docSELF-MUTILATION IN A LESCH
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SELF-MUTILATION IN A LESCH
SELF-MUTILATION SELF-MUTILATIONIN IN LESCH-NYHAN LESCH-NYHANSYNDROME SYNDROME --------------------------------------------BERLIN BERLIN--2004 2004 Rita CAUWELS, DDS Dept. Paediatric Dentistry and special Care PAECAMED-research GHENT UNIVERSITY - BELGIUM LESCH-NYHAN SYNDROME BERLIN-2004-RC 1 SELF-MUTILATION SELF-MUTILATION DELIBIRATE DESTRUCTION OF BODY TISSUE WITHOUT CONSCIOUS SUICIDAL INTENT SEVERAL PSYCHIATRIC DISORDERS VARIOUS DEVELOPMENTAL DISABILITIES SYNDROMES LESCH-NYHAN SYNDROME BERLIN-2004-RC 2 LESCH-NYHAN SYNDROME BACKGROUND X-linked recessive condition Metabolic error: absence of enzyme HPRT excessive production of uric acid LESCH-NYHAN SYNDROME BERLIN-2004-RC 3 LESCH-NYHAN SYNDROME CLINICAL MANIFESTATIONS 1 Severe mental retardation 2 Spasticity 3 Choreoathetosis 4 Self-mutilation 5 Aggression towards others LESCH-NYHAN SYNDROME BERLIN-2004-RC 4 LESCH-NYHAN SYNDROME CASE LESCH-NYHAN SYNDROME BERLIN-2004-RC 5 Self-injurious behavior involving his lower lip LESCH-NYHAN SYNDROME BERLIN-2004-RC 6 TREATMENT TREATMENT ?? NO NO PREDICTABLE PREDICTABLE RESULT RESULT !! MOUTHGUARD ERKODENT ® LESCH-NYHAN SYNDROME BERLIN-2004-RC 7 22 WEEKS WEEKS LATER LATER LESCH-NYHAN SYNDROME BERLIN-2004-RC 8 SELF-MUTILATION 1 month later It is not unusual that patients may be mutilative for weeks and months and then, without appliance, return to normal behavior and restart afterwards LESCH-NYHAN SYNDROME BERLIN-2004-RC 9 SELF-MUTILATION SELF-MUTILATION RESTARTED UPPER AND LOWER MOUTHGUARD LESCH-NYHAN SYNDROME BERLIN-2004-RC 10 APPLIANCE CONSTRUCTION DISTANCE BETWEEN LOWER ARCH AND LOWER LIP DO NOT ALLOW SUCKING IN OF THE LOWER LIP. LESCH-NYHAN SYNDROME BERLIN-2004-RC 11 LIP-BUMPER LESCH-NYHAN SYNDROME BERLIN-2004-RC 12 LIP-BUMPER RECALL 2 MONTHS LATER LESCH-NYHAN SYNDROME BERLIN-2004-RC 13 C E H S U O U N I T ON LESCH-NYHAN SYNDROME G N I AL BERLIN-2004-RC 14 CONCLUSION CONCLUSION TO CONTROL SELF-MUTILATION • Extraction ? • Restrictive approach ? • TRIAL AND ERROR • The least restrictive but most effective treatment ! LESCH-NYHAN SYNDROME BERLIN-2004-RC 15
