Movement Disorders
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Movement Disorders

Movement Disorders

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Movement Disorders Presentation Transcript

  • 1. Movement disorders* * Except Parkinsonism and tremor Submitted to AskTheNeurologist.Com in 2007 Author Anon.
  • 2. 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
  • 3. Types of abnormal movement
    • Myoclonus
    • Ballismus
    • Chorea
    • Athetosis
    • Dystonia
    • Tics
    • Tremor ( discussed elsewhere )
  • 4. 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
  • 5. 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. Wilson’s 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
  • 6. 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.
  • 7. Treatment of Myoclonus
    • Valproic acid is drug of choice
    • May respond to benzodiazepines
    • e.g. clonazepam
  • 8. 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?
  • 9. 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.
  • 10. 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
  • 11. 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
  • 12. 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
  • 13. 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)
  • 14. 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).
  • 15. Examples of focal dystonias
    • Blepharospasm:
    • - involuntary forceful closure of eyes
    • Torticollis:
    • - Tendency of neck to twist to one side.
    • Writer’s cramp:
    • - Dystonic posturing of arm when hand used to perform specific tasks e.g. writing, playing piano
  • 16. 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.
  • 17. Causes of Chorea, Dystonia and athetosis
    • Hereditary
    • Static Encephalopathy ( Cerebral Palsy )
    • Drugs
    • Cerebrovascular ( ischaemia, haemorrhage )
    • Structural lesions ( subthalamic nucleus)
    • Secondary to medical disorders
    • Miscellaneous - Sydenham’s chorea
    • - Chorea Gravidarum
    • - Sporadic idiopathic torsion dystonia
    • - Focal dystonias
  • 18. Hereditary
    • Huntington’s disease
    • Wilson’s disease
    • Neuroacanthocytosis
    • Hereditary dystonias - idiopathic torsion dystonia
            • - dopa - responsive dystonia
  • 19. Wilson’s 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.
  • 20. 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
  • 21. 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
  • 22. Drug-induced
    • Drugs acting on dopaminergic system
    • - Antiparkinsonian
    • - Antipsychotic
    • Lithium
    • Phenytoin
    • Oral contraceptives
  • 23.
    • 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)
  • 24. Sydenham’s 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
  • 25. Chorea gravidarum
    • Chorea of any cause that begins in pregnancy
    • May represent recurrence of Sydenham’s chorea.
    • Most commonly associated with anti-phospholipid syndrome +/- SLE
    • Usually resolves spontaneously
  • 26. 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
  • 27. 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. Wilson’s disease
  • 28. Submitted to AskTheNeurologist.Com in 2007 Author Anon.