Therapeutics

Gammagard®

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Overview

Name: Gammagard®
Synonyms: Intravenous Immunoglobulin, IVIg
Therapy Type: Immunotherapy (passive) (timeline)
Target Type: Amyloid-Related (timeline), Inflammation (timeline)
Condition(s): Alzheimer's Disease
U.S. FDA Status: Alzheimer's Disease (Discontinued)
Company: Baxter Healthcare
Approved for: Immunodeficiency conditions

Background

Gammagard® is a preparation of pooled human plasma antibodies from blood donations. It has an established safety record from human use in immunodeficiency and certain autoimmune conditions. Gammagard® has been used off-label for the treatment of a small number of Alzheimer's disease patients for years. The proposed treatment rationale is twofold. Intravenous immunoglobulin mixtures contain a small fraction of polyclonal antibodies directed against Aβ that are thought to effect clearance or counteract synaptic toxicity caused by Aβ (Szabo et al., 2010). The concentration of natural Aβ antibodies is lower in IVIg preparations than in engineered monoclonal Aβ immunotherapies. In addition, IVIg has immunomodulatory effects, which are proposed to change patients' cytokine profiles to induce a noninflammatory, phagocytic microglial phenotype (see Aug 2011 news story).

Findings

An open-label dose-finding study in eight patients with mild AD reported increases of Aβ antibodies in serum, decreases of Aβ in CSF, and stabilization of MMSE scores over 18 months (see Relkin et al., 2009).

A blinded Phase 2 trial of 24 people with mild to moderate AD tested two doses of Gammagard®, at two treatment regimens each, for six months against placebo. Each treatment group had four patients. This trial reported a benefit on the Clinical Global Impression of Change primary outcome that persisted at three years of follow up, beneficial changes in plasma cytokine concentrations, and reduced ventricular enlargement on MRI scans (see Jul 2013 conference storyApr 2010 conference story). 

Two pivotal Phase 3 trials were run. The first, run by the Alzheimer's Disease Cooperative Study at 45 sites in the United States and Canada, compared 18-month treatment with 400 mg/kg and 200 mg/kg of Gammagard® to placebo in 390 patients with mild to moderate Alzheimer's disease against cognitive and global function as assessed by the Alzheimer's Disease Assessment Scale-Cognitive Subscale and the Alzheimer's Disease Cooperative Study Activities of Daily Living. Substudies measured CSF, cerebral metabolism by FDG-PET, and brain amyloid load by PET. This trial was completed. Primary endpoints showed no difference between study drug and placebo.  Prespecified subgroup analysis showed a trend toward benefit for the higher dose, but only in moderate AD and with insufficient power to be statistically significant (see May 2013 news story, see full results on clinicaltrials.gov). 

Baxter subsequently terminated a second pivotal trial, which started in 2012 and was to have run until 2015 in 530 patients with mild to moderate AD in countries around the world. A long-term follow-up extension to the first pivotal trial was also terminated.

For all trials on this treatment, see clinicaltrials.gov

Last Updated: 03 Oct 2023

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Therapeutics

Idebenone

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Overview

Name: Idebenone
Synonyms: Catena, Sovrima
Chemical Name: '2-(10-hydroxydecyl)-5,6-dimethoxy-3-methyl-cyclohexa-2,5-diene-1,4-dione
Therapy Type: Small Molecule (timeline)
Target Type: Other (timeline)
Condition(s): Alzheimer's Disease
U.S. FDA Status: Alzheimer's Disease (Discontinued)
Company: Takeda Pharmaceutical Company
Approved for: None

Background

Idebenone, a synthetic analogue of coenzyme Q10, has potent antioxidant activity and facilitates the flux of electrons along the mitochondrial electron transport chain, increasing the production of ATP.

Last Updated: 16 Oct 2013

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Therapeutics

Ibuprofen

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Overview

Name: Ibuprofen
Synonyms: Advil™, Nuprin™ , Motrin™
Therapy Type: Small Molecule (timeline)
Target Type: Amyloid-Related (timeline), Inflammation (timeline)
Condition(s): Alzheimer's Disease
U.S. FDA Status: Alzheimer's Disease (Discontinued)
Approved for: Pain and Fever

Background

Ibuprofen is an over-the-counter non-steroidal anti-inflammatory drug (NSAID). It inhibits cyclooxygenase activity and reduces inflammation through reduced prostaglandin synthesis. 

In the Alzheimer's field, interest in NSAIDs such as ibuprofen arose when epidemiological studies started reporting lower rates of Alzheimer's among people who had been taking these drugs for chronic treatment of inflammatory conditions (e.g., Mar 1997 news story; in't Veld et al., 1998; Nov 2001 news story; Vlad et al., 2008). Interest grew when ibuprofen was shown to be part of a subset of NSAIDs that reduce Aβ42 levels by modulating the activity of the γ-secretase enzyme complex. The protective epidemiological effect of these NSAIDs was then attributed to this γ-secretase modulating activity, not their cyclooxygenase inhibition (Nov 2001 news story).

In subsequent years, a host of experimental studies reported Aβ-lowering effects of NSAIDS including ibuprofen, in cell-based and animal models of Alzheimer's disease (e.g., Aug 2000 news story; Sep 2004 news story)

Findings

Because of its known gastrointestinal toxicity and low exposure in the brain, few clinicial trials have evaluated ibuprofen for the treatment of Alzheimer's disease. In 2003 and 2004, a one-year, multicenter trial compared 400 mg of ibuprofen twice daily to placebo in 132 people with mild to moderate AD. Outcomes included the ADAS-cog, MMSE, CDR, and other standard measures. There was no difference between the ibuprofen and placebo groups on any outcome (Pasqualetti et al., 2009).

