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. Author manuscript; available in PMC: 2013 Jul 9.
Published in final edited form as: Curr Treat Options Neurol. 2011 Jun;13(3):324–332. doi: 10.1007/s11940-011-0116-y
Methylprednisolone
 Standard dosage No standard has been set. The recommended dose for adults is 1.0 g intravenously per day for 5 days.
 Contraindications Documented hypersensitivity or concurrent serious infection.
 Main drug interactions Many potentially serious pharmacokinetic interactions. May increase levels of cyclosporine. Concurrent use of drugs that induce hepatic P450 enzymes can effectively decrease the steroid effects, and there are variable effects on anticoagulants.
 Main side effects Short courses of methylprednisolone are usually well tolerated. but it may cause dizziness, nausea, indigestion, increased appetite, weight gain, hyperglycemia, weakness, or sleep disturbances. More serious side effects include myopathy, hypokalemia, peptic ulcer disease, and psychosis.
 Special points Long-term treatment should be avoided because of adverse effects. Use caution with diabetic patients because of potential hyperglycemia.
 Cost/cost-effectiveness One gram of methylprednisolone is approximately $17, and there are incremental costs for infusion and nursing care.
Intravenous Immunoglobulins
 Standard dosage No standard dose has been set. Most patients have received 0.4 g/kg intravenously per day for 5 consecutive days each month.
 Contraindications IgA deficiency, previous hypersensitivity, hyperviscosity syndrome, vascular disease, renal insufficiency.
 Main drug interactions None.
 Main side effects Headache, nausea, fever, aseptic meningitis. Some effects are mitigated if infusions start slowly and the rate is increased as tolerated.
 Special points The use of 60 mg of intravenous methylprednisolone may decrease the severity of headache.
 Cost/cost-effectiveness Approximately $50 to $100 per gram an infusion can cost $10,000, but clinical improvement likely reduces time in intensive care and inpatient facilities.
Cyclophosphamide
 Standard dosage No standard dose has been determined. We use 750 mg/m2 (body surface area calculation) infused over 1 h once every 4 weeks (longer interval based on blood counts). We recommend premedication with dexamethasone and ondansetron and post-treatment medication with prochlorperazine as needed. If patients are taking adequate oral intake, no additional hydration is needed at this dose, but for patients with reduced oral intake, hydration should be encouraged or provided. This dose of cyclophosphamide does not require mesna to prevent hemorrhagic cystitis and can be given on an outpatient basis.
 Contraindications Patients with diabetes, underlying infections, hypertension, or peptic ulcer disease need to be monitored closely.
 Main drug interactions Allopurinol.
 Main side effects Nausea, vomiting, myelosuppression, headache, dizziness, and increased risk of myeloproliferative or lymphoproliferative malignancies.
 Special points Blood counts need to be monitored for leukopenia.
 Cost/cost-effectiveness Cyclophosphamide is relatively inexpensive, but costs are increased by the need for intravenous hydration and laboratory monitoring.
Rituximab
 Standard dosage 375 mg/m2 (body surface area calculation) intravenously weekly for 4 weeks.
 Contraindications Previous anaphylactic reactions to rituximab or other murine proteins.
 Main drug interactions Live-virus vaccines should not be given while receiving rituximab and for several months afterwards.
 Main side effects Infusion reactions with respiratory distress, urticaria, and hypotension can occur and are mitigated by delivering the first infusion over 90 min, with the use of diphenhydramine and acetaminophen as preinfusion medications.
 Cost/cost-effectiveness About $600 for 100 mg. One infusion may cost over $10,000 because of incremental infusion charges.
Mycophenolate Mofetil
 Standard dosage One gram twice a day.
 Contraindications Pregnancy, hypersensitivity to this drug or any of its components, including polysorbate 80.
 Main drug interactions Should not be given with drugs that interfere with enterohepatic recirculation. Patients should not receive live vaccines while being treated.
 Main side effects Among the many side effects are constipation, diarrhea, leukopenia, sepsis, vomiting, and increased risk of opportunistic infection and lymphoproliferative disorders.
 Special points Consult with a practitioner with prior experience with this medication for advice on safe medical monitoring.
 Cost/cost-effectiveness No data available.
Azathioprine
 Standard dosage Two to 3 mg/kg per day.
 Contraindications Pregnancy, renal impairment (use with caution), and prior use of alkylating agents.
 Main drug interactions Allopurinol; use caution with warfarin and phenytoin as the efficacy of these drugs may be decreased.
 Main side effects Multiple side effects include myelosuppression, nausea, vomiting, diarrhea, and muscle aches.
 Special points Consult with a practitioner with prior experience with this medication for advice on safe medical monitoring.
 Cost/cost-effectiveness No data available.
Antiepileptic drugs
Seizures are treated with standard antiepileptic medications. Because many of the stereotypic movements do not have an EEG correlate, however, a thorough video EEG evaluation should be done before starting these drugs.