Shock treatments give hope to people left speechless due to mystery brain disorder

© Edgard Garrido
Researchers are making inroads in how to diagnose and treat a rare, mysterious brain disorder that robs patients of their words. Current diagnostics commonly mistake it for Alzheimer’s or age-related dementia, though it affects speech and not memory.

Primary progressive aphasia (PPA) is a neurological syndrome that begins gradually, often before age 65, and worsens over time, according to the Mayo Clinic. Patients will have trouble expressing thoughts, as well as a understanding or finding words.  In time they lose their ability to speak and write and, eventually, to understand written or spoken language.

"I'm using a speech device to talk to you," Robert Voogt, a 66-year-old who was diagnosed with PPA a decade ago, ‘said’ by playing a recording from a phone-sized assistive device at a meeting of the American Association for the Advancement of Science on Sunday. "I have trouble speaking, but I can understand you."

PPA patients are often able to continue caring for themselves and participate in daily activities for several years after symptoms begin. Families don’t seek treatment after that because they assume that the increasingly garbled attempts to communicate are because of age-related dementia, Dr. Argye Elizabeth Hillis of Johns Hopkins University told AP.

"Nobody's talking to them, nobody's involving them. It's very sad," Dr. Margaret Rogers of the American Speech-Language-Hearing Association told the wire service. Yet for many, "they can handle their own finances, they can drive, they can appreciate music. There's a lot that still works for them."

The symptoms are similar to those of stroke or brain injury victims, but PPA is a degeneration of the brain regions that control language caused by disease, rather than a specific trauma to the organ.

Those doctors who are aware of the rare form of aphasia use special MRI scans to tell the difference between PPA and other neurological problems, Hillis said. They are also used to help identify patients whose aphasia will worsen faster, as well as who has a subtype that can morph into a condition similar to Alzheimer's, where the patients eventually do lose memory and their ability to understand language.

Hillis’ team at Hopkins is testing to see whether a a kind of brain stimulation that sends electrical signals through the skull can increase the effects of the standard PPA treatment of language therapy, which has patients match pictures to the correct word. The goal of the treatment is to keep the brain ‘wiring’ involved with communication as active as possible.

Two months after receiving the electrical stimulation, the first 19 patients in the study performed better at retrieving the rights words compared to getting a placebo “zap,” Hillis said. The PPA sufferers were able to name more objects they hadn’t practiced with, and brain scans showed better connectivity in the affected region.

The research is still preliminary, and the Hopkins team still needs to study whether the treatment produces long-term effects, she noted.

Richard Tsai at the University of California, San Francisco is also studying how to better diagnose PPA and similar diseases through PET imaging. He focuses on tau mutation carriers, and studied three with a clinical diagnosis of the nonfluent variant of primary progressive aphasia (nfvPPA) among a larger sample of similar brain diseases.

He found that, while the distribution and strength of the tau signal varied, by and large the ligand bound to regions affected by each specific disease, Tsai told Alzforum. On top of that, the imaging brought out subtle differences among each person that fit with their individual pathologies.

Among the three nfvPPA patients, PET imaging correlated with subtle differences, such as the  tracer bound predominantly on the left side of the brain for the two right-handed volunteers, while the lefty had a more even distribution between the two hemispheres of the brain.