A subsequent meta-analysis of clinical trials of NSAIDs including ibuprofen confirmed that the NSAID groups had no significant cognitive benefit, but more nausea and vomiting, and that ibuprofen cannot be recommended for the treatment of Alzheimer's disease (Jaturapatporn et al., 2012). More broadly, randomized clinical trials of NSAIDs as therapeutic approaches to Alzheimer's disese have been negative and remain at odds with the epidemiological observations (May 2008 news story).

Early research on ibuprofen shifted attention to a structurally related compound, R-flurbiprofen. This drug was clinically evaluated through Phase 3, where it failed.

Last Updated: 16 Oct 2014

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Therapeutics

Huperzine A

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Overview

Name: Huperzine A
Synonyms: ZT-1, DEBIO 9902 , Qian ceng ta, Cerebra capsule, Pharmassure Memorall capsule
Therapy Type: Small Molecule (timeline), Supplement, Dietary (timeline)
Target Type: Cholinergic System (timeline)
Condition(s): Alzheimer's Disease
U.S. FDA Status: Alzheimer's Disease (Inactive)
Company: Debiopharm Group™, Neuro-Hitech, Inc.
Approved for: None

Background

Huperzine A is an alkaloid extract derived from the Chinese club moss Huperzia serrata. Huperzine A for many years has been widely used in traditional Chinese medicine to enhance memory and to treat fever and inflammation. In the United States it is commercially available as a food supplement, but has not been approved by the FDA as a treatment for AD or other cognition disorders.

Multiple proposed mechanisms of action for huperzine A can be found in the literature. It is a selective AChE inhibitor. It also been shown to have antioxidant and neuroprotective properties counteracting glutamate-induced toxicity, possibly by interacting with  NMDA receptors (Ashani et al., 1994Ved et al., 1997Zhang et al., 2001; Gordon et al., 2001Bai et al., 2000).

Research on huperzine A originated with the Chinese Academy of Sciences. The Mayo Clinic, Jacksonville, Florida, generated a version of huperzine A that was tested at Georgetown University, Washington, D.C., and licensed to the biotechnology company Neuro-Hitech to develop an oral formulation for clinical development. Preclinical studies of this compound suggested blood-brain barrier penetration and good tolerability and the same mechanism of action as the original Chinese extract (Neuro Hi-Tech media release). Additional formulations of huperzine A exist, as well.

Findings

Between 2004 and 2007, Neuro-Hitech's oral formulation of huperzine A completed a Phase 2 clinical trial in the United States for the treatment of Alzheimer's disease. Conducted by the Alzheimer Disease Cooperative Study, this multicenter, four-month trial compared two doses of huperzine to placebo in 210 people with mild to moderate AD. This trial was negative on its primary endpoint, but secondary analyses indicated a trend toward cognitive improvement on the higher dose (Rafii et al., 2011). Side effects, mostly nausea and vomiting, were mild to moderate. Development of this formulation has been discontinued.

A second trial, of a sustained-release formulation developed by Shandong Luye Pharmaceutical Co., Ltd., began in 2010 and was estimated to run for two years. According to clinicaltrials.gov, this Phase 2/3 study was conducted at the Shanghai Mental Health Center, to compare a six-month course of two doses to placebo in 390 people with mild to moderate Alzheimer's disease. The status of this trial is unknown and data are unavailable.

The Swiss company Debiopharm tested a synthetic huperzine derivative and prodrug, ZT-1, aka DEBIO 9902. It was reported to be well-tolerated, rapidly absorbed, and converted into huperzine A, in a small Phase 1 trial in healthy Chinese volunteers ( Jia et al., 2013). However, clinical development of DEBIO 9902 has been discontinued.

Previously, clinical trials conducted in China had reported beneficial effects on cognition. These were two-week to two-month trials in 60, 103, and 160 patients with dementia, respectively (see Xu et al., 1999; Xu et al., 1995). 

A published meta-analysis of 20 huperzine clinical trials conducted in China, Europe, and the United States leaves open the question of whether huperzine could be therapeutically useful. Trials reporting beneficial effects have tended to be small and of short duration (Yang et al., 2013). A separate attempt to evaluate clinical trials of huperzine A for mild cognitive impairment found no studies that met inclusion criteria for meta-analysis, that is, randomised, parallel-group, placebo-controlled trials (Yue et al., 2012). Research on huperzine A continues.

Last Updated: 11 Dec 2013

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Therapeutics

Galantamine

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Overview

Name: Galantamine
Synonyms: Razadyne™, Reminyl™, Nivalin®
Chemical Name: (4aS,6R,8aS)-4a,5,9,10,11,12-Hexahydro-3-methoxy-11-methyl-6H-benzofuro [3a,3,2-ef] [2] benzazepin-6-ol
Therapy Type: Small Molecule (timeline)
Target Type: Cholinergic System (timeline)
Condition(s): Alzheimer's Disease
U.S. FDA Status: Alzheimer's Disease (Approved)
Company: Janssen, Ortho-McNeil Pharmaceutical, Sanochemia Pharmazeutika, Shire, Takeda Pharmaceutical Company
Approved for: Mild to Moderate Alzheimer's disease

Background

Discovered in the 1950s, galantamine is an alkaloid isolated from Galanthus nivalisthe common snowdrop. Galantamine crosses the blood-brain barrier and has been used in humans for decades in anaesthesia and the treatment of neuropathic pain. Galantamine enhances cholinergic function in two ways: It is a weak acetyl cholinesterase inhibitor and an allosteric potentiator of both nicotinic and muscarinic acetylcholine receptors. Synthetic galantamine was first registered for the treatment of Alzheimer's disease in Sweden in the year 2000; it was subsequently approved for this indication in the European Union, the United States, Canada, Japan, and many other countries around the world. 

