPress.com · Movement Disorders Charles Braun, MD IHA Neurology Consultants . Case 1: •73 year old right handed woman presents for initial neurological

Ataxia: symptoms/signs

• Gait Disturbance – sx: insecure walking, falls. si: sway with stance (lateropulsion), wide based stance, poor tandem, etc

• Limb ataxia – sx: clumsiness with tasks, si: dysmetria, dysdiadochokinesia

• Truncal ataxia – titubation; midline cerebellar lesions.

• Dysarthria and Bulbar Symptoms – sx/si: slurred speech, abn of pitch, tone, volume (scanning speech), dysphagia (incoordination of swallowing muscles), ineffectiveness of cough.

• Visual – sx: blurriness or sense of movement of environment due to ocular oscillations si: gaze evoked nystagmus, VOR dysfunction

• Sensory Ataxia – sx: no visual, speech, swallowing problem. si:

• Tone – decreased

• Cognitive/Affective: executive dysfunction, defective visual/verbal memory, verbal fluency, cerebellar mutism

Page 24: Movement Disorders - WordPress.com · Movement Disorders Charles Braun, MD IHA Neurology Consultants . Case 1: •73 year old right handed woman presents for initial neurological

Ataxia: Etiology

• Acquired • Ischemia

• Trauma

• Infectious/post-infectious • HTLV, Lyme, HIV, viral cerebellitis,

ADEM, Miller-Fisher, TB, WNV, Whipple’s dz

• Inflammatroy/Autoimmune • SLE, GAD, Sjogren’s, Celiac, Behcet’s

• Neoplastic/paraneoplastic • Yo, Ma, Tr, VGCC, Hu, Ri,CV2/CRMP5

• Toxic/Metabolic • Alcohol, Hypo/hyperglycemia,

hypothyroid, AEDs, lithium, cyclosporine, MTX, azoles, amiodarone, 5FU, thallium, toluene, bismuth, mercury

• Vitamin Def • Thiamine, vit B12, vit E

• Degenerative • CJD, MSA, PSP

• Hereditary/Congenital • Autosomal Dominant

• SCA 1-36

• Episodic Ataxia

• Autosomal Recessive • Friedrich’s ataxia, ataxia-telangiectasia,

ataxia with oculomotor apraxia, Wilson’s, ataxia with vit E def, cerebrotendinosus xanthomatosis, CoQ10 Def, Refsum Dz, lysosomal storage diseases, PKAN

• X linked • Fragile X tremor ataxia synd

• Pyruvate dehydrogenase def

• X linked adrenoleukodystrophy

• Mitochondrial • Myoclonic epilepsy with ragged red

fibers (MERRF)

• Kearns-Sayre syndrome

• Leigh syndrome

• Arnold-Chiari

• Congenital cerebellar hypoplasia

• Dandy Walker

• Joubert Syndrome (absence of vermis)

Page 25: Movement Disorders - WordPress.com · Movement Disorders Charles Braun, MD IHA Neurology Consultants . Case 1: •73 year old right handed woman presents for initial neurological

Ataxia: Diagnosis

Page 26: Movement Disorders - WordPress.com · Movement Disorders Charles Braun, MD IHA Neurology Consultants . Case 1: •73 year old right handed woman presents for initial neurological

Ataxia: treatment

• Treat underlying cause for acquired disorders

• For genetic disorders there’s no specific tx

• Case reports of SSRI, Idebenone

• Physical Therapy

Page 27: Movement Disorders - WordPress.com · Movement Disorders Charles Braun, MD IHA Neurology Consultants . Case 1: •73 year old right handed woman presents for initial neurological

Chorea and athetosis:

Signs/Symptoms, other

clues

• Definition: irregular, unpredictable, brief, jerky

movements that flow randomly from one part of the body

to another. Choreoathetosis is slow chorea, typically seen

in patients with cerebral palsy.

