Post on 07-May-2015
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