Galantamine is available as a tablet, as solution, and, since 2005, as a once-daily extended-release capsule. Also in 2005, the trade name the name Reminyl® was replaced with RazadyneTM to avoid confusion and prescribing errors arising from the similarity of the name Reminyl® to the diabetes drug Amaryl® (glimepiride). Since then, several generic equivalents have been approved by the FDA.

In keeping with the other cholinesterase inhibitors approved to treat Alzheimer's—donepezil and rivastigmine—the most common side effects of galantamine are gastrointestinal, including nausea, vomiting, and diarrhea. Dizziness, insomnia or nightmares, agitation, mild arryhthmia, and other effects have also been reported. Adverse effects tend to be milder when the dose is titrated up to the final therapeutic dose slowly, over the course of two months. A study directly comparing galantamine and donepezil treatment for a year found a similar side effect profile for both drugs (Wilcock et al., 2003).

Findings

More than 90 clinical trials have evaluated galantamine's effect on Alzheimer's and other conditions. Overall, they find a consistent symptomatic benefit on cognition and clinical measures in AD for several years of treatment; however, as with all cholinesterase inhibitors, galantamine's effect size is small.

For example, in a six-month study of 653 patients with mild to moderate AD, 24 or 32 mg/day of galantamine led to a roughly 3-point treatment effect over placebo on both the ADAS-cognition scale and the CIBIC-plus and DAD scales of global clinical impression (Wilcock et al., 2000). A separate, five-month study of 978 patients with mild to moderate Alzheimer's compared placebo to galantamine slowly escalated up to final doses of 16 and 24 mg/day. It similarly showed a 3- to 3.6-point treatment effect on ADAS-cog, as well as benefits in global clinical function, behavioral symptoms, and activities of daily living (Tariot et al., 2000). 

After galantamine was approved, trials assessed whether it affected behavioral symptoms in patients with mild to moderate Alzheimer's disease. Post-hoc analysis of data on 2,033 patients, pooled from three separate studies, suggested a galantamine treatment benefit as measured by the Neuropsychiatric Inventory (NPI), particularly on symptoms of agitation, anxiety, disinhibition, and aberrant movements; a subsequent analysis of four trials found much the same result (Herrmann et al., 2005; Kavanagh et al., 2011). Other postmarketing studies evaluated additional aspects of galantamine treatment in Alzheimer’s disease, for example the drug’s effect on attention and its efficacy in mild AD (Vellas et al., 2005; Orgogozo et al., 2004). 

Several studies assessed galantamine's long-term efficacy. Overall, they reported modest but consistent cognitive and clinical benefits of continuous treatment over three to four years (e.g., Raskind et al., 2004). Claims that long-term galantamine treatment delays a patient's placement in a nursing home have been reported at conferences (see company press release); similar studies have been published in the peer-reviewed literature (Feldman et al., 2009).

A separate question concerns whether older patients and patients with advanced disease respond as well to cholinesterase treatment as do patients in their 70s and those with mild to moderate disease. An analysis of data on patients 80 and older, pooled from four different Phase 3 trials, suggested a treatment benefit for galantamine in patients in that age group (Marcusson et al., 2003). A retrospective analysis of data pooled from four three- to six-month trials reported a treatment benefit for moderate to severe Alzheimer's disease, as did the subsequent, international SERAD study specifically on severe AD (Wilkinson et al., 2002; Burns et al., 2009).

In 2005, safety flags were raised temporarily when more deaths occurred in the galantamine than placebo groups in two mild cognitive impairment trials, and the drug's prescribing information was updated accordingly (see Jan 2005 news storyWHO Pharmaceuticals newsletter). Subsequent meta-analysis contradicted this finding (e.g., Feldman et al., 2009). In 2012, a separate two-year trial of 2,051 patients with mild to moderate AD reportedly found 8 to 24 mg per day of galantamine to be associated not only with less decline in cognition and function, but also with lower mortality than placebo (see company press release). This last study has not been published in the peer-reviewed literature.

Even so, while galantamine is no longer seen as raising mortality in MCI, it was found to be ineffective in that population both in the two original MCI trials and in a broader meta-analysis of MCI patients in the cholinesterase inhibitor clinical trial literature (see Winblad et al., 2008Tricco et al., 2013).

Overall, the acetylcholinesterase therapies galantamine, donepezil, and rivastigmine are seen as having similar efficacy and safety, but few side-by-side comparisons have been conducted. One such trial, comparing galantamine to donepezil for one year, reported a slightly larger cognitive benefit and more caregiver relief for galantamine, with similar results on tolerability and activities of daily living (Wilcock et al., 2003). In clinical practice, galantamine—or donepezil or rivastigmine—is frequently prescribed in combination with the NMDA antagonist memantine (Atri 2011).

Phase 4 studies have also evaluated galantamine for conditions other than AD. These include vascular dementia, tardive dyskinesia, attention deficit hyperactivity disorder, post-traumatic headache, postoperative delirium, depression, Tourette’s syndrome, bipolar disorder, cognition in schizophrenia, and stroke. Development attempts for chronic fatigue and fibromyalgia have been discontinued. Galantamine is still being actively evaluated as a cognitive enhancer in bipolar disorder and schizophrenia, and as an aid to end nicotine or cocaine dependence. However, despite extensive testing of galantamine, as of 2014 its regulatory approval has not been expanded beyond its original indication of mild to moderate Alzheimer’s disease. 

For some years after cholinesterase inhibitor therapies were initially approved for Alzheimer's disease, their modest effect size created controversy about their cost-effectiveness (see Jul 2004 news story and extensive commentary). Pharmacoeconomic studies in the United States, Canada, and European countries have generally found that cholinesterase inhibitor treatment reduces the cost of care. In the United Kingdom, this debate called into question coverage of cholinesterase inhibitors by its universal health care system. The U.K.'s National Institute for Health and Care Excellence (NICE) in 2007 restricted use of these drugs, but in 2010 it re-evaluated the issue and in 2011 issued a guidance recommending the use of galantamine in the treatment of mild to moderate AD (see, e.g.,  Garfield et al., 2002; NICE guidance). Recent pharmacoeconomic studies confirm the cost-effectiveness of galantamine (e.g., Hyde et al., 2013).

