DSA32 - Movement Disorders

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43 Terms

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-Substantia Nigra

-Subthalamic Nucleus

-Striatum

-Thalamus

-Connects of system with the CST

The basal ganglia component of the Extrapyramidal Motor System consists of:

<p>The basal ganglia component of the Extrapyramidal Motor System consists of:</p>
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-Dopamine

-Glutamate

-GABA

-ACh

What are the major neurotransmitters of the Extrapyramidal Motor System?

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Abnormalities of muscle tone abnormal body (tremors)

Disturbance of Extrapyramidal Motor System leads to what?

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regular oscillation around a plane in space; can be physiologic or pathologic

Define Tremor

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Physiologic tremors PERSIST during sleep (largely ballisto-cardiographic effect), but pathologic tremors DISAPPEAR during sleep (due to disease state)

What is the difference between physiologic tremors and pathologic tremors?

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Postural Tremor ("Action/Essential Tremor")

Define Condition:

Most common movement disorder (ET); affects males & females equally; All ages, but more with older age; May be due to dysfunction of the purkinje cells in cerebellar cortex/projections to deep cerebellar nuclei

-Hx: Usually around 65 y/o; +FHx (Autosomal Dominant)

-Sx: BILAT & SYMM at ONSET; Starts in UE, but can affect all even head and neck (titubation) or jaw - increased by stress, fatigue or caffeine

-PE: Worse when limbs outstretched/maintaining posture, attempting delicate movement; may stop with complete relaxation (ex: reclining supine) --> (-)Archimedes Spiral w/o touching paper

-Dx: Normal Imaging

-Tx: Propanolol --> Alprazolam --> More intense like barbituate (Primidone), Botox, GABA)

<p>Define Condition:</p><p>Most common movement disorder (ET); affects males &amp; females equally; All ages, but more with older age; May be due to dysfunction of the purkinje cells in cerebellar cortex/projections to deep cerebellar nuclei</p><p>-Hx: Usually around 65 y/o; +FHx (Autosomal Dominant)</p><p>-Sx: BILAT &amp; SYMM at ONSET; Starts in UE, but can affect all even head and neck (titubation) or jaw - increased by stress, fatigue or caffeine</p><p>-PE: Worse when limbs outstretched/maintaining posture, attempting delicate movement; may stop with complete relaxation (ex: reclining supine) --&gt; (-)Archimedes Spiral w/o touching paper</p><p>-Dx: Normal Imaging</p><p>-Tx: Propanolol --&gt; Alprazolam --&gt; More intense like barbituate (Primidone), Botox, GABA)</p>
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Senile Tremor

When a Postural Tremor occurs in the elderly, it is known as a ()

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Familial Tremor

When a Postural Tremor occurs in families, it is known as a ()

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Benign Essential Tremor (ET)

When a Postural Tremor occurs in neither families nor is it elderly, it is known as a ()

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B/c ET typically does not produce an ataxic gait like PD does

Why should gait (walking) and station (standing) ALWAYS be checked with ETs?

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Resting Tremor (a/w Parkinsons)

Define Condition:

Loss of the pigmented neurons of the parscompacta of the substantia nigra in the midbrain --> overactivity of local-circuit, cholinergic neurons

-Hx: Older adults

-Sx: Usually begins as ASYMMETRIC; Preceded by slowness (bradykinesia) and stiffness (rigidity in FLEXION), Possible Depression/REM sleep disorder/Anosmia

-PE: (+)"Pill Rolling", Decrease with action/increase at rest

-Dx: Normal Imaging, except DaT scan (shows abnormal dopamine uptake)

-Tx: Carbidopa /Levodopa (Sinemet), Dopamine Agonists, MAOi, Anticholinergics +/- Pallidotomy +/- Thalamotomy +/- Deep Brain Stimulation (DBS)

