PD & Drug Therapy Of Basal Ganglia Disorders Flashcards

1
Q

Ballismus

  • Description
  • Pathophysiology
  • Causes
A

A high amplitude flailing of the limbs
Hemiballismus- one side of the body

Pathophysiology

Disruption of the STN
Indirect pathway is not excited so direct pathway is promoted

Cause
Stroke

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2
Q
Chorea 
-Description
-Pathophysiology
-Causes
.
A

Jerky, brief, irregular contractions
non-repetitive, non-rhythmic, flow from one muscle to the next
Fidgety, restless

Pathophysiology
-Disruption in STN, indirect pathway is inhibited, direct pathway is promoted

Causes

  • Huntingdon’s Disease (degenerative)
  • Neuroleptics (antipsychotics)
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3
Q

Tics

  • Description
  • Exacerbating and relieving factors
A
Short, repetitive movements with a premonitory urge.
Simple movements
- blinking, coughing
Complex movements
- jumping or twirling
Plus: motor disorder

Coprolalia (rare): swearing -

Worsened with

  • fatigue
  • anxiety

Reduced by

  • Distractions
  • Concentration
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4
Q

Causes of Tic Disorder

A

Co-morbid conditions
ADHD 50%
OCD 33.3%
Anxiety 50%

Complex genetic inheritance

Post infectious immune

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5
Q

Tourette syndrome

A

more severe expression of a spectrum of tic disorders

multiple motor tics and at least one phonic tic

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6
Q

Huntington’s chorea

-genetics

A

Autosomal dominant with complete penetrance

Trinucleotide repeat on chromosome 4
-longer the repeat sequence the earlier the disease presents

Repeat sequence tends to enlarge with each generation

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7
Q

Huntington’s chorea

-clinical presentation

A

Cognitive

-Inability to make decisions, multitask, slow in thought.

Behavioural

Irritability, depression, apathy, anxiety, delusions.

Physical

Chorea, motor persistence, dystonia, eye movements.

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8
Q

Myoclonus

  • Description
  • Causes
A

Quick, brief, involuntary muscle jerk.
Rapid onset and offset

Positive=muscular contractions

Negative=muscular inhibitions

Causes

  • Juvenile Myoclonic Epilepsy
  • Brain hypoxia
  • Prion disease
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9
Q

Describe the pathophysiology of myoclonus

A

Unknown

1) Possibly an imbalance between excitatory and inhibitory neurotransmitters

(can be treated with antiepileptic drugs)

2) Perturbations of the motor control system leading to a brief disequilibrium

Explain why present at multiple levels e.g cortical, subcortical , spinal etc.

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10
Q

Dystonia

  • Description
  • Causes
A

Abnormal twisting posture
(often axial, facial, truncal, associated with jerky tremor)

Causes

Stroke
Brain injury
Encephalitis
Parkinsons disease
Huntington’s disease
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11
Q

Dystonia

-pathophysiology

A
  • abnormal activity in the motor cortex, supplementary motor areas, cerebellum, basal ganglia
  • Abnormal dopaminergic activity in basal ganglia (dystonia causes by blocking DA receptors, some dystonias are Levodopa responsive)
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12
Q

What is a tremor?

A

Involuntary, rhythmic, sinusoidal alternating movements of part of the body.
in limbs, head, chin, soft palate

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13
Q

When can a tremor occur?

A

At rest

Postural (in a particular body position)

Kinetic (when performing an action)

Most common:
Essential tremor (simple kinetic tremor)
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14
Q

Describe the pathophysiology of a tremor

A

Increased activity in the cerebellothalamocortical circuit.

In PD: Dopamine dysfunction in the pallidum results in this.

In Essential Tremors:
GABAergic dysfunction in the cerebllum causes this.

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15
Q

MRI focussed ultrasound therapy

A

Treatment method for treatment resistant Essential Tremor

-used for PD

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16
Q

What are the drug treatments for tics, chorea, ballismus?

A

Dopamine (D2) receptor blocking agents

Dopamine depleting agents

Atypical anti-psychotics

17
Q

Problems with D2 receptors

A

Acute problems

  • Oculogyric crisis
  • Neuroleptic malignant syndrome

Subacute problems
-Drug induced Parkinsonism

Long term (tardive) dyskinesias

18
Q

Oculogyric crisis

A

Very characteristic acute response to certain drugs

Fixed stare, upward deviation of eyes

Neck and trunk extensions

Jaw spasms +/- tongue protrusion

‘Acute dystonic’ reaction

19
Q

Tetrad of features of Neuroepileptic malignant syndrome

A

1) Fever
2) rigidity
3) autonomic instability
4) confusion

20
Q

Tardive dyskinesia

A

Clinical Manifestations: chorea, athetosis, dystonia, akathisia, stereotyed behaviours, rarely tremor.

21
Q

Hypokinetic movement disorders

-Parkinsonism

A

Symptoms

  • Slowness of movement (also thought/ psychomotor retardation)
  • Stiffness
  • Shaking.

Physical signs include:

  • Slowness and poverty of movement (bradykinesia) e.g. loss of facial expression and arm swing, difficulty with fine movements
  • Voluntary movements harder to initiate (akinesia)
  • Rigidity
  • Rest tremor.
22
Q

What is Parkinson’s Disease?

What is the Histopathological hallmark?

A

A neurodegenerative condition, primarily affecting dopaminergic cells of the substantia nigra

Histopathological hallmark: Lewy bodies and intraneuronal protein inclusion

23
Q

Pathophysiology of PD

A
  • Decreased dopamine input to striatum
  • reduced activation of direct pathway
  • reduced inhibition (higher activity) of indirect pathway
  • reduced movements.
24
Q

Causes of Parkinsonism

A

Neurodegenerative
-PD

Secondary

  • Drugs(haloperidol)
  • Hydrocephalus
  • Cerebrovascular disease
  • Toxicity/ metal deposition disoders

Genetic

  • Metabolic - Wilson’s disease (copper deposition)
  • Rare familial (dominant/ recessive) causes
25
Q

What enzyme breaks down Levodopa to Dopamine?

A

DOPA decarboxylase

26
Q

Treatment for PD [7]

A
L-DOPA
Amantidine 
DA agonist 
COMT inhibitors
Anticholinergics
MAO-IB
Deep Brain Stimulation
27
Q

DA Agonist

-give 2 examples

A

L-DOPA
-Gold standard

-Always combined with Dopa decarboxylase inhibitor to prevent peripheral conversion to DA which then can’t cross BBB

Apomorphine
s/c
reduces dyskinesias

28
Q

COMT inhibitors

  • give 2 examples
  • side effects
A

Entacapone/Tolcapone

-worsens dyskinesias, diarrhoea, liver disease (tolacpone)

29
Q

Anticholinergics

A

helps with tremors and parkinsonian symptoms

30
Q

Amantadine

A

Antiviral

glutamate antagonist

31
Q

MAO-IB

A

treatment of PD

32
Q

Deep Brain Stimulation

A

For PD, tremous, dystonias
implantable device
directly stimulates STN