CHOREA AND BALLISMUSDr PS Deb

Director Neurology

GNRC Hospitals Assam

Guwahati

CHOREA State of excessive, spontaneous movement,

irregularly timed, non repetitive, randomly distributed and abnormal in character.

It may very in severity from restlessness with mild intermittent exaggeration of gesture and expression, fidgeting movement of hands unstable, movement of hands.

Unstable gait to continuous flow of disabling violent movement.

Random – distribution, time, and duration

CHOREA Associated with

Hypotonia – Reduced long latency reflex Motor impersistence → inability to sustain vol.

movement Pronator sign Milkmaids sign Trombone tongue

Hung-up reflex – Prolonged contraction of stretched muscles by late sensory provoked choric movement.

PATHOPHYSIOLOGY

Stiatum Pallidum

ThalamusPC PRSN

Cortex

Cord

Chorea

DA GABA

GABA

GABA

GABA

CHOREA MECHANISM - NEUROTRANSMITTER DISTURBANCE Reduced: GABA, Sub P, Ach Increased: Dopamine, NA in Striatum and pallidum,

Somatostatine GABA in SN and GP due to degeneration of

striatum, but gabargic drugs does not reduce chorea Dopamine Tyrosin hydroxylase enzyme in SN,

chorea reduced by anti dopa drugs and by Ldopa Ach marginally reduced, choline esterase enzyme

mild reduced, large striatal interneuron well maintained, Ach agonist does not improve chorea

CHOREA PATHOGENESIS PET - Histochemistry Drug effect Surgical

Chorea reduced by Pallidotomy, Nigrotomy, Thalamotomy

Pathological

Animal Model

ELECTROPHYSIOLOGICAL Choric discharges on EMG resemble normal

voluntary contraction Active inhibition of innervation Readiness potential not tested

id

Idea Programming ExecutionDischarge Discharge Chorea

Association Cortex

Basal Ganglia

Motor Cortex

MECHANISM OF CHOREA Chorea appears to be a fragments of normal

movements, appearing in inappropriate circumstances and lacking any purpose

They may be determined by peripheral stimuli which in ordinary circumstances would be ignored.

Basal ganglia normally filter the mass of cortical input, they receive to select movement appropriate to the circumstances.

Striatal damage might prevent normal suppression of unwanted motor response to external stimuli → Chorea

CAUSE OF CHOREA AND CHOREO-ATHETOSIS

1. Trauma1. Concussion

2. Neoplasm1. Primary brain tumor2. Metastasis3. CNS leukemia

3. Cerebrovascular disease1. Epidural subdural hemorrhage2. Hemorrhage3. Lacunar infarct4. AVM5. Polycythemia6. Migraine

4. Infection1. Post streptococcal2. Typhoid3. Pertusis4. Dyphtheria5. Neurosyphilis6. Tuberculosis7. Mycoplasma

5. Viral infection1. Exanthema2. Encephalitis

6. Collagen vascular disease1. MSV

CAUSE OF CHOREA AND CHOREO-ATHETOSIS CONT….7. Metabolic

1. Electrolyte imbalance2. Glucose metabolic disorder3. Endocrinal – Thyroid, Adrenal4. Copper metabolic impairment –

Wilson5. Renal failure6. Nutritional – thiamine, Niacin, B12

8. Intoxication1. Alcohol2. CO3. Mercury4. Manganese5. Thallium6. Glue sniffing

9. Drugs1. Neurolaptics2. Dopa agonists3. Anti cholinergic4. Sympathomimetics5. Steroid, estrogen6. Opiates7. INH8. Reserpine9. Anti-histaminics10. Tricycle antidepressants11. Lithium12. Metaclopromide13. Digoxin14. Lithium15. Diazoxide

CAUSE OF CHOREA AND CHOREO-ATHETOSIS CONT….

10. Hereditary1. Aminoaciduria2. Lipid 3. Glucose4. Protein metabolic 5. Huntington’s chorea6. Benign familial chorea7. Chorea with Acanthocytosis8. Familial inverted

choreoathetosis9. Familial striatal necrosis10. Familial basal ganglionic

calcification11. Spinocerebellar degeneration

11. Other1. Hallevorden spatz disease2. Ataxia Telengectasia3. Tuberous sclerosis4. Stuge Weber Syndrome5. Myoclonus epilepsy with

chorea6. Paraxysmal dystonic

choreoathetosis7. Kernicterus8. Cerebral palsy9. Electrical injury10. Thalamic dementia

THERAPY Drugs

Dopamine antagonist Haloperidol Tetrabenazine Pimozide Perphenazine

Cholinergic drugs Lecithine → ↑ cerebral choline

GABA agonist INH Sod. Valproate

SURGICAL THERAPY

BALLISMProximal, flinging, violent, involuntary movement

MECHANISM OF BALLISMUS Surgical Pallidotomy, Nigrotomy,

thalamotomy reduces ballismus Animal Substantia Nigra lesion causes

hemiballismus Experimental destruction of Striata nigra no

ballismus, 20% destruction → hemiballismus Striatal dopamine increased Subthalamic GABA reduced

SITE OF LESION CAUSES BALLISMUS Sbuthalamus Pallidum Substantia nigra Thalamus Post. Central gyrus Superior frontal gyrus Precentral gyrus

PATHOPHYSIOLOGY

Subthalamic.neucli Pallidum

ThalamusPC PRSN

Cortex

Cord

Ballismus

GABA

GABA

GABA

GABA

ETIOLOGY BALLISMUS Vascular – commonest

Lacunar infarct, TIA Hemorrhage Subarachnoid hemorrhage AVM Venous angioma

Tumor Secondaries Cyst

Infection Tuberculloma, TBM Syphilis

Metabolic Hyperglycemia

Drugs Contraceptive L-dopa

Truama Head injury Post surgical

(Parkinson disease) Multiple sclerosis

TYPES Monoballismus Hemiballismus Paraballismus Biballismus Prognosis

Variable – few days to years

TREATMENT Drug

Dopamine antagonists Halloparidol Tetrabenazine Thiopropazate Pimozide Perphenazine

GABAargic Sod. Valproate Benzodiazepine

Diazepam Clonazepam

Surgical – Pallidotomy, Nigrotomy, Thalamotomy

THANKS