<p>Define Condition:</p><p>Loss of the pigmented neurons of the parscompacta of the substantia nigra in the midbrain --&gt; overactivity of local-circuit, cholinergic neurons</p><p>-Hx: Older adults</p><p>-Sx: Usually begins as ASYMMETRIC; Preceded by slowness (bradykinesia) and stiffness (rigidity in FLEXION), Possible Depression/REM sleep disorder/Anosmia</p><p>-PE: (+)"Pill Rolling", Decrease with action/increase at rest</p><p>-Dx: Normal Imaging, except DaT scan (shows abnormal dopamine uptake)</p><p>-Tx: Carbidopa /Levodopa (Sinemet), Dopamine Agonists, MAOi, Anticholinergics +/- Pallidotomy +/- Thalamotomy +/- Deep Brain Stimulation (DBS)</p>
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Neurodegenerative disorder primarily caused by degeneration of the substantia nigra in the basal ganglia --> Not enough Dopamine to suppress inhibition → overactivity in inhibitory functions = lower ability to initiate movement

Define Parkinson's Disease

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Eosinophilic intracytoplasmic inclusion body with a dense core consisting of a-synuclein protein; found in the cytoplasm of nigrostriatal neurons in people with Parkinson's disease

Define Lewy Bodies

<p>Define Lewy Bodies</p>
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-Resting Tremor

-Bradykinesia

-Rigidity in FLEXION

-Postural Disequilibrium

What are the 4 Cardinal Signs of Parkinson's? (dx is based on 2/4 of these signs)

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Involves the coordination of movement strategies to stabilize the centre of body mass during both self-initiated and externally triggered disturbances of stability (ex: difficulty getting out of low chair)

Define Postural Disequilibrium

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Antidopaminergic Drugs (antipsychotic drugs and anti-emetics such asmetoclopramide and compazine)

DRUG INDUCED Parkinsonism is a common S/E for what kind of drugs?

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Progressive Supranuclear Palsy/PSP

Define Condition:

*Mistaken as PD*

-Hx: Usually > 60 y/o; Gradual onset

-Sx: Gait instability/Falls, Disturbance of speech articulation and swallowing, Impairment of eye movements (issues seeing)

-PE: Parkinsonism w/o tremor, Rigidity in EXTENSION Increased AXIAL muscle tone, Unstable gait/falls, dysarthric speech, dysphagia, Impaired eye movements (Downward vertical, conjugate ophthalmoplegia with intact VOR = “supranuclear lesions”), Intellect preserved until very late

-Dx: Brain MRI (r/o other disorders but may show classic midbrain atrophy, "hummingbird sign")

-Tx: Supportive Tx --> Full Care

<p>Define Condition:</p><p>*Mistaken as PD*</p><p>-Hx: Usually &gt; 60 y/o; Gradual onset</p><p>-Sx: Gait instability/Falls, Disturbance of speech articulation and swallowing, Impairment of eye movements (issues seeing)</p><p>-PE: Parkinsonism w/o tremor, Rigidity in EXTENSION Increased AXIAL muscle tone, Unstable gait/falls, dysarthric speech, dysphagia, Impaired eye movements (Downward vertical, conjugate ophthalmoplegia with intact VOR = “supranuclear lesions”), Intellect preserved until very late</p><p>-Dx: Brain MRI (r/o other disorders but may show classic midbrain atrophy, "hummingbird sign")</p><p>-Tx: Supportive Tx --&gt; Full Care</p>
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Intention Tremor

Define Condition:

Occurs with attempt at purposeful movement; due to disturbances of the cerebellum or connections to the cerebellum

-Hx: May have FHx of similar issues

-Sx: Occurs with balance issues, gait problems, and speech/eye issues (*If lesions are FOCAL = Sx are ipsilateral*)

-PE: Worsens as target is reached (increases in amplitude/frequency) --> disappears at rest; May see cerebellar dysfunction (limb ataxia, dysmetria, dysarthria, nystagmus, hypotonia, etc)

-Dx: MRI (Cerebellar Lesions or Atrophy)

-Tx: Depends on etiology; MUST prevent injuries from ataxia; DBS may help if severe

