Movement Disorders

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Movement Disorders

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Movement Disorders

  1. 1. Movement disorders* * Except Parkinsonism and tremor Submitted to AskTheNeurologist.Com in 2007 Author Anon.
  2. 2. What is a Movement disorder? <ul><li>“ A disorder which impairs the regulation of voluntary motor activity without directly affecting strength, sensation or cerebellar function ” </li></ul><ul><li>Sometimes also known as “ extrapyramidal disorders” </li></ul><ul><li>Many neurologists restrict the term “ extrapyramidal” to refer only to Parkinsonism </li></ul>
  3. 3. Types of abnormal movement <ul><li>Myoclonus </li></ul><ul><li>Ballismus </li></ul><ul><li>Chorea </li></ul><ul><li>Athetosis </li></ul><ul><li>Dystonia </li></ul><ul><li>Tics </li></ul><ul><li>Tremor ( discussed elsewhere ) </li></ul>
  4. 4. Myoclonus <ul><li>“ sudden, brief, shock-like involuntary movements” </li></ul><ul><li>May be caused by active muscle contraction </li></ul><ul><li>- positive myoclonus </li></ul><ul><li>May be caused by inhibition of ongoing muscle activity </li></ul><ul><li>- negative myoclonus ( eg. Asterixis ) </li></ul><ul><li>Generalised - widespread throughout body </li></ul><ul><li>Focal / segmental – restricted to particular part of body </li></ul>
  5. 5. Generalised myoclonus <ul><li>Physiologic - Nocturnal ( usually on falling asleep ) </li></ul><ul><li>- Hiccups </li></ul><ul><li>Essential - Occurs in the absence of other abnormality </li></ul><ul><li>- Benign and sometimes inherited </li></ul><ul><li>Epileptic - Demonstrable cortical source </li></ul><ul><li>Symptomatic i.e secondary to disease process </li></ul><ul><li>- Neurodegenerative eg. Wilson’s disease </li></ul><ul><li>- Infectious e.g CJD, Viral encephalitis </li></ul><ul><li>- Toxic e.g. penicillin, antidepressants </li></ul><ul><li>- Metabolic - anoxic brain damage </li></ul><ul><li>- hypoglycemia </li></ul><ul><li>- hepatic failure ( “ asterixis” ) </li></ul><ul><li>- renal failure </li></ul><ul><li>- hyponatremia….. And others </li></ul>
  6. 6. Segmental Myoclonus <ul><li>Can arise from lesions anywhere in the </li></ul><ul><li>CNS </li></ul><ul><li>Can arise from many of the processes that can cause generalised myoclonus </li></ul><ul><li>Example is “palatal myoclonus” which </li></ul><ul><li>arises due to any lesion that interrupts </li></ul><ul><li>pathway between red nucleus, </li></ul><ul><li>cerebellum and olivary nucleus. </li></ul>
  7. 7. Treatment of Myoclonus <ul><li>Valproic acid is drug of choice </li></ul><ul><li>May respond to benzodiazepines </li></ul><ul><li>e.g. clonazepam </li></ul>
  8. 8. Ballismus, Chorea, Athotosis and Dystonia <ul><li>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 </li></ul><ul><li>WHY? </li></ul>
  9. 9. Because…….. <ul><li>They often co-exist </li></ul><ul><li>Even neurologists may often not be able to agree as to how a particular movement should be classified! </li></ul><ul><li>They often ( with some notable exceptions ) have the same significance in terms of aetiology. </li></ul>
  10. 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. 11. Ballismus <ul><li>Violent “flinging” movement of entire limb </li></ul><ul><li>Almost always unilateral and therefore </li></ul><ul><li>use term “ HEMIBALLISMUS” </li></ul><ul><li>Involves proximal musculature and is sometimes thought of as a </li></ul><ul><li>“ proximal unilateral chorea ” </li></ul><ul><li>Usually due to a CVA in contralateral subthalamic nucleus </li></ul>
  12. 12. Chorea ( “dance” in Greek) <ul><li>Rapid irregular muscle jerks </li></ul><ul><li>May affect limbs, head, face and tongue </li></ul><ul><li>In the limbs chorea refers more to distal movements ( as proximal movements usually called ballismus) </li></ul><ul><li>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 </li></ul>
  13. 13. Athetosis “ changeable” in Greek <ul><li>Slow, flowing, often twisting movements </li></ul><ul><li>Occurs mainly distally ( hands, fingers) </li></ul><ul><li>Can also affect face and tongue </li></ul><ul><li>Often use term “ choreoathetosis ” due to overlap between syndromes ( chorea referring to less smooth , more jerky movements) </li></ul>
  14. 14. Dystonia <ul><li>“ condition in which the patient assumes a sustained, abnormal posture or limb position” </li></ul><ul><li>Due to co-contraction of agonist and antagonist muscles in part of body </li></ul><ul><li>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. </li></ul><ul><li>The part of the movement when the limb was held, unmoving, in an abnormal position would be considered a dystonia ( may occur alone). </li></ul>
  15. 15. Examples of focal dystonias <ul><li>Blepharospasm: </li></ul><ul><li>- involuntary forceful closure of eyes </li></ul><ul><li>Torticollis: </li></ul><ul><li>- Tendency of neck to twist to one side. </li></ul><ul><li>Writer’s cramp: </li></ul><ul><li>- Dystonic posturing of arm when hand used to perform specific tasks e.g. writing, playing piano </li></ul>
