Nasu Hakola Disease: A Rare Cause of Dementia and Cystic Bone Lesions, Report of a New Turkish Family.

The differential diagnosis of young-onset progressive dementia is an issue that requires effort. Recording the family history and careful clinical evaluation are useful tools in the diagnosis. In case of genetic bases, definitive diagnosis requires molecular analysis. We report consanguineous two patients presenting with young-onset progressive dementia characterized by behavioral changes and with bone cysts. Concomitant bone pathology and inheritance pattern directed us to investigate TREM2 gene, for differential diagnosis, which resulted with the identification of a causative mutation that confirmed the diagnosis of Nasu Hakola disease. The mutation (c.113A>G) is the same for a Turkish family with Nasu Hakola disease reported before. But the presence of bone cysts and absence of epilepsy in our patients are the different findings. Molecular analysis should be considered in patients with young age onset behavioral and cognitive deficits, with white matter lesions in brain magnetic resonance imaging, if especially associated with cystic bone lesions.

Female Parkinson's disease caregivers have much anxiety and depressive symptom.

OBJECTIVE: Parkinson's Disease Caregivers (PDC) play an important role, especially in the medium and advanced phase of the disease for patients' daily life activities, treatment, and follow-up. The aim of this study is to attract attention to the factors which place PDC at risk of psychological problems and to give consideration to these factors. MATERIALS AND METHODS: First of all, the 80 participants, who were PDC, filled in the demographic information form. The Hospital Anxiety and Depression Scale (HADS) was applied in order to determine the psychological status of the PDC. RESULTS: The average age of PDC in the study was found as 47.94. While 11 (13.8%) of the PDC had undergone psychiatric treatment in the past, four of them (5%) were currently receiving treatment. Twenty-eight (35%) of those who provide care have experience in patient care, whereas 52 (65%) of them have no prior experience in caring for patients. Thirty-six (45%) of the PDC reported that they had difficulties, which were mostly psychological. According to the HADS which was applied, anxiety was seen in 26 of those who provide care (32.5%), while depression was seen in 41 (51.3%). CONCLUSION: This study is the first to provide data on the psychological status of PDC in our country. It is important that PDC's psychological problems are reduced by psychotherapy or, if necessary, by treatment. This situation has a direct negative effect on the patient's daily life activities.

The Level of Knowledge of Parkinson's Disease among Nonprofessional Caregivers in a Movement Disorders Center in Turkey.

Introduction. Only a few studies have been conducted to determine the level of knowledge among caregivers about Parkinson's disease (PD). The aim of the current study was to determine the knowledge of PD among caregivers at a movement disorder clinic in Turkey. Methods. We conducted a questionnaire based interview with the subjects in a tertiary care neurology facility in Turkey. The questions were divided into two parts covering the symptomatology and treatment of PD. A questionnaire consisting of 10 questions was applied to the subjects who had to mark the correct option in a stipulated time. Results. Eighty caregivers were included in the study. The caregivers' mean age was 47.94 years (SD = 12.40). There were 47 female caregivers (58.8%). The most well-known question was that the number of drugs given to the patient may vary with time (76.3%), whereas "the benefit noted in the patient's treatment decreases over time" was the least known question (11.3%). Discussion. This study is the first in our country and shows the necessity to increase the knowledge of PD among caregivers and the public. Education programs may have a positive role in imparting knowledge to the caregivers of PD patients.