Hyperkinetic Movement Disorders Flashcards
what type of tremor does Parkinsons’s cause, and is it uni or bilateral
resting - pill rolling
often unilateral at first, progressing to bilateral
what are tremors classified by
position, distribution, frequency, amplitude
what can you ask the pt to draw to examine a tremor
write or draw a spiral
what blood tests should be done when diagnosing tremor
thyroid function, copper and ceruloplasmin conc (Wilsons)
what is the most common cause of a cerebellar pathway tremor (intention)
multiple sclerosis
what is Wilsons disease
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
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management of Wilsons
life long copper chelating drugs - penicillamine
a cerebellar tremor is typically slow/fast and high/low amplitude
slow and high of extremities
what test can be done for intentino tremor
finger to nose test
what are the most common causes of cerebellar tremor (intention and holmes)
MS, stroke or trauma, chronic alcoholism can damage cerebellum
when is a holmes tremor present
rest, intention and postural
cause of essential tremor, and at what age does it present
prevalence increases with age
can be inherited in autosomal dominant fashion
which body part does essential tremor affect
upper extremities
can involve voice, palate, head and jaw - quivering sound to voice
when is essential tremor present
posture and action eg when holding a glass
does essential tremor usually cause signficant impairement
is usually mild and stable for years, it does slowly progress but rarely causes severe disability - does impair ADL
what exacerbates and relieves essential tremor
exacerbated - anxiety, sympathomimetics (eg salbutamol)
made better by small amounts of alcohol
is there good treatment for essential tremor?
not really, often unecessary and unsatisfactory
what pharmacological agents are used in teh management of essential tremor
propanolol and primidone
what is the class and action of primidone
agonist of GABA A
anticonvulsant of barbiturate class
how does the pt feel if they try to suppress tics?
anxious and uncomfortable, when allowed to relax they will respond with a flurry of tics
what sort of things make tics worse
excitement, stress, anxiety
decrease with distraction
when do tics usually develop
childhood, adult onset is rare
what is a common first tic
blinking
where do tics usually start
in head and face
give some examples of simple tics
sniffing, coughing, throat clearing, snorting
give some examples of complex vocal tics
barking, making of animal noises, inappropriate voice intonations and uttering strings of words.
what is copropraxia and coprolalia
production of obscene gesture and words
echopraxia and echolalia
copying movements/words of others
what is palilalia
repetition of same phrase, word or syllable
which psychiatric disorders are commonly seen alongside tics
90% psychiatric co morbidity - ADHD and OCD common
what time frame distinguishes between simple transient and chronic tics of childhood
1 year
what type of tics must be present in Tourettes?
multiple motor and at least one vocal
what is the most common cause of tics
Tourettes
criteria for Tourettes diagnosis
tics occur many times a day, nearly every day/intermittent for more than 1 year
no longer than 3 months w/out tics
is Tourettes more common in M or F
males
when must onset be for a diagnosis of Tourettes
<18 (mean age of 6), adult onset Tourettism is a separate diagnosis
what is the first line psychotherapy for Tourettes
habit reversal training
what medication is considered the best option for tic control in Tourettes
risperidone
what medication is used for co existing ADHD and Tourettes
clonidine
describe chorea
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
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what are the 2 main types of chorea
Huntingtons and Syndenhams
what is syndenhams chorea caused by
rheumatic fever
who gets syndenhams chorea
children (5-15), mainly girls, rare now in developed countries
what other features os sydnenhams chorea associated with
behavioural disturbance and OC symptoms
management and prognosis of syndenhams chorea
self limiting and usually resolves within 6 months
what causes myoclonus
brief activation of group of muscles - body part jerks
what is negative myoclonus
muscle jerks resulting from a brief cessation of muscle activity, eg liver flap in those with liver failure
physiological myoclonus
common and nonpathological feature - nocturnal myclonus is sudden jerk (with a feeling of falling) when dropping off to sleep or waking up
what is dystonia
an involuntary muscle spasm which leads to a sustained abnormal posture of the affected body part
what are the muscles doing to cause dystonia
co contraction of agonist and antagonist muscle
does the abnormal posture in dystonia remain fixed?
no, often there is slow writhing movements (athetosis) - dominant muscle activity switches back and forth from agonist to antagonist
does dystonia affect one body part or whole body?
focal or generalised
what tricks can improve dystonia
sensory trick - geste antagoniste - touching face/head with arm
which medications can be used for dystonia
anticholinergics
anti spasmodics
botulinum toxin in focal dystonias
are early onset or late onset dystonias more common
late onset
what mutationis found in early onset dystonias
DYT1
torticollis
a primary dystonia, SCM problem - head is turned or drawn backwards (retrocollis)
what is the hemiballismus movement like
dance like, contorting and continuous
usually of arm and leg on one side
a lesion in which area causes hemiballismus
sub thalamic lesion
what does a pt with restless leg syndrome describe
unpleasant senation/urge to move legs
at what time of the day is restless leg syndrome most prominent
at night
what relieves restless leg syndrome
getting up and walking about - instant relief
pharmacoloigcal management of restless leg syndrome
dopamine agonists eg pramipexole and ropinirole
task specific dystonias
Specific inability to perform a previous highly developed repetitive skill, e.g. writing, musicians