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    Movement Disorders 4335

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    Fitri Amelia Rizki
    Movement disorders impair voluntary motor activity without affecting strength, sensation or cerebellar function. They include myoclonus, ballismus, chorea, athetosis, dystonia and tics. These disorders exist on a spectrum, can overlap, and often have the same underlying causes such as genetic conditions, drugs, medical illnesses or brain lesions. Treatment depends on the specific type and cause but may include medications like valproic acid or botulinum toxin injections. Movement disorders can be difficult to precisely define and classify, but understanding their etiology is important for guiding treatment and management.

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    Movement Disorders 4335

    Uploaded by

    Fitri Amelia Rizki
    57 views28 pages
    Movement disorders impair voluntary motor activity without affecting strength, sensation or cerebellar function. They include myoclonus, ballismus, chorea, athetosis, dystonia and tics. These disorders exist on a spectrum, can overlap, and often have the same underlying causes such as genetic conditions, drugs, medical illnesses or brain lesions. Treatment depends on the specific type and cause but may include medications like valproic acid or botulinum toxin injections. Movement disorders can be difficult to precisely define and classify, but understanding their etiology is important for guiding treatment and management.

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    movement disorder

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    Movement disorders impair voluntary motor activity without affecting strength, sensation or cerebellar function. They include myoclonus, ballismus, chorea, athetosis, dystonia and tics. These disorders exist on a spectrum, can overlap, and often have the same underlying causes such as genetic conditions, drugs, medical illnesses or brain lesions. Treatment depends on the specific type and cause but may include medications like valproic acid or botulinum toxin injections. Movement disorders can be difficult to precisely define and classify, but understanding their etiology is important for guiding treatment and management.

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    © All Rights Reserved
    Download as PPT, PDF, TXT or read online from Scribd
    Download as ppt, pdf, or txt
    57 views28 pages

    Movement Disorders 4335

    Uploaded by

    Fitri Amelia Rizki
    Movement disorders impair voluntary motor activity without affecting strength, sensation or cerebellar function. They include myoclonus, ballismus, chorea, athetosis, dystonia and tics. These disorders exist on a spectrum, can overlap, and often have the same underlying causes such as genetic conditions, drugs, medical illnesses or brain lesions. Treatment depends on the specific type and cause but may include medications like valproic acid or botulinum toxin injections. Movement disorders can be difficult to precisely define and classify, but understanding their etiology is important for guiding treatment and management.

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    © All Rights Reserved
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    Download as ppt, pdf, or txt
    of 28

    Movement disorders*

    * Except Parkinsonism and tremor


    Submitted to
    AskTheNeurologist.Com
    in 2007
    Author Anon.
    What is a Movement 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
    Types of abnormal movement
    Myoclonus
    Ballismus
    Chorea
    Athetosis
    Dystonia
    Tics
    Tremor ( discussed elsewhere )
    Myoclonus
    sudden, brief, shock-like involuntary movements

    May be caused by active muscle contraction
    - positive myoclonus

    May be caused by inhibition of ongoing muscle activity
    - negative myoclonus ( eg. Asterixis )

    Generalised - widespread throughout body
    Focal / segmental restricted to particular part of body
    Generalised myoclonus
    Physiologic - Nocturnal ( usually on falling asleep )
    - Hiccups
    Essential - Occurs in the absence of other

    abnormality
    - Benign and sometimes inherited
    Epileptic - Demonstrable cortical source
    Symptomatic i.e secondary to disease process
    - Neurodegenerative eg. Wilsons disease
    - Infectious e.g CJD, Viral encephalitis
    - Toxic e.g. penicillin, antidepressants
    - Metabolic - anoxic brain damage

    - hypoglycemia

    - hepatic failure ( asterixis )

    - renal failure

    - hyponatremia.. And others
    Segmental Myoclonus
    Can arise from lesions anywhere in the
    CNS
    Can arise from many of the processes that
    can cause generalised myoclonus
    Example is palatal myoclonus which
    arises due to any lesion that interrupts
    pathway between red nucleus,
    cerebellum and olivary nucleus.
    Treatment of Myoclonus
    Valproic acid is drug of choice


    May respond to benzodiazepines
    e.g. clonazepam
    Ballismus, Chorea, Athotosis
    and Dystonia
    These should NOT be thought of as
    separate entities amenable to specific
    definition but rather as a SPECTRUM of
    movements that blend into one-another



    WHY?
    Because..
    They often co-exist
    Even neurologists may often not be
    able to agree as to how a particular
    movement should be classified!
    They often ( with some notable
    exceptions ) have the same
    significance in terms of aetiology.
    The spectrum
    Ballismus Dystonia Chorea 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
    Ballismus
    Violent flinging movement of entire limb
    Almost always unilateral and therefore
    use term HEMIBALLISMUS
    Involves proximal musculature and is
    sometimes thought of as a
    proximal unilateral chorea
    Usually due to a CVA in contralateral
    subthalamic nucleus
    Chorea ( dance in Greek)
    Rapid irregular muscle jerks
    May affect limbs, head, face and tongue

