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Flashcards in Movement disorders Deck (82):
1

what is the role of the basal ganglia

initiation and modulation of movements

receives input from cerebral cortex and relays it back to the cerebral cortex via the thalamus via direct (enhance outflow of thalamus) or indirect (inhibits thalamus outflow) pathways

2

what are the two types of movement disorders

hypokinetic (too little movement)
hyperkinetic (too much movement)

3

what descending pathway do basal ganglia disorders affect

extra pyramidal

4

what features are seem in pyramidal (corticospinal)/ UMN lesions

pyramidal weakness= arms: extensor weaker than flexor
legs: flexors weaker than extensors

spascticity

5

what are the features of a extrapyramidal/ basal ganglia lesion

hyperkinetic:
-dystonia
-tics
-myoclonus
-chorea
-tremor

hypokinetic:
-parkinsonism

6

what are the motor features of a cerebellar lesion

ataxia- lack of voluntary coordination of muscle movements that can include gait abnormality, speech changes and abnormalities in eye movements

7

what is the most common neurodegenerative disease

alzeheimers, followed by parkinsons

8

what is the pathology of parkinsons

loss of black pigment correlating to the degeneration of dopaminergic neurones in basal ganglia (substantia nigra (pars compacta region) and locus coeruleus)

presence of lewy bodies

9

what causes parkinsons

idiopathic
advancing age
genetic and environmental factors
5% familial

10

what are the clinical features of parkinsons

tremor (resting, slow reemergence of tremor when in posture, often asymmetrical)
bradykinesia
rigidity
postural instability

non motor:
sleep disorders (RBD)
hallucinations
GI dysfunction (constipation)
depression
cognitive impairment
dementia
anosmia

11

what are the three subtypes of parkinsons motor features

1. tremor dominant (with relative absence of other motor symptoms)
2. non tremor dominant (such as akinetic, rigid syndrome, postural instability)
3. mixed

12

which motor subtype of parkinsons has the best prognosis

tremor dominant has slowest progression and less rate of physical disability

13

what are the common pre motor/ prodromal symptoms of PD

constipation, RBD, excessive daytime sleepiness, hyposmia, depression

14

how do you test bradykinesia

finger tapping
will have decrements in amplitude/ speed of movement and fatiguing

15

what are the features of advanced PD

urinary symptoms
orthostatic hypotension
dementia
dysphagia
postural instability
freezing of gait
falls
fluctuations
dyskinesia
psychosis

16

what are the essential features for a PD diagnosis

bradykinesia and one more of the following: resting tremor, rigidity (cog wheel or lead pipe), postural instability).

Additional motor features: stooped, fixed posture, dystonic postures, hypomimia (masked face), shuffling, short stepped gait (+/- festination= running after their centre of gravity).

Additional non motor: late onset hyposmia, depression, anxiety, constipation, bladder problems, pain, subtle mental or cognitive impairment

17

what should patient with suspected PD not present with

early onset bulbar problems (CN 9-12)
dementia
hallucinations
preferential involvement of lower limbs
prominent eye movement disorder (e.g. supranuclear palsy= problems in voluntary upwards and downwards movement)
intrusive early autonomic problems (e.g. bladder control)

18

what imaging can be done for a PD diagnosis

brain imaging
DaTSCAN (nuclear medicine presynaptic dopaminergic imagine- if comma shaped = possible essential tremor. If period shape= possible parkinsons tremor)

19

who gets parkinsons

all ethnicities
men more likely
old age

20

what are the risk factor for parkinsons

old age
family Hx
male
environmental:
-pesticides
-head injury
-rural living
-beta blocker use
-farmer
-well water drinker

21

what decreases you risk of parkinsons

tobacco smoking
coffee drinking
NSAID use
CCB use
alcohol consumption

22

what are the monogenic forms of parkinsons

LRRK2
PARKIN

23

what do symptomatic treatments do in PD

enhance intracerebral dopamine concentrations/ stimulate dopamine receptors

24

what are the symptomic PD drugs

levodopa (best, given >65/70s)
dopamine agonists (if patient young)
monoamine oxydase type B inhibitors (adjuvant/ early disease)
amantadine (NMDA antagonist)

