Movement Disorders

Question 0

To diagnose PD

Answer 0

Requires two of –> slowness, tremor, stiffness
Must respond to dopamine
Must check for–> neuroleptics, disorders of eye movement, signs of early onset dementia
If early onset check copper and genetics

Question 1

Movement disorders include

Answer 1

Disorders of abnormal movements –> myoclonus, chorea, tics, tremors (dyskinesia)
Disorders of lack of movement –> Parkinsonism (dystonia)

Question 2

Parkinson’s disease (PD)

Answer 2

A degenerative disorder of the motor system due to death of dopamine cells in the substantial nigra
Characterised by the accumulation of alpha-synclein in inclusions called Lewy bodies

Question 3

Symptoms of PD

Answer 3

Initial symptoms are a slowness, tremor and rigidity with difficulty walking and gait
This develops into cognitive and behavioural problems often leading to dementia and depression

Question 4

Epidemiology of PD

Answer 4

Prevalence –> 1.6/1000
Incidence –> 1/100 at 70
Increasing risk with age

Question 5

Clinical signs of PD

Answer 5

Reduced movement (tapping speed, walking, ADL)
Lead-pipe rigidity
Resting tremor
Fixed posture without arm swinging
Biological signs are loss of SN pigment and Lewy bodies

Question 6

Aetiology of PD

Answer 6

Multiple identified genes, both dominant and recessive

In most cases age is only risk factor

Question 7

Prognosis of PD

Answer 7

Progressive, significant mortality –> on average 10 good years
slower progression in early onset
Motor symptoms fluctuate
Significant non-motor problems, most important is psychiatric
Depression most common comorbidity

Question 8

PD treatment

Answer 8

DOPA -+ decarboxylase inhibitor (Co-careldopa/-benyldopa)
Can also use DA agonists (ropinirole, pramipexole, rotigotine patch, apomorphine infusion)
Others –> MAOi (rasagiline) or COMTi (entacapone)
Late stage –> Dudopa infusion or DBS

Question 9

Parkinson’s plus diseases

Answer 9

Primary Parkinsonisms with additional features. They generally carry a worse prognosis
Multiple system atrophy (MSA)
Progressive supraventricular palsy (PSP)
Dementia with Lewy bodies

Question 10

Multiple systems atrophy (MSA)

Answer 10

Primary Parkinsonisms with additional features
No dopamine response
Autonomic and cerebellar features
‘Hot cross bun’ appearance on MRI

Question 11

Progressive supraventricular palsy (PSP)

Answer 11

Primary Parkinsonisms with additional features
Early onset balance problems
Upper Eye movement disorder
Perseveration (applause sign)

Question 12

Tremor

Answer 12

Most often essential and non-pathological
May be thyroid or familial
In most cases only requires symptomatic treatment

Question 13

Causes of dyskinesia

Answer 13

Neuroleptic drugs –> tardative dyskinesia
Huntington’s disease –> chorea
Cerebral palsy –> athetosis

Question 14

Huntington’s disease

Answer 14

Chr 4 CAG repeats
More than 35 repeats causes full disease
Disease shows complex inheritance, with increasing severity and early onset across generations
Treat with counselling and suppression of chorea by neuroleptics

Question 15

Dystonia

Answer 15

Can be focal,generalised or task specific (musicians or writer’s block)
Mostly idiopathic or psychological. Can be caused by Brain trauma or rare diseases

Question 16

Treatments of non-Parkinson’s dystonia

Answer 16

If minor therapy, reassurance or task modification
Can use Botox or drugs (neuroleptics, benzos, baclofen, trihexyphenidyl)
If generalised and severe can use DBS

Question 17

Additional movements

Answer 17

Choreaiform movements (huntingtons’s or neuroleptics)
Myoclonus –>involuntary muscle twitches.may be pathological, epileptic or essential
Tics –> often innocent in childhood. Vocal tics suggest Tourette’s
Hemifacial spasm can be treated with Botox