Movement DisordersMovement DisordersGeneral OverviewGeneral Overview

BASAL GANGLIA CIRCUITRYBASAL GANGLIA CIRCUITRY

HypokinesiasHypokinesiasParkinsonismParkinsonism– Akinesia/BradykinesiaAkinesia/Bradykinesia– RigidityRigidity– Motor FreezingMotor Freezing

SpasticitySpasticityApraxiasApraxiasTics (Blocking & Holding Tics, Obsessional Tics)Tics (Blocking & Holding Tics, Obsessional Tics)PsychiatricPsychiatric– CatatoniaCatatonia– Psychomotor DepressionPsychomotor Depression– Psychogenic ParkinsonismPsychogenic Parkinsonism

Cataplexy/Drop AttacksCataplexy/Drop AttacksHypothyroidism or other Endocrinologic DisordersHypothyroidism or other Endocrinologic DisordersHesitant Gait of the ElderlyHesitant Gait of the Elderly

Hyperkinesias and DyskinesiasHyperkinesias and Dyskinesias

TremorTremorChoreaChoreaDystoniaDystoniaMyoclonusMyoclonusAtaxia and DysmetriaAtaxia and DysmetriaStereotypies and TicsStereotypies and TicsAkathisiaAkathisiaMyokymia and SynkinesiasMyokymia and SynkinesiasRestless LegsRestless LegsPeriodic Hypnogogic Movements (PLMS)Periodic Hypnogogic Movements (PLMS)

Movement Disorders -- Movement Disorders -- ParkinsonismsParkinsonisms

Parkinson’s DiseaseParkinson’s DiseaseDiffuse Lewy Body DiseaseDiffuse Lewy Body DiseaseMultiple Systems Atrophy (+/- Ataxia)Multiple Systems Atrophy (+/- Ataxia)Progressive Supranuclear PalsyProgressive Supranuclear PalsyCorticobasal DegenerationCorticobasal DegenerationVascular ParkinsonismVascular ParkinsonismPost Traumatic ParkinsonismPost Traumatic ParkinsonismSecondary (Toxin, Medication, Metabolic)Secondary (Toxin, Medication, Metabolic)

PARKINSONISMSPARKINSONISMS

Idiopathic Parkinson’s DiseaseIdiopathic Parkinson’s Disease

Usually AsymmetricUsually AsymmetricCardinal SignsCardinal Signs– TremorTremor– RigidityRigidity– BradykinesiaBradykinesia

Levodopa ResponsiveLevodopa ResponsiveLater FindingsLater Findings– Postural InstabilityPostural Instability– Levodopa-unresponsive gait disorderLevodopa-unresponsive gait disorder– Non Motor Features (Depression, Sexual Dysfunction, Non Motor Features (Depression, Sexual Dysfunction,

Cognitive Dysfunction of Variable Severity)Cognitive Dysfunction of Variable Severity)

Diffuse Lewy Body DiseaseDiffuse Lewy Body Disease

Unlike PD, Lewy Bodies rapidly spready Unlike PD, Lewy Bodies rapidly spready throughout the brain, including the throughout the brain, including the cerebral cortexcerebral cortex

Levodopa Responsive ParkinsonismLevodopa Responsive Parkinsonism

Rapidly Progressive DementiaRapidly Progressive Dementia– HallucinationsHallucinations

Multiple Systems AtrophyMultiple Systems Atrophy

Cardinal FindingsCardinal Findings– Parkinsonism that is poorly responsive to LevodopaParkinsonism that is poorly responsive to Levodopa– Autonomic Failure (Low Blood Pressure)Autonomic Failure (Low Blood Pressure)– Cerebellar SignsCerebellar Signs– Long Tract Signs (Spasticity)Long Tract Signs (Spasticity)

Striato-Nigral Type (Parkinsonism First)Striato-Nigral Type (Parkinsonism First)

Shy-Drager Syndrome (Autonomic Failure First) Shy-Drager Syndrome (Autonomic Failure First)

Olivo-ponto-cerebellar Type (OPCA – Ataxia Olivo-ponto-cerebellar Type (OPCA – Ataxia First)First)

