Ms. A was a 33-year-old woman who was admitted to the general medical hospital for rhabdomyolysis. She had been diagnosed with neuroacanthocytosis 4 years earlier in a university setting. She had continuous, uncontrolled, and rapid involuntary movements; a heart rate of 132 bpm; a WBC count of 13.1/mm3; and a creatine phosphokinase level of 35673 U/liter. She arrived at the hospital taking 5 mg t.i.d. of diazepam, 0.5 mg t.i.d. of benztropine mesylate, and 0.5 mg of haloperidol, as needed for agitation.
We recommended that she be placed into an intensive care unit and intubated. Molindone hydrochloride, 50 mg t.i.d., was introduced; diazepam, benztropine mesylate, and haloperidol were discontinued. After 5 days of taking propofol and with ventilator support, Ms. A’s creatine phosphokinase level had fallen to 911 U/liter. Ms. A was extubated on day 8 when her creatine phosphokinase level was 876 U/liter. On day 9, the molindone hydrochloride was titrated to 100 mg t.i.d. On day 11, divalproex sodium, 250 mg t.i.d., was introduced. Upon discharge on day 14, Ms. A’s creatine phosphokinase level was 794 U/liter.
The combination of molindone and divalproex was effective in reducing her extreme involuntary movements. Ms. A was calm, alert, aware, conversant, and oriented to the clinical setting, her age, the month, and the year. She regained some level of independent function in her upper extremities and was able to ambulate on a treadmill for brief periods. Upon discharge, her parents took her to their home.
Treatment for this disorder is symptomatic and supportive. Maintenance of proper nutrition is a challenge. A feeding tube may be needed for some patients as the disorder progresses. Antipsychotic drugs can provide stage-dependent relief from chorea and tics. Benzodiazepines may be used to reduce anxiety and diminish the intensity of movement disorders.