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55 Cards in this Set
- Front
- Back
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Define five global conditions which exhibit hyperkinetic movemnet
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tremour
tics chorea myoclonus dystonia |
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Which classic condition demonstrates hypokinesia
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Parkinson's Disease
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Define 'tremour'
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rhythmic sinusoidal ossiclliations of a body part
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Define 'tic'
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involuntary sterotyped movements or vocalizations
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Define 'Chorea'
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brief irregular, purposeful movements, which flit and flow from one body part to another 'dance like'
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Define 'moyclonus'
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Brief shock like jerks
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Define 'Dystonia'
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abnormal posture of the affected body part
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Name 4 ways to classify tremor
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1. Position (at rest or during movement)
2. Distribution (body part affected) 3. Frequency (measure in Hertz, ie low or high) 4. Amplitude ('fine' or 'coarse') |
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Name 3 common causes of a resting tremor
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Parkinson's Disease (resting tremor, hypokinesia, mask like face)
Drug Induced Parkinsonism Psychogenic tremor |
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Name 3 common causes of postural tremor
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essential tremor
Enhanced physiological tremor Tremor associated with neuropathy |
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Name 2 causes of kinetic tremor
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Cerebellar disease (demyelination, haemorrhagic, degenerative, toxic etc)
Wilson's Disease |
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Dystonia and cerebellar disease are two causes of which type of tremor
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Head Tremor
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Jaw Tremor can be a feature of
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parkinson's disease
Dystonia |
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Palatal Tremor can occur in....
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conjunction with ataxia
symptomatic essential tremor |
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Core Criteria for ESSENTIAL TREMOR
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bilateral action tremor of hands, absence of neurolgoical signs (except cog-wheeling)
+/- isolated head tremor with no signs of dystonia |
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Secondary criteria for ESSENTIAL TREMOR
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Long duration (usually longer than 3 years)
+ family history Beneficial response to Alcohol |
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What is the prevalence of Essential Tremor
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5% General population (UK!)
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What is the DDx of Essential Tremor
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enhanced physiological tremor, PD, adult onset idiopathic dystonia, Wilson's Disease
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What is the first line Treatment of Essential Tremor
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Propranolol, primidone
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What other medications are available for Essential Tremor
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atenolol, gabapentin, sotalol, alpraxolam, toiramate, clonazepam, nadolol, nimodipine
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Which proceedure can be considered in disabiling Essential Tremor
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Deep Brain Stimulation
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copropraxia
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producing obscene gestures
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Echopraxia
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Copying the movement of others
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Coprolalia
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Saying obscene words
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Echolalia
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Copying words of others
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Palilalia
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repetition of the same phrase, word or syllable
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Primary Tic
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almost always begin in childhood
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Secondary Tics
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Onset of tic due to underlying cause
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Name 4 types of Primary Tic Disorder
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Simple transient childhood ticks
Chronic Tics of childhood Gilles de La Tourette's Syndrome Adult onset Tourettism |
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Name 6 causes of secondary tic disoders of neurodegenerative origin
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Huntington's
Wilson's Neuroacanthocythosis neuronal Brain Iron Accumulation Syndrome Rett's Lesch-Nyhan Syndrome |
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Name 4 causes of secondary tic disoders of developemental origin
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Downs syndrome and other chromosomal abnormalities
Fragile X Syndrome Autism Non-specific mental retardation |
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Name 2 type of structural abnormalities that could cause Secondary Tic disorder
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Post-encephalopathy
Basal Ganglia lesion (caudate nucleus) Syndenham's Chorea PANDA's |
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Name 4 drugs/toxins that could cause secondary tic disorder
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Cociane
CO poisioning Amphetamines anticonvulsants |
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Outline 4 criteria for diagnosis of Tourette's Syndrome
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Motor and at least one vocal tic must be present
Tics must occur either: Multiple times a day, nearly every day, or occur intermittently with at no more than 3 tic free months in 1 year onset at Age <18 Exclusion of secondary symptoms |
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Name 6 inherited conditions which can exhibit Chorea
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Huntingtons
Wilsons Neuroacanthocythosis Benign Hereditary Chorea Ataxia Telangactasia Spinocerebellar axataxia type 17 |
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Name 6 Autoimmune diseases that can exhibit Chorea
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SLE
Anti-phospholipid syndrome Bechets Coeliac disease Hasimotos Thyroiditis |
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Name 5 catagories of drugs which can cause Chorea?
