Summary of Movement Disorders

Summary of Movement DisordersSamyuktha BalabhadraHypokinesiaReduced amount of normal free flowing, fluid movementUsually accompanied by abnormally increased toneHyperkinesiaIncreased movementSpontaneous, uncontrolled movementsOverviewHypokineticParkinsons DiseaseParkinsonian syndromesProgressive Supranuclear PalsyMultisystem AtrophyOlivopontocerebellar Degeneration (Sporadic Form)Striatonigral DegenerationShy-Drager SyndromeDiffuse Lewy Body DiseaseCorticobasal DegenerationDrug-Induced ParkinsonismDopa responsive DystoniaOther Non-Parkinsons Akinetic Rigid SyndromesHuntingtons Disease (Rigid or Juvenile Form)Wilsons DiseaseEssential TremorDepressionArthritis, Polymyalgia, FibromyalgiaHyperkineticRegular/PredictableTremorHemiballismPalatal MyoclonusIntermediateDystoniaMyokymiaAthetosisTicStereotypyMyorythmiaFleeting/UnpredictableFasciculationsMyoclonusChoreaDyskinesiasSpectrum. Do not think of a disease as strictly falling under 1 category. i.e. Parkinsons.3

Evaluating Abnormal MovementsLocationExtent of movementPattern, rhythm, recurrenceCourse, speed, frequencyAmplitude, forceRelationship to posture, rest, activity/exertion, time of dayResponse to heat and coldRelationship to emotional tension and excitementDegree of movement suppression by attention or use of sensory tricksPresence or absence of movements during sleepFor this evaluation, we make use of the one of the key components of the neuro exam. Observation.6Hyperkinetic: TremorSeries of involuntary, relatively rhythmic, purposeless, oscillatory movementsDistribution: unilateral or bilateral; distal parts of extremitiesClassification: Simple vs. CompoundRest vs. ActionPostural vs. KineticMany ways to classifyAssociated Conditions: Parkinsons disease: 2-6 Hz; resting tremorEssential tremor: most common movement disorder; high frequency; low amplitudePhysiologic: 8-12 HzAnxiety: postural tremorCerebellar disease: low frequency; intention tremorMedicationsMost commonly in distal but can occur in other parts as wellSimple vs Compound: one or many muscle groupsRest vs. Action: relaxed phase or appear in an activityPostural vs. Kinetic: when limbs maintained in antigravity i.e. arm stretch or when making voluntary movementCan classify by variations in location, rate, amplitude, etc. Intention Tremor: type of action tremor; finger to nose failureIf a tremor looks irregular, think about myoclonus.

7Hyperkinetic: ChoreaInvoluntary, irregular, purposeless, random, nonrhythmic, spontaneous, abrupt, brief, rapid, jerky, unsustainedPresent at rest; increased with activity, tension, stress, self-consciousnessDistribution: Distal part of upper extremitiesAssociated Conditions: Huntingtons Disease: chorea and athetosisRheumatic Fever (Sydenhams Chorea)Lesch-Nyhan SyndromeNiemann-PickMedications

Patients may be able to suppress movementsDistribution: most commonly in distal part of upper extremities; but can involve lower extremities, trunk, face, etc. , may affect vocal cords also; one extremity, half of the body, generalized, distal, proximalDisappears in sleepAppearance: piano playing; milkmaidHuntingtons: CAG repeat on Chr. 4 with anticipation; Huntingtin protein causes progressive degeneration of caudate and putamen; personality changesMany diseases can cause chorea; one special one in peds postpump chorea in kids after 8Hyperkinetic: AthetosisSlower, more sustained, larger amplitude than choreaInvoluntary, irregular, coarse, somewhat rhythmic, writhing or squirmingFlow from one part to anotherExacerbated by voluntary activity of another body partCauses: Usually congenital perinatal injury to basal gangliaOverlap with chorea

