Hyperkinetic Movement Disorders

Question 1

what type of tremor does Parkinsons’s cause, and is it uni or bilateral

Answer 1

resting - pill rolling

often unilateral at first, progressing to bilateral

Question 2

what are tremors classified by

Answer 2

position, distribution, frequency, amplitude

Question 3

what can you ask the pt to draw to examine a tremor

Answer 3

write or draw a spiral

Question 4

what blood tests should be done when diagnosing tremor

Answer 4

thyroid function, copper and ceruloplasmin conc (Wilsons)

Question 5

what is the most common cause of a cerebellar pathway tremor (intention)

Answer 5

multiple sclerosis

Question 6

what is Wilsons disease

Answer 6

genetic defect (AuR) resulting in loss of ceruloplasmin, which normally binds copper. Leads to accumulation of excess copper

• tremor, CNS signs, mood, movement, Kayler-Fleischer rings, liver disease

Question 7

management of Wilsons

Answer 7

life long copper chelating drugs - penicillamine

Question 8

a cerebellar tremor is typically slow/fast and high/low amplitude

Answer 8

slow and high of extremities

Question 9

what test can be done for intentino tremor

Answer 9

finger to nose test

Question 10

what are the most common causes of cerebellar tremor (intention and holmes)

Answer 10

MS, stroke or trauma, chronic alcoholism can damage cerebellum

Question 11

when is a holmes tremor present

Answer 11

rest, intention and postural

Question 12

cause of essential tremor, and at what age does it present

Answer 12

prevalence increases with age

can be inherited in autosomal dominant fashion

Question 13

which body part does essential tremor affect

Answer 13

upper extremities

can involve voice, palate, head and jaw - quivering sound to voice

Question 14

when is essential tremor present

Answer 14

posture and action eg when holding a glass

Question 15

does essential tremor usually cause signficant impairement

Answer 15

is usually mild and stable for years, it does slowly progress but rarely causes severe disability - does impair ADL

Question 16

what exacerbates and relieves essential tremor

Answer 16

exacerbated - anxiety, sympathomimetics (eg salbutamol)

made better by small amounts of alcohol

Question 17

is there good treatment for essential tremor?

Answer 17

not really, often unecessary and unsatisfactory

Question 18

what pharmacological agents are used in teh management of essential tremor

Answer 18

propanolol and primidone

Question 19

what is the class and action of primidone

Answer 19

agonist of GABA A

anticonvulsant of barbiturate class

Question 20

how does the pt feel if they try to suppress tics?

Answer 20

anxious and uncomfortable, when allowed to relax they will respond with a flurry of tics

Question 21

what sort of things make tics worse

Answer 21

excitement, stress, anxiety

decrease with distraction

Question 22

when do tics usually develop

Answer 22

childhood, adult onset is rare

Question 23

what is a common first tic

Answer 23

blinking

Question 24

where do tics usually start

Answer 24

in head and face

Question 25

give some examples of simple tics

Answer 25

sniffing, coughing, throat clearing, snorting

Question 26

give some examples of complex vocal tics

Answer 26

barking, making of animal noises, inappropriate voice intonations and uttering strings of words.

Question 27

what is copropraxia and coprolalia

Answer 27

production of obscene gesture and words

Question 28

echopraxia and echolalia

Answer 28

copying movements/words of others

Question 29

what is palilalia

Answer 29

repetition of same phrase, word or syllable

Question 30

which psychiatric disorders are commonly seen alongside tics

Answer 30

90% psychiatric co morbidity - ADHD and OCD common

Question 31

what time frame distinguishes between simple transient and chronic tics of childhood

Answer 31

1 year

Question 32

what type of tics must be present in Tourettes?

Answer 32

multiple motor and at least one vocal

Question 33

what is the most common cause of tics

Answer 33

Tourettes

Question 34

criteria for Tourettes diagnosis

Answer 34

tics occur many times a day, nearly every day/intermittent for more than 1 year no longer than 3 months w/out tics

Question 35

is Tourettes more common in M or F

Answer 35

males

Question 36

when must onset be for a diagnosis of Tourettes

Answer 36

\<18 (mean age of 6), adult onset Tourettism is a separate diagnosis

Question 37

what is the first line psychotherapy for Tourettes

Answer 37

habit reversal training

Question 38

what medication is considered the best option for tic control in Tourettes

Answer 38

risperidone

Question 39

what medication is used for co existing ADHD and Tourettes

Answer 39

clonidine

Question 40

describe chorea

Answer 40

Continuous, spontaneous jerky movements, irregularly timed and randomly distributed. Brief irregular purposeless movements which flit and flow from one body part to another, pt appears constantly restless or fidgety

Question 41

what are the 2 main types of chorea

Answer 41

Huntingtons and Syndenhams

Question 42

what is syndenhams chorea caused by

Answer 42

rheumatic fever

Question 43

who gets syndenhams chorea

Answer 43

children (5-15), mainly girls, rare now in developed countries

Question 44

what other features os sydnenhams chorea associated with

Answer 44

behavioural disturbance and OC symptoms

Question 45

management and prognosis of syndenhams chorea

Answer 45

self limiting and usually resolves within 6 months

Question 46

what causes myoclonus

Answer 46

brief activation of group of muscles - body part jerks

Question 47

what is negative myoclonus

Answer 47

muscle jerks resulting from a brief cessation of muscle activity, eg liver flap in those with liver failure

Question 48

physiological myoclonus

Answer 48

common and nonpathological feature - nocturnal myclonus is sudden jerk (with a feeling of falling) when dropping off to sleep or waking up

Question 49

what is dystonia

Answer 49

an involuntary muscle spasm which leads to a sustained abnormal posture of the affected body part

Question 50

what are the muscles doing to cause dystonia

Answer 50

co contraction of agonist and antagonist muscle

Question 51

does the abnormal posture in dystonia remain fixed?

Answer 51

no, often there is slow writhing movements (athetosis) - dominant muscle activity switches back and forth from agonist to antagonist

Question 52

does dystonia affect one body part or whole body?

Answer 52

focal or generalised

Question 53

what tricks can improve dystonia

Answer 53

sensory trick - geste antagoniste - touching face/head with arm

Question 54

which medications can be used for dystonia

Answer 54

anticholinergics anti spasmodics botulinum toxin in focal dystonias

Question 55

are early onset or late onset dystonias more common

Answer 55

late onset

Question 56

what mutationis found in early onset dystonias

Answer 56

DYT1

Question 57

torticollis

Answer 57

a primary dystonia, SCM problem - head is turned or drawn backwards (retrocollis)

Question 58

what is the hemiballismus movement like

Answer 58

dance like, contorting and continuous usually of arm and leg on one side

Question 59

a lesion in which area causes hemiballismus

Answer 59

sub thalamic lesion

Question 60

what does a pt with restless leg syndrome describe

Answer 60

unpleasant senation/urge to move legs

Question 61

at what time of the day is restless leg syndrome most prominent

Answer 61

at night

Question 62

what relieves restless leg syndrome

Answer 62

getting up and walking about - instant relief

Question 63

pharmacoloigcal management of restless leg syndrome

Answer 63

dopamine agonists eg pramipexole and ropinirole

Question 64

task specific dystonias

Answer 64

Specific inability to perform a previous highly developed repetitive skill, e.g. writing, musicians