**5.3.11 Branched-chain amino acids (BCAA)**

The branched-chain amino acids comprehend leucine, isoleucine, and valine. In clinical trials, branched chain amino acids did not show a beneficial effect in ALS patients (Tandan et al., 1996).

### **5.3.12 Bromocriptine**

The dopamine agonist bromocriptine did not show a benefit in ALS patients (Szulc-Kuberska et al., 1990).

### **5.3.13 Buspirone**

The anxiolytic agent buspirone did not show a benefit in ALS patients (The ALS Association, 2010).

### **5.3.14 Ceftriaxone**

Ceftriaxone is a ß-lactam antibiotic which also shows anti-oxidant and anti-excitotoxity effects (Traynor et al., 2006).

Currently, ceftriaxone is evaluated in a phase III clinical trial in patients with ALS (NCT00349622).

### **5.3.15 Celecoxib**

Celecoxib is an inhibitor of cyclooxygenase-2 (COX-2), an enzyme which promotes inflammation by releasing of inflammatory substances, such as prostaglandin E(2) (PGE2). The rationale of celecoxib treatment is the reduction of PGE2 in the cerebrospinal fluid (CSF), thus preventing neuroinflammation and neuronal loss.

In a phase II clinical trial (NCT00355576), celecoxib did not show a beneficial effect on the decline in muscle strength, motor unit number estimates, vital capacity, ALS Functional Rating Scale-Revised, and overall survival (Cudkowicz et al., 2006). Moreover, PGE2 levels in the CSF were not elevated at baseline and did not decline during treatment (Cudkowicz et al., 2006). In this trial, celecoxib was combined with creatinine and/or minocycline (Gordon et al., 2008).
