Prion disease, always fatal, is the most common cause of rapidly progressive dementia (RPD), but about a third of RPD cases have other roots. Michael Geschwind, M.D., Ph.D., of the UCSF Memory and Aging Center, where a team of experienced experts uses a frequently updated diagnostic algorithm to guide people to appropriate treatment, says, "The challenge is diagnosing the cause because patients with non-prion RPD can see significant improvements in quality of life, and even recover in some cases."
Any patient with RPD progresses from normal cognition to dementia in less than two years, and sometimes more quickly, even within days. Outside of prion disease, the most common causes are other neurodegenerative diseases such as:
Autoimmune neurological diseases are the next most common cause, Geschwind says. More rare are cancers and a few infectious diseases. Understanding the cause can significantly affect care and outcomes.
"Patients with RPD due to Lewy body dementia often experience dramatic improvements with current treatments,” Geschwind says. "And RPD due to autoimmune diseases or cancer often might be reversed if identified early.”
Yet, because RPD is relatively rare, most physicians are not trained in evaluating these patients, and many common diagnostic tests are misunderstood. Looking for markers in cerebrospinal fluid can produce many false positives, Geschwind says. An ongoing study run by the UCSF Memory and Aging Center suggests that as often as two-thirds of the time, indications of prion disease in a patient's MRI are overlooked or poorly understood.
"Experience is critical in knowing what to look for," says Geschwind. The UCSF Memory and Aging Center likely sees more of these patients than anywhere in the world. It also has time to do the necessary detective work in a variety of its clinics, including a recently established bimonthly clinic that brings together Geschwind, a neurobehavioral expert, with Jeffrey Gelfand, M.D., a neuroimmunologist.
Prior to any visit, a thorough review of patient records allows doctors to distinguish between patients who genuinely have RPD and patients who have developed dementia symptoms imperceptibly over time and only recently experienced an accelerated decline. "For patients with true RPD, we do everything we can to expedite their evaluation because timing is crucial," Geschwind says.
New patients typically have an initial half- or full-day visit, during which physicians apply a constantly evolving diagnostic algorithm. An initial evaluation helps eliminate common causes of dementia-like symptoms, such as electrolyte or metabolic disturbances.
Then the emphasis turns to providing a swift, accurate diagnosis, which is when things can get complicated.
Finally, says Geschwind, "Because these patients require extensive, ongoing care, we also carefully maintain continuity of care with their primary physicians."
While versions of the UCSF diagnostic algorithm have been published and are in use in other settings, it remains a work in progress. “We are continually learning from the patients we see,” says Geschwind. “The more we can refine our diagnostic procedure, the more we can provide future patients with expert care.”
For more information, contact Dr. Geschwind at (415) 476-2900.
For patient referrals, contact Shirley Reeder at (415) 476-3898.
New MS Drug Shows Promise
An experimental drug called Ocrelizumab has shown promise in a Phase 2 clinical trial involving 220 people with multiple sclerosis (MS). The study was carried out by researchers at UCSF Medical Center and other hospitals in the U.S., Canada and Europe.
UCSF Automated Pharmacy Wins Popular Science Innovation Award
UCSF Medical Center's automated pharmacy has won a 2011 Best of What's New award from Popular Science. The awards recognize 100 innovations that indicate where technology is headed in the future.