For a comprehensive view of galantamine trials, see clinicaltrials.gov.

Last Updated: 06 Mar 2014

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Therapeutics

Flurizan™

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Overview

Name: Flurizan™
Synonyms: tarenflurbil , r-flurbiprofen, MPC-7869
Therapy Type: Small Molecule (timeline)
Target Type: Amyloid-Related (timeline)
Condition(s): Alzheimer's Disease
U.S. FDA Status: Alzheimer's Disease (Discontinued)
Company: Myriad Genetics & Laboratories
Approved for: None

Background

Flurizan™ is the R-enantiomer of the nonsteroidal anti-inflammatory flurbiprofen, an NSAID that is structurally and pharmacologically related to ibuprofen and is used to treat several inflammatory conditions. As a selective Aβ42-lowering agent (aka SALA), Flurizan™ would have been the first in its class to treat Alzheimer's disease. The rationale for testing Flurizan™ in Alzheimer's is that it can modulate γ-secretase, the enzyme complex that cleaves the Aβ peptide off its precursor protein, to selectively reduce generation of long, aggregation-prone forms of Aβ without affecting γ-secretase cleavage of other substrates, such as Notch.

Epidemiological research has shown that long-term use of NSAIDs is associated with a reduced risk for development of Alzheimer disease. In contrast to S-fluriprofen, the R-enantiomer is inactive toward the cyclooxygenase enzyme, and hence causes fewer gastrointestinal side effects. In cell-based and animal studies, Flurizan™ has been shown to lower Aβ42, probably through a direct effect on y-secretase (see Weggen et al., 2001; Weggen et al., 2003). The presenilin component of the y-secretase intramembrane complex is thought to be the target of Flurizan™'s activity (see Eriksen et al., 2003; Beher et al., 2004).

Mouse studies reported that chronic dosing with R-flurbiprofen improved performance of Tg2576 APP-transgenic mice (see Kukar et al., 2007).

Findings

A 12-month Phase 2 study in 210 people with mild to moderate Alzheimer's disease compared 400–800 mg of Flurizan™ twice daily to placebo for effects on cognition and function. A 12-month extension was added to the placebo-controlled phase of the trial. This trial found no effect on cognition, but did report a small treatment benefit on function, only for the higher dose and only in mild AD. Side effects included nausea, dizziness, and diarrhea, but overall Flurizan™ was tolerated well  (see Wilcock et al., 2008).

However, a Phase 1 trial of 400 to 1,600 mg/day of Flurizan™ in 48 healthy volunteers generated CSF pharmacodynamic data, suggesting low exposure of the drug in the brain. In particular, CSF Aβ42 did not change with 21 days of dosing (see Galasko et al., 2007).

A subsequent 18-month Phase 3 trial in 1,600 AD patients carried out at 133 centers in the United States initially compared twice-daily doses of 400 and 800 mg of Flurizan™ to placebo.  After analysis of the Phase 2 trial data, the Phase 3 trial was modified, with the approval of the FDA, so that only the highest dose was administered and only to patients with mild AD; patients with moderate AD were dropped from the trial. There was no difference between the treatment and placebo arms in either the co-primary outcomes (the ADAS-Cog and the ADCS activities of daily living scales) or a range of secondary outcomes that included measures of function (the CDR sum-of-boxes), cognition (the MMSE), psychopathology (Neuropsychiatric Inventory), quality of life (QQL-AD), and caregiver burden (see Aug 2008 conference storyGreen et al., 2009). The failure of Flurizan™ is generally attributed to its insufficient pharmacodynamics, i.e., inadequate ability to penetrate the brain and engage its target protein at doses sufficient to yield an effect. Two additional Phase 3 trials were terminated and further development of Flurizan™ was discontinued. Separate clinical development of Flurizan™ for prostate cancer has also been discontinued following negative Phase 2 results.

Last Updated: 04 Mar 2016

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Therapeutics

Rivastigmine

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Overview

Name: Rivastigmine
Synonyms: Exelon™, Rivastigmine tartrate , Rivastach® Patch, Prometax®, SDZ ENA 713
Chemical Name: (S)-3-[1-(dimethylamino)ethyl]phenyl N-ethyl-N-methylcarbamate
Therapy Type: Small Molecule (timeline)
Target Type: Cholinergic System (timeline)
Condition(s): Alzheimer's Disease, Parkinson's Disease Dementia
U.S. FDA Status: Alzheimer's Disease (Approved), Parkinson's Disease Dementia (Approved)
Company: Novartis Pharmaceuticals Corporation
Approved for: Mild to moderate Alzheimer’s disease and mild to moderate dementia related to Parkinson's disease

Background

Rivastigmine is a reversible inhibitor of both the acetylcholinesterase and butyrylcholinesterase enzymes. It is widely used for the treatment of Alzheimer's across its mild, moderate, and severe stages, as well as for the treatment of dementia associated with Parkinson's disease (PDD). Rivastigmine was first approved to be marketed for AD in 1997 in Switzerland, and in the years since has come to be available in some 80 countries worldwide, including the United States, Canada, and Europe, for both AD and PDD.

This drug was originally formulated as a twice-daily oral capsule. It was the first AD therapy to be made available as a skin patch that continuously delivers the drug over 24 hours. The patch is sold in three doses. By way of transdermal absorption, the patch provides steady plasma concentrations of rivastigmine and bypasses first-pass metabolism in the intestine and liver. Because it generates fewer gastrointestinal side effects, it enables patients to receive a higher therapeutic dose (Cummings et al., 2007). Generic versions of different doses of rivastigmine capsules started becoming available in 2010; a generic patch came out in 2015.

