Movement Disorders

Question 1

describe an essential tremor?

Answer 1

postural or action tremor

(rather than resting tremor seen in parkinsons)

Question 2

essential tremor is a trait gained throigh which pattern of inheritance?

Answer 2

autosomal dominant

Question 3

what can imrpove essential tremor?

Answer 3

often improves after alcohol

can be helped by B blockers (propanolol)

Question 4

lesions in the cerebellum or its pathways can cause which tremor?

Answer 4

intention tremor

Question 5

other than intention tremor what other cerebellar pathway tremor is there?

Answer 5

Holmes (rubral) tremor

Question 6

describe holmes tremor?

Answer 6

tripartite tremor incorporating tremor at rest, postural tremor and intention tremor

Question 7

what common movement disorder is characterised by involuntary muscle spasms leading to abnormal postures of affected body part?

Answer 7

dystonia

Question 8

what are the possible treatment for dystonia?

Answer 8

anticholinergics

botulinum toxin (used first line if focal dystonia)

surgery

Question 9

describe chorea?

Answer 9

continuous spontaneous jerky movements, irregularly timed and randomly distributed

Question 10

what pattern of inheritance is seen in Huntingtons?

Answer 10

autosomal dominant

Question 11

mutation in which gene causes huntingtons?

Answer 11

HTT gene

Question 12

the HTT mutation invloved which segment of DNA?

Answer 12

CAG segment

usually repeats 10-30 times, mutation result in 30 to >120 repeats

Question 13

what are early signs of Huntingtons?

Answer 13

personality changes

depression

apathy

dysarthria

Question 14

progression of huntigntons will lead to what?

Answer 14

chorea

rigidity

dementia

Question 15

what neurotransmoitters are affected in Huntington’s?

Answer 15

decreased GABA

decreased ACh

increased dopamine

Question 16

what drug can be of use in Huntongton’s?

Answer 16

tetrabenazine (anti-choreic)

works by inhibiting vesicular monoamine transporter (VMAT)- limits dopamine vesicle packing and release

Question 17

what is Sydenhams chorea?

Answer 17

manifestation of rheumatic fever

occurs n children 5-15yrs old

Question 18

what is seen in sydenhams chorea?

Answer 18

widespread chorea

behvaioural disturbance

OCD symtpoms

Question 19

how is Sydenhams chorea treated?

Answer 19

self limiting and usually resolves within 6 months

Question 20

describe tics

Answer 20

brief rapid intermittent stereotyped involuntary movements or sounds

(can be simple/complex)

Question 21

what is seen in Gilles de la Tourette syndromewhat ?

Answer 21

multiple motor tics and one or more phonic/ vocal tics

Question 22

what is the maximum age of onset of tourettes syndrome?

Answer 22

18 years

Question 23

what is the most common first tic?

Answer 23

blinking

most tics usally begin in head and face

Question 24

what are simple vocal tics and complex vocal tics?

Answer 24

simple: sniffing, throat clearing, snorting, coughing

complex: barking, animal noises, inappropriate voice intonations

Question 25

what percentage of those with tourettes have a psychiatric co-morbidity?

Answer 25

90% most common is ADHD followed by OCD

Question 26

what is the preferred treatment if mild tourettes?

Answer 26

counselling behavioural/ psychosocial interventions habit reversal training

Question 27

if medicaiton is to be used in tourettes what is considered best option for tic control?

Answer 27

risperidone

Question 28

if tourettes with co-existing ADHD what is the treatment of choice?

Answer 28

clonidine (wiht or without risperidone)

Question 29

what movement disorder if characterised by a sudden brief, shock-like movement caused by muscular contraction or inhibition?

Answer 29

myoclonus

Question 30

myoclonus can be a symtpom of which neurodegenerative disease?

Answer 30

wilsons huntingtons multiple system atrophy severe hypoxia (lance adams)

Question 31

what is hemiballism?

Answer 31

wild flinging/throwing of one arm or leg usually result of cerebrovascular lesion to subthalamic nucleus

Question 32

restless leg syndrome is classically seen at what time?

Answer 32

night time also occurs when tryign to relax when sitting or lying down during the day

Question 33

what can instantly relieve restless leg syndrome?

Answer 33

getting up and wlakign about

Question 34

iron deficiency, uraemia and pregnancy are assoc w what movement disorder?

Answer 34

restless leg syndrome

Question 35

restless leg syndrome has a positive response to which drug?

Answer 35

dopamine agonists | (pramipexole)