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Med School > Ataxia > Flashcards

Ataxia Flashcards

1
Q

Role of the Basal Ganglia

A
  • Modulating voluntary motor activity
  • amplitude and direction of movement, body language, decision to move
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2
Q

Role of the Cerebellum
- 3 areas and their roles?

A
  • proprioception, vestibular info/ balance, fine movement, hand-eye coordination, predicts sensory consequences

Spinocerebellum (anterior lobe + vermis) –> extremity synergy

Cerebrocerebellum (posterior lobe) –> topography, eye movement, speech coordination

Vestibulocerebellum (flocculonodular lobe + vermis) –> trunk control

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3
Q

Vestibulocerebellar Loop

A

Paraveramal Area –> Fastigial Nucleus –> Vestibular nuclei on both sides and the reticular formation –> Spinal cord and skeletal muscle

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4
Q

Spinocerebellar Loop

A
  • Stabilize trunk –> EGF/ red nuclei/ reticular formation/ vestibular nuclei
  • Stabilize extremities –> EGF/thalamus/ contralateral motor cortex/ lateral cst
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5
Q

Cerebrocerebellar Loop

A

Info from corticopontine tract –> dentate nucleus –> dentatorubrothalamic tract –> output to sc via lateral cst

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6
Q

Results of lesion to:
- Midline cerebellum (vermis)
- Lateral cerebellum
- Flocculonodular node
- Anterior lobe

A
  • Midline –> issues with gait, trunk balance, head postures, nystagmus, fall with eyes open on Romberg
  • Lateral –> issues with hand-eye coordination, speech, dysmetria and dysdiadokochinesia
  • Flocculonodular –> truncal ataxia, nystagmus, issues with fixation of gaze and smooth pursuit
  • Anterior –> gait ataxia
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7
Q

Direct Pathway vs Indirect Pathway

A

Direct –> stimulation from cortex stimulates the C/P to inhibit GPi which is normally inhibiting the thalamus
- D1 from SN helps with this

Indirect –> stimulation from cortex stimulates the C/P to inhibit GPe via Ach which is normally inhibiting the STh
- now that STh is active it strengthens the inhibition of GPi on the thalamus
- D2 from the thalamus weakens the indirect pathway by inhibting the C/P (why you get extra movements in PD treatment with Levodopa)

*both occur simultaneously
- role is to inhibit competing movements

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8
Q

PD is an extrapyramidal disorder - what does this mean?

A
  • It does not involve UMN/LMN which are involved in GENERATING movement
  • Pyramidal symptoms include spasticity, weakness, hyperreflexia
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9
Q

Definition of PD

A
  • Hypokinetic Disorder
  • Bradykinesia plus one of: Tremor @ rest, rigidity, akinesia (bradykinesia), or postural instability
  • Usually asymmetric at onset
  • Bradykinesia –> slow initiation and progressive decrease in speed/ amplitude w repetitive actions (decrement), micrographia
  • Will see neurodegeneration of the SN with Lewy Bodies
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10
Q

Defintion of Tremor

A
  • involuntray rhythmic oscillation of a body part often around a joint
  • alternating contractions of muscles
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11
Q

Definition of Chorea (+Ballism)

A
  • brief flitting movements, dance-like
  • often multiple body parts
  • ballism –> flinging movements of proximal limbs
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12
Q

Definition of Dystonia

A
  • sustained or intermittent contractions causing abnormal and repetitive movements and postures
  • patterned, tremulous, twisting
  • i.e. Cerebral Palsy
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13
Q

Definition of Myoclonus
Examples?

A
  • brief jerks generated from anywhere along the neural axis
  • i.e. hiccups!
  • hemifacial spasm (facial nerve, could be Bell’s Palsy)
  • Asterixis (negative myocolonus due to liver/ renal failure)
  • ACUTE post-anoxic (generalized, brainstem, poor prognosis)
  • DELAYED post-anoxic (non-progressive, multifocal, cortical, affects gait and speed)
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14
Q

Definition of Tics
Defintion of Tourette’s

A
  • repetitive semi/purposeful movements proceeded by an urge relieved by the action
  • supressible, distractable, suggestable
  • 2+ motor tics (at least one vocal), 1 year duration, onset before 18
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15
Q

Definition of Ataxia

A
  • incoordination of voluntary movements due to cerebellar dysfunction causing irregular timing/ precision (speech, swallowing, gait, eyes, limbs, etc)

-Oscillopsia (moving environment), issues with dexterity/ sitting unsupported/ walking

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16
Q

What are neuroleptics?