• Age at onset and acuity of onset are big clues

• Originally noticed as fidgitiness, patients try to mask,

then clumsiness, usually hard to diagnose

• Motor impersistence: trombone tongue, milkmaid grip,

irregular/lurching gait

Page 28: Movement Disorders - WordPress.com · Movement Disorders Charles Braun, MD IHA Neurology Consultants . Case 1: •73 year old right handed woman presents for initial neurological

Chorea: Etiology

Acquired

• Ischemia

• Autoimmune/Inflammatory • APLS, celiac, Hashimoto’s, SLE,

syndenham’s (ASO titer), PAN, Behcet’s, Sarcoidosis, Sjogren’s

• Infectious • HIV/AIDS, CJD, diptheria, viral

encephalitis, Legionnaire’s, Lyme, Malaria, NCC, syphilis, PML, TB

• Neoplastic/Paraneoplastic

• Metabolic/Endocrine • Hepatic failure, hyperthyroidism,

hypo/hypercalcemia, hypo/hyperglycemia, hypo/hypernatremia, hypomagnesemia, polycythemia, pregnancy (chorea gravidarum), renal failure, vit def (thiamine, vit b12, niacin)

• Toxic • Alcohol, CO, glue, manganese, mercury,

thallium, toluene, drugs (neuroleptics, antiparkinsonian drugs, amphetamines, cocaine, TCAs, OCPs,

Hereditary

• Autosomal Dominant • Huntington’s Dz (CAG repeat>50), benign

hereditary chorea, SCA1-3, 17, dentatorubral pallidoluysian atrophy, Fahr’s Dz, Neuroferritinopathy, Paroxysmal Kinesigenic choreoathetosis, Paroxysmal nonkinesigenic dyskinesia

• Autosomal Recessive • Ataxia with oculomotor apraxia types 1&2,

ataxia-telangiectasia, choreoacanthocytosis, Friedreich ataxia, neuronal ceroid lipofuscinosis, PKAN, phenylketonuria, porphyria, Wilson’s Dz

• X linked • McLeod Syndrome

• Lesch-Nyhan Disease

• Mitochondrial Disorders

Page 29: Movement Disorders - WordPress.com · Movement Disorders Charles Braun, MD IHA Neurology Consultants . Case 1: •73 year old right handed woman presents for initial neurological
Page 30: Movement Disorders - WordPress.com · Movement Disorders Charles Braun, MD IHA Neurology Consultants . Case 1: •73 year old right handed woman presents for initial neurological

Chorea

Page 31: Movement Disorders - WordPress.com · Movement Disorders Charles Braun, MD IHA Neurology Consultants . Case 1: •73 year old right handed woman presents for initial neurological

Chorea: Diagnosis

Page 32: Movement Disorders - WordPress.com · Movement Disorders Charles Braun, MD IHA Neurology Consultants . Case 1: •73 year old right handed woman presents for initial neurological

Chorea: treatment

• Treat underlying cause

• Huntington’s

• Tetrabenazine (depletes dopamine)

• Antipsychotics (haldol)

• Antiepileptics (depakote, tegretol)

Page 33: Movement Disorders - WordPress.com · Movement Disorders Charles Braun, MD IHA Neurology Consultants . Case 1: •73 year old right handed woman presents for initial neurological

Tardive Dyskinesia

• Caused by neuroleptics and other dopamine blocking drugs like reglan, compazine, etc. Risk with atypical antipsychotics is not lowered. Can happen days to weeks after stopped.

• Chorea is random flowing movements involving multiple body parts whereas TD is stereotyped usually isolated to orolingual region (often lower facial muscles).

• Can be subdued. Often a sensory trick like pressing finger to mouth.

• Distractibility often makes TD worse.

• No motor impersistence (unlike true chorea)

• Gait is often normal in TD

• Akithisia often coincides with TD

• Treatment is removal of offending medicine

• Acute treatment involves anticholinergic like Benadryl.

Page 34: Movement Disorders - WordPress.com · Movement Disorders Charles Braun, MD IHA Neurology Consultants . Case 1: •73 year old right handed woman presents for initial neurological

Dystonia: key points

• Sustained muscle contractions, which often cause twisting and repetitive movements or abnormal postures

• Often a sensory trick to abate the symptoms

• Most often focal, but can be generalized

• Can be task specific (eg – writing, violin, running)

• Can occur after injury to affected limb (associated with casting, CRPS

• Examples of dystonia include: torticollis, blepharospasm, laryngeal dystonia

• Age of onset and location of symptoms are often helpful clues for diagnosis

• Medication history is huge historical factor

• Family history should be reviewed in detail since family members may have symptoms in different distributions that go unrecognized

• Dopa-responsive dystonia often have strong diurnal component (worsens as day goes on)

• Sleep lessens or remits symptoms usually, so if present during sleep consider musculoskeletal abnormality or contracture.

