Neuroacanthocytosis Syndromes Medication

Updated: Oct 16, 2018
  • Author: Kenneth B V Gross, MD; Chief Editor: Selim R Benbadis, MD  more...
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Medication Summary

Medication for chorea and dyskinesia is usually inadequate but is worth trying.

Haloperidol, tetrabenazine, and diazepam have been used in high doses in selected patients with NA. Only modest temporary benefit can be expected. Levetiracetam and topiramate have shown some benefit in secondary choreas and may be considered in treating NA. Levetiracetam has also been shown to help a patient with truncal tic.

Rhabdomyolysis and the neuroleptic malignant syndrome are always concerns in patients with NA, particularly because compliance and swallowing problems may lead to undesired daily variations of the dopamine blocking agent blood levels.

Even when no dramatic haloperidol dosing changes could be documented, Robinson noted rhabdomyolysis in a patient with NA. [35] After treatment for the life-threatening condition, the patient was switched from haloperidol to a combination of molindone and divalproex to effectively reduce involuntary movements.

Epilepsy management is very individualized. Phenytoin has been used successfully. Carbamazepine and lamotrigine have been reported to worsen the involuntary movements.

Digitalis and diuretics have been used successfully for congestive heart failure in patients with NA and cardiomyopathy.

Anticonvulsant therapy with phenytoin has been successful in patients with generalized tonic-clonic seizures.

In view of potential anticholinergic problems in patients with cardiomyopathy, selective serotonin reuptake inhibitors (SSRIs) are probably a better choice than tricyclic antidepressants.

Citalopram was found to help a patient with NA and obsessive-compulsive disorder. [36]

Generally, if hemolytic anemia is noted owing to acanthocyte accumulation/degradation, it is mild and does not require treatment.

In a case report, electroconvulsive therapy helped only speech but not chorea and other progressive neurologic problems. [37]

New protocols similar to those for Huntington disease are worth discussing with the medical treatment team. Agents such as the atypical antipsychotics for chorea and anti-Parkinson disease drugs for patients with NA and Parkinson disease features may be of value. Newer antidepressant drugs may also have a role, particularly SSRIs.

Neuroprotective agents and gene therapy may also have key future roles.


Antipsychotic Agents

Class Summary

Chorea, tics, and other adventitious movements such as oral dyskinesia may respond to drugs that block dopamine receptors and facilitate GABA transmission.

Haloperidol (Haldol)

In general, can decrease chorea, tics, and dyskinesia through blockade of dopamine receptors of CNS.



Class Summary

An agent that can decrease anxiety, such as a benzodiazepine, can also decrease movement disorders often made worse by associated stress.

Diazepam (Valium)

Long-acting benzodiazepine that can decrease anxiety in patients with NA throughout the day.