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As it achieved clinical, then regulatory successes developing Relyvrio™ (sodium phenylbutyrate and taurursodiol) to treat amyotrophic lateral sclerosis (ALS) in adults, Amylyx Pharmaceuticals (AMLX) saw its stock price more than quadruple over the past five months from its low closing price for the year of $6.85 on May 11.

Yet when Amylyx won the FDA’s first approval for an ALS drug in five years late last week, investors did not follow suit with another surge in the stock price. Instead, Amylyx shares have yo-yoed since the company and the FDA announced Relyvrio’s approval after the close of the markets September 29.

The first trading day afterward, shares actually fell nearly 7%, from $30.19 to $28.15 on September 30.

“We were surprised by the stock’s reaction,” acknowledged Marc Goodman, Senior Managing Director, Neuroscience, and a senior research analyst with SVB Securities, in a Sunday research note. “We still expect the stock to grind higher as we believe the product should have a strong launch.”

Three days earlier, Goodman shared SVB’s sales projections for Relyvrio, an oral, fixed-dose combination therapy: “We expect a fairly fast US ramp with estimated sales of ~$220M in 2023 and >$500M in 2024.” Such a strong launch, he predicted, will propel the stock to further gains.

Those projected gains had not begun to surface at deadline, as investors appear to continue having concerns, judging by Amylyx’s performance this week. After bouncing back about 11%, to $31.16 on Tuesday (just below its 2022 high of $32.90 on February 28), Amylyx shares fell 4.5% Wednesday to $29.77.

Goodman observed that the unexpected decline appeared to have two explanations. One was fear that Relyvrio could be yanked from the market if the drug were to fail the Phase III PHOENIX trial (NCT05021536), designed to confirm the safety and efficacy of the drug in up to 600 adults with ALS. Amylyx co-CEO Justin Klee and Billy Dunn, MD, director of the FDA’s Office of Neuroscience, have both said Amylyx will voluntary remove Relyvrio from the market should the PHOENIX trial fail.

The other was “the usual financing overhang post good biotech news,” namely investors quickly cashing in to take profits.

“We continue to believe that it is not very likely that Relyvrio will be pulled from the US market even if the PHOENIX study is negative given the significant enthusiasm and support from the patient and physician community for the drug; it would have to miss significantly for our view to change,” Goodman wrote on September 29.

Positive feedback

Goodman cited positive comments from two unnamed key opinion leaders or KOLs—identified only as practicing medicine at Centers of Excellence (CoEs)—that they planned to prescribe Relyvrio to their patients on top of two standard-of-care ALS drugs, riluzole, a generic that is still sold by Sanofi under the brand name Rilutek®; and Mitsubishi Tanabe Pharma America’s Radicava® (edaravone).

“We believe that the positive KOL feedback/comments are supportive of our positive view that Relyvrio addresses a high unmet need, should have a fast ramp, and should become a meaningful product in the space,” Goodman reported.

While Radicava use has historically been lower than that of riluzole since it must be administered intravenously, an oral suspension formulation approved by the FDA in May is expected to boost use of that drug.

Goodman noted that Amylyx hopes to stake a competitive position for Relyvrio by pricing its drug lower than the $170,000-a-year Radicava after consulting with the ALS patient community, at $12,504 for a 28-day supply, equivalent to ~$158,000 per year. Prices for generic riluzole (50 mg tablets) started as low as $30.15, according to a check of the drug discount information website GoodRx on Thursday.

Amylyx said its Amylyx Care Team (ACT) Support Program will ensure that all eligible patients who would benefit from Relyvrio can access the drug, in part by providing financial assistance options to eligible patients with out-of-pocket costs.

To fund the commercial launch of Relyvrio, Amylys has launched a public offering of 6 million shares of common stock, with an option for underwriters to purchase 900,000 more shares. Based on Monday’s closing price of $29.75, net proceeds from the initial offering would be at least $167 million ($192.2 million if the underwriter option is exercised in full), of which:

  • Approximately $100 million would fund production and commercialization activities for Relyvrio in the U.S. and Canada, plus regulatory approval activities and market preparation in Europe.
  • Approximately $30 million to fund completion of the PHOENIX trial, including the open label extension phase, and the completion of post-marketing requirements.
  • Approximately $20 million to fund development and expansion of Amylyx’s pipeline to address other neurodegenerative indications, as well as formulations and derivatives of Relyvrio.
  • The rest for working capital and other general corporate activities.

Relyvrio has been conditionally approved in Canada as Albrioza™, that decision tied to success in a Phase III study, while a marketing authorization application for the drug is under review by the European Medicines Agency.

In the U.S., Relyvrio’s approval was based on data from a single 137-patient clinical study, the Phase II CENTAUR trial (NCT03127514), and an unusual two hearings held by an FDA advisory panel, the agency’s Pe­riph­er­al and Cen­tral Ner­vous Sys­tem Drugs Ad­vi­so­ry Com­mit­tee. The first hearing ended with a 6-4 vote that effectively recommended against agency approval of the treatment by asserting that CENTAUR and an open label extension did not establish the conclusion that the drug was effective in the treatment of patients with ALS. That vote touched a 54% plunge in Amylyx shares in the weeks that followed, from $14.93 on September 30 to $6.85 on May 11.

