Treatment of Secondary Chorea: A Review of the Current Literature

Erin Feinstein; Ruth Walker

Treatment of Secondary Chorea: A Review of the Current Literature

Tremor and Other Hyperkinetic Movements

Volume 10 (2020): Issue 0

By: Erin Feinstein and Ruth Walker

Open Access

|Jul 2020

Figures & Tables

Table 1

Summary of causes of secondary chorea and empiric and symptomatic treatment recommendations. Defined abbreviations: ANNA-1, 2: anti-neuronal nuclear antibodies type 1 and 2, aCP: aceruloplasminemia, ASA: aspirin, ASP: antiphospholipid syndrome, CBZ: carbamazepine, CASPER2: contactin-associated protein-like 2, CRMP5: collapsin response mediator protein 5, DA: dopamine, DBS: deep brain stimulation, deuTBZ: deuterated TBZ, D2: dopamine 2 receptor, GAD65: glutamic acid decarboxylase, GLT1: glucose transport 1, GPi: globus pallidus interna, HAART: highly-active antiretroviral therapy, HIV: human immunodeficiency virus, HSV-6: herpes virus-6, IVIg: intravenous immunoglobulin, LID: levodopa induced dyskinesia, NBIA: neurodegeneration with brain iron accumulation disorders, Nf: neuroferritinopathy, PKD: kinesigenic dyskinesia, PnKD: non-kinesigenic dyskinesia, SLE: Systemic lupus erythematous, STN: subthalamic nucleus, TD: tardive dyskinesia, TBZ: tetrabenazine, WNV: West Nile virus, VPA: valproic Acid, VZV: varicella zoster virus.

Cause of secondary chorea	Etiologic treatment	Symptomatic treatment
1) Infectious
Sydenham chorea	Acute phase: penicillin 500 mg BID for 10 days or 1 IM dose Chronic phase: penicillin G 1.2 million daily for 21 days	VPA 5–20 mg/kg/day CBZ 15–20 mg/kg/day IVIg 1 mg/kg/day
VZV		ASA 81 mg/day VPA 5 mg/kg/day
HSV-6	Foscarnet 40 mg/day for 2 weeks	IVIg 1 g/kg/day for 5 days
Tick encephalitis		Haloperidol Dexamethasone Pentoxifylline Nitrazepam Midazolam
Influenza A, parvovirus B19 encephalitis, WNV		Supportive care
Syphilis	Benzathine penicillin 2.4 million units IV Penicillin G 2.4 million units IM for 14 days
HIV	HAART
HIV with toxoplasmosis	HAART Pyrimethamine sulfadiazine
2) Drug-induced
Cocaine		Drug cessation Supportive care
Methamphetamine		Drug cessation Supportive care
TD	TBZ 12–100 mg/day deuTBZ 24 or 36 mg/day VBZ 40 or 80 mg/day	Levetiracetam 500 mg- 300 mg daily
LID	DBS of GPi or STN Amantadine >200 mg/day	Medication cessation Medication reduction
3) Metabolic and Pregnancy-related
Diabetic non-ketotic hyperglycemia	Insulin	Supportive care
Hyperthyroidism	Methimazole 3mg/day	Beta-blocker (propranolol 20–60 mg/day, metoprolol 25 mg/day) DA D2 blocking agent (chlorpromazine)
Chorea gravidarum	Birth
Estrogen supplementation		Cessation of estrogen
Cobalamin deficiency	Acute: cyanocobalamin 1000 mcg/day for 10 days Chronic: cyanocobalamin 1000 mg/daily for 6 months
4) Autoimmune and paraneoplastic
APD	Anti-platelets Anticoagulation	IVIg Plasmapheresis Immunosuppressive agents (mycophenolate, methotrexate)
SLE	Anticoagulation	Methylprednisolone 500 mg/daily for 5 days IVIg
LGI1		Immunosuppression (methylprednisolone 1000 mg/day)
Celiac disease	Gluten-free diet
Paraneoplastic and non-paraneoplastic diseases (ANNA-1,2, anti-CASPR2, anti-CRMP5, anti-LGI1, anti-NMDA, anti-Hu, anti-Ri, anti-Yo, anti-GAD65)	Oncologic targeted treatment	Immunosuppression Corticosteroids Cyclophosphamide IVIg
5) Genetic non-HD
GLUT1 deficiency	Ketogenic diet
PKD		Phenytoin CBZ Benzodiazepines VPA Gabapentin Lamotrigine Levetiracetam Oxcarbazepine
PnKD	Trigger avoidance	Benzodiazepines
aCP	Chelating agents (deferasirox 1000 mg/day or desferrioxamine 1000 mg/day for 5 day) Deferiprone 500 mg/day with fresh frozen plasma Ceruloplasmin
NBIA	Chelating agents (deferiprone 15 mg/kg BID
Nf		DA modulating agents (sulpiride 400 mg/day, TBZ up to 125 mg/day)
Wilson’s disease	Chelating agents (Trientine, D-Penicillamine, tetrathiomolybdate) Zinc