<p>Define Condition:</p><p>Occurs with attempt at purposeful movement; due to disturbances of the cerebellum or connections to the cerebellum</p><p>-Hx: May have FHx of similar issues</p><p>-Sx: Occurs with balance issues, gait problems, and speech/eye issues (*If lesions are FOCAL = Sx are ipsilateral*)</p><p>-PE: Worsens as target is reached (increases in amplitude/frequency) --&gt; disappears at rest; May see cerebellar dysfunction (limb ataxia, dysmetria, dysarthria, nystagmus, hypotonia, etc)</p><p>-Dx: MRI (Cerebellar Lesions or Atrophy)</p><p>-Tx: Depends on etiology; MUST prevent injuries from ataxia; DBS may help if severe</p>
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postural

If the neuro exam is normal besides the tremor, it is likely a(n) (postural/resting/intention) tremor

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Resting/Parkinsonian

If the neuro exam shows rigidity, slowness of movement, and/or postural disequilibrium, it is likely a(n) (postural/resting/intention) tremor

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Intention

If the neuro exam shows ataxia, it is likely a(n) (postural/resting/intention) tremor

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Guide center for movement - provides for coordinated, fluid and precise movement

What is the function of the Cerebellum?

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-Visual System

-Vestibular System

-Proprioceptive system

From where does the Cerebellum RECEIVE input?

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Pyramidal motor system (CST)

Where does Cerebellar input go to?

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Cerebellar Ataxia

Define Condition:

-Hx: Child/adult onset, ACUTE = trauma/stroke, SUBACUTE = Intoxication/Abscess, CHRONIC = hereditary/degenerative/Neoplastic; May have neg FHx if recessive

-Sx/PE (ispilat to lesion): Limb/Gait ataxia (looks drunk), Dysmetria (past pointing), Dysarthria, Nystagmus, Scanning Speech, Hypotonia, Intention Tremor = Dysdiadochokinesia (Ex: can't quickly pronate/supinate)

-Dx: MRI WWOC (to view posterior fossa/cerebellum)

-Tx: Genetic counseling if hereditary, Surgery for hemorrhage/neoplasm, Tx if MS, Rehab if traumatic

<p>Define Condition:</p><p>-Hx: Child/adult onset, ACUTE = trauma/stroke, SUBACUTE = Intoxication/Abscess, CHRONIC = hereditary/degenerative/Neoplastic; May have neg FHx if recessive</p><p>-Sx/PE (ispilat to lesion): Limb/Gait ataxia (looks drunk), Dysmetria (past pointing), Dysarthria, Nystagmus, Scanning Speech, Hypotonia, Intention Tremor = Dysdiadochokinesia (Ex: can't quickly pronate/supinate)</p><p>-Dx: MRI WWOC (to view posterior fossa/cerebellum)</p><p>-Tx: Genetic counseling if hereditary, Surgery for hemorrhage/neoplasm, Tx if MS, Rehab if traumatic</p>
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When Speech is "slowed" or "staccato"

Define Scanning Speech

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Define Scanning Speech

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Spinocerebellar Ataxia/SCA

Define Condition:

-Hx: Rarely purely spinal; +FHx (ex: Machado-Joseph’sDisease = AD/14q, Friedreich’s Ataxia = ar/9q)

-Sx/PE (*IF SPINAL, no dysarth/nystag): +/- Nystagmus & Dysarthric Speech, Gait ataxia (looks drunk), Intention Tremor = Dysdiadochokinesia (Ex: can't quickly pronate/supinate), Dysmetria (past pointing), Hypotonia, Dysdiadochokinesia (Ex: can't quickly pronate/supinate) + SC sensory and motor disturbances

-Dx: MRI (may show spinal and/or cerebellar degeneration/atrophy, or white matter changes)

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Friedreich's Ataxia

Define Condition:

MOST COMMON SCA in YOUNG PEOPLE; Due to Autosomal recessive trinucleotide repeat disorder (GAA, frataxin gene). Leads to impairment in mitochondrial functioning & destruction of myelin in Spinal Cord.

-Sx/PE: Ataxia/frequent falling, nystagmus, dysarthria, pes cavus, hammer toes, hypertrophic cardiomyopathy (Cause of death). Presents in childhood with kyphoscoliosis.