Flowdirection of movement changes randomly; Limbs in constant motionDisappears in sleepCauses: changes to caudate and putamen9Hyperkinetic: DystoniaSpontaneous, involuntary, sustained muscle contractionsForce affected parts of body into abnormal movements or posturesExtremities, neck, trunk, eyelids, face, vocal cordsPatterned and recur in same locationDuration varies may be seconds to hoursAssociated Conditions: Dystonia musculorum deformansWilsons DiseaseHallervorden-SpatzKernicterusAdvanced HuntingtonsCervical Dystonia (Spasmodic Torticollis)BlepharospasmWriters CrampMedicationsDescribed more as writhing movements, twistingDurationbrief=spasm; prolonged=postureSpasmodic Torticollis: tx w/ botoxMedications: dopaminergic drugs, cimetidine, anticonvulsants, CCBs10Hyperkinetic: MyoclonusSingle or repetitive, abrupt, brief, rapid, lightning like, jerky, arrhythmic, asynergic, involuntary contractionsPortions of muscles, entire muscles, or groupsMost commonly in extremities and trunkSymmetrically on both sides of bodyIrregular intervalsAssociated Conditions: Epileptic or NonepilepticProgressive Myoclonic EpilepsiesCreutzfeldt-Jakob DiseaseOpsoclonus-Myoclonus SyndromeMetabolic DisordersPsychogenicMost commonlymay involve facial muscles, jaws, tongue, pharynx, larynx; may be multifocal, diffuse, widespreadToo weak to cause joint movementSymmetrical appearance is unique to myoclonusMany classifications. Most significant is epileptic vs nonepileptic. PME: group of disorders with myoclonic or tonic-clonic seizures and neurologic declineOpso: neuroblastomaMetabolic: uremic and anoxic encephalopathy11Hyperkinetic: HemiballismusDramatic neurologic syndrome of wild, flinging, incessant movements on one side of the bodyRapid and forcefulDisappear only with deep sleepAssociated Conditions: Infarction or hemorrhage in contralateral subthalamic nucleusDescribed at times as an exaggerated choreaDifficult to tx12Hyperkinetic: DyskinesiaAll hyperkinetic movements are technically dyskinesias.Term for complex involuntary movements that do not fit into another category.

Orofacial dyskinesia: involuntary facial movements; tardive dyskinesiaOrofacialafter use of psychotropics13Hyperkinetic: OtherAsterixisMyorhythmiaTicAkathisiaStereotypyHyperekplexia Sleep-Related DyskinesiaFasciculationsMyokymiaSpasmAsterixis: inability to sustain normal muscle tone; liver flapMyorhythmia: slow, rhythmic alternating movements that resemble tremor; specific for Whipples diseaseTic: compulsive movement; Tourette syndromeAkathisia: inner restlessness and urge to move; psychotropic drugsStereotypy: repetitive, purposeless, involuntary, patterned; Retts syndrome hand wringing

Hyperekplexia: pathologic startle n absence of neurologic diseaseSleep-Related Dyskinesia: RLSFasciculations: fine, rapid, flickering or vermicular twitching movements due to contraction of a bundle of muscle fibersMyokymia: involuntary, spontaneous, localized, transient or persistent quivering movements; affect a few muscle bundlesSpasm: involuntary contraction of a muscle or group of muscles; Tetanus

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Hypokinetic: Parkinsons DiseasePresentation:Hypertonia: Cogwheel rigidityBradykinesia: slow movementMasked faciesLoss of arm swingTremor: at rest; rhythmic pill rollShuffling gait with en-bloc turnsPoor balance and increased risk of fallsPathophysiology:degeneration of substantia nigra and presence of Lewy bodies in surviving neurons Dopamine deficiencyIncreased output from Globus Pallidus interna and subthalamic nucleiExcessive inhibition of thalamus and suppression of cortical motor systemTreatment:Pharmacologic Dopamine ReplacementDeep Brain Stimulation

Hypokinetic: Parkinsonian DiseasesMultisystem Atrophy:Cause: degeneration of basal ganglia, cerebellum, anterior horn cells, cerebral cortex, and brainstem in varying combinationsPresentation: cerebellar ataxia, dementia, amyotrophy, parkinsonism, corticospinal tract dysfunction, dysautonomia, urinary dysfunctionVarious subtypesProgressive Supranuclear Palsy:Cause: degenerative changes in rostral brainstem and thalmusPresentation: impairment of downgaze, upgaze, then global gaze paresis; frontal lobe dementia; postural instability; pseudobulbar palsy; extensor axial rigidity; facial dystoniaCorticobasal DegenerationCause: abnormalities in basal ganglia and cerebral cortexPathology: asymmetric frontoparietal neuronal loss and gliosisDiffuse Lewy Body DiseasePresentation: Dementia + Parkinson features + PsychosisHypokinetic: Wilsons DiseaseAbnormal copper deposition in brain, especially basal ganglia, liver, eye and other tissuesDefect in ATP7B, ATPase involved in copper transportLabs: low ceruloplasmisnFeatures: Wing beating tremorKayser-Fleischer ringsCirrhosisHemolytic AnemiaRenal DiseaseTreatment: PenicillamineHypokinetic: Hallervorden-Spatz SyndromeMacroscopic rust-brown discoloration of globus pallidus and substantia nigra due to iron depositionClinical Features:Begins in fourth decadeRigidityInvoluntary movementsAtaxiaDystoniaPyramidal signsProgressive dementiaMRI: bilaterally symmetric low signal intensity in globus pallidus, due to iron deposition, surrounding a focus of high intensity due to gliosis Hypokinetic: Dentarubropallidoluysian AtrophyCAG repeatsChanges to dentate, globus pallidus externa, red nucleus, subthalamic nucleusClinical manifestations: ChoreoathetosisDystoniaDementiaMyoclonusAtaxiaReferencesCampbell, W. 2005.DeJong's The Neurologic Examination. 6th ed. Philadelphia (PA): Lippincott Williams & Wilkins.