After the first hearing, the advisory committee majority concluded that data from CENTAUR did not meet the panel’s standard for substantial evidence and persuasiveness. The majority cited the rate of decline in the Amyotrophic Lateral Sclerosis Functional Rating Scale-Revised (ALSFRS-R) 24 weeks after treatment. However, Amylyx insisted that its primary endpoint was met, reporting that participants treated with its ALS drug scored, on average, 2.32 points higher than placebo patients on ALSFRS-R over 24 weeks.

At the urging of ALS patient groups, the advisory committee met a second time last month, where based on new analyses submitted by Amylyx, the panel concluded 7-2 that the available evidence of effectiveness was sufficient to support approval of the combination drug, then known as AMX0035.

In May, researchers involved in the CENTAUR trial published in Muscle & Nerve  long-term survival analyses from the study suggesting a longer survival benefit for AMX0035 when adjusting for placebo crossover ranging from 10.6 to 18.8 months, compared with 6.9 months seen in the original pre-specified intent-to-treat (ITT) analysis in participants with ALS.

Clene falls on ALS results; company cites >90% risk reduction

The development of an ALS drug also figured in Clene (CLNN) shares tumbling 28% on Monday, from $2.80 to $2.01, then falling another 13% on Tuesday, to $1.74 (before rising 4%, to $1.81 on Wednesday). The early-week declines occurred after the company acknowledged Monday that its lead pipeline candidate CNM-Au8® missed its primary endpoint in the Healey ALS Platform Trial (NCT04414345  and NCT04297683), namely a statistically significant slope of change in ALS Functional Rating Scale Revised (ALSFRS-R) scores adjusted for mortality. That slope was only 2% slowing at 24 weeks.

CNM-Au8 also missed its secondary endpoints of Combined Assessment of Function and Survival (CAFS) and slow vital capacity (SVC) at 24 weeks across both 30 mg and 60 mg doses, Clene reported, adding that the 60 mg dose “did not demonstrate a survival benefit.”

However, Clene emphasized another, more favorable, finding of Healey-ALS in a secondary survival endpoint of the study—namely a >90% reduction in risk of death or risk of death/permanently assisted ventilation at 24 weeks for the 30 mg dose. Clene has selected the CNM-Au8 30 mg dose as it continues to develop the drug in ALS.

“We are very pleased to see a survival benefit in a broad population of people who had already been living with ALS for up to three years,” Robert Glanzman, MD, FAAN, Clene’s Chief Medical Officer, said in a statement.

That survival signal also caught the attention of one analyst: “Given the survival signal, we are maintaining our BUY rating as we head into two important Healey trial-related events that are yet to come,” wrote Sumant Kulkarni, Managing Director, Biotechnology Equity research with Canaccord Genuity, in a note to investors on Monday.

Those two events include the release of biomarker data, expected by the end of this year; and fuller data on the survival signal after the 52-week open label extension portion of the study concludes, something Kulkarni estimated could emerge in the summer of 2023.

“The second event is especially crucial as it could pave the way for substantive discussions with the FDA on a path forward on CnM-Au8,” Kulkarni added.

In his statement, Glanzman suggested a longer treatment duration may be needed to better assess the treatment’s efficacy vs. ALS: “CNM-Au8’s mechanism of enabling energy metabolism and efficiency may not be reflected in the slope of ALSFRS-R change after only 24 weeks of treatment.”

According to Glanzman, Healey marked the second Phase II study to show a survival benefit following CNM-Au8 treatment. Last year in the Phase II RESCUE-ALS trial (NCT04098406), CNM-Au8 showed what Clene said was an efficacy signal for slowing disease progression as measured by Motor Unit Number Index (MUNIX) at week 12, and cited a pre-specified analysis in the subset of limb onset ALS in which, the company said, CNM-Au8 showed a “significant treatment effect” in MUNIX at week 12 and a “trend” for improvement at week 36.

However, Clene has acknowledged that CNM-Au8 missed its primary endpoint in RESCUE-ALS of slowing disease progression as measured by MUNIX, as well as the study’s secondary endpoint (forced vital capacity or FVC).

The Healey ALS Platform Trial is designed to assess CNM-Au8 and at least four other potential ALS treatments—zilucoplan (being developed by UCB), verdiperstat (Biohaven Pharmaceuticals, acquired by Pfizer for $11.6 billion in a deal completed Monday), pridopidine (Prilenia Therapeutics), and SLS-005 (also called trelahose injection; Seelos Therapeutics). The 800-patient Phase II/III trial is led by the Sean M. Healey & AMG Center for ALS at Massachusetts General Hospital, and being conducted at more than 50 centers nationwide.

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