<p>Define Condition:</p><p>MOST COMMON SCA in YOUNG PEOPLE; Due to Autosomal recessive trinucleotide repeat disorder (GAA, frataxin gene). Leads to impairment in mitochondrial functioning &amp; destruction of myelin in Spinal Cord. </p><p>-Sx/PE: Ataxia/frequent falling, nystagmus, dysarthria, pes cavus, hammer toes, hypertrophic cardiomyopathy (Cause of death). Presents in childhood with kyphoscoliosis.</p>
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Sensory Ataxia

Define Condition:

-Hx: Subacute or Chronic, +/- FHx; +/- Hx Alcoholism, STD (Tabes Dorsalis), Peripheral Neuropathy

-Sx: Numbness, Tingling, Pain in feet, frequent tripping/stumbling on irregular surfaces

-PE: Ataxia WORSE in dark (loss of proprioception --> balance depends on sight), Sensory deficit in LE (feet), Decreased vibratory/joint position in feet, (+) ROMBERG, Argyll-Robertson Pupils (Tabes Dorsalis), ABSENT RELFEXES

-Dx: Labs for neuropathy (DM, Hypothyroid, B12, +HIV, +RPR), EMG/NCV (neuropathic), Nerve biopsy for etiology

-Tx: Depends on etiology

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When pupils reactive to accommodation but not to light, associated with tabes dorsalis

Define Argyll-Robertson Pupils

<p>Define Argyll-Robertson Pupils</p>
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Huntington's Chorea/Disease

Define Condition:

Trinucleotide repeat produces a “toxic gain of function,” affectingthe protein “Huntingtin,” on Chromosome 4p.

-Hx: +FHx of early-onset dementia w/ dyskinesias due to AD of high penetrance; Onset in 40s-50s (but issues in childhood)

-Sx: Gradual onset of "fidgetiness" (choreiform ataxia) --> jerky movements, Personality changes

-PE: Jerks becoming incapacitating + personality changes

-Dx: Brain MRI (atrophy of the caudate nuclei + large ant horns of ventricles) + Genetic Testing (CAG trinucleotide repeats > 38)

-Tx: Tx Chorea with Tetrabenazine (deplete monoaminergic synaptic vesicles, thus resetting a balance between dopamine/NE and ACh)

<p>Define Condition:</p><p>Trinucleotide repeat produces a “toxic gain of function,” affectingthe protein “Huntingtin,” on Chromosome 4p.</p><p>-Hx: +FHx of early-onset dementia w/ dyskinesias due to AD of high penetrance; Onset in 40s-50s (but issues in childhood)</p><p>-Sx: Gradual onset of "fidgetiness" (choreiform ataxia) --&gt; jerky movements, Personality changes</p><p>-PE: Jerks becoming incapacitating + personality changes</p><p>-Dx: Brain MRI (atrophy of the caudate nuclei + large ant horns of ventricles) + Genetic Testing (CAG trinucleotide repeats &gt; 38)</p><p>-Tx: Tx Chorea with Tetrabenazine (deplete monoaminergic synaptic vesicles, thus resetting a balance between dopamine/NE and ACh)</p>
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Normal Pressure Hydrocephalus/NPH

Define Condition:

-Hx: Adult onset (45-75 y/o), +/- Hx of meningitis/SDH/TBI affecting CSF reabsorption

-Sx: Urinary incontinence, Gait Ataxia (falls), cognitive decline (dementia)

-PE: Frontal Lobe ataxia (magnetic gait - "stuck to the floor"), LE hypertonia, NO PAPILLEDEMA (*think "wet, wacky, wobbly"*)

-Dx: CT WOC (ENLARGED LATERAL VENTRICLES but NO ATROPHY)/Brain MRI (communicating hydrocephalus WITHOUT atrophy - VENTRICULAR ENLARGEMENT) + LP (normal, or 5-20 cm H2O)