Page 35: Movement Disorders - WordPress.com · Movement Disorders Charles Braun, MD IHA Neurology Consultants . Case 1: •73 year old right handed woman presents for initial neurological

Dystonia: diagnosis

• Focal – localized to a specific body part (most common)

• Segmental – involves 2 or more adjacent body parts (second most common)

• Multifocal – involves 2 or more non-adjacent body parts

• Hemidystonia (Unilateral) – involves arm and leg on only one side

• Generalized – Trunk plus two other regions

• If <40 yrs, draw ceruloplasmin and 24 hour urinary copper (Wilson’s)

• If generalized, likely to be genetic. Some respond to levodopa

• Focal/Segmental dystonia: MRI and Wilson’s labs

• Causes

• Genetic: http://www.cmdg.org/Dystonia/Dystonia_genetics/dystonia_genetics.htm

• Acquired: Tumor, stroke, MS, trauma, meds with tardive dystonia, Parkinson’s, PSP, MSA, CBD, Huntington’s, Wilson’s, encephalitis (WNV), paraneoplastic,APLS, psychogenic.

Page 36: Movement Disorders - WordPress.com · Movement Disorders Charles Braun, MD IHA Neurology Consultants . Case 1: •73 year old right handed woman presents for initial neurological

Dystonia: Treatment

• In young/generalized dystonia: try levodopa

• Generalized: 1st line is anticholinergics like Artane. Other

meds to try are tetrabenazine, muscle relaxants like benzos,

flexeryl, baclofen pump, AEDs, deep brain surgery.

• Focal/Segmental: 1st line is botox. Can add muscle relaxants

like benzos, baclofen, etc.

• Status Dystonicus: medical emergency. I’ve seen caused by

withholding anti-parkinsonian drugs in advanced PD.

Manifests with severe generalized stiffness, fever, markedly

elevated CK, respiratory, renal failure. Tx is Versed gtt add

propofol pentobarb coma baclofen infusion surgery,

DBS.

Page 37: Movement Disorders - WordPress.com · Movement Disorders Charles Braun, MD IHA Neurology Consultants . Case 1: •73 year old right handed woman presents for initial neurological

Dyskinesias

Page 38: Movement Disorders - WordPress.com · Movement Disorders Charles Braun, MD IHA Neurology Consultants . Case 1: •73 year old right handed woman presents for initial neurological

Myoclonus

• Sudden, brief, shock-like involuntary movement possibly caused by active muscle contraction (positive myoclonus) or inhibition of ongoing muscle activity (negative myoclonus)

• Huge Differential

• Huge variation in Classification

• Body parts afffected: Focal, multifocal or generalized

• Origination in Nervous system: Cortical, subcortical, spinal or peripheral

• Spontaneous or Reflex

• Etiology: Physiological, essential, epileptic, symptomatic or psychogenic

Page 39: Movement Disorders - WordPress.com · Movement Disorders Charles Braun, MD IHA Neurology Consultants . Case 1: •73 year old right handed woman presents for initial neurological

Myoclonus

Page 40: Movement Disorders - WordPress.com · Movement Disorders Charles Braun, MD IHA Neurology Consultants . Case 1: •73 year old right handed woman presents for initial neurological

Myclonus

• Post-anoxic Myoclonus

• Lance Adams Syndrome (delayed post-anoxia)

• Benign Essential Myoclonus

• Degenerative Disease

Page 41: Movement Disorders - WordPress.com · Movement Disorders Charles Braun, MD IHA Neurology Consultants . Case 1: •73 year old right handed woman presents for initial neurological

Myoclonus: Diagnosis

• BMP

• LFTs

• Thyroid antibodies and function

• Drug and toxin screen (if history suggests)

• Paraneoplastic antibodies

• Brain imaging Spine imaging (if focal or segmental)

• Cerebrospinal fluid analysis (eg, for infectious and inflammatory disorders, prion disease)

• EEG, may need long-term to capture multiple events to characterize

• EMG

• SSEPs

• EMG/EEG

If not otherwise ruled out, the following should be considered:

• Body imaging for occult cancer, even in the absence of paraneoplastic antibodies

• Evaluation for malabsorption disorders such as Celiac sprue and Whipple disease

• Enzyme assays for neuraminidase deficiency (sialidosis) and biotinidase deficiency (multiple carboxylase deficiency)

• Evaluation for Wilson disease

• Evaluation for ataxia-telangiectasia

• Genetic testing (and tissue biopsy if indicated) for inherited disorders, such as Unverricht-Lundborg disease (EPM1 gene), Lafora body disease (EPM2A and EPM2B genes), neuronal ceroid lipofuscinosis, and Huntington disease