  16. 16. Botulinum toxin <ul><li>Derived from Clostridium botulinum </li></ul><ul><li>Prevents pre-synaptic release of Ach vesicles and therefore blocks neuro-muscular junction transmission </li></ul><ul><li>May be injected selectively into muscles responsible for focal dystonias, allowing relief for up to several months </li></ul><ul><li>Requires expertise; can cause more extensive paralysis than intended. </li></ul>
  17. 17. Causes of Chorea, Dystonia and athetosis <ul><li>Hereditary </li></ul><ul><li>Static Encephalopathy ( Cerebral Palsy ) </li></ul><ul><li>Drugs </li></ul><ul><li>Cerebrovascular ( ischaemia, haemorrhage ) </li></ul><ul><li>Structural lesions ( subthalamic nucleus) </li></ul><ul><li>Secondary to medical disorders </li></ul><ul><li>Miscellaneous - Sydenham’s chorea </li></ul><ul><li>- Chorea Gravidarum </li></ul><ul><li>- Sporadic idiopathic torsion dystonia </li></ul><ul><li>- Focal dystonias </li></ul>
  18. 18. Hereditary <ul><li>Huntington’s disease </li></ul><ul><li>Wilson’s disease </li></ul><ul><li>Neuroacanthocytosis </li></ul><ul><li>Hereditary dystonias - idiopathic torsion dystonia </li></ul><ul><ul><ul><ul><ul><li>- dopa - responsive dystonia </li></ul></ul></ul></ul></ul>
  19. 19. Wilson’s disease <ul><li>Autosomal recessive defect of copper excretion in which there is defective copper-binding to ceruloplasmin </li></ul><ul><li>Leads to copper deposition in: </li></ul><ul><li>- liver causing cirrhosis </li></ul><ul><li>- brain ( especially basal ganglia ) leading to movement disorders and other abnormalities </li></ul><ul><li>- Cornea leading to the appearance of rusty brown “ Kayser-Fleischer rings” around cornea (usually only seen with slit-lamp). </li></ul><ul><li>Diagnosis is via low serum ceruloplasmin, increased urinary Cu, liver biopsy ( excessive Cu) and brain MRI changes </li></ul><ul><li>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. </li></ul>
  20. 20. Idiopathic torsion dystonia <ul><li>Hereditary and sporadic forms </li></ul><ul><li>Variable inheritence </li></ul><ul><li>High incidence in Ashkenazi Jews </li></ul><ul><li>Onset may be in childhood / adulthood </li></ul><ul><li>Affects limbs, trunk, neck, face </li></ul><ul><li>Trial of L-dopa usually initiated </li></ul><ul><li>Level of disability variable </li></ul>
  21. 21. Dopa-responsive dystonia <ul><li>Autosomal dominant inheritence </li></ul><ul><li>Onset usually in childhood </li></ul><ul><li>Girls more severely affected </li></ul><ul><li>Some parkinsonian features - rigidity </li></ul><ul><ul><ul><ul><li>- bradykinesia </li></ul></ul></ul></ul><ul><li>Low levels of L-Dopa very effective </li></ul>
  22. 22. Drug-induced <ul><li>Drugs acting on dopaminergic system </li></ul><ul><li>- Antiparkinsonian </li></ul><ul><li>- Antipsychotic </li></ul><ul><li>Lithium </li></ul><ul><li>Phenytoin </li></ul><ul><li>Oral contraceptives </li></ul>
  23. 23. <ul><li>Anoxic brain damage ( post – CPR ) </li></ul><ul><li>Systemic lupus erythematosis </li></ul><ul><li>Hepatic failure </li></ul><ul><li>Endocrine - Thyrotoxicosis </li></ul><ul><li>- Addisons </li></ul><ul><li>Electrolyte - Low Ca, Mg, </li></ul><ul><li>- High Na </li></ul><ul><li>Polycythemia rubra vera </li></ul>Secondary to medical disorders (A SHEEP)
  24. 24. Sydenham’s chorea <ul><li>Mainly children / adolescents </li></ul><ul><li>Complication of previous group A streptococcal infection </li></ul><ul><li>Usually no recent history of infection </li></ul><ul><li>Acute / subacute onset </li></ul><ul><li>May have behavioural problems </li></ul><ul><li>Usually remits spontaneously </li></ul>
  25. 25. Chorea gravidarum <ul><li>Chorea of any cause that begins in pregnancy </li></ul><ul><li>May represent recurrence of Sydenham’s chorea. </li></ul><ul><li>Most commonly associated with anti-phospholipid syndrome +/- SLE </li></ul><ul><li>Usually resolves spontaneously </li></ul>
  26. 26. Tics <ul><li>Recurrent, sterotyped abnormal movements </li></ul><ul><li>May be suppressed voluntarily or with distraction </li></ul><ul><li>Voluntary suppression leads to anxiety and a build-up of internal unrest. </li></ul><ul><li>Worsen under stress </li></ul>
  27. 27. Summary <ul><li>Movement disorders are often difficult to define precisely, but have similar differential diagnoses. </li></ul><ul><li>They are often a manifestation of a more widespread neurological or internal medical problem. </li></ul><ul><li>Other than the specific treatments mentioned, most details of therapy are beyond the scope of this course. </li></ul><ul><li>In some cases treatment includes treatment of underlying cause e.g. Wilson’s disease </li></ul>
  28. 28. Submitted to AskTheNeurologist.Com in 2007 Author Anon.

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