An Experimental Tau Inhibitor Could Treat bvFTD, A Rare Form of Dementia


If you think about it, our brains are pretty amazing. It is one of our most complex features and contains tens of billions of neurons which help control the way your body acts. But in neurodegenerative conditions, neurons die. Many times, this is caused by an over-accumulation of a protein called tau. As a result, the brain degenerates and atrophies, inhibiting communication between the brain and body, and causing issues with movement, behavior, and much more. While tau inhibitors are effective treatments for Alzheimer’s disease, researchers also wanted to understand if it could be used to treat a rare form of dementia called behavioral variant frontotemporal dementia (bvFTD). Find the full results of their study in Journal of Alzheimer’s Disease.


Behavioral variant frontotemporal dementia (bvFTD) is a rare form of dementia that occurs before the age of 65. Despite it being rare, it is also the 2nd most common cause of dementia in that age group. Although there is no clear cause, people with bvFTD usually have a buildup of one of three proteins in their brain:

  • Tau
  • Fused-in sarcoma (FUS)
  • TDP-43

Symptoms of bvFTD include:

  • Behavioral Issues: In addition to having trouble controlling behaviors, people with bvFTD may be impulsive, inappropriate, or lack manners. They may also exhibit rule and law-breaking behaviors. Changes in behavior and inhibition may result in touching or acting inappropriately towards strangers, throwing temper tantrums, public urination, or acting out sexually.
  • Changes in Personality: This can include becoming apathetic, self-centered, and socially isolated. Additionally, individuals may become restless, angry, aggressive, violent, or overly sentimental.
  • Overeating and/or only eating specific types of food
  • Reduced sympathy and empathy
  • Impaired judgment
  • Issues with memory, organization, and planning
  • Delusions
  • Compulsive behaviors such as hoarding, acting out certain movements, or frequent urination
  • Forgetfulness
  • Muscle weakness
  • Problems with speech

Most patients do not notice that their behavior has changed. However, many patients with bvFTD do understand the world around them until later stages of the disease. Learn more about bvFTD.

Utilizing Tau Inhibitors

Developed by TauRx Therapeutics, hydromethylthionine is designed to treat patients with Alzheimer’s disease. The experimental tau inhibitor prevents TDP-43 and tau from building up in the brain.

Researchers ran a Phase III clinical trial which examined 220 patients with bvFTD. Of these, the mean age was 63. 139 patients (63%) were males. During the trial, researchers sought to understand whether 200mg of hydromethylthionine taken daily would be more effective than a “control” dose of 8mg taken daily.

At first, the results showed that there were no differences in efficacy between the 200mg and 8mg doses. However, researchers then ran more tests. They used plasma concentration data from 176 patients (80%) and performed an analysis. As a result, they found that the 200mg doses resulted in higher plasma concentrations than the smaller doses. Ultimately, these were linked to worse patient outcomes.

Based on their findings, researchers believe that 30mg hydromethylthionine taken daily would be an ideal dose to treat bvFTD. Moving forward, TauRx Therapeutics will test whether this is true. Additionally, their next trial will be placebo-controlled to allow for a clearer understanding of hydromethylthionine’s benefits.

Read the original source here.

Jessica Lynn

Jessica Lynn

Jessica Lynn has an educational background in writing and marketing. She firmly believes in the power of writing in amplifying voices, and looks forward to doing so for the rare disease community.

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