Tremor, Dystonia, Chorea

Question 1

Tremor

Answer 1

Involuntary sinusoidal oscillation of a body part. Usually due to alternate activation of agonist and antagonist muscles.

Question 2

Tics

Answer 2

Involuntary stereotyped movements or vocalisations.

Can be simple or complex.

Question 3

Primary Tic Disorders

Answer 3

Start in childhood.

Adult onset is rare, and is usually due to a secondary cause.

Question 4

Tourette Syndrome (Diagnostic Criteria)

Answer 4

Autosomal dominant inheritance is seen in some families (no gene identified).

Both multiple motor tics and ≥1 vocal tics.
Tics occur many times a day, for more than a year.
Age of onset <18.
Exclusion of secondary causes.

Question 5

Tourette Syndrome (Treatment)

Answer 5

Symptomatic treatment:

Clonidine, Tetrabenazine

Question 6

Dystonia

Answer 6

Abnormal posture of the affected body part. Involuntary . sustained muscle contraction. Leads to twisting and repetitive movement or abnormal posturing.

Question 7

DYT1 - Torsion Dystonia

Answer 7

<28 (usually childhood)
Starts in limbs
Missing Torsin
Often positive FH

Question 8

Chorea

Answer 8

Brief irregular purposeless movements that flit and flow . from one body part to another. Patients appear constantly restless or fidgety.
Can be difficult to distinguish between chorea and myoclonus (short and not flying around) and tics (suppressible)

Question 9

Chorea (Causes)

Answer 9

Inherited - HD, Wilsons Disease
Autoimmune - SLE, Bechet, Anti-phospholipid syndrome
Infectious - HIV
Drugs - Levodopa
Paroxysmal Chorea - PD
Metabolic - Thyroid

Question 10

Chorea (Treatment)

Answer 10

Symptomatic treatment with tetrabenazine or dopamine receptor blocker drugs.

Question 11

Huntington’s Disease

Answer 11

Autosomal dominant neurodegenerative disorder

Question 12

Huntington’s Disease (Clinical Features)

Answer 12

Progressive behavioural disturbance
Dementia
Movement disorder (usually chorea)
Age of onset usually fourth decade

Question 13

Huntington’s Disease (Genetics)

Answer 13

Autosomal dominant

CAG triplet repeat expansion disorder affecting huntingtin gene on chromosome 4

Question 14

Myoclonus

Answer 14

Brief electric shock like jerks. Hiccups or hypnic jerks are common and normal forms of myoclonus.

Question 15

Symptomatic Myoclonus

Answer 15

With encephalopathy

Without encephalopathy

Question 16

Juvenile Myoclonus Epilepsy (Clinical Features)

Answer 16

Onset of myoclonic jerks and generalised seizures.
Worse in morning
EEG shows characteristic 3-5Hz polyspike and wave pattern.

Question 17

Juvenile Myoclonus Epilepsy (Precipitants)

Answer 17

Alcohol and sleep deprivation.

Question 18

Juvenile Myoclonus Epilepsy (Treatment)

Answer 18

Sodium Valproate
Levetiracetam

AED usually required longterm
(Carbamazepine can aggravate the epilepsy syndrome).