Gilles de la Tourette Syndrome (GTS) is a neuropsychiatric disorder defined by the motor and phonic tics affecting approximately 1% of the children worldwide. The symptoms of GTS typically arise at the age of 5 to 7 and generally improve with increasing age. Affected individuals can have a social stigma and poor quality of life, especially when tics are severe or accompanied by other neuropsychiatric disorders. Abnormalities in neurotransmitter signaling affecting basal ganglia circuits have been suggested as representatives of neurobiological mechanisms underlying GTS. While several evidences suggest GTS as an inherited disorder, the detailed genetic abnormalities responsible for the pathophysiology of GTS remain poorly understood. Currently, there is no satisfactory treatment option for moderate-to-severe GTS due to the limited efficacy, often complicated with side effects of available pharmacological drugs. Therefore, a number of animal models have been established to explore potential pathophysiological targets in GTS and to further screen candidate drugs. In this review, we revisit the experimental findings that describe the genetic and immunologic abnormalities in GTS as well as animal models established for studying GTS.
Parosteal osteosarcoma, a subtype of juxtacortical osteosarcoma, has a better prognosis compared to central osteosarcoma with a relatively low risk for recurrence and metastasis. Rarely, it can arise synchronously with other malignant tumors. Synchronous malignancies are defined as the occurrence of a second primary malignancy within 6 months of the appearance of the first malignancy. Here in, we introduce a 64-year-old woman who visited the Department of Oral & Maxillofacial Surgery, Yonsei University Dental Hospital with a 2 year history of a whitish verrucous lesion on the palate. She presented an exophytic mass on mandible during the following visits. Histopathologic evaluation revealed a synchronous parosteal osteosarcoma and squamous cell on right mandible and a precancerous verrucous leukoplakia on the palate.
A 20-year-old woman who had a main symptom of intermittent pain in both mandibular third molar visited our hospital. In CBCT findings, the #38, 48 teeth were located on the lingual side of the inferior alveolar nerve. In addition, a large stone about 12mm in length was observed in the right Wharton's duct in CBCT findings. Neck CT scan was performed and a large stone about 12mm in length was observed, and the accumulation of saliva in the rear of the stone was observed. The patient underwent sialolithotomy and #38, 48 tooth extraction through intraoral approach under general anesthesia. The removed stone was similar in shape and color to a tooth. The removed stone was markedly fragmented and composed of dentin and enamel-like tissue which showed lamellate arrangement in microscopic examination. Patient was presently symptom free after 10-months postoperatively.
Juvenile xanthogranuloma (JXG) is an unusual histiocytosis by an unknown cause, which rarely occurs in the oral cavity. Its clinical presentation is non-specific, so prevents to differentiate it from other oral mucosal diseases. In addition, limited oral JXG cases have been reported so far, and clinical features and therapy of oral JXG are more and less different from cutaneous JXG. The purpose of this report is to present a case of JXG on the dorsal tongue and a review of relevant literature.