• Evaluation for mitochondrial disorders, including serum lactate, muscle biopsy, and genetic testing

Page 42: Movement Disorders - WordPress.com · Movement Disorders Charles Braun, MD IHA Neurology Consultants . Case 1: •73 year old right handed woman presents for initial neurological

Myoclonus

• Treat underlying cause

• AEDs – keppra, depakote, phenobarbital, benzos

Page 43: Movement Disorders - WordPress.com · Movement Disorders Charles Braun, MD IHA Neurology Consultants . Case 1: •73 year old right handed woman presents for initial neurological

Tics

• Sudden, abrupt, repetitive, and stereotyped movements that may resemble gestures and mimic fragments of normal behavior, vary in intensity, and repeated at irregular intervals.

• Onset in childhood/adolescence

• Inner urge to perform

• Can subdue for period of time

• When subdued, often burst of multiple

Page 44: Movement Disorders - WordPress.com · Movement Disorders Charles Braun, MD IHA Neurology Consultants . Case 1: •73 year old right handed woman presents for initial neurological

Parkinsonism

Page 45: Movement Disorders - WordPress.com · Movement Disorders Charles Braun, MD IHA Neurology Consultants . Case 1: •73 year old right handed woman presents for initial neurological

Parkinsonism: Features

Cardinal Features:

• Rest Tremor

• Rigidity

• Bradykinesia

• Postural Instability

Secondary Features:

• Micrographia

• Masked face

• Slowing of ADLs

• Stooped, shuffling gait

• Decreased arm swing with walking

• Difficulty arising from a chair

• Difficulty turning in bed

• Hypophonic speech

• Sialorrhea

• Loss of the sense of smell

• Foot dystonia

• Constipation

Page 46: Movement Disorders - WordPress.com · Movement Disorders Charles Braun, MD IHA Neurology Consultants . Case 1: •73 year old right handed woman presents for initial neurological

Parkinsonism

• Primary PD ~ 85%

• Drug-induced PDism 7% - 9%

• MSA ~ 2.5%

• PSP ~ 1.5%

• Vascular PDism ~ 3%

• PS due to MPTP, CO, Mn, recurrent head trauma is

extremely rare

• No new cases of postencephalitic parkinsonism since

l960s

Page 47: Movement Disorders - WordPress.com · Movement Disorders Charles Braun, MD IHA Neurology Consultants . Case 1: •73 year old right handed woman presents for initial neurological

Parkinsonism

DDx of primary PD

• Drug-induced (Reglan, neuroleptics)

• Toxin-induced

• Metabolic

• Genetic (Wilson’s Disease)

• Structural lesions (vascular parkinsonism, etc.)

• Hydrocephalus (NPH)

• Infections

• Neurodegenerative

• Progressive Supranuclear Palsy – limitation in vertical gaze, severe postural instability, Hummingbird sign

• Multiple Systems Atrophy – early severe autonomic involvement

• Cortico-basal Degeneration – alien limb phenomenon

• Diffuse Lewy Body Disease – 1. visual hallucinations, 2. motor features of parkinsonism, 3. fluctuating cognition

Page 48: Movement Disorders - WordPress.com · Movement Disorders Charles Braun, MD IHA Neurology Consultants . Case 1: •73 year old right handed woman presents for initial neurological

Parkinsonism

Clues to diagnosis of PD:

• Asymmetric

• Response to levodopa

Non-motor symptoms of PD:

• Depression/Anxiety

• RLS/RBD

• Constipation

• Urinary sx

• Orthostatic hypotension

• Hypophonia

• Dementia

• Impotence

• Psychosis

• Sialorrhea

• Seborrhea

Suggestive of another diagnosis (parkinson’s plus syndrome)

• Lack of response response to dopamine (all)

• Early postural instability (PSP)

• Early onset dementia (PSP, MSA, LBD)

• Limitation in vertical eye movements (PSP)

• Severe autonomic involvement (MSA)

• Alien limb phenomenon (CBD)

• Prominent motor apraxia (CBD, DLBD)

• Visual hallucinations (DLBD)

Page 49: Movement Disorders - WordPress.com · Movement Disorders Charles Braun, MD IHA Neurology Consultants . Case 1: •73 year old right handed woman presents for initial neurological

Parkinson’s Disease

Treatment

• Treat non-motor symptoms

• Monoamine B inhibitors (selegiline 5 BID, rasagiline 1 QD) • Possible neuroprotective, may modify disease course, interacts with TCAs/SSRIs

