Section 08
ALS
Disease-Modifying & Symptomatic Therapies
7 AGENTS
Riluzole
Rilutek / Tiglutik / ExservanGlutamate release inhibitor
- Forms
- Tab: 50mg | Susp: 5mg/mL | Film: 50mg
- Dose
- 50mg BID
- MOA
- ↓Presynaptic glutamate release
Edaravone
Radicava / Radicava ORSFree radical scavenger
- Forms
- IV: 30mg/100mL | Oral susp: 105mg/5mL
- Dose
- Cyclic dosing: 60mg/day x14d, then 10d off
- MOA
- Antioxidant, reduces oxidative stress
Sodium phenylbutyrate-taurursodiol
Relyvrio (WITHDRAWN Apr 2024)Neuroprotective combination
- Forms
- Oral packet: 3g/1g
- Dose
- No longer marketed (PHOENIX trial negative); legacy patients only
- MOA
- ↓ER stress + mitochondrial dysfunction
Tofersen
QalsodyAntisense oligonucleotide
- Forms
- Intrathecal: 100mg/15mL
- Dose
- 3 loading doses q14 days, then maintenance q28 days
- MOA
- Reduces SOD1 mRNA/protein
Dextromethorphan-quinidine
NuedextaSigma-1/NMDA agonist + CYP2D6 inhibitor
- Forms
- Cap: 20mg/10mg
- Dose
- 1 cap BID (pseudobulbar affect)
- MOA
- Sigma-1 & NMDA modulation (pseudobulbar affect)
Baclofen
Lioresal / genericGABA-B agonist
- Forms
- Tab: 10, 20mg | Intrathecal: 0.05-2mg/mL
- Dose
- titrate per spasticity protocol
- MOA
- Presynaptic GABA-B agonism, ↓spasticity
Glycopyrrolate
Robinul / CuvposaAnticholinergic
- Forms
- Tab: 1, 2mg | Sol: 1mg/5mL
- Dose
- Sialorrhea (off-label in ALS): 1-2mg TID
- MOA
- Muscarinic receptor blockade, ↓salivation