A
  • block dopamine receptors
  • used in the treatment of schizophrenia/ psychosis/ BPD
  • can induce tremor/ parkinsonism
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17
Q

Tardive Parkinsonism

Tardive Dyskinesia

Tardive Dystonia

Treatment? If you have to use a neuroleptic, which one should you use?

A
  • persistent parkinsonism (NOT PD) after prolonged neuroleptic withdrawal
  • Dyskinesia –> choreifrom oromandibular movements, ELDERLY
  • Dystonia –> axial dystonia w trunk and neck hyperextension, YOUTH

*dyskinesia and dystonia are often overlapping and caused by chronic neuroleptic exposure (6m)

Tx –> wean off neuroleptic, anticholinergics, tetrabenzine, botox for cervical dystonia, DBS
- clozapine if you must

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18
Q

What are some drugs that can induce parkinsonism/ tremor?

A
  • Antipsychotics –> haloperidol, risperidone, clozapine, metoclopramide
  • Tetrabenzine (DA depleter)
  • Valproic acid, lithium, amiodarone, L-thyroxine
  • Amphetmaines (cocaine)
  • Corticosteroids
  • Caffeine, nicotine
  • SSRIs/ TCAs
  • Alcohol and benzo withdrawal
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19
Q

Acute Dystonic Reaction (ADR)
Tx?

A
  • Occurs days after giving a neuroleptic/ increasing the dose/ switching to IV
  • Prodrome of restlessness, fixed gaze, followed by torticollis/ laryngospasm (stridor)/ oromandibular dystonia/ oculogyric crisis/ opisthotonus (neck and trunk hyperextension)/ retrocollis
  • treat with ABC, benztropine, diphenhydramine, benzodiazepines
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20
Q

Neuroleptic Malignant Syndrome
Tx?

A
  1. Encephalopathy, 2. Rigidity, 3. Hyperthermia,
  2. Dysautonomia
  • high risk for patients w Parkinson’s/ Lewy Body Dementia
  • NMS-like syndrome can occur in PD patients who abruptly stop levodopa/ DA agonists (DO NOT DO THIS)
  • Treat by stopping offending agents (neuroleptics), benzodiazepines
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21
Q

Cause of malignant hypothermia?

A

Use of succinylcholine or halogenated inhaled anesthetics in a genetically predisposed individual

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22
Q

Serotonin Syndrome
- Tx?

A
  • caused by SSRIs/ opioids
  • hyperreflexia, increased tone, tremor, myoclonus
  • Tx is same as NMS –> stop offending agents, benzodiazepines
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23
Q

Describe the course of PD

A
  • Costipation/ REM sleep behaviour disorder
  • One shoulder stiffness, stooped posture, difficult to turn, dragging one leg
  • Bradykinesia/ resting tremor (not everyone!)/ rigidity/ fatigue/ pain
  • Dementia/ urinary issues/ orthostatic hypoTN
  • Dysphagia/ falls
  • Psychosis

*PD more common in males, incidence has increased in higher GDP countries (Canada the most!)

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24
Q

What are protective factors for PD? What are risk factors?

A
  • Exercise
  • Smoking, mediterranean diet (whole grains, nuts, legumes, flavonoids, decreased red meat intake), coffee, vitamin D, NSAIDs, higher urate levels
  • Pesticides, rural farm work, chlorinated solvents, lead, head injuries, increased dairy
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25
Q

What are supportive signs of PD?

A
  • Clear benefit from dopamine
  • On/off fluctuations
  • levodopa induced dyskinesias (chorea)
  • Olfactory loss (anosmia)
  • Cardiac SNS denervation
26
Q

What are red flags when considering PD? What are exclusion signs?

A
  • wheelchair, dysphonia/phagia, autonomic issues, no progression in 5 years
  • stridor and inspiratory sighs
  • Falls in 3 years
  • pyramidal signs
  • symmetric at onset
  • cannot look down, restricted to legs for more than 3 years, no response to Levodopa, agraphesthesia, astereognosis, progressive aphasia, normal neuroimaging of DA system
27
Q

Multiple Systems Atrophy

A
  • progressive, earlier onset than PD
  • Must include autonomic failure –> ED/ urinary incontinence, orthostatic hypoTN
  • Parkinsonism features
  • MSA-P is poorly Levodopa responsive, MSA-C is cerebellar (gait and limb ataxia, dysarthria, stridor)
28
Q

Dementia with Lewy Bodies

A
  • Dementia, visual hallucinations, REM sleep disorder, parkinsonism
  • Severe sensitivity to neuroleptics, falls, syncope, delusions, anxiety and depression, autonomic dysfunction
29
Q

Progressive Supranuclear Palsy

A
  • Surprised look, cannot look down, axial rigidity (early falls), pseudobulbar palsy (dysarthria/phagia), dementia
  • More commin in men 60-70s
30
Q

Corticobasal Degeneration

A
  • Asymmetric rigidity, bradykinesia, dystonia, alien hand, myocolonus, apraxia, dementia, agraphesthesia/astereognosis
  • Often overlaps with PSP
31
Q

Essential Tremor

A
  • bilateral, postural hand tremor
  • often alcohol responsive
32
Q

What is the leading cause of death in PD? Who deals with this?