Rivastigmine's side effects are consistent with class effects of cholinesterase inhibition. They include most commonly nausea, vomiting, diarrhea, and loss of appetite; less frequently, agitation, depression, dizziness, fatigue/sleeplessness, and others. Side effects tend to be strongest in the beginning weeks, when the dose is titrated up to the therapeutic level, and milder in the maintenance phase. Apart from rare skin sensitivity reactions, the patch is generally better tolerated than the capsules.

Findings

About 75 clinical trials Phase 2 and higher have been registered with rivastigmine. For example, in a six-month North American trial comparing a dose of 6-12 mg/day to 1-4 mg/day and to placebo in 699 patients with mild to moderate AD, rivastigmine capsules showed a modest but dose-dependent benefit on cognition, function, and activities of daily living as measured by the ADAS-cog, the CIBIC-plus, and the Progressive Deterioration Scale (PDS), respectively. Similar results were shown in a European trial in 725 patients with mild to moderate AD that compared 6-12 mg/day to 1-4 mg/day of rivastigmine and placebo, also for six months and using the same outcome measures. Only a quarter to a third of patients had a significant treatment response to rivastigmine capsules (Corey-Bloom et al., 1998; Rösler et al., 1999).

The skin patch formulation was evaluated in the six-month, Phase 3 IDEAL (Investigation of transDermal Exelon in ALzheimer's disease) study in 1,195 patients with mild to moderate AD. Participants received either one of two doses of the rivastigmine patch (9.5 or 17.4 mg over the course of 24 hours), 6 mg twice-daily capsules, or placebo. On efficacy, both patch doses outperformed placebo; the lower-dose patch with a similar effect to that of the capsules, the high-dose patch adding a small benefit on cognition. With the patch, the incidence of gastrointestinal complaints dropped nearly to that of placebo, and two-thirds of caregivers said they preferred the patch. A six-month, 716-patient trial comparing a 4.6 mg/24-hour to a 13.3 mg/24-hour patch reported that the higher dose was more efficacious at comparable tolerability (e.g., Winblad et al., 2007Grossberg et al., 2011Farlow et al., 2013). An open-label study in a real-life clinical setting outside of randomized controlled trials reported that 969 patients with mild to moderate AD treated with the rivastigmine patch tended to maintain cognitive and global function over 18 months (Gauthier et al., 2013).

Most double-blind RCTs of rivastigmine lasted three to six months. Some longer-term treatment data are available from open-label extensions or from analyses of clinical observation. For example, one study of 1,998 patients treated for up to five years found that their mean baseline MMSE score of 19.3 declined to above 10 points after five years of rivastigmine treatment, while model-based projections predicted that without treatment it would have declined to below 10 points by three years. Other studies reported clinically meaningful treatment benefits compared with historical controls for two years of treatment (Small et al., 2005Grossberg et al., 2004).

Overall, the acetylcholinesterase therapies donepezil, rivastigmine, and galantamine are viewed as having similar efficacy and safety, though few side-by-side comparisons exist. One three-month study randomized 111 patients with mild to moderate AD to either donepezil titrated up to 10 mg once daily, or rivastigmine capsules up to 6 mg twice daily. Both regimens led to comparable symptomatic benefits on cognition. Donepezil was better tolerated, though this has since changed with the rivastigmine patch (Wilkinson et al., 2002). A two-year trial comparing rivastigmine to donepezil in 994 patients with moderate to severe AD found that almost half dropped out, most due to gastrointestinal side effects. Those who continued had a similar benefit with either therapy on measures of cognition and behavior, though rivastigmine appeared to perform slightly better on activities of daily living and global function. Subgroup analyses hinted at better results for rivastigmine in patients with a particular butyrylcholinesterase genotype and patients who had symptoms of concomitant Lewy body disease, as well (Bullock et al, 2005). A two-year observational study associated long-term use of rivastigmine with a higher risk of death than long-term use of donepezil (Kazmierski et al., 2018).

Several trials have evaluated rivastigmine for dementia with Lewy bodies and Parkinson's disease dementia. This came up when routine clinical use of cholinesterase inhibitors in dementia patients with symptoms other than typical AD seemed to show a cognitive and clinical benefit in a large minority of such patients (Pakrasi et al., 2003). One multicenter, five-month trial in 120 patients with DLB showed a benefit on apathy, anxiety, delusions, hallucinations, as well as on cognitive performance and attention in the treatment group. About two-thirds of the treated patients had a clinically signifcant response to rivastigmine (McKeith et al., 2000). Based on this and subsequent trials of other cholinesterase inhibitors (see Wang et al, 2014), these medicines are now widely used to treat cognitive and behavioral symptoms in patients with DLB (see Hershey and Coleman-Jackson, 2019).

Hallucinations are a hallmark symptom of DLB and PDD. They represent an area of overlap between these forms of dementia and AD. Retrospective analysis of several trials indicates that AD patients with hallucinations respond better to rivastigmine treatment than those without (Cummings et al., 2010). More broadly, rivastigmine treatment appears to reduce somewhat the concomitant use of antipsychotic medications in people with AD (Scharre et al., 2010). How to manage behavioral symptoms of AD has become a pressing issue since 2005, when the FDA responded with a black-box warning to reports of increased mortality in dementia patients on antipsychotics (see Feb 2011 newsOct 2005 news).

