Kohlschütter-Tönz Syndrome - Report of an additional case.
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. Key words:Kohlschütter-Tönz syndrome, amelogenesis imperfecta, seizures, mental deterioration.
DDS, MSc. Oral Diagnosis Department, Semiology and Oral Pathology, Piracicaba Dental School, State University of Campinas (UNICAMP), Piracicaba, Sao Paulo, Brazil ; Assistant professor. Oral Pathology and Diagnosis, Facultad de Odontología, Universidad de Valparaíso, Valparaíso, Chile.
Wilfredo A
WA
González-Arriagada
DDS. Oral Pathology Section, Centro Clínico de Cabeza y Cuello/Hospital Herrera Llerandi, Guatemala City, Guatemala.
Román
R
Carlos-Bregni
DDS. Oral Pathology Section, Centro Clínico de Cabeza y Cuello/Hospital Herrera Llerandi, Guatemala City, Guatemala.
Elisa
E
Contreras
DDS, PhD. Oral Diagnosis Department, Semiology and Oral Pathology, Piracicaba Dental School, State University of Campinas (UNICAMP), Piracicaba, Sao Paulo, Brazil.
Oslei P
OP
Almeida
DDS, PhD. Oral Diagnosis Department, Semiology and Oral Pathology, Piracicaba Dental School, State University of Campinas (UNICAMP), Piracicaba, Sao Paulo, Brazil.
Marcio A
MA
Lopes