Movement disorders Flashcards
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
what are the two types of movement disorders
hypokinetic (too little movement)
hyperkinetic (too much movement)
what descending pathway do basal ganglia disorders affect
extra pyramidal
what features are seem in pyramidal (corticospinal)/ UMN lesions
pyramidal weakness= arms: extensor weaker than flexor
legs: flexors weaker than extensors
spascticity
what are the features of a extrapyramidal/ basal ganglia lesion
hyperkinetic:
- dystonia
- tics
- myoclonus
- chorea
- tremor
hypokinetic:
-parkinsonism
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
what is the most common neurodegenerative disease
alzeheimers, followed by parkinsons
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
what causes parkinsons
idiopathic
advancing age
genetic and environmental factors
5% familial
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
what are the three subtypes of parkinsons motor features
- tremor dominant (with relative absence of other motor symptoms)
- non tremor dominant (such as akinetic, rigid syndrome, postural instability)
- mixed
which motor subtype of parkinsons has the best prognosis
tremor dominant has slowest progression and less rate of physical disability
what are the common pre motor/ prodromal symptoms of PD
constipation, RBD, excessive daytime sleepiness, hyposmia, depression
how do you test bradykinesia
finger tapping
will have decrements in amplitude/ speed of movement and fatiguing
what are the features of advanced PD
urinary symptoms orthostatic hypotension dementia dysphagia postural instability freezing of gait falls fluctuations dyskinesia psychosis
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
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)
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)
who gets parkinsons
all ethnicities
men more likely
old age
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
what decreases you risk of parkinsons
tobacco smoking coffee drinking NSAID use CCB use alcohol consumption
what are the monogenic forms of parkinsons
LRRK2
PARKIN
what do symptomatic treatments do in PD
enhance intracerebral dopamine concentrations/ stimulate dopamine receptors
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)
when should you start Tx in parkinsons
when symptoms cause disability/ discomfort
what in PD responds well to dopaminergic treatment
bradykinesia and rigidity in early phase of disease
tremor is inconsistently responsive
what drugs can be effective for tremor
anticholinergics
trihexphenidyl
clozapine
what type of drug is levodopa and cardibopa
levo- dopamine precurosor
carbi- carboxylase inhibitor
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)
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
what eye movement problems can you get in parkinsons
vertical gaze palsy and slow saccade = progressive supranuclear palsy
what eye movement problems in huntingtons
difficulties initiating saccades
what are the two main types of movement disorder
hypokinetic- bradkinesia: parkinsons, akinetic rigid syndromes
hyperkinetic- dyskinesia: tremor, tics, chorea, myoclonus, dystonia
what is a tremor
rhythmic sinusoidal oscillation of a body part
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
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
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)
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
what is the different between chorea and myoclonus and tics
myoclonus- short and not flying around
tics- suppressible
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
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?
what is myoclonus
brief electric shock like jerks
hiccups or hypnic jerks (when falling asleep) are normal forms of myoclonus
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
what is negative myoclonus
produced by a temporary cessation of muscle activity e,g, asterixis (liver flap) in patients with liver failure
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
what are the types of tic
MOTOR: eye blinking head jerks arm/leg jerks complex sequence VOCAL: - sniffing - grunting - snorting
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
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]
what is dystonia
movement disorder characterised by sustained or intermittent muscle contractions causing abnormal, often repetitive, movements, postures or both
what are dystonic movements like
patterned, twisting and may be tremulous
what often initiates or worsens dystonia
voluntary action/ voluntary maintained posture
is associated with overflow muscle activation
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.
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.
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.
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.
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)
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)
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
where is the problem in an intention tremor
cerebellum
what are the features of cerebellar problems
staccato speech, saccade problems, nystagmus, intention tremor
what is the pathological hallmark of PD
prominent dopaminergic neuron loss in the substantia nigra pars compacta (SNpc) with a-synuclein-containing Lewy bodies
what is hypomimia
decreased facial expression and eye blinking
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
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)
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
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
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
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
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
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)
which type of parkinsonsim is responsive to levodopa
idiopathic parkinsons disease (this supports clinical diagnosis)
what are lewey bodies
eosinophillic intracytoplasmic inclusion bodies
are present in PD
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)
what antiemetic can you give in PD
clomperidone
does levodopa slow the progression of PD
no
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
what should you be careful of with anticholinergics in the eldelry
causes confusion
what are the types of secondary parkinsons
vascular and drug induced
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
what is lewey body dementia
dementia occurring before/ at the time of onset of PD
will have progressive dementia, visual hallucinations and parkinsonism
what is parkinsons disease dementia
dementia occuring 1 year after onset of PD
what is corticobasal degeneration
akinetic regidity involving one limb
cortical sensory loss- astereognosis, apraxia, alien limb phenomenom
(parkinson plus syndrome)
