home
***
CD-ROM
|
disk
|
FTP
|
other
***
search
/
CD-ROM Today (UK) (Spanish) 15
/
CDRT.iso
/
dp
/
0391
/
03916.txt
< prev
next >
Wrap
Text File
|
1994-01-17
|
6KB
|
158 lines
$Unique_ID{BRK03916}
$Pretitle{}
$Title{Landau-Kleffner Syndrome}
$Subject{Landau-Kleffner Syndrome Infantile Acquired Aphasia Epilepsy }
$Volume{}
$Log{}
Copyright (C) 1986, 1987, 1990, 1991 National Organization for Rare
Disorders, Inc.
260:
Landau-Kleffner Syndrome
** IMPORTANT **
It is possible the main title of the article (Landau-Kleffner Syndrome)
is not the name you expected. Please check the SYNONYMS listing to find the
alternate names, disorder subdivisions, and related disorders covered by this
article.
Synonyms
Infantile Acquired Aphasia
Information about the following disorder can be found in the Related
Disorders section of this report:
Epilepsy
General Discussion
** REMINDER **
The information contained in the Rare Disease Database is provided for
educational purposes only. It should not be used for diagnostic or treatment
purposes. If you wish to obtain more information about this disorder, please
contact your personal physician and/or the agencies listed in the "Resources"
section of this report.
Landau-Kleffner Syndrome is a disorder that occurs exclusively in
childhood. It is primarily a speech disorder characterized by loss of
ability to speak (aphasia), paroxysmal changes in the electroencephalogram
(EEG) and occasionally, epileptic seizures. It may also be associated with
an inability to recognize the significance of sounds (auditory agnosia).
Symptoms
The symptoms of Landau-Kleffner Syndrome include regression in previous
speech development, paroxysmal changes in the electroencephalogram (EEG), and
occasionally epileptic seizures. A slowly evolving disorder, it may also be
associated with an inability to recognize the significance of sounds
(auditory agnosia). This disorder seems to improve with time in 60 percent
of cases, and be persistent in forty percent of the cases reported. In
general, the prognosis seems to be more serious in cases of very early onset,
and less serious in cases of later onset.
Causes
The cause of Landau-Kleffner Syndrome is unknown at this time. It is a
neurological disorder affecting the speech centers of the brain.
Affected Population
Landau-Kleffner syndrome seems to be exclusively a childhood disease.
Identified in 1957, only eighty cases of this extremely rare disorder had
been reported as of 1982.
Related Disorders
Other childhood aphasias may have similar symptoms to Landau-Kleffner. The
major difference is that the later Landau-Kleffner begins, the better the
prognosis for language development. The aphasia tends to vary greatly among
patients. The type and severity of language loss are related to the location
and extent of the brain dysfunction. Disabilities can range from a temporary
slurring of speech to total loss of communication.
Epilepsy, which is a symptomatic component of Landau-Kleffner Syndrome,
is a central nervous system disorder characterized by a sudden, aimless, and
uncontrollable discharge of nerves in the brain. This discharge is sometimes
preceded by an aura and is often characterized by a convulsion which is
accompanied by the loss of consciousness. There are many different types of
epilepsy. The disorder is not usually life-threatening and those affected
can often lead a full and active life with medication. (For more
information, choose "epilepsy" as your search term in the Rare Disease
Database).
Therapies: Standard
Treatment of Landau-Kleffner Syndrome is generally symptomatic and
supportive. Antiepileptic drugs have not been consistently effective in all
patients. Speech therapy may be of benefit in some cases. Neuroradiological
investigations are useful for diagnostic purposes.
Therapies: Investigational
There is a need for more postmortem cerebral examinations to further research
on Landau-Kleffner Syndrome.
A new type of surgery is being investigated for children suffering from
Landau-Kleffner Syndrome. The Subpial Transection may restore hearing and
speech and eliminate seizures. For more information on this procedure,
contact:
Rush Presbyterian, St. Lukes Medical Center
1753 West Congress Parkway
Chicago, IL 60612
(312) 942-5000 or (312) 942-5939
A new non-surgical treatment is being tested on Landau-Kleffner patients.
Corticosteroid therapy, if given early, can in many cases restore speech and
eliminate seizures. Further study is needed to determine the long-term
safety and effectiveness of this treatment.
This disease entry is based upon medical information available through
July 1991. Since NORD's resources are limited, it is not possible to keep
every entry in the Rare Disease Database completely current and accurate.
Please check with the agencies listed in the Resources section for the most
current information about this disorder.
Resources
For more information on Landau-Kleffner Syndrome, please contact:
National Organization for Rare Disorders (NORD)
P.O. Box 8923
New Fairfield, CT 06812-1783
(203) 746-6518
C.A.N.D.L.E.
(Childhood Aphasia, Neurological Disorders, Landau-Kleffner Syndrome &
Epilepsy)
4414 McCampbell Dr.
Montgomery, AL
205-271-3947
NIH/National Institute of Deafness & Other Communication Disorders
(NIDCD)
9000 Rockville Pike
Bethesda, MD 20892
(301) 496-5751
American Speech-Language-Hearing Association
10801 Rockville Pike
Rockville, Maryland 20852
References
THE LANDAU-KLEFFNER SYNDROME: INFANTILE "ACQUIRED" APHASIA, PAROXYSMAL
ELECTROENCEPHALOGRAPHIC CHANGES AND EPILEPTIC SEIZURES: M. Dugas; Nouv
Presse Med. (Dec. 18, 1982: 11(51)). Pp. 3787-91. (Published in French).
AGE OF ONSET AND OUTCOME IN 'ACQUIRED APHASIA WITH CONVULSIVE DISORDER'
(LANDAU-KLEFFNER SYNDROME): D.V. Bishop; Dev Med Child Neurol (Dec. 1985:
27(6)). Pp.705-12.
CONTRIBUTION TO OUR KNOWLEDGE OF THE LANDAU AND KLEFFNER "ACQUIRED
APHASIA WITH EPILEPSY" SYNDROME: A. Lorizio and A. Franciosi; Riv Patol Nerv
Ment. (Sept.-Oct. 1982 103(5)). Pp. 201-214. (Published in Italian).