Title : Neurogentic Disorders with Diagnostic Oro-Dental Anomalies
Developmental oral and dental anomalies can occur as an isolated anomaly or as a part of a syndrome. The aim of this study was to document the most common neurogenetics disorders presenting with oro-dental anomalies which aid in the diagnosis of these disorders. The dental records of neurogenetic patients attending Oro-dental Genetics and neurogenetics clinics, National research centre, Egypt were reviewed and evaluated and the most frequent disorders with diagnostic oral and dental anomalies were recorded. Extensive oral ulceration commencing with eruption of teeth in addition to premature loss of teeth were frequent oral findings in congenital insensitivity to pain. Cleft lip/palate and/or solitary median central incisors were found in holoprosencephaly. OFD6 patients showed median notching of upper lip and lobulated tongue. Other Oro-denal anomalies were premature loss of teeth in Coffin Lowry syndrome patients, tongue fasciculation in spinal muscular atrophy and severe skeletal open bite and malocclusion in myotonic dystrophy patients. Microdontia was found in microcephalic osteodysplastic primordial dwarfism type II (MOPD II) and the presence of macrodontia was diagnostic in KBG syndrome. High caries index was present in Cockayne syndrome. To conclude, oral and dental anomalies represent important constituents of several neurogenetic disorders acting as a diagnostic clue in identifying a large number of these disorders. Oro-dental examination should be an integral part of clinical evaluation of patients with neurogenetic disorders.