TY - JOUR
T1 - Kohlschütter-tönz syndrome-report of an additional case
AU - González-Arriagada, Wilfredo Alejandro
AU - Carlos-Bregni, Román
AU - Contreras, Elisa
AU - Almeida, Oslei Paes de
AU - Lopes, Marcio Ajudarte
PY - 2013/4
Y1 - 2013/4
N2 - Kohlschütter-Tönz Syndrome is a rare disorder clinically characterized by amelogenesis imperfecta, epilepsy and progressive mental deterioration. We present an additional case of this syndrome of a nine year-old boy who was referred by pigmented teeth. The mental deterioration was associated with speech delay, impulsive behavior, attention-deficit/hyperactivity disorder, and learning problems. The physical examination revealed a reduction of lower third, slightly palpebral fissures, low ear and hair implantation, coarse hair and hypertrichosis. The intraoral examination showed alteration in teeth pigmentation diagnosed as amelogenesis imperfecta. Although rare, the present case report illustrates a syndrome that has dental anomalies and systemic alterations. It is important to recognize this syndrome as early as possible and paediatric dentist may contribute to the diagnosis and consequently to better manage the patients.
AB - Kohlschütter-Tönz Syndrome is a rare disorder clinically characterized by amelogenesis imperfecta, epilepsy and progressive mental deterioration. We present an additional case of this syndrome of a nine year-old boy who was referred by pigmented teeth. The mental deterioration was associated with speech delay, impulsive behavior, attention-deficit/hyperactivity disorder, and learning problems. The physical examination revealed a reduction of lower third, slightly palpebral fissures, low ear and hair implantation, coarse hair and hypertrichosis. The intraoral examination showed alteration in teeth pigmentation diagnosed as amelogenesis imperfecta. Although rare, the present case report illustrates a syndrome that has dental anomalies and systemic alterations. It is important to recognize this syndrome as early as possible and paediatric dentist may contribute to the diagnosis and consequently to better manage the patients.
KW - Amelogenesis imperfecta
KW - Kohlschütter-Tönz syndrome
KW - Mental deterioration.
KW - Seizures
UR - http://www.scopus.com/inward/record.url?scp=84880401672&partnerID=8YFLogxK
U2 - 10.4317/jced.51018
DO - 10.4317/jced.51018
M3 - Article
AN - SCOPUS:84880401672
SN - 1989-5488
VL - 5
SP - e108-e111
JO - Journal of Clinical and Experimental Dentistry
JF - Journal of Clinical and Experimental Dentistry
IS - 2
ER -