Progressive Supranuclear PalsyProgressive Supranuclear Palsy

Early Postural Instability and FallsEarly Postural Instability and Falls

Parkinsonism (Unresponsive to Parkinsonism (Unresponsive to Levodopa)Levodopa)

““Stone Face”Stone Face”

DementiaDementia

Ocular SignsOcular Signs

Corticobasal DegenerationCorticobasal Degeneration

Asymmetric Parkinsonism with Poor Asymmetric Parkinsonism with Poor Response to LevodopaResponse to Levodopa

Apraxia and Alien LimbApraxia and Alien Limb

Spasticity, Rigidity, DystoniaSpasticity, Rigidity, Dystonia

Gait and Balance ProblemsGait and Balance Problems

Dementia always occurs, but may be a Dementia always occurs, but may be a late featurelate feature

Drug Related ParkinsonismDrug Related Parkinsonism

Haldol and other antipsychotic Haldol and other antipsychotic medications cause symmetric findings that medications cause symmetric findings that are indistinguishable at times from are indistinguishable at times from Idiopathic Parkinson’s DiseaseIdiopathic Parkinson’s Disease

ReglanReglan is a dopamine blocker and is an is a dopamine blocker and is an important cause of Parkinsonism in elderly important cause of Parkinsonism in elderly patients (currently used as an anti-nausea patients (currently used as an anti-nausea medication)medication)

HYPERKINESIASHYPERKINESIAS

Movement Disorders -- TremorMovement Disorders -- Tremor

Essential TremorEssential Tremor– Limbs, Head, VoiceLimbs, Head, Voice

Dystonic TremorDystonic Tremor– Task Specific TremorsTask Specific Tremors

Midbrain Outflow TremorsMidbrain Outflow Tremors– Cerebellar TremorCerebellar Tremor– Rubral TremorRubral Tremor

Rest Tremor (Parkinsonism)Rest Tremor (Parkinsonism)Orthostatic TremorOrthostatic TremorExaggerated Physiologic Tremor Exaggerated Physiologic Tremor – Caffiene, Medication WithdrawalCaffiene, Medication Withdrawal

Other (Drug Related Tremor)Other (Drug Related Tremor)

Essential TremorEssential TremorCommonCommonUpper Extremity Tremor with Posture and/or Upper Extremity Tremor with Posture and/or ActionActionBilateral, usually roughly symmetric Bilateral, usually roughly symmetric Typically of Long-Standing DurationTypically of Long-Standing DurationOther Areas May Be InvolvedOther Areas May Be Involved– Head or JawHead or Jaw– VoiceVoice– Lower ExtremitiesLower Extremities

Tremor may produce disability, but often doesn’tTremor may produce disability, but often doesn’tNo clear association with other diseases or No clear association with other diseases or disordersdisorders

MyoclonusMyoclonus

Not a TremorNot a TremorSudden, shock-like muscle contractionsSudden, shock-like muscle contractionsFocal, Multifocal, or GeneralizedFocal, Multifocal, or GeneralizedMay be regular and rythmic, but usually May be regular and rythmic, but usually irregular and jerkyirregular and jerkyEpileptic or Non-EpilepticEpileptic or Non-Epileptic– Cortical (Epileptic)Cortical (Epileptic)– Brainstem or Spinal Segmental (Non-Brainstem or Spinal Segmental (Non-

Epileptic)Epileptic)

ChoreaChorea

Excessive Spontaneous MovementsExcessive Spontaneous Movements

Irregular, Random, Brief and AbruptIrregular, Random, Brief and Abrupt

Non-repetitiveNon-repetitive

Distal PredominanceDistal Predominance

Flitting randomly from one body part to anotherFlitting randomly from one body part to another

Purposeless, but may be disguised Purposeless, but may be disguised (parakinesias)(parakinesias)

Facial grimacing and abnormal respiratory Facial grimacing and abnormal respiratory soundssounds

