[lg policy] A Thief That Robs the Brain of Language

Fri May 6 18:56:38 UTC 2011

A Thief That Robs the Brain of Language By JANE E.
BRODY<http://topics.nytimes.com/top/reference/timestopics/people/b/jane_e_brody/index.html?inline=nyt-per>

Steve Riedner of Schaumburg, Ill., was a 55-year-old tool-and-die maker, a
job that involves difficult mental calculations, and a frequent speaker at
community meetings when he found himself increasingly at a loss for words
and unable to remember numbers. He even began to have difficulty reading his
own written comments.

The neurologist he consulted thought Mr. Riedner had suffered a
stroke<http://health.nytimes.com/health/guides/disease/stroke/overview.html?inline=nyt-classifier>and
for three years treated him with
cholesterol<http://health.nytimes.com/health/guides/nutrition/cholesterol/overview.html?inline=nyt-classifier>-lowering
medication. But instead of his language ability stabilizing or improving, as
should happen following a stroke, it got worse.

A second neurologist concluded after further testing that Mr. Riedner might
have a condition called primary progressive
aphasia<http://health.nytimes.com/health/guides/disease/picks-disease/overview.html?inline=nyt-classifier>,
or P.P.A., a form of
dementia<http://health.nytimes.com/health/guides/disease/dementia/overview.html?inline=nyt-classifier>affecting
the brain’s language center.

Having seen only one other case in his career, the neurologist referred Mr.
Riedner and his wife, Mary Beth, to the Cognitive Neurology and Alzheimer’s
Disease<http://health.nytimes.com/health/guides/disease/alzheimers-disease/overview.html?inline=nyt-classifier>Center
at Northwestern
University<http://topics.nytimes.com/top/reference/timestopics/organizations/n/northwestern_university/index.html?inline=nyt-org>,
whose director, Dr. M. Marsel Mesulam, is perhaps the world’s leading expert
on this relatively rare disorder.

P.P.A. is a clinical syndrome, one of several forms of brain disease lost in
the medical shadow of their much better known relative Alzheimer’s disease.
While hardly as common as Alzheimer’s, P.P.A. is often misdiagnosed, and
many patients like Mr. Riedner lose valuable time trying inappropriate and
ineffective treatments. Though there is no cure, patients and families can
learn ways to minimize the disabilities it causes.

Unlike Alzheimer’s, P.P.A. does not affect
memory<http://health.nytimes.com/health/guides/test/mental-status-tests/overview.html?inline=nyt-classifier>,
at least not initially. It also tends to occur at younger ages, often in the
late 50s, and affects twice as many men as women. While symptoms of
Alzheimer’s are readily recognized by friends and relatives but not those
affected, people with P.P.A. are painfully aware of their struggle to
communicate, often long before it is apparent to others.

“Early symptoms are usually very subtle, and patients may be the only ones
aware of the problem because they have to work harder to communicate, even
though they may not make obvious errors,” said Joseph R. Duffy, head of
speech pathology at the Mayo
Clinic<http://topics.nytimes.com/top/reference/timestopics/organizations/m/mayo_clinic/index.html?inline=nyt-org>in
Rochester, Minn. “Initially, their communicative errors are like those
we
all make and may be written off as due to chronic
fatigue<http://health.nytimes.com/health/guides/disease/chronic-fatigue-syndrome/overview.html?inline=nyt-classifier>.
But gradually the errors of speech occur with increasing frequency.”

*‘Like Being a Parent Again’*

The disorder is also commonly misdiagnosed, usually as Alzheimer’s disease
or a result of depression or anxiety, Dr. Duffy said in an interview. But
while a definitive diagnosis can be made only at autopsy, the results of
neuropsychological testing and the patient’s progressive difficulty with
communication despite a normal memory can pinpoint P.P.A. as the problem.

As the condition worsens, cognitive difficulties may become apparent. Now
seven years into the disorder, Mr. Riedner is having increasing problems
performing the tasks of daily living.

“He can no longer punch in the numbers to operate the garage door or the
microwave or the remote for the TV,” Mrs. Riedner, his wife of 38 years,
said in an interview. “He might open the car window, then not know how to
close it. We bought him an
iPad<http://topics.nytimes.com/top/reference/timestopics/subjects/i/ipad/index.html?inline=nyt-classifier>,
but he can’t learn how to use it.”

New ways of communicating have to be learned, like using gestures or making
lists of things to say or do or eat, so that the person can point to them
when needed. Unfortunately, sign language doesn’t work because it, too,
depends on the region of the brain that is diseased.