-Tx: VPS/ventriculo-peritoneal shunt within 2 yrs of onset

<p>Define Condition:</p><p>-Hx: Adult onset (45-75 y/o), +/- Hx of meningitis/SDH/TBI affecting CSF reabsorption</p><p>-Sx: Urinary incontinence, Gait Ataxia (falls), cognitive decline (dementia)</p><p>-PE: Frontal Lobe ataxia (magnetic gait - "stuck to the floor"), LE hypertonia, NO PAPILLEDEMA (*think "wet, wacky, wobbly"*)</p><p>-Dx: CT WOC (ENLARGED LATERAL VENTRICLES but NO ATROPHY)/Brain MRI (communicating hydrocephalus WITHOUT atrophy - VENTRICULAR ENLARGEMENT) + LP (normal, or 5-20 cm H2O)</p><p>-Tx: VPS/ventriculo-peritoneal shunt within 2 yrs of onset</p>
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These conditions can scar the arachnoid granulations that allow for egress of CSF

Why might Ataxia due to NPH be more common in patients who have a prior history of head trauma, meningitis, or subarachnoid hemorrhage?

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Ataxia = INCOORDINATED movements; Dyskinesia = ABNORMAL movements

Distinguish Ataxia vs Dyskinesia

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Dyskinesia

Define Condition:

Abnormal movement that is not tremors or chorea; often irreversible - focal, asynchronous muscle movements which may occur in any muscle groups but are particularly common in the oro-facial-lingual and limb muscles

-Hx: ACUTE with drug reactions, but usually Subacute or Gradual; Hx of Anti-Dopa or Antipsych/Anti-emetics

-Dx: Normal

-Tx: STOP any drug implicated (if it can be safely stopped), Sometimes Botulinum Toxin

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discontinuation

Acute iatrogenic dyskinesias begin shortly after beginning or increasing the causative agent --> will cease with () of agent

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Tardive

() dyskinesias are usually seen in patients taking certain drugs chronically

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True

T/F - Phenothiazine can induce Extrapyramidal Symptoms like dyskinesia or dystonia

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Basal Ganglia

What lesions typically cause Involuntary Dyskinesia (tremors when pt isn't doing anything)?

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Cerebellar

What lesions typically cause Voluntary Dyskinesia (tremors ONLY when using body/limbs)?

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Dystonia

Define Condition:

Abnormal movement that is not tremors or chorea; often irreversible - prolonged muscle contractions more like increased muscle tone in specific muscle groups

-Hx: ACUTE with drug reactions, but usually Subacute or Gradual; Hx of Anti-Dopa or Antipsych/Anti-emetics

-Sx/PE: Cervical (torticollis, aka "wry neck"), Generalized ("Dystonia musculorum deformans"), Focal Limb ("writer's cramp")

-Dx: Normal - EXCEPT in Wilson's Disease

-Tx: STOP any drug implicated (if it can be safely stopped), Anticholinergic Drugs (ex: Benadryl), Benzodiazepines, Sometimes Botulinum Toxin

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Wilson's Disease

Define Condition:

Hepatolenticular degeneration due to genetic defect, “ar” on Chromosome 13 causing abnormal metabolism and deposition of copper

-Hx: +FHx, Young Onset

-Sx/PE: Unexplained dyskinesia/tremor (typically produces dystonic tremors); deposition of copper in brain, liver (cirrhosis) & eye (kaiser-flesher ring)

-Dx: Labs for 24 hr copper UA (high) and serum ceruloplasmin (low) + Liver Biopsy

-Tx: Chelation Therapy

<p>Define Condition:</p><p>Hepatolenticular degeneration due to genetic defect, “ar” on Chromosome 13 causing abnormal metabolism and deposition of copper</p><p>-Hx: +FHx, Young Onset</p><p>-Sx/PE: Unexplained dyskinesia/tremor (typically produces dystonic tremors); deposition of copper in brain, liver (cirrhosis) &amp; eye (kaiser-flesher ring)</p><p>-Dx: Labs for 24 hr copper UA (high) and serum ceruloplasmin (low) + Liver Biopsy</p><p>-Tx: Chelation Therapy</p>