25

when should you start Tx in parkinsons

when symptoms cause disability/ discomfort

26

what in PD responds well to dopaminergic treatment

bradykinesia and rigidity in early phase of disease
tremor is inconsistently responsive

27

what drugs can be effective for tremor

anticholinergics
trihexphenidyl
clozapine

28

what type of drug is levodopa and cardibopa

levo- dopamine precurosor
carbi- carboxylase inhibitor

29

what are the side effects of PD medications

dopamine agonists and levodopa- nausea, daytime somnolence, oedema

dopamine agonists- impulse control disorders (gambling, hypersexuality, binge eating, spending) - avoid in Hx of addiction/ impulsive personality. hallucinations (avoid in elderly with CI)

levodopa best symptomatic benefit but longterm use is associated with motor complications (dyskinesia and motor fluctuations)

30

what are the long term complications of dopamingeric therapies for PD

motor fluctuations- (periods of good and bad motor symptom control)

non motor fluctuations

dyskinesia (involuntary choreiform or dystonic movements)- happens when levodopa is at highest conc

drug induced psychosis- hallucinations

31

what eye movement problems can you get in parkinsons

vertical gaze palsy and slow saccade = progressive supranuclear palsy

32

what eye movement problems in huntingtons

difficulties initiating saccades

33

what are the two main types of movement disorder

hypokinetic- bradkinesia: parkinsons, akinetic rigid syndromes

hyperkinetic- dyskinesia: tremor, tics, chorea, myoclonus, dystonia

34

what is a tremor

rhythmic sinusoidal oscillation of a body part

35

what are the types of tremor

REST: occurs when the affected body part is at rest (5 Hz)
POSTURAL: occurs when the arms are outstretched
KINETIC: occurs during movement of a body part
INTENTION: worsening during voluntary movement, gets worse as reaches target

36

what is an essential tremor

the most common type of postural tremor
most commonly seen when hands outstretched
may continue throughout motor function
can be familial - AD
age of onset varies
progression is slow but can be disabeling
often slightly asymmetrical at onset
improved by alcohol intake

37

what conditions can present with a postural tremor

physiological (symmetrical, fast and small amplitude- e.g. nervous)
wilsons disease (wing beating)
hepatic encephalopathy (asterixis, repetitive drops of extended hands)
titubation (oscillations of the head in the anteroposterior or vertical direction in standing position)

38

what is chorea

brief irregular purposeless movements that flit and flow from one body part to another

patient appear constantly restless or fidgety

often generalised but may be confined to one region

39

what is the different between chorea and myoclonus and tics

myoclonus- short and not flying around
tics- suppressible

40

what can cause clonus

INHERITED / DEGENERATIVE DISORDERS
Huntington’s disease (HD) and HD-like syndromes
Wilson’s disease
Neuroacanthocythosis
Benign hereditary chrorea
Ataxia teleangiectasia
Spinocerebellar ataxia Typ 17
AUTOIMMUNE (including post infectious)
Systemic lupus erythematosus
Anti-phospholipid syndrome
Bechet syndrome
Coeliac disease
Sydenham’s chorea
Hashimotos’ thyreoiditis
INFECTIOUS
DRUGS
Dopamine-receptor blocking drugs, levodopa, stimulants, oral contraceptive pill, anticonvulsants, ….
PAROXYSMAL CHOREA
Parkinson’s disease
METABOLIC
Chorea gravidarum, glucose, thyroid, parathyroid, sodium, magnesium metabolism

41

what are the main consideration with a patient who has chorea

age of onset
type of onset (acute/ sub/ chronic/ progressive)
family/ drug history
distribution of chorea/ other clinical features- dementia?

