movement disorders

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Movement Disorders

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Page 1: Movement Disorders

Movement Movement disorders*disorders*

* Except Parkinsonism and tremor

Submitted to Submitted to AskTheNeurologist.ComAskTheNeurologist.Com

in 2007in 2007

Author Anon.Author Anon.

Page 2: Movement Disorders

What is a Movement What is a Movement disorder?disorder?

“ A disorder which impairs the regulation of voluntary motor activity without directly affecting strength, sensation or cerebellar

function ”

• Sometimes also known as “ extrapyramidal disorders”

• Many neurologists restrict the term “ extrapyramidal” to refer only to Parkinsonism

Page 3: Movement Disorders

Types of abnormal Types of abnormal movementmovement

• Myoclonus

• Ballismus

• Chorea

• Athetosis

• Dystonia

• Tics

• Tremor ( discussed elsewhere )

Page 4: Movement Disorders

MyoclonusMyoclonus

•“ “ sudden, brief, shock-like involuntary movements”sudden, brief, shock-like involuntary movements”

• May be caused by active muscle contraction May be caused by active muscle contraction

- positive myoclonus- positive myoclonus

• May be caused by inhibition of ongoing muscle activityMay be caused by inhibition of ongoing muscle activity

- negative myoclonus ( eg. Asterixis ) - negative myoclonus ( eg. Asterixis )

• Generalised - widespread throughout bodyGeneralised - widespread throughout body

• Focal / segmental – restricted to particular part of bodyFocal / segmental – restricted to particular part of body

Page 5: Movement Disorders

Generalised myoclonusGeneralised myoclonus• PhysiologicPhysiologic - Nocturnal ( usually on falling asleep )- Nocturnal ( usually on falling asleep )

- Hiccups- Hiccups• EssentialEssential - Occurs in the absence of other - Occurs in the absence of other

abnormality abnormality- Benign and sometimes inherited- Benign and sometimes inherited

• EpilepticEpileptic - Demonstrable cortical source- Demonstrable cortical source• Symptomatic Symptomatic i.e secondary to disease processi.e secondary to disease process

- Neurodegenerative eg. Wilson’s disease- Neurodegenerative eg. Wilson’s disease- Infectious e.g CJD, Viral encephalitis- Infectious e.g CJD, Viral encephalitis- Toxic e.g. penicillin, antidepressants- Toxic e.g. penicillin, antidepressants- Metabolic - Metabolic - anoxic brain damage- anoxic brain damage

- hypoglycemia- hypoglycemia- hepatic failure ( “ asterixis” )- hepatic failure ( “ asterixis” )- renal failure- renal failure- hyponatremia….. And others- hyponatremia….. And others

Page 6: Movement Disorders

Segmental MyoclonusSegmental Myoclonus

• Can arise from lesions anywhere in the Can arise from lesions anywhere in the

CNSCNS

• Can arise from many of the processes Can arise from many of the processes that that can cause generalised myoclonuscan cause generalised myoclonus

• Example is “palatal myoclonus” which Example is “palatal myoclonus” which

arises due to any lesion that interrupts arises due to any lesion that interrupts

pathway between red nucleus, pathway between red nucleus,

cerebellum and olivary nucleus.cerebellum and olivary nucleus.

Page 7: Movement Disorders

Treatment of MyoclonusTreatment of Myoclonus

• Valproic acid is drug of choiceValproic acid is drug of choice

• May respond to benzodiazepines May respond to benzodiazepines

e.g. clonazepame.g. clonazepam

Page 8: Movement Disorders

Ballismus, Chorea, Athotosis Ballismus, Chorea, Athotosis and Dystoniaand Dystonia

These should NOT be thought of as These should NOT be thought of as separate entities amenable to specific separate entities amenable to specific definition but rather as a SPECTRUM definition but rather as a SPECTRUM of movements that blend into one-of movements that blend into one-anotheranother

WHY?WHY?

Page 9: Movement Disorders

Because……..Because……..

• They often co-exist They often co-exist

• Even neurologists may often not Even neurologists may often not be be able to agree as to how a able to agree as to how a particular particular movement movement should be classified!should be classified!

• They often ( with some notable They often ( with some notable exceptions ) have the same exceptions ) have the same

significance in terms of aetiology. significance in terms of aetiology.

Page 10: Movement Disorders

The spectrumThe spectrum

Ballismus DystoniaChorea Athetosis

Movements become - Less violent / explosive / jerky

- Smoother and more flowing

- More sustained

They differ from tics in that they cannot be suppressed by voluntary control

Page 11: Movement Disorders

BallismusBallismus

• Violent “flinging” movement of entireViolent “flinging” movement of entirelimblimb

• Almost always unilateral and thereforeAlmost always unilateral and therefore

use term “ HEMIBALLISMUS”use term “ HEMIBALLISMUS”

• Involves proximal musculature and is Involves proximal musculature and is sometimes thought of as a sometimes thought of as a

“ “ proximal unilateral chorea ”proximal unilateral chorea ”

• Usually due to a CVA in contralateral Usually due to a CVA in contralateral subthalamic nucleussubthalamic nucleus