The EXPRESS (EXelon in PaRkinson's disEaSe dementia Study) trial tested 3 to 12 mg/day of rivastigmine capsules in 541 patients with Parkinson's disease dementia, first for six months in a double-blind and placebo controlled phase, and then in an open-label extension. At six months, treated patients improved modestly on cognition, overall function, and psychiatric symptoms. By 48 weeks, the mean ADAS-cog score for the treatment group remained above baseline, and placebo patients who switched to rivastigmine for the extension phase had a treatment benefit similar to that of the original rivastigmine group during the double-blind trial. Again, patients with hallucinations tended to respond better (e.g., Poewe et al., 2006). Rivastigmine also appeared to improve apathy in patients with advanced PD who did not yet have dementia; this finding is from a small trial conducted in France (Devos et al., 2014). A university-sponsored trial directly assessed the effect of rivastigmine capsules on hallucinations in people with Parkinson’s. It intended to enroll 168 participants with minor visual hallucinations at baseline, with a primary outcome of time to develop psychosis or dementia. Slow recruitment and lack of funding forced this trial to stop at 91 participants; in this study population rivastigmine did not alter the primary endpoint compared to placebo (van Mierlo et al., 2021).

From 2008-2010, Novartis ran an 18-month trial comparing the capsule and patch formulations in 583 patients with mild to moderately severe PDD. This trial assessed whether long-term treatment with rivastigmine led to worsening of motor symptoms while improving cognitive and psychiatric symptoms of PDD. Traditionally, cholinergic agents have been contraindicated for the motor and autonomic aspects of PD. This is in part because anticholinergic drugs, particularly muscarinic receptor antagonists, have been used for the symptomatic treatment of PD since the late 19th century. According to published results, rivastigmine in this trial caused no additional decline in motor function beyond that expected due to natural disease progression. The most common side effects were nausea, vomiting, and tremor, consistent with other studies (Emre et al., 2014).

Gait stability and falls are related to cholinergic deficits in people with Parkinson’s disease. The Phase 2 Rivastigmine for Gait Stability in Parkinson’s Disease (ReSPonD) trial randomized 130 Parkinson’s patients without dementia, who had fallen at least once in the previous year, to 32 weeks of rivastigmine or placebo. Treatment improved the primary outcome of gait variability (Henderson et al., 2016). A Phase 3 trial running through November 2023 is assessing the effect of a rivastigmine patch on the number of falls over one year in 600 patients. The protocol is published (Neumann et al., 2021).

Some studies indicate that rivastigmine may affect cerebrovascular factors influencing dementia. Alzheimer's patients who also have cardiovascular risk factors such as hypertension have been reported to respond better to rivastigmine than patients with more "pure" Alzheimer's disease, and some evidence exists for a treatment benefit of rivastigmine for vascular dementia  (e.g., Erkinjuntti et al., 2003Farlow et al., 2011Birks et al., 2013). A more recent study found no difference in the response to rivastigmine in people with either mild or moderate MRI-documented signs of ischemic damage along with Alzheimer’s (Park et al., 2017).

For some years after cholinesterase inhibitor therapies were initially approved for Alzheimer's disease, their small effect created controversy about cost-effectiveness (see Jul 2004 news and extensive commentary). Since then, studies have shown that rivastigmine is modestly effective in the long-term treatment of AD, see above. Pharmacoeconomic studies in the United States and European countries have generally found that cholinesterase inhibitor treatment reduces the cost of care (e.g., Nagy et al., 2011). In the United Kingdom, where this debate had called into question coverage of rivastigmine by this country's universal health care system, NICE in 2011 issued a guidance recommending use of rivastigmine in the treatment of mild to moderate AD (see, e.g. Fillit et al., 1999; Wimo et al., 2003; NICE guidance). In contrast, in May 2018, France’s national health care authority stopped paying for rivastigmine and other cholinesterase inhibitors, claiming an unfavorable risk/benefit ratio for these drugs (see published commentary). 

Post-marketing data on long-term safety continues to accrue. According to worldwide adverse-event monitoring systems, rivastigmine was associated with frequent neuropsychiatric and cardiovascular serious adverse events (Krõger et al., 2015). Rivastigmine use was associated with more deaths than other cholinesterase inhibitors (Ali et al., 2015), partly owing to “patch overdose.” This occurs when patients or caregivers apply a new patch without removing the old one, or apply more than one patch at a time (e.g., Lövborg et al., 2012). Rivastigmine overdose is a medical emergency that can lead to sudden cardiac death. 

More recent analysis of data from the FDA Adverse Events Reporting System (FAERS) revealed an apparently increased risk of pneumonia with rivastigmine compared to other symptomatic medications (Morris et al., 2021). This was possibly related to an increase in reports of difficulty swallowing, also in FAERS (Bu et al., 2022). A different study found a slightly increased risk of pneumonia in people using rivastigmine patches, but not capsules, in an analysis of community dwellers with Alzheimer’s disease in Finland (Lampela et al., 2017). However, a study of U.S. Medicare users noted a lower risk of pneumonia among new users of rivastigmine (Lai et al., 2015).

In other trials, rivastigmine decreased postoperative delirium in older people with cognitive dysfunction (e.g., Youn et al., 2016). It also is suggested to aid recovery after traumatic brain injury, though a recent review calls the evidence for this indication limited (Florentino et al., 2022). 

A small trial in 42 children and adolescents with Down's syndrome found no benefit on measures of cognition, language, and overall function (Spiridigliozzi et al., 2016). Trials for cognitive problems in people with multiple sclerosis were likewise negative (Cotter et al., 2018).

In 2018, Novartis began testing the rivastigmine patch in 102 Alzheimer’s patients with severe dementia. The trial, at nine sites in India, will finish in January 2023. An ongoing trial for progressive supranuclear palsy will also end in 2023.

For a list of rivastigmine trials, see clinicaltrials.gov.

