Clinical Syndromes of Basal Ganglia and their Drug Treatment

How Do We Move?

Brain Movement Program- Recap

How do we Describe Movement?

Language Related to Pathology in the Basal Ganglia- Phenotypical Description

Phenotypical description (axis 1)

• Describe the movements (previous slide)

• Which parts of the body are affected

• Are there non-movement features?

Language Related to Pathology in the Basal Ganglia- Aetiological Description

Aetiological description (axis 2)

• Blood tests

• Brain imaging

• Genetics

Hypokinetic Movement Disorders- parkinsonism

Hypokinetic Movement Disorders- Axis 1- parkinsonian Symptoms

Hypokinetic Movement Disorders- Axis 1- parkinsonian Signs

Hypokinetic Movement Disorders- Axis 2- Aetiology

How does Dopamine Cause Parkinson’s?

Hypokinetic Movement Disorders- Treatment

Hypokinetic Movement Disorders- Treatment- Levodopa Adjuncts

Hypokinetic Movement Disorders- Treatment- Dopamine Agonists

Different formulations: tablets, patches, infusions
Pros: long acting or quick acting, effective, also treat Restless Leg Syndrome

Cons: Risk of  impulse control disorders / behaviours

Hypokinetic Movement Disorders- Treatment- MAOB-inhibition

Hypokinetic Movement Disorders- Treatment- COMT-Inhibition

Hypokinetic Movement Disorders- Treatment- Non-tablet Therapies

Hypokinetic Movement Disorders- Treatment- Deep Brain Stimulation

Hyperkinetic Movement Disorders- Tremor

Hyperkinetic Movement Disorders- Tremor Pathophysiology

Hyperkinetic Movement Disorders- Tremor Treatment

Hyperkinetic Movement Disorders- Chorea- Definition

1. Involuntary, continuous flow of brief, non-rhythmical and unpredictable movements, which appear to move in a stream from one body part to another.

2. Can be described as “dance-like” due to the fluidity and variability in direction, amplitude and timing. 

3. Patient appears fidgety, restless, when asked will struggle to suppress the movements.

Hyperkinetic Movement Disorders- Chorea- Causes

Hyperkinetic Movement Disorders- Chorea- Huntington’s Disease

Hyperkinetic Movement Disorders- Chorea- Huntington’s Disease Presentation

1. Cognitive: impaired decision making, slowed thinking

2. Behavioural: Irritability, depression, apathy, anxiety, delusions

3. Physical: Chorea, motor persistence, eye movement abnormalities

Hyperkinetic Movement Disorders- Treatments

Hyperkinetic Movement Disorders- Dystonia Definition

1. Abnormal twisting posture:

• axial/ facial/ truncal / global

• may be associated with jerky tremor

• Due to overactivity of muscles

Hyperkinetic Movement Disorders- Dystonia Pathophysiology

Hyperkinetic Movement Disorders- Dystonia Causes

• Stroke

• Brain injury

• Parkinson’s disease

• Huntington’s disease

• Wilson’s disease

• Genetic

• Idiopathic

Hyperkinetic Movement Disorders- Dystonia Treatment

Hyperkinetic Movement Disorders- Tics Definition

Hyperkinetic Movement Disorders- Tics and Co-occurrence

Hyperkinetic Movement Disorders- Tics Treatments

1. Main stay of treatment in through neuro-behavioural approaches, such as CBT

2. Some people with Tics access pharmacological treatments through specialist clinics, such as clonidine

3. Deep brain stimulation is undertaken in some parts of the world, but is not licenced for tics in the UK

Hyperkinetic Movement Disorders- Drugs Causing Movement Disorders

What is Oculogyric Crisis?

Characteristic acute response to certain drugs

• Fixed stare, upward deviation of eyes

• Neck extension

• Trunk extension

• Jaw spasms +/- tongue protrusion

• ‘Acute dystonic’ reaction

What is Neuroleptic Malignant Syndrome?

What is Tardive Dyskinesia?

1. Chorea-like oral-facial movements

2. dystonic trunk posturing

3. Exact mechanism unclear – likely dopamine sensitivity of basal ganglia –i.e. secondary receptor/ plastic changes

What are the Treatments for Tardive Dyskinesia?

Treatment

• gradual withdrawal of offending agent

• use of a dopamine depleting agent (tetrabenazine)

• use of a benzodiazepine (clonazepam) if distressing