A
  • aspiration pneumonia
  • speech language pathologists (also deals with swallowing)
33
Q

Non-motor symptoms of PD
What explains this?

A
  • depression/ anxiety/ apathy/ delusions
  • Paresthesias, insomnia, dry eyes, diplopia, weight loss
  • Constipation (lewy bodies in gut, decreases absorption of Levodopa)
  • Psychosis, dementia

*psychosis is mainly visual hallucinations and delusions

  • deposition of alpha-synuclein in different areas of the brain
34
Q

Resting vs Action tremor

A

Resting - supported against gravity (including dangling while walking)
- should subside with action, almost exclusive to PD

Action - either postural (maintaining limb against gravity, physiological) or kinetic (which includes intention tremor - at end of goal-directed movement)
- intention tremor is suggestive of cerebellar disease

35
Q

Physiologic Tremor

A
  • normal phenomenon enhanced by certain conditions, not associated with an underlying condition
  • stress, fatigue, drugs, hypoglycemia, hyperthyroid, etc.
  • postural, normally upper limbs
  • high frequency (8-12) and fine amplitude
36
Q

Cerebellar Tremor

A
  • Due to underlying cerebellar disease - presents with ataxia
  • Includes intention tremor
  • Can be caused by MS, posterior stroke, alcoholic degeneration
  • Low frequency (3-5)
37
Q

Essential Tremor
Treatment?

A
  • Common in older, family Hx
  • Postural and kinetic, commonly symmetric in the upper extremities
  • Starts high frequency and ends up low frequency with higher amplitude

*If it is an isolated head tremor that is NOT essential tremor. That is cervical dystonia.

1st Line –> propranolol (NO if asthma, hypotn, bradycardia) , primidone
2nd Line –> Gabapentin, clonazepam, topiramate
3rd Line –> DBS

38
Q

Idiopathic cervical and Idiofocal upper extremity dystonia - tx?

Dopa-responsive dystonia

A
  • both see normal brain and C-spine imagery
  • head tremor vs interference with writing
  • both treat with botox injections
  • genetic, childhood onset, lower extremities and worse in the evening
39
Q

Huntington’s
Imaging signs?

A
  • cause of Chorea
  • dementia, psych disturbances
  • autosomal dominant, CAG repeats (more severe with more repeats)
  • initally affects the indirect pathway more
  • see atrophy of caudate head, loss of neurons, reactive astrocytes and microglia, and intranuclear inclusions (ubiquitin) which represents abnormal huntingtin aggregates
40
Q

What are some causes of Chorea?

A
  • Huntington’s
  • Stroke (contralateral to the lesion, normally in the STh)
  • Levodopa induced
  • Sydenham (autoimmune, delayed onset following group A strep pharyngitis)
  • SLE, antiphospholipid syndrome
41
Q

What causes the motor symptoms of PD?

A
  • loss of dopaminergic projections from the Substantia Nigra Pars Compacta to the striatum
42
Q

Levodopa

A
  • prodrug of dopamine, coverted by dopa-decarboxylase present in the CNS and PNS
  • causes nausea and vomiting in the PNS so taken with Carbidopa (sinemet) to inhibit dopa-decarboxylase
  • Most potent treatment but highest risk of motor fluctuations
43
Q

Dopamine Agonists
- S/E?

A
  • Moderate benefit, less motor fluctuations (but more fatigue, nausea, ortho, hallucinations)
  • May cause impulse control disorders (hypersexuality, gambling)
  • i.e. Bromocriptine, pramipexole
  • also Amantadine –> mild benefit, mostly used for dyskinesias (constipation, ankle edema, livedo reticularis)
44
Q

MAO-B Inhibitors
- S/E?

A
  • inhibit metabolism of DA by MAO-B
  • weak benefit, used to extend duration of levodopa
  • i.e Selegiline
  • nausea, dizziness, insomnia, maybe serotonin syndrome if on lots of SSRIs
45
Q

COMT Inhibitors
- S/E?