Last Updated: 02 Nov 2022

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Therapeutics

Encenicline

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Overview

Name: Encenicline
Synonyms: EVP-6124 , MT-4666, α7-nAChR agonist
Chemical Name: (R)-7-chloro-N-quinuclidin-3-yl)benzo[b]thiophene-2-carboxamide
Therapy Type: Small Molecule (timeline)
Target Type: Cholinergic System (timeline)
Condition(s): Alzheimer's Disease, Schizophrenia
U.S. FDA Status: Alzheimer's Disease (Discontinued), Schizophrenia (Discontinued)
Company: FORUM Pharmaceuticals Inc., Mitsubishi Tanabe Pharma
Approved for: None

Background

Encenicline hydrochloride is a partial, selective agonist of the α-7 nicotinic acetylcholine receptor (α7-nAChR). It is being developed for the treatment of cognitive deficits in schizophrenia and Alzheimer's disease. Cholinergic function declines in Alzheimer's, and currently approved acetylcholinesterase inhibitor therapies modestly improve cognitive deficits in patients with AD by way of boosting cholinergic transmission. The rationale of selective α7-nAChR agonists is that they will enhance cognition without causing side effects associated with over-activation of other nAChRs such as α4β2, or muscarinic AChRs. In rats, encenicline penetrates the blood-brain barrier and improves memory performance by potentiating the acetylcholine response. Encenicline has been reported to act as a co-agonist with acetylcholine. It sensitizes the α-7 nACh receptor to its natural ligand and renders sub-efficacious doses of AChEI drugs effective in restoring memory function in an object recognition task (Prickaerts et al., 2012).

Findings

This compound was originally developed at Bayer Healthcare and in 2004 licensed to Envivo Pharmaceuticals, which subsequently licensed development in Asia to Mitsubishi Tanabe Pharma Corporation. In 2014, Envivo changed its name to FORUM Pharmaceuticals Inc.

Encenicline is being tested in Alzheimer's disease and schizophrenia. In Alzheimer's, an ascending-dose Phase 1/2 study showed 0.1 to 1 mg/day of EVP-6124 to be safe and well-tolerated when given to 49 people with mild to moderate AD for 28 days. No serious side effects were reported. Secondary efficacy endpoints suggested that EVP-6124 given in addition to therapy with the acetyl cholinesterase inhibitors donepezil or rivastigmine appeared to improve attention, verbal fluency, and executive function as measured on  tests in the CogState or NTB batteries (see Jul 2009 conference news). This study has posted results on clinicaltrials.gov.

A 24-week Phase 2 trial conducted in 409 people with mild to moderate Alzheimer's disease in the United States and Eastern Europe compared 0.3, 1, and 2 mg of EVP-6124 per day to placebo, measuring cognition with ADAS-Cog as the primary outcome plus cognitive, functional, and psychiatric secondary outcomes. EVP 6124 was given as adjunct therapy to donepezil or rivastigmine. This trial was reported to have met its primary and most secondary endpoints, showing that people on the highest dose improved over baseline. EVP-6124 dose-dependently improved measures of attention, verbal and language fluency, and executive function. In this trial, all treatment groups initially improved, possibly due to a placebo effect, but by 12 weeks the groups separated and the placebo and low-dose groups declined (see Jul 2012 conference news). EVP-6124 was well-tolerated.

Mitsubishi Tanabe Pharma Corporation is conducting a Phase 2 trial for the treatment of Alzheimer's disease in Japan.

In October 2013, two international Phase 3 trials began enrolling what are to be 790 patients in each trial with mild to moderate Alzheimer's who are already taking an acetylcholinesterase inhibitor. The trials will compare two fixed, undisclosed add-on doses of EVP-6124 to placebo, all given as once-daily tablets for six months, for cognitive benefit as measured by the ADAS-Cog, clinical benefit as measured by the Clinical Dementia Rating Sum of Boxes (CDR-SB), as well as for safety and tolerability. Called COGNITIV AD, this Phase 3 program is set to run through 2016.

For schizophrenia, a Phase 1 study comparing 0.3 and 1 mg/day of EVP-6124 to placebo in 28 people with the disease gave preliminary evidence for the compound's safety, tolerability, and pharmacokinetics in this population. In addition, the compound yielded signals of bioactivity in the brain by way of EEG tests of evoked potentials, a measure of sensory gating affected in this disease. See study results on clinicaltrials.gov.

A subsequent 12-week Phase 2 trial compared 0.3 and 1 mg/day of EVP-6124 to placebo in 317 people with schizophrenia and measured the compound's safety and efficacy on cognitive function. As presented at the American College of Neuropsychopharmacology meeting held in Hawaii December 2011, EVP 6124 met its primary endpoint of improvement on the CogState overall cognition index. The study also met secondary endpoints, showing improvement in clinical function as assessed by the Schizophrenia Cognition Rating Scale, and a decrease in negative symptoms. Two six-month, 700-patient Phase 3 studies, plus a six-month extension study, are ongoing. 

In September 2015, the FDA placed a clinical hold on three Phase 3 Alzheimer studies and one schizophrenia safety extension study following reports of gastrointestinal side effects (see Sep 2015 news). In November 2015, the FDA lifted a partial clinical hold on encenicline evaluation for cognitive impairment in schizophrenia; two global Phase 3 trials in this indication are expected to deliver results in the first half of 2016  (see company press release). As of January 2016, the clinical hold on the AD trials remained in place. 

In March 2016, Forum announced topline from the two cognitive function in schizophrenia phase 3 studies. Encenicline missed the co-primary endpoints in both trials (see company press release). Development of this compound has ended, and Forum Pharmaceuticals has been shut down.