Page 12: Movement Disorders

Chorea ( “dance” in Greek)Chorea ( “dance” in Greek)

• Rapid irregular muscle jerksRapid irregular muscle jerks• May affect limbs, head, face and tongueMay affect limbs, head, face and tongue

• In the limbs chorea refers more to distal In the limbs chorea refers more to distal movements ( as proximal movements movements ( as proximal movements usually called ballismus)usually called ballismus)

• Patients often attempt to conceal involuntary Patients often attempt to conceal involuntary movements by superimposing voluntary movements by superimposing voluntary movements onto them e.g. an involuntary movements onto them e.g. an involuntary movement of arm towards face may be movement of arm towards face may be adapted to look-like an attempt to look at adapted to look-like an attempt to look at watchwatch

Page 13: Movement Disorders

Athetosis “ changeable” in Athetosis “ changeable” in GreekGreek• Slow, flowing, often twisting Slow, flowing, often twisting

movementsmovements• Occurs mainly distally ( hands, Occurs mainly distally ( hands,

fingers)fingers)• Can also affect face and tongueCan also affect face and tongue• Often use term “ choreoathetosis ” Often use term “ choreoathetosis ”

due to overlap between syndromes due to overlap between syndromes ( chorea referring to less smooth , ( chorea referring to less smooth , more jerky movements)more jerky movements)

Page 14: Movement Disorders

DystoniaDystonia

• “ “ condition in which the patient assumes a sustained, condition in which the patient assumes a sustained, abnormal posture or limb position”abnormal posture or limb position”

• Due to co-contraction of agonist and antagonist Due to co-contraction of agonist and antagonist muscles in part of bodymuscles in part of body

• Can be thought of as an athetoid movement that “gets Can be thought of as an athetoid movement that “gets stuck” for a period of time; thus, a patient with stuck” for a period of time; thus, a patient with choreoathetosis may perform an involuntary movement choreoathetosis may perform an involuntary movement in which his hand and fingers are twisted behind his in which his hand and fingers are twisted behind his head. He may hold this position for a few moments head. He may hold this position for a few moments before his hand moves back in front of his body.before his hand moves back in front of his body.

• The part of the movement when the limb was held, The part of the movement when the limb was held, unmoving, in an abnormal position would be considered unmoving, in an abnormal position would be considered a dystonia ( may occur alone).a dystonia ( may occur alone).

Page 15: Movement Disorders

Examples of focal dystoniasExamples of focal dystonias

• Blepharospasm:Blepharospasm:

-- involuntary forceful closure of eyesinvoluntary forceful closure of eyes

• Torticollis:Torticollis:

- - Tendency of neck to twist to one Tendency of neck to twist to one side.side.

• Writer’s cramp:Writer’s cramp:

- - Dystonic posturing of arm when Dystonic posturing of arm when hand hand used to perform specific tasks used to perform specific tasks e.g. e.g. writing, playing piano writing, playing piano

Page 16: Movement Disorders

Botulinum toxinBotulinum toxin

• Derived from Derived from Clostridium botulinumClostridium botulinum• Prevents pre-synaptic release of Ach Prevents pre-synaptic release of Ach

vesicles and therefore blocks neuro-vesicles and therefore blocks neuro-muscular junction transmissionmuscular junction transmission

• May be injected selectively into May be injected selectively into muscles responsible for focal muscles responsible for focal dystonias, allowing relief for up to dystonias, allowing relief for up to several monthsseveral months

• Requires expertise; can cause more Requires expertise; can cause more extensive paralysis than intended.extensive paralysis than intended.

Page 17: Movement Disorders

Causes of Chorea, Dystonia Causes of Chorea, Dystonia and athetosisand athetosis

• HereditaryHereditary

• Static Encephalopathy ( Cerebral Palsy )Static Encephalopathy ( Cerebral Palsy )

• DrugsDrugs

• Cerebrovascular ( ischaemia, haemorrhage )Cerebrovascular ( ischaemia, haemorrhage )

• Structural lesions ( subthalamic nucleus)Structural lesions ( subthalamic nucleus)

• Secondary to medical disordersSecondary to medical disorders

• MiscellaneousMiscellaneous - Sydenham’s chorea- Sydenham’s chorea

- Chorea Gravidarum- Chorea Gravidarum

- Sporadic idiopathic torsion - Sporadic idiopathic torsion dystonia dystonia

- Focal dystonias- Focal dystonias

Page 18: Movement Disorders

HereditaryHereditary

• Huntington’s diseaseHuntington’s disease

• Wilson’s diseaseWilson’s disease

• NeuroacanthocytosisNeuroacanthocytosis

• Hereditary dystoniasHereditary dystonias - idiopathic torsion dystonia- idiopathic torsion dystonia

- dopa - - dopa - responsiveresponsive dystoniadystonia

Page 19: Movement Disorders

Wilson’s diseaseWilson’s disease• Autosomal recessive defect of copper excretion in which Autosomal recessive defect of copper excretion in which

there is defective copper-binding to ceruloplasminthere is defective copper-binding to ceruloplasmin• Leads to copper deposition in:Leads to copper deposition in:

- - liver causing cirrhosisliver causing cirrhosis-- brain ( especially basal ganglia ) brain ( especially basal ganglia )

leading to movement disorders and leading to movement disorders and other abnormalitiesother abnormalities

-- Cornea leading to the appearance of rusty Cornea leading to the appearance of rusty brown “ Kayser-Fleischer rings” around brown “ Kayser-Fleischer rings” around cornea (usually only seen with slit-lamp).cornea (usually only seen with slit-lamp).