A
  • inhibit metabolism of DA by COMT
  • must be used with levodopa, extends its duration
  • i.e Entacapone
  • Orange urine
46
Q

What classifies wearing off? How to fix?

A
  • under 4 hour duration of an adequate does of LD
  • increased dose, decrease dose intervals, add MAO-B COMT inhibitors or DA agonists, long acting or continuous dose LD (intestinal gel via PEG-J), DBS
47
Q

What classifies as delayed kicking in? Why does it happen? How to fix?

A
  • takes over 30 minutes for LD to work, more common in morning dose
  • delayed gastric emptying, constipation, poor absorption due to protein
  • manage constipation, space 30 minutes from protein, crush/chew, long acting LD, take meds w carbonated drink, MAO-B inhibitor
48
Q

What is off vs on?

A

Off –> medication has worn off and symptoms come back
- common in the AM, leg cramps/ toe-curling/ foot inversion

49
Q

What are the two types of dyskinesia onset with DA drugs? How to fix?

A
  • Peak dose –> onset at peak of anti-PD effects of meds
  • Diphasic (DID) –> minutes after dose or several hours later
  • use lowest effective LD dose, if its peak dose use smaller more frequent doses, clozapine, amantadine
50
Q

Freezing of Gait - Tx?

A
  • more frequent when off
  • LD intestinal gel, physio, sensory tricks
  • acetylcholinesterase inhibitors/ gait aids to prevent falls
51
Q

How does DBS work? S/E?

A
  • Stimulates the GPi and STh –> Mimics the on state, but does not treat LD unresponsive symptoms
  • generally for younger cognitively intact patients
  • Stroke, sensory and cognitive disturbances, personality changes
52
Q

Which atypical neuroleptics can you give to prevent non-motor fluctuations such as psychosis?

A

Quietapine, clozapine

53
Q

What is the role of a social worker in PD?

A
  • advance care planning, power of attorney, long term care
  • support groups, employment
54
Q

Scanning dysarthria

A
  • issues with articulation, prosody, volume
  • both cerebellar hemispheres involved
55
Q

Different inabilities to stabilize gaze? Broken saccades?

A
  • square wave jerks (small saccade away and back), ocular flutter (back to back horizontal), opsoclonus (saccadomania)
  • hypermetric (overshoot), hypometric (have to catch up), nystagmus
  • impaired VOR cancellation (cannot stabilize gaze while spinning)
56
Q

Causes of ataxia

A
  • Drugs –> alcohol, lithium, metronidazole, tacrolimus, amiodarine
  • vitamin B12 and E and thiamine deficiency
  • cancer, paraneoplastic (pancerebellar targeting purkinjes)
  • infections, ischemia, congenital, etc.
57
Q

Alcoholic cerebellar degeneration

A
  • direct toxin, causes thiamine deficiencies
  • anterior midline (vermis) structures are most affected
  • see midline cerebellar atrophy on MRI, as well as increased MCV and AST
58
Q

SCA and EA-1/2

A

Autosomal dominant causes of ataxia

Spinocerebllar ataxia –> CAG repeats

Episodic Ataxia 1 –> seconds to minutes of trunk/limb/ ataxia, normal inbetween and not-progressive

Episodic Ataxia 2 –> hours of N/V, vertigo, progressive inbetween and treated with acetazolamide

59
Q

Autosomal Recessive Causes of Ataxia

A

Friedrich’s –> GAA repeat, dorsal column loss, atrophy of cervical cord, cardiac issues common

Isolated Vitamin E Deficiency –> Purkinje cells are sensitive, cord atrophy (like Friedrich’s)

Abetalipoprotinemia –> decreased lipid absorption leads to decreased vitamins ADEK

Cerebrotendinous Xanthomatosis –> cataracts in youth, spatiscity, distal neuropathy
tx –> chenodeoxycholic acid

60
Q

Signs of cerebellar vs peripheral nerves vs spinal cord ataxia?

A

Cerebellar –> walking like drunk
Peripheral nerves –> worsens in the dark, decreased ankle jerks, stocking sensory loss
Spinal –> same as peripheral plus wide gait and weakness

61
Q

Lewy Bodies

A
  • characteristic of PD
  • made of alpha-synuclein which accumulates abnormally in neuronal bodies, dendrites, cortex
62
Q

Topgraphy of cereblleum

A
  • Lateral –> extremities
  • Intermediate –> distal limb
  • Midline/ vermis –> proximal limb and trunk
  • Flocculonodular –> balance, and vestibulo-ocular reflexes

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