Chorea -- CausesChorea -- Causes

MEDICATIONS (e.g. tardive dyskinesia)MEDICATIONS (e.g. tardive dyskinesia)– Haldol, other antipsychoticsHaldol, other antipsychotics– Reglan is an important cause of tardive Reglan is an important cause of tardive

dyskinesiadyskinesia

Huntington’s DiseaseHuntington’s DiseaseHemiballism (Stroke)Hemiballism (Stroke)Post-Infectious (Strep Infection)Post-Infectious (Strep Infection)PregnancyPregnancyOther DisordersOther Disorders

TicsTics

Semi-voluntary (e.g. suppressible), Semi-voluntary (e.g. suppressible), rapid,non-rythmic movements or soundsrapid,non-rythmic movements or sounds

Background of normal activityBackground of normal activity

Associated CompulsionsAssociated Compulsions

May be associated with OCDMay be associated with OCD

Occasionally tics are disabling, but the Occasionally tics are disabling, but the OCD is usually the more disabling OCD is usually the more disabling condition, if it existscondition, if it exists

AkathisiaAkathisia

Motor restlessness with a “desire to move”Motor restlessness with a “desire to move”

Difficult for patients to describeDifficult for patients to describe– ““I feel like I’m always crawling out of my skin”I feel like I’m always crawling out of my skin”

Often with associated vocalizations and Often with associated vocalizations and gruntinggrunting

Often very disablingOften very disabling

Cause: MEDICATIONS – antipsychotics Cause: MEDICATIONS – antipsychotics and Metoclopromide (Reglan)and Metoclopromide (Reglan)

DystoniaDystonia

Sustained, patterned muscle contractionsSustained, patterned muscle contractionsAgonists and AntagonistsAgonists and AntagonistsClinical FindingsClinical Findings– Repetitive Twisting or Squeezing MovementsRepetitive Twisting or Squeezing Movements– Fixed PosturesFixed Postures

LocalizationLocalization– FocalFocal

Face (Blepharospasm and Meige Syndrome)Face (Blepharospasm and Meige Syndrome)Neck (Cervical Dystonia)Neck (Cervical Dystonia)Limbs (Task Specific Dystonias)Limbs (Task Specific Dystonias)

– SegmentalSegmental– GeneralizedGeneralized

Causes of DystoniaCauses of Dystonia

Idiopathic (Most Cases)Idiopathic (Most Cases)

DRUG RELATEDDRUG RELATED– Antipsychotics and ReglanAntipsychotics and Reglan

Genetic (DYT-1 Gene)Genetic (DYT-1 Gene)

StructuralStructural– TraumaTrauma– StrokeStroke– Multiple Rare DiseasesMultiple Rare Diseases

AtaxiaAtaxia

Symptomatic Interruption of the Cerebellar Symptomatic Interruption of the Cerebellar PathwaysPathways– Dysmetria (Poor targeting of planned movements)Dysmetria (Poor targeting of planned movements)– Dysdiadochokinesia (Poor sequencing of planned Dysdiadochokinesia (Poor sequencing of planned

movements)movements)– Intention Tremor (Past Pointing)Intention Tremor (Past Pointing)

Gait AbnormalitiesGait Abnormalities– Wide based gaitWide based gait– Poor balancePoor balance

““Drunken Sailor’s Walk”Drunken Sailor’s Walk”

Causes of AtaxiaCauses of Ataxia

Lesion in the following areas of the brainLesion in the following areas of the brain– CerebellumCerebellum– PonsPons– Midbrain (Red Nucleus)Midbrain (Red Nucleus)– Olivary NucleusOlivary Nucleus– Thalamus (Cerebellar Inflow Nucleus)Thalamus (Cerebellar Inflow Nucleus)

Causes of AtaxiaCauses of Ataxia

Acute intoxication (Alcohol, Dilantin, others)Acute intoxication (Alcohol, Dilantin, others)Chronic Substance Abuse (including Alcohol)Chronic Substance Abuse (including Alcohol)StrokeStrokeMultiple SclerosisMultiple SclerosisBrain TumorsBrain TumorsGenetic AtaxiasGenetic Ataxias– Autosomal Dominant Ataxias Autosomal Dominant Ataxias – Autosomal Recessive AtaxiasAutosomal Recessive Ataxias

