Mulvihill-Smith syndrome (MSS) is characterized by premature aging, multiple pigmented nevi, decreased facial subcutaneous fat, microcephaly, short stature, mental retardation and recurrent infections, however the adult phenotype of MSS has yet to be delineated. We report a 28-year-old woman with Mulvihill-Smith syndrome, who had a solid pseudopapillary cystic tumor of her pancreas at age 17 years. Her distinctive sleep pattern includes severe insomnia with disappearance of sleep spindles and K-complexes, persisting muscle tone, and loss of slow wave sleep. The clinical and neurophysiological studies are compatible with agrypnia excitata, a sleep disorder attributable to a dysfunction of the thalamo-limbic system. Brain magnetic resonance imaging and single photon emission computed tomography revealed structural and functional deficits in the dorsomedial region of the thalamus and indicated that an alteration in the thalamo-limbic system may underlie the sleep disturbances in MSS. Furthermore, the rapid and severe decline in acquired cognitive function showed the distinct cognitive impairments resembling dementia, including intellectual deficits, memory disorder and executive dysfunction. We posit that an early onset tumor, sleep disorder and cognitive decline are adult manifestations of Mulvihill-Smith syndrome.

The number of patients with BRONJ has increased in Japan. Attention to oral BP and proper treatment is required to minimize the number of cases. Surgical therapy seems to be effective for BRONJ stage 2.

The use of mandibular subperiosteal implants was first reported by Dahl in the 1940s. It was followed by a more comprehensive report by Goldberg and Gershkoff, who published the first case series in the United States. We describe a case of an orocutaneous fistula that developed secondary to a chronic infection attributed to a failing subperiosteal implant. Elemental analysis of the metal framework revealed an implant composed of mainly a cobalt-chromium alloy. Cobalt, which was frequently used in subperiosteal implant manufacturing, is associated with a higher corrosion rate than other metals and is no longer used to fabricate subperiosteal implants. The strength of subperiosteal implants is their ability to be used in a mandible with an atrophic alveolar ridge. Unfortunately, this feature of the hardware is also its weakness in that reconstructive procedures after their removal are difficult. The failing subperiosteal implant in our patient was subsequently removed, and primary closure of the intraoral wound and extraoral fistula as well as resolution of the patient's symptoms was obtained with no lasting complications.

No abstract

We report on the case of a severely osteoporotic elderly Japanese woman with bisphosphonate-associated osteonecrosis of the jaw (ONJ), who was treated successfully with teriparatide. A 79-year-old woman with severe osteoporosis and bisphosphonate-associated ONJ was treated with teriparatide after debridement of the necrotic tissue in the jaw bone. Computed tomography (CT) images revealed the bone defect in the mandible after debridement of the necrotic tissue associated with ONJ. According to the attending dentist, the ONJ healed after 2 months of therapy. After 3 months of treatment, a robust increase in the serum level of the bone formation marker, serum intact procollagen type 1 N-terminal propeptide, was noted and a repeat CT revealed improvement of the bone defect of the mandible. These results suggest the beneficial effects of teriparatide therapy in the severely osteoporotic elderly woman with ONJ.