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Dopamine receptor blocking drugs
Levodopa Stimulants OCP Anti-convulsant drugs |
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Which condition can anti-psychotic drugs cause that demonstrates Chorea?
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Neuroleptic Malignancy Syndrome
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Which infection can cause Chorea?
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HIV
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In which condition can patients present with paroxysmal chorea
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Parkinson's Disease
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Name 6 metabolic disturbances which can cause Chorea
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Chorea gravidarum
Glucose upset Thryoid problems parathyroid problems Sodium disturbance Magnesium metabolism |
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Causes of primary myoclonus (5)
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Hypnic jerks, hiccups
Benign infantile myoclonus with feeding/sleep Essential Myoclonus Myoclonus dystonia cortical tremor |
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Name 6 types of epilepsy disorders asssociated with myoclonus
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Benign myoclonus epilepsy of infancy
Juvenile myoclonic epilepsy Early infancy myoclonic encephalopathy West's Syndrome Lennox Gastaut Syndrome Epilepsy partialis continua |
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Name 6 forms of progressive myoclonic epilepsy and ataxia
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Lafora body disease
Unverricht Lindborg disease Neuronal ceroid Lipofuscinosis sialidosis Myonclonic epilepsy with ragged red fibres Dentatorubropallidoluysian atrophy |
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Name 6 types of symptomatic myoclonus + encephalopathy
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liver failure
renal failure drug intoxication (alcohol, lithium) Toxins (lead) Post hypoxia progressive encephalomyelitis with rigidity |
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Name 4 catagories of symptomatic myoclonus without associated encephalopathy
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dementia (alzheimers, lewy-body, CJD)
Parkinsonism (corticobasal degeneration, MSA, spinocerebellar ataxias( Focal (spinal cord, plexus, root, palatal myoclonus) Other (whipples, coeliac disease, paraneoplastic, durgs) |
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Which type of epilepsy is associated with 3-5Hz polyspike and wave patterns
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Juvinile Myoclonic epilepsy
(sleep deprevation and alcohol Hx) |
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Describe 5 distrubutions of dystonia?
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focal : single body region
Segmental: contiguous body region Hemi-body: Ipsilateral arm and leg Multi-focal: > 2 non-contiguous body parts Generalized: entire body |
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Name 6 broad descriptions of the aetiology of dystonia
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primary dystonia
Dystonia + syndromes (no secondary cause and no degeration) Secondary dystonia (clear secondary cause is present such as brain injury and drug exposure) Heredodegenerative dystonia (neurodeg. process) Paoxysmal dystonia (episodic attack, no intervening signs/attacks) Psychogenic |
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Which gene is associated with Primary young onset dystonians?
What type of mutation is DYT1? What type of inheritance pattern does this exhibit? Which drug should be trialled in all young dsytonians |
DYT1 in 50-60% patients
GAG deletion on DYT1 coding for Torsin A Autosomal dominant inheritence with reduced penetrance Levodopa |
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What are the clinical characteristics of cervical dystonia (spinal torticollis)
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Abnormal head posture, head tremour and head pain
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Blepharospasm is characterised by?
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increased blink rate
forced eye closure difficulty opening eyes |
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Oromandibular Dystonia is characterised by?
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Jaw clenching (bruxism)
jaw in the open position lateral jaw shift |
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Spasmoidic dysphonia is characterised by?
(adductors type and abductor type or mixed) |
Voice breaks and strain
breathy voice |
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Limb dystonia
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action dystonias affecting fine motor tasks such as writing, playing an instrument and handling tools
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