• Diagnosis is via low serum ceruloplasmin, increased urinary Diagnosis is via low serum ceruloplasmin, increased urinary Cu, liver biopsy ( excessive Cu) and brain MRI changesCu, liver biopsy ( excessive Cu) and brain MRI changes

• Although it is rare it is very important to think-of and Although it is rare it is very important to think-of and diagnose as it is TREATABLE:- Penicillamine is given to diagnose as it is TREATABLE:- Penicillamine is given to chelate the copper and promote excretion.chelate the copper and promote excretion.

Page 20: Movement Disorders

Idiopathic torsion dystoniaIdiopathic torsion dystonia

• Hereditary and sporadic formsHereditary and sporadic forms

• Variable inheritenceVariable inheritence

• High incidence in Ashkenazi JewsHigh incidence in Ashkenazi Jews

• Onset may be in childhood / Onset may be in childhood / adulthoodadulthood

• Affects limbs, trunk, neck, faceAffects limbs, trunk, neck, face

• Trial of L-dopa usually initiatedTrial of L-dopa usually initiated

• Level of disability variableLevel of disability variable

Page 21: Movement Disorders

Dopa-responsive dystoniaDopa-responsive dystonia

• Autosomal dominant inheritenceAutosomal dominant inheritence

• Onset usually in childhoodOnset usually in childhood

• Girls more severely affectedGirls more severely affected

• Some parkinsonian features Some parkinsonian features - rigidity- rigidity

- - bradykinesiabradykinesia Low levels of L-Dopa very effectiveLow levels of L-Dopa very effective

Page 22: Movement Disorders

Drug-inducedDrug-induced

• Drugs acting on dopaminergic systemDrugs acting on dopaminergic system

- Antiparkinsonian- Antiparkinsonian

- Antipsychotic- Antipsychotic

• LithiumLithium

• PhenytoinPhenytoin

• Oral contraceptivesOral contraceptives

Page 23: Movement Disorders

• Anoxic brain damage ( post – CPR ) Anoxic brain damage ( post – CPR )

• Systemic lupus erythematosisSystemic lupus erythematosis

• Hepatic failureHepatic failure

• EndocrineEndocrine - Thyrotoxicosis- Thyrotoxicosis

- Addisons- Addisons

• ElectrolyteElectrolyte - Low Ca, Mg, - Low Ca, Mg,

- High Na- High Na

• Polycythemia rubra veraPolycythemia rubra vera

Secondary to medical Secondary to medical disordersdisorders(A SHEEP)(A SHEEP)

Page 24: Movement Disorders

Sydenham’s choreaSydenham’s chorea

• Mainly children / adolescentsMainly children / adolescents

• Complication of previous group A Complication of previous group A streptococcal infectionstreptococcal infection

• Usually no recent history of infectionUsually no recent history of infection

• Acute / subacute onsetAcute / subacute onset

• May have behavioural problems May have behavioural problems

• Usually remits spontaneouslyUsually remits spontaneously

Page 25: Movement Disorders

Chorea gravidarumChorea gravidarum

• Chorea of any cause that begins in Chorea of any cause that begins in pregnancypregnancy

• May represent recurrence of May represent recurrence of Sydenham’s chorea.Sydenham’s chorea.

• Most commonly associated with Most commonly associated with anti-phospholipid syndrome +/- SLEanti-phospholipid syndrome +/- SLE

• Usually resolves spontaneouslyUsually resolves spontaneously

Page 26: Movement Disorders

TicsTics

• Recurrent, sterotyped abnormal movementsRecurrent, sterotyped abnormal movements

• May be suppressed voluntarily or with May be suppressed voluntarily or with distractiondistraction

• Voluntary suppression leads to anxiety and Voluntary suppression leads to anxiety and a build-up of internal unrest.a build-up of internal unrest.

• Worsen under stressWorsen under stress

Page 27: Movement Disorders

SummarySummary

• Movement disorders are often difficult to define Movement disorders are often difficult to define precisely, but have similar differential diagnoses.precisely, but have similar differential diagnoses.

• They are often a manifestation of a more widespread They are often a manifestation of a more widespread neurological or internal medical problem.neurological or internal medical problem.

• Other than the specific treatments mentioned, most Other than the specific treatments mentioned, most details of therapy are beyond the scope of this course.details of therapy are beyond the scope of this course.

• In some cases treatment includes treatment of In some cases treatment includes treatment of underlying cause e.g. Wilson’s disease underlying cause e.g. Wilson’s disease

Page 28: Movement Disorders

Submitted to Submitted to AskTheNeurologist.ComAskTheNeurologist.Com

in 2007in 2007

Author Anon.Author Anon.