• Dopamine Agonists (pramipexole, ropinirole) • Side effects of impulsivity risky behavior

• Carbidopa/Levodopa +/- COMT inhibitors (entacapone – inhibits degr) • Ask about “wearing off” and “dyskinesias”

• Amantadine • Help with dyskinesias, tremor, and activating

• Anti-cholinergics (trihexyphenidyl, benztropine) • Good for tremor

• DBS

• PT/SPL/Yoga/Exercise

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Case 1: Parkinson’s Disease

• 73 year old right handed woman presents for initial neurological evaluation for complaint of “right sided weakness”. She describes progressive difficulty over the last 2 years with fine movements in the right hand, problems writing and change in walking.

• On review of systems she endorses chronic constipation and decreased sense of smell/taste.

• Her PCP obtained an MRI C spine which showed moderate cervical stenosis with spondylosis with multilevel neuroforaminal narrowing but no cord compression.

• Her exam shows moderate to severe bradykinesia in the right hand, decreased blink rate, asymmetric right>left rigidity, mild postural instability on pull test. She has no tremor.

Page 51: Movement Disorders - WordPress.com · Movement Disorders Charles Braun, MD IHA Neurology Consultants . Case 1: •73 year old right handed woman presents for initial neurological

Case 2: anti NMDA receptor encephalitis

• 23 year old woman transferred to MICU from psychiatry service after prolonged GTC seizure. She was originally admitted to psychiatry service 2 weeks ago with paranoid delusions and auditory hallucinations for 1 week. She originally endorsed rare cocaine use in college and 1 episode of MDMA use 3 months ago. She was treated with olanzapine and prn haldol. On day 5 of admit she became catatonic for 2 days and was given ativan 1 mg q6h.

• Her basic labs and head CT were unremarkable when she presented originally.

• Her exam shows T: 38C, BP: 144/94, HR: 94, RR: 8, cessation of convulsive activity, no response to verbal commands, diffuse rigidity, occasional bizarre tongue protruding movements and brisk reflexes throughout. Her eyes are open but she does not acknowledge anyone or blink to threat.

Page 52: Movement Disorders - WordPress.com · Movement Disorders Charles Braun, MD IHA Neurology Consultants . Case 1: •73 year old right handed woman presents for initial neurological

Case 3: Progressive Supranuclear

Palsy

• 64 year old man with recent falls. The family describes

cognitive changes with more impulsive behavior and a

change in gait. He fell down stairs and sustained a

subdural hematoma that was evacuated 1 year ago.

• On review of systems, the patient describes difficulty

with aspirating both liquids and solids.

• Exam shows symmetric rigidity, severe postural

instability with exaggerated findings on pull test and

limitation in down gaze.

• MRI brain shows atrophy in midbrain described as

hummingbird sign.

Page 53: Movement Disorders - WordPress.com · Movement Disorders Charles Braun, MD IHA Neurology Consultants . Case 1: •73 year old right handed woman presents for initial neurological

Case 4: Akithisia and

Dystonia from Reglan

• 42 year old obese woman with poorly controlled type II

diabetes x 10 years with gastroparesis and migraine

headaches. She presents to ED with 5 days of intractable

migraine. She is given migraine cocktail that she

typically has responded to in the past which includes IV

toradol, reglan, and valium. 3 hours later she wakes up

from sleep with agitation and tightness in the right side of

her neck.

• Exam shows her agitated, pacing with rigid right sided

cervical paraspinal muscles and trapezius.

Page 54: Movement Disorders - WordPress.com · Movement Disorders Charles Braun, MD IHA Neurology Consultants . Case 1: •73 year old right handed woman presents for initial neurological

Case 5: Multifocal Myoclonus from

polypharmacy (opiates)

• 64 year old veteran male with hepatitis C, alcoholism, cirrhosis, HTN, DM type II and CKD stage 3 admitted for worsening volume overload and abdominal pain for which he has been getting breakthrough oxycodone. For concern of SBP he was started on levaquin. Over the last 12 hours he reports multifocal brief jerks involving hands, arms, and legs, with retained awareness.

• Medication list includes: aspirin, gabapentin, citalopram, insulin, lactulose, amlodipine, lisinopril, morphine

• Exam shows ascites, slowed responses to questions but appropriate, bilateral ptosis, normal strength, bulk and tone, without asterixis.