Rapidly Progressive Dementias: Current Treatments

Rapidly Progressive Dementias Current Treatment

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[Anonymous]

Slide 42 is entitled compassionate use of quinacrine.

We are now in a neuroimaging era. Early diagnostic differentiation of the dementias via this route is being undertaken and attempted. As such, findings on PET scans in CJD patients must be compassionately dealt with. My loved one's PET demonstrated diffusely reduced metabolism to the frontal lobes; decreased to the parietal lobes, thalamus, and basal ganglia.

The "off label" use of intranasal insulin for AD has been brought up at least twice this week in World News.

It would appear to me that the most compassionate use of an "off label" medication regimen for CJD would include intranasal insulin.

An NIH doctor presenting at an educational seminar told me and a small group of internal medicine clinicians that he treats his LBD patients with intranasal insulin and DDAVP. We were discussing examples of how to manage some of the more troubling systemic symptoms of a rapidly dying ANS and the cardiovascular needs of this particular RPD population. How dehydration factors in, etc.

CJD patients present with similar complications, they simply are not managed, discussed or addressed in the literature or with the family. It is not that they are not happening. "Off label" use of a cholinesterase inhibitor was improperly prescribed for my loved one who already had sinus bradycardia and a first degree AVB with a longstanding history decreased heart rate variability and dizziness.

If a drug is to be given, lets start looking a little more closely at what really does a body good shall we. These patients can be eased to death rather than catapulted there! This population of patients is slowly called upon to trial drugs that may slow the progression of all dementias. (An issue that medicine really cannot decide if it wants to commit to as evidenced by lack of adequate funding and lack of transparent discussion).

CJD patients really should not be last in line for truly therapeutic invterventions. And who is standing up to ensure these issues and needs are addressed in real time?! It is not memory and computation we are seeking to enhance; their calculus days are over. Withholding basic therapy that helps to meet the metabolic needs at the cellular level is addressing the problem.

Every 2 years or so the professional groups weigh in on best practice measures for cardiac patients and these are published in the AHA guidelines.

What professional group is responsible for the lackluster direction for updating the treatment of CJD? I'd like to be invited to tell my tale and present my legitimate complaints for review and discussion.

Performing brain autopsy only does not excuse American neurology and psychiatry from addressing what is going on elsewhere as a result of that dying ANS... especially when you scan early and often, run screening EKG's, and now know to look closely at those sleep studies and ERG's. Insulin verses cholinesterase inhibitor. Which "off label" use is therapeutic for CJD patients? Which should have been indicated for my loved one? I wish I had had the informed choice and guidance of practitioners who cared. In his own words: "I feel like I have been hit by a train. Just no energy to do anything."

[cjdblogger]

I usually offer the off-label use of doxycycline to my patients. The advantages are that I can tell them a little bit about the European observational studies, it's a rather benign drug (as opposed to quinacrine), and it's readily available. Whenever I do discuss any of the investigational treatments, I am sure to have a discussion regarding the lack of any quality of life measures in all treatment studies of CJD thus far. As we can see from the UK patient preference trial, that may be one of the most important things for family members to consider when discussing possible treatments.