Amyotrophic Lateral Sclerosis Drug Summary

Drug	Dosage	Remarks
Edaravone	Initial cycle: 105 mg PO 24 hourly x 14 days followed by 14-day drug-free period Subsequent cycle: 105 mg PO 24 hourly x 10 days within a 14-day period followed by 14-day drug-free period  or Initial cycle: 60 mg IV 24 hourly x 14 days followed by 14-day drug-free period Subsequent cycle: 60 mg IV 24 hourly x 10 days within a 14-day period followed by 14-day drug-free period	Adverse Reactions CNS effects (abnormal gait, headache); Other effects (bruising, injection site reactions, fatigue, allergic reaction)  Special Instructions Use with caution in patients with asthma or sulfite allergy Avoid mixing Edaravone with other medications in the infusion bag Patients on IV Edaravone may be switched to oral with the same dose schedule
Riluzole	50 mg PO 12 hourly Max dose: 100 mg/day	Adverse Reactions CNS effects (headache, dizziness, vertigo, somnolence, anorexia); GI effects (elevated liver enzymes, nausea/vomiting, abdominal pain, diarrhea, constipation); Other effects (anaphylactoid reactions, neutropenia, asthenia, tachycardia, decreased lung function) Special Instructions Take on an empty stomach, at least 1 hour prior to or 2 hours after a meal Avoid in patients with hepatic disease or raised liver enzyme values Monitor liver function prior to and during treatment Use with caution in patients with renal impairment or history of liver disorders Patients should be advised that Riluzole may cause dizziness or somnolence; use caution in performing tasks that require alertness  Patient and caregiver should report signs of infection to physician immediately (eg sore throat, fever)
Tofersen	100 mg intrathecally every 14 days x 3 doses Followed by 100 mg intrathecally every 28 days	Adverse Reactions Most common: Musculoskeletal effects (arthralgia, myalgia, stiffness); CNS effect (neuralgia); Other effects (pain, fatigue, increased white blood cell and protein in the cerebrospinal fluid) Less common: CNS effects (myelitis and/or radiculitis, papilledema, elevated intracranial pressure, aseptic meningitis) Special Instructions Instruct patients and caregivers to contact their healthcare provider if symptoms consistent with adverse reactions develop

Drug	Dosage	Remarks
Baclofen	Initial dose: 5 mg PO 8 hourly x 3 days Increase gradually to 10 mg, 15 mg and 20 mg PO 8 hourly at 3-day intervals Max dose: 80 mg/day	Adverse Reactions CNS effects (dizziness, euphoric mood, hallucinations, ataxia, tremor); GI effects (nausea, GI disturbance, retching); Renal effects (polyuria, enuresis, dysuria); Other effects (lassitude, exhaustion, muscular weakness, myalgia, nightmares, visual disturbances, nystagmus, decreased cardiac output, rash) Special Instructions Should be taken with food Avoid in patients with peptic ulcer disease Avoid abrupt withdrawal Use with caution in patients with psychotic disorders, schizophrenia, confusional states or Parkinson’s disease, epilepsy, CVA or respiratory insufficiency, hepatic insufficiency, renal impairment, during pregnancy and lactation
Eperisone	50 mg PO 8 hourly	Adverse Reactions CNS effects (dizziness, weakness, insomnia, drowsiness, numbness or trembling in the extremities); GI effects (hepatic dysfunction, GI disturbances); Hypersensitivity effects (skin rashes, urticaria, angioedema); Other effects (hematological changes, urinary disorders) May worsen muscle weakness  Special Instructions Should be taken with food Use with caution in patients with hepatic disorders Monitor for signs of severe allergic reactions or shock

All dosage recommendations are for non-elderly adults with normal renal and hepatic function unless otherwise stated.   
Not all products are available or approved for above use in all countries. 
Products listed in the Drug Summary are based on indications stated in the locally approved product monographs.   
Please refer to local product monographs in Related MIMS Drugs for country-specific prescribing information.