For all clinical trials of encenicline, see clincialtrials.gov

Clinical Trial Timeline

  • Phase 2
  • Phase 3
  • Study completed / Planned end date
  • Planned end date unavailable
  • Study aborted
Sponsor Clinical Trial 2008 2009 2010 2011 2012 2013 2014 2015 2016 2017 2018 2019 2020 2021 2022 2023 2024 2025 2026 2027 2028 2029 2030 2031 2032 2033 2034 2035
FORUM Pharmaceuticals Inc. NCT01073228
N=409
FORUM Pharmaceuticals Inc. NCT01969123
N=790
FORUM Pharmaceuticals Inc. NCT01969136
N=790

Last Updated: 15 Jan 2016

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Therapeutics

Etanercept

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Overview

Name: Etanercept
Synonyms: Enbrel™
Therapy Type: Immunotherapy (passive) (timeline)
Target Type: Inflammation (timeline)
Condition(s): Alzheimer's Disease
U.S. FDA Status: Alzheimer's Disease (Phase 2)
Company: Amgen, Inc., Pfizer
Approved for: Rheumatoid Arthritis, Psoriatic Arthritis, Ankylosing Spondilysis

Background

Etanercept inhibits the function of a pro-inflammatory cytokine called tumor necrosis factor alpha (TNF-α). Etanercept is a fusion protein consisting of two copies of the TNF-α receptor and the Fc end of the immunoglobulin G (IgG) antibody. Also called a decoy receptor, etanercept binds both the soluble and the membrane-bound forms of TNF-α, counteracting its signaling.

Etanercept is administered by injection under the skin, and in this form is FDA-approved for the treatment of various rheumatological and inflammatory skin conditions. Etanercept is effective and widely prescribed for these conditions. Its side effects include, among others, increased risk for serious infections including tuberculosis, invasive fungal infections, and bacterial infections such as listeria (see Enbrel.com).

The rationale of evaluating peripheral administration of etanercept as a treatment for Alzheimer's disease derives from studies suggesting that low-grade, chronic, systemic inflammation releases TNF-α, which reportedly can communicate from the periphery to the brain to induce a central immune response involving adverse microglial activation. In Alzheimer's disease patients, elevated serum TNF-α has been linked to faster decline and worse psychiatric symptoms (Perry et al., 2007; Drake et al., 2001; Holmes et al., 2009; Holmes et al., 2011).

In mouse models of neurodegenerative disease, several studies have shown that microglia are primed to activation in response to systemic inflammatory stimuli (Mar 2015 conference news; Perry and Hughes, 2014; Cunningham et al., 2005).

Findings

As of spring 2015, one double-blind, randomized, placebo-controlled trial of subcutaneous etanercept in Alzheimer's disease has been conducted, at the University of Southampton, U.K. In 2011, this investigator-initiated trial began enrolling 41 people with mild to moderate Alzheimer's disease whose MMSE fell between 10 and 27 and who had no evidence of prior exposure to tuberculosis. They were randomized to 50 mg of enbrel or matching placebo injected under the skin once a week for six months. Primary outcomes were tolerability as measured by compliance, and safety as measured by the number of and type of adverse events. Secondary outcomes were cognitive, functional, and behavioral measures, as well as cytokine measures in blood.

This trial has been published in the peer-reviewed literature (Butchart et al., 2015). In brief, etanercept was reported to be well tolerated, with 18 of 20 patients in the etanercept group completing the study compared to 15 of 21 in the placebo group. No new side effects appeared in this small AD population. As expected, infections were more common in the etanercept group. They included gastroenteritis, respiratory and urinary-tract infections, pharyngitis, and cellulitis. Eleven reports of infection were captured in nine people on etanercept compared to seven infections in six people on placebo. Of 97 side effects recorded in this study, one was serious but occurred in the placebo group. 

The secondary clinical outcomes showed no statistically significant differences, but did show trends favoring the peripheral etanercept group. Of note, decline on the ADAS-cog in the placebo group was twice what had been anticipated, and the randomization results indicated slightly worse neuropsychiatric symptoms in the placebo group at baseline. Analysis of serum inflammatory markers showed no differences between the groups at baseline, but higher serum TNF-α levels at weeks 12 and 24 weeks of treatment, as well as four weeks later after washout. This is consistent with the increased half-life of the dimeric fusion protein after binding TNF-α. 

According to the authors, this study calls for independent validation in a larger, more heterogeneous AD patient population. It does not by itself support off-label use of subcutaneous etanercept for the treatment of AD dementia (Butchart et al., 2015). It also, according to the authors, is different in concept from a hypothesized rapid change of central TNF-α through a perispinal etanercept injection. This approach has not been evaluated in RCTs and remains controversial (Novella S, Science-based Medicine, accessed 11 May 2015).

Last Updated: 12 May 2015

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Therapeutics

Estrogen

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Overview

Name: Estrogen
Synonyms: Premarin™
Therapy Type: Other
Target Type: Other (timeline)
Condition(s): Alzheimer's Disease
U.S. FDA Status: Alzheimer's Disease (Discontinued)
Approved for: Hormone Replacement Therapy

Background

There are a number of biological mechanisms through which estrogens might exert neuroprotective effects. These include the promotion of cholinergic activity (Gibbs, 2010), increase in the morphological complexity of neurons associated with learning and memory (Brinton et al., 2000), protection from toxic insult (Brinton et al., 2000), stimulation of neuron formation (Tanapat et al., 1999), and reduction of the formation of Aβ (Pike et al., 2009). 

Estrogen is a steroid hormone important in the development and maintenance of the female reproductive system and secondary sex characteristics. In addition, in vitro experiments have demonstrated that estrogen can protect cultured neurons from Aβ-related toxicity (Behl et al., 1995Goodman et al., 1996Mook-Jung et al., 1997), as well as oxidative stress-related damage (e.g., Behl, 2002). Animal models based on these mechanisms also support potential benefits of estrogen in the CNS. Estrogen is capable of decreasing amyloid accumulation and improving memory performance in ovariectomized rats (Carroll et al., 2007Shang et al., 2010), and has been shown to enhance long-term potentiation in the hippocampus in these rats (Foy et al., 1999).

Last Updated: 26 Oct 2015

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