42

what is myoclonus

brief electric shock like jerks
hiccups or hypnic jerks (when falling asleep) are normal forms of myoclonus

43

what is the pathology of myoclonus

brief activation of a group of muscles leading to a jerk of the affected body part
activation can arise from the cortex, subcortical structures, spinal cord, or nerve root and plexus

44

what is negative myoclonus

produced by a temporary cessation of muscle activity e,g, asterixis (liver flap) in patients with liver failure

45

what is a tic

'un'voluntary repetitive, stereotypes movements or vocalisations
are suppressible by the patient for a short period of time - will experience growing anxiety and discomfort during tic suppression
when relax will respond with a flurry of tics

46

what are the types of tic

MOTOR:
eye blinking 
head jerks 
arm/leg jerks 
complex sequence
VOCAL:
- sniffing 
- grunting 
- snorting 

47

when do tics usually start

primary tic disorder almost always start in childhood
adult onset of tics is rare, almost always due to a secondary cause

48

what is gilles de la tourette syndrome

, onset of persistent multiple motor and vocal tics, often with associated psychiatric disturbance [Attention deficit hyperactivity syndrome (ADHD); Obsessive compulsive disorder (OCD); copropraxia; coprolalia] 

49

what is dystonia

movement disorder characterised by sustained or intermittent muscle contractions causing abnormal, often repetitive, movements, postures or both

50

what are dystonic movements like

patterned, twisting and may be tremulous

51

what often initiates or worsens dystonia

voluntary action/ voluntary maintained posture
is associated with overflow muscle activation

52

what is a dystonic tremor

A spontaneous oscillatory, rhythmical, patterned movement produced by contractions of dystonic muscles often exacerbated by an attempt to maintain primary (normal) posture.

53

what is overflow muscle activation

in dystonia overflow= a unintentional muscle contraction which accompanies, but is anatomically distinct from the primary dystonic movement. Commonly occurs at the peak of dystonic movements.

54

what is mirror dystonia

Unilateral posture or movement that is the same or similar in character to a dystonic feature that can be elicited, usually in the more severely affected side, when contralateral movements or actions are performed.

55

what are sensory ticks

Voluntary actions that specifically correct the abnormal posture or alleviate dystonic movements. Usually simple movements (“gestes”) involving, or directed to, the body region affected by dystonia.

56

what are the features of the tremor in parkinsons

Rhythmic oscillatory involuntary movement of affected body part at rest
Vanishes with active movement
Typically reappears after few sec when arms held outstreched (‘reemerging tremor’)
Tremor frequency in low to mid-range (3-6 Hz) with variable amplitude
Most distinguishing resting tremor is “Pill-rolling”-type or
Finger flexion-extension or abduction-adduction
Tremor can also affect lower limbs, jaw, and tongue
Head tremor is not typical for PD
In clinical practice, tremor is best observed while patient is focused on a particular mental task (counting backwards from 100)

57

what are the features of rigidity in parkinsons

Increased muscle tone felt during examination by passive movement
Resistance is felt throughout full range of movement
No increase with higher mobilizing speed
This distinguishes rigidity from spasticity owing to UMN lesions
Rigidity + resting tremor = “cog wheel” rigidity (especially felt at wrist)
Positive Froment’s maneuver (rigidity increases in examined body segment by voluntary movement of other body parts)

58

what are the features of posture and gait in parkinsons

Stooped posture owing to impaired postural reflexes (major contributor to falls risk)
In some cases extreme anterior truncal flexion “Camptocormia”
Parkinsonian gait is slow, occurs at narrow base with short, shuffling steps
Decreased arm swing
Slow turning with multiple small steps
Freezing
Festination (very fast succession of steps and difficulties stopping)
Clinically, assess gait and posture in open space as well as while passing through narrow doorways or spaces
Pull test

59

where is the problem in an intention tremor

cerebellum

60

what are the features of cerebellar problems

staccato speech, saccade problems, nystagmus, intention tremor

61

what is the pathological hallmark of PD

prominent dopaminergic neuron loss in the substantia nigra pars compacta (SNpc) with a-synuclein-containing Lewy bodies