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

    Patients often attempt to conceal involuntary
    movements by superimposing voluntary
    movements onto them e.g. an involuntary
    movement of arm towards face may be adapted
    to look-like an attempt to look at watch
    Athetosis changeable in Greek
    Slow, flowing, often twisting
    movements
    Occurs mainly distally ( hands, fingers)
    Can also affect face and tongue
    Often use term choreoathetosis due
    to overlap between syndromes ( chorea
    referring to less smooth , more jerky
    movements)
    Dystonia
    condition in which the patient assumes a sustained,
    abnormal posture or limb position
    Due to co-contraction of agonist and antagonist
    muscles in part of body
    Can be thought of as an athetoid movement that
    gets stuck for a period of time; thus, a patient with
    choreoathetosis may perform an involuntary
    movement in which his hand and fingers are twisted
    behind his head. He may hold this position for a few
    moments before his hand moves back in front of his
    body.
    The part of the movement when the limb was held,
    unmoving, in an abnormal position would be
    considered a dystonia ( may occur alone).
    Examples of focal dystonias
    Blepharospasm:
    - involuntary forceful closure of eyes
    Torticollis:
    - Tendency of neck to twist to one side.
    Writers cramp:
    - Dystonic posturing of arm when hand
    used to perform specific tasks e.g.
    writing, playing piano
    Botulinum toxin
    Derived from Clostridium botulinum
    Prevents pre-synaptic release of Ach
    vesicles and therefore blocks neuro-
    muscular junction transmission
    May be injected selectively into muscles
    responsible for focal dystonias, allowing
    relief for up to several months
    Requires expertise; can cause more
    extensive paralysis than intended.
    Causes of Chorea, Dystonia and
    athetosis
    Hereditary
    Static Encephalopathy ( Cerebral Palsy )
    Drugs
    Cerebrovascular ( ischaemia, haemorrhage )
    Structural lesions ( subthalamic nucleus)
    Secondary to medical disorders
    Miscellaneous - Sydenhams chorea
    - Chorea Gravidarum
    - Sporadic idiopathic torsion
    dystonia
    - Focal dystonias
    Hereditary
    Huntingtons disease

    Wilsons disease

    Neuroacanthocytosis

    Hereditary dystonias - idiopathic torsion dystonia
    - dopa - responsive dystonia

    Wilsons disease
    Autosomal recessive defect of copper excretion in which there is
    defective copper-binding to ceruloplasmin
    Leads to copper deposition in:
    - liver causing cirrhosis
    - brain ( especially basal ganglia )
    leading to movement disorders and
    other abnormalities
    - Cornea leading to the appearance of rusty
    brown Kayser-Fleischer rings around
    cornea (usually only seen with slit-lamp).
    Diagnosis is via low serum ceruloplasmin, increased urinary Cu,
    liver biopsy ( excessive Cu) and brain MRI changes

    Although it is rare it is very important to think-of and diagnose
    as it is TREATABLE:- Penicillamine is given to chelate the copper
    and promote excretion.
    Idiopathic torsion dystonia
    Hereditary and sporadic forms
    Variable inheritence
    High incidence in Ashkenazi Jews
    Onset may be in childhood / adulthood
    Affects limbs, trunk, neck, face
    Trial of L-dopa usually initiated
    Level of disability variable
    Dopa-responsive dystonia
    Autosomal dominant inheritence

    Onset usually in childhood
    Girls more severely affected
    Some parkinsonian features - rigidity
    - bradykinesia
    Low levels of L-Dopa very effective
    Drug-induced
    Drugs acting on dopaminergic system
    - Antiparkinsonian
    - Antipsychotic
    Lithium

    Phenytoin

    Oral contraceptives
    Anoxic brain damage ( post CPR )
    Systemic lupus erythematosis
    Hepatic failure
    Endocrine - Thyrotoxicosis
    - Addisons
    Electrolyte - Low Ca, Mg,
    - High Na
    Polycythemia rubra vera
    Secondary to medical disorders
    (A SHEEP)
    Sydenhams chorea
    Mainly children / adolescents
    Complication of previous group A
    streptococcal infection
    Usually no recent history of infection
    Acute / subacute onset
    May have behavioural problems
    Usually remits spontaneously
    Chorea gravidarum
    Chorea of any cause that begins in
    pregnancy
    May represent recurrence of
    Sydenhams chorea.
    Most commonly associated with anti-
    phospholipid syndrome +/- SLE
    Usually resolves spontaneously
    Tics
    Recurrent, sterotyped abnormal movements

    May be suppressed voluntarily or with
    distraction

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

    Worsen under stress
    Summary
    Movement disorders are often difficult to define precisely, but
    have similar differential diagnoses.

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

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

    In some cases treatment includes treatment of underlying
    cause e.g. Wilsons disease
    Submitted to
    AskTheNeurologist.Com
    in 2007
    Author Anon.

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