“In some ways it’s like being a parent again. He acts in childlike ways
sometimes, but he still wants to be himself, an independent adult,” Mrs.
Riedner said. “Coping with the problem takes an immense amount of patience
on both our parts.”

Mrs. Riedner said the center at Northwestern has been “a lifesaver for us,”
a source of support, speech therapy and rehabilitation, help with practical
matters like applying for Social
Security<http://topics.nytimes.com/top/reference/timestopics/subjects/s/social_security_us/index.html?inline=nyt-classifier>disability
insurance, and tips on how to minimize the inevitable
frustrations that can accompany lost language skills.

Dr. Mesulam was the first in modern times to recognize the distinction
between P.P.A. and other forms of dementia.

In 1975, as a newly minted neurologist at Beth Israel Hospital in Boston, he
became puzzled by a group of patients who seemed to know what they wanted to
say but couldn’t string words together in an intelligible sequence. Their
communicative problems got progressively worse, although there were no
lesions apparent in their brains.

His report in 1982 on six such cases stimulated modern interest in P.P.A. As
Dr. Mesulam explained in a retrospective article in the journal Alzheimer
Disease and Associated
Disorders<http://journals.lww.com/alzheimerjournal/Abstract/2007/10000/Primary_Progressive_Aphasia__A_25_year.9.aspx>in
the fall of 2007, “The
aphasia<http://health.nytimes.com/health/guides/symptoms/speech-impairment-adult/overview.html?inline=nyt-classifier>can
interfere with word-finding, object naming, syntax, phonology,
morphology, spelling or word comprehension. The progression occurs in the
course of years rather than months, and the primary nature of the aphasia is
demonstrated by showing that memory for recent events, recognition of
familiar faces and objects, reasoning, and basic aspects of comportment are
relatively preserved at the initial stages.”

*Focus on What Remains*

With no therapy available to treat the underlying disease, treatment
involves determining which aspects of language are strongest, then providing
speech therapy that emphasizes those areas and avoids areas in which
patients are weakest, Dr. Mesulam said.

Dr. Duffy said that early intervention is important and should focus on
alternative approaches to communication like using a computer, picture
icons, or iPhones or iPads that have apps that can talk for a person. Family
members are taught how to aid comprehension by communicating simply and
clearly: telling patients only one thing at a time, reducing noise and other
distractions, and avoiding multitasking.

Family members are also taught coping skills and helped to prepare for the
future as the disease progresses, said Darby Morhardt, a social worker at
the Northwestern center. “There’s inevitably a grieving process as patients
change right before their eyes, but at the same time families want to grasp
as much as possible of what remains.”

Patients often are encouraged to develop new hobbies and skills that don’t
rely on communication, like woodworking, gardening, fishing, sculpting or
painting. Among Northwestern’s patients is one who began breeding pigeons
and another who started judging dog races.

Unfortunately, if patients live long enough, other deficits — including
those involving memory and cognitive abilities, and motor problems
resembling symptoms of Parkinson’s
disease<http://health.nytimes.com/health/guides/disease/parkinsons-disease/overview.html?inline=nyt-classifier>—
often become apparent, as is now happening to Mr. Riedner.

Like Alzheimer’s disease, P.P.A. shortens life expectancy: The average age
of death is 67. Ms. Morhardt said it’s important for patients and their
families not to delay getting financial matters in order. Many would be wise
to consult a lawyer who specializes in elder law, she said.

This article has been revised to reflect the following correction:

*Correction: May 5, 2011*

The Personal Health column on Tuesday, about primary progressive aphasia, a
type of dementia that affects the brain’s language center, misspelled the
hometown of Steve Riedner, who struggled with symptoms for years before the
disease was correctly diagnosed. It is Schaumburg, Ill., not Schaumberg.

http://www.nytimes.com/2011/05/03/health/03brody.html?scp=1&sq=A%20thief%20that%20robs%20the%20brain&st=cse

-- 
=+=+=+=+=+=+=+=+=+=+=+=+=+=+=+=+

 Harold F. Schiffman

Professor Emeritus of
 Dravidian Linguistics and Culture
Dept. of South Asia Studies
University of Pennsylvania
Philadelphia, PA 19104-6305

Phone:  (215) 898-7475
Fax:  (215) 573-2138

Email:  haroldfs at gmail.com
http://ccat.sas.upenn.edu/~haroldfs/

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