62

what is hypomimia

decreased facial expression and eye blinking

63

what are the features of vascular parkinonism

Parkinsonism affects predominantly lower limbs (“lower body” Parkinsonism)
Rest tremor is uncommon
Other signs of brain vascular lesions might be present (spasticity, hemiparesis, and pseudobulbar pasly)
Poor levodopa response
Structural brain imaging will guide diagnosis

64

what are the features of drug induced parkinonism

Parkinsonism tends to be symmetrical
Often coarse postural tremor
Presence of other drug induced disorders:
Orolingual dyskinesias, tardive dystonia, akathisia
Series of events is important: emergence of symptoms after drug exposure
Improvement / resolution within few months of complete drug withdrawal

-any drug that blocks the action of dopamine (especially neuroleptic drugs)

65

what is multi system atrophy

Common cause of degenerative Parkinsonism (parkinson plus syndrome)
Age of onset in late 6th or 7th decades
Core triad of dysautonomia,\ (AUTONOMIC FEATURES) cerebellar features, and Parkinsonism
Jerky postural tremor, pyramidal signs (generalized hyperreflexia and extensor plantar responses)
Suboptimal and shor-tlived levodopa response in 1/3 of patients
AUTONOMIC FEATURES: POSTURAL HYPOTENSION, ERECTILE DYSFUNCTION, URINARY URGENCY
MRI shows putaminal atrophy, hot cross bin appearance of pons axially

66

what is a progressive supranuclear palsy

is a parkinson plus syndrome
Symmetric akinesia and rigidity syndrome with predominantly axial involvement- loss of balance
loss of eye movements- supranuclear gaze palsy (vertical plane)
MRI shows midbrain atrophy with hummingbird sign on saggital view

67

what are the features of progressive supranuclear palsy

Gait and balance impairement (early falls)
Tremor is infrequently seen in these patients
Vertical gaze supranuclear palsy
Pseudobulbar symptoms
Continuous activity of the frontalis muscle with eyes wide open (staring)
Frontal-subcortical cognitive deficits
No response to levodopa
retrocollis

68

what is fragile x tremor ataxia syndrome

Late-onset (>50 years) neurodegenerative disorder in pts with an abnormal number of CGG repeats in the FMR1 gene

69

what are the symptoms of fragile X tremor ataxia syndrome

cerebellar gait ataxia, postural / intention tremor, variably Parkinsonism, dysautonomia, cognitive decline of frontal type, and peripheral neuropathy
Slow disease progression

70

what is the golden rules for parkinsons treatment

if over 65/70 then levodopa
if younger then give dopamine agonist (pramipexole, ropinerol) to delay use of levodopa (loses efficacy and causes movement disorder)

71

which type of parkinsonsim is responsive to levodopa

idiopathic parkinsons disease (this supports clinical diagnosis)

72

what are lewey bodies

eosinophillic intracytoplasmic inclusion bodies
are present in PD

73

what is prescribed along side levodopa in the tx of PD

decarboxylase inhibitor to prevent the peripheral conversion (levodopa crosses BBB and is converted into DA by the substantia nigra) of the drug to dopamine to prevent SEs (nausea)

74

what antiemetic can you give in PD

clomperidone

75

does levodopa slow the progression of PD

no

76

when are MAO-B inhibitors used in PD

in combo with levodopa or in early disease to dealy levodopa
causes hypertensive crisis (tryamine, cheese), AF, hallucinations, vivid dreams, sudden uncontrolled movements

77

what should you be careful of with anticholinergics in the eldelry

causes confusion

78

what are the types of secondary parkinsons

vascular and drug induced

79

what are the parkinson plus syndrome

parkinsonism + other features that differentiate it from idiopathic PD:
progressive suprannuclear palsy
lewey body dementia
mutliple system atrophy
corticobasal degeneration

80

what is lewey body dementia

dementia occurring before/ at the time of onset of PD
will have progressive dementia, visual hallucinations and parkinsonism

81

what is parkinsons disease dementia

dementia occuring 1 year after onset of PD

82

what is corticobasal degeneration

akinetic regidity involving one limb
cortical sensory loss- astereognosis, apraxia, alien limb phenomenom
(parkinson plus syndrome)