MetabolicMetabolic– Vitamin E, Thiamine, Vitamin E, Thiamine,

Multiple Systems AtrophyMultiple Systems Atrophy

Restless LegsRestless Legs

Bothersome urge to move legs at nightBothersome urge to move legs at night

Prevents sleepPrevents sleep

Treatment of Parkinson’s DiseaseTreatment of Parkinson’s Disease

Levodopa and Dopamine AgonistsLevodopa and Dopamine Agonists

Amantidine, MAO-B InhibitorsAmantidine, MAO-B Inhibitors

Deep Brain Stimulation (Subthalamic Deep Brain Stimulation (Subthalamic Nucleus, Globus Pallidus Interna)Nucleus, Globus Pallidus Interna)

Treatment of DystoniaTreatment of Dystonia

Artane, Valium, occasionally BaclofenArtane, Valium, occasionally Baclofen

Botulinum Toxin InjectionBotulinum Toxin Injection

Deep Brain Stimulation (Globus Pallidus Deep Brain Stimulation (Globus Pallidus Interna)Interna)

Treatment of Essential TremorTreatment of Essential Tremor

PrimidonePrimidone

Propanolol and other Beta BlockersPropanolol and other Beta Blockers

Deep Brain Stimulation (Thalamus)Deep Brain Stimulation (Thalamus)

Tremors of some patients are quite responsive Tremors of some patients are quite responsive to alcohol, and patients may self-medicateto alcohol, and patients may self-medicate

Treatment of Tardive (Post Treatment of Tardive (Post Dopamine-Blockade) Movement Dopamine-Blockade) Movement

DisordersDisordersPREVENTIONPREVENTION

Dopamine Depleting Agents (Difficult to Obtain Dopamine Depleting Agents (Difficult to Obtain in the U.S.)in the U.S.)

Ramp up dopamine blockade (often worsens the Ramp up dopamine blockade (often worsens the underlying pathologic process)underlying pathologic process)

Life Long Botulinum Toxin InjectionsLife Long Botulinum Toxin Injections

Deep Brain StimulationDeep Brain Stimulation

Complications of Dopamine Complications of Dopamine Blocking AgentsBlocking Agents

Reversible? Sequelae IncludeReversible? Sequelae Include– TremorTremor– ParkinsonismParkinsonism

Permanent and Difficult to Treat Sequelae Permanent and Difficult to Treat Sequelae IncludeInclude– Tardive Dyskinesia (Chorea-like syndrome)Tardive Dyskinesia (Chorea-like syndrome)– Tardive AkathisiaTardive Akathisia

– Tardive DystoniaTardive Dystonia (Long Term Botox, may need DBS)(Long Term Botox, may need DBS)

DEEP BRAIN STIMULATIONDEEP BRAIN STIMULATION

HypokinesiasHypokinesiasParkinsonismParkinsonism– Akinesia/BradykinesiaAkinesia/Bradykinesia– RigidityRigidity– Motor FreezingMotor Freezing

SpasticitySpasticityApraxiasApraxiasTics (Blocking & Holding Tics, Obsessional Tics)Tics (Blocking & Holding Tics, Obsessional Tics)PsychiatricPsychiatric– CatatoniaCatatonia– Psychomotor DepressionPsychomotor Depression– Psychogenic ParkinsonismPsychogenic Parkinsonism

Cataplexy/Drop AttacksCataplexy/Drop AttacksHypothyroidism or other Endocrinologic DisordersHypothyroidism or other Endocrinologic DisordersHesitant Gait of the ElderlyHesitant Gait of the Elderly

Hyperkinesias and DyskinesiasHyperkinesias and Dyskinesias

TremorTremorChoreaChoreaDystoniaDystoniaMyoclonusMyoclonusAtaxia and DysmetriaAtaxia and DysmetriaStereotypies and TicsStereotypies and TicsAkathisiaAkathisiaMyokymia and SynkinesiasMyokymia and SynkinesiasRestless LegsRestless LegsPeriodic Hypnogogic Movements (PLMS)Periodic Hypnogogic Movements (PLMS)