Movement disorders. Dr Rick Leventer Royal Children s Hospital
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1 Movement disorders Dr Rick Leventer Royal Children s Hospital richard.leventer@rch.org.au
2 Movement disorders! conditions that affect the speed, fluency, quality, and ease of movement! usually affect TONE and POSTURE! Abnormal movements may be divided into: " Voluntary and involuntary " Positive and negative " Just plain BIZARRE
3 Movement disorders! Generally restricted to disorders secondary to pathology in the brain " Basal ganglia extrapyramidal " corticospinal tracts - pyramidal " Cerebellum WHITE MATTER BASAL GANGLIA CEREBELLUM
4 What do the basal ganglia do?! work away quietly (i.e. involuntarily and automatically) in the background to SET UP the MUSCLE TONE and POSTURE upon which voluntary movements can occur! extrapyramidal and pyramidal work together to produce normal movements! basal ganglia also involved with BEHAVIOUR and COGNITION i.e. psychiatric disturbances may co-exist (think Tourettes / Huntingtons / Parkinsons)
5 Circuits and chemicals! Neurotransmitters " acetylcholine " dopamine " GABA " glutamine " substance P
6 Movement disorders - POSITIVE
7 Movement disorders - NEGATIVE! rigidity! hypokinesia! bradykinesia
8 Definitions! Tremor " regular alternating or oscillatory movements involuntary movements " Resting or intention or both! Dystonia " Non-rhythmic sustained (unbalanced) muscle contractions often distorting body posture
9 Definitions! Dyskinesia: abnormal speed or fluency of movement " Hypokinesia: slowed or absent voluntary movement " Hyperkinesia: excessive involuntary movement " Bradykinesia: difficulty initiating movement! Tics " Idiosyncratic, nonrhythmic, rapid, suppressible, and repetitive " can be suppressed only for brief periods with conscious effort. " simple or complex
10 Definitions! Chorea " nonrhythmic, jerky, rapid, and nonsuppressible " primarily in distal muscles or the face. " Sometimes abnormal movements merge imperceptibly into purposeful or semipurposeful acts that mask the involuntary movements. " often occurs with athetosis as choreoathetosis! Athetosis " Movements are nonrhythmic, slow, writhing, sinuous, primarily in distal muscles; alternating postures of the proximal limbs often blend continuously to produce a flowing stream of movement.
11 Definitions! Hemiballismus " Movements are nonrhythmic, rapid, nonsuppressible, violent, and flinging; they occur unilaterally, primarily in the proximal arm! Myoclonus " Nonrhythmic, rapid, nonsuppressible, shocklike twitches occur, sometimes in multiple muscles simultaneously " Epileptic (cortical) or non-epileptic (subcortical)
12 Pathologies! Infections " primary " parainfectious, autoimmune! Ischaemia " HIE " stroke! Trauma! Tumours " neoplastic " paraneoplastic! Metabolic " neurotransmitter disorders " mitochondrial " Wilsons " PKAN! Autoimmune " SLE! Medications " anticonvulsants " antipsychotic
13 Chorea! Causes " Sydenham chorea " medications! AED! antipsychotics " Juvenile Huntingtons " SLE " NMDA antibodies " hyperthyroidism! Treatment " valproate " benzodiazepines " haloperidol " chlorpromazine " (penicillin)
14 Athetosis! Causes " HIE (cerebral palsy) " phenylketonuria! Treatment "? L-dopa "? DBS " Wilson disease " Niemann-Pick " PKAN " Leigh mitochondrial " kernicterus " medications
15 Dystonia! Causes " Primary! idiopathic torsion dystonia (DYT1)! paroxysmal torticollis! Segawa disease (DRD)! Paroxysmal " Secondary! HIE! Huntington! PKAN! Wilsons! Niemann-Pick! glutaric aciduria! medications! hyperthyroidism! Treatment "? AED "? benzodiazepines " benztropine for neurolepticinduced " Botox for focal dystonia " L-dopa trial " Artane for torsion dystonia " alcohol for torsion dystonia! * when you think functional, think dystonia / when you think dystonia, think functional*
16 Tremor! Causes " Benign essential physiological " Juvenile Parkinsons " Wilsons " medications " (Bobble-headed doll)! IIIrd V lesions " (Spasmus nutans)! episodic abnormal head posturing with nystagmus! Treatment " beta blockers " clonidine " primadone " DBS (Parkinsonian)
17 Myoclonus! Causes " Physiological (sleep) " Epileptic " HIE " autoimmune! opsoclonus / myoclonus " metabolic! Tay Sachs! Wilsons! NCL! mitochondrial " Medications! Treatment " benzodiazepines " valproate " levetiracetam
18 Tics! Causes " idiopathic! simple " transient " chronic! Tourette " Secondary! Sydenham! CO! post HIE! medications! Treatment " reassurance " clonidine " haloperidol " pimozide! * the main disabilities in tics disorders are not the tics* " 50% ADHD " 30% OCD
19 VIDEO VIGNETTES
20 Movement disorders - pearls! often present at rest, but usually increase with activity, stress and anxiety! usually absent during sleep (except severe torsion dystonia & ballismus)! not purposeful, but often masked by combining with normal movements! often mixed i.e. choreoathetosis! neither purely ipsilateral or contralateral! CP with diurnal variation give L-dopa trial! ask parents to video abnormal movements!
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