Reduced orexin-A levels in frontotemporal dementia: possible association with sleep disturbance.

Sleep disturbances including excessive daytime sleepiness (EDS) are encountered in frontotemporal dementia (FTD). To investigate the relationship between the plasma orexin-A levels and sleep disturbance patterns, we measured the plasma orexin-A levels and performed sleep studies in patients with FTD. The orexin-A levels were measured in 10 consecutive patients with FTD and controls by enzyme-linked immunosorbent assay. Nocturnal polysomnography (PSG) and Multiple Sleep Latency Test (MSLT) were performed in 2 patients with FTD. The orexin-A levels were significantly lower in patients with FTD compared to controls. The PSG revealed increased rapid eye movement (REM) latency in patients, whether or not they reported EDS. Mean sleep latency in MSLT was less than 10 minutes in both the patients, being shorter in patient without EDS, but none of them had REM sleep onset. Some patients with FTD may develop narcolepsy-like involuntary sleep attacks, even without complaining of EDS. Involvement of hypothalamus and a subsequent alteration in the orexin levels might be one of the determining factors in this sleep disturbance.

Destructive stereotactic surgery for treatment of dystonia.

BACKGROUND: This study is a retrospective review of the results of stereotactic destructive surgery in selected cases of drug-resistant dystonia. METHODS: Fifty-eight patients with drug-resistant dystonia were treated with stereotactic surgery between 1991 and 1999 in our institution. These patients' charts were retrospectively analyzed. The timing of the conducted evaluations was as follows: preoperatively, postoperatively, in the postoperative 1st week, 6th month, 12th month, and also thereafter every year. RESULTS: Symptoms of dystonia occurred before the age of 10 years in 30 patients (51.8%) and after the age of 10 years in 28 patients (48.2%). Generalized dystonia was detected in 41 patients, whereas 11 patients had hemidystonia, 5 patients had focal dystonia, and 1 patient had segmental dystonia. The most common etiologic factor was CP (n = 34). A total of 103 ablative lesions were created in 86 surgical sessions. Thalamotomy, pallidotomy, subthalamotomy, and the region of Forel lesions were performed either separately or in combination. In this series, the mean follow-up time was 102.2 months. Except for 2 cases of temporary hemiparesis, no other complications were observed. Minor improvement was obtained in 17 patients (19.7%), improvement of a medium degree was obtained in 17 patients (19.7%), high-degree improvement was obtained in 11 (12.8%), and very high degree improvement was obtained in 16 (18.6%) patients. A final evaluation revealed permanent improvement in 32 patients (55.2%). CONCLUSION: Production of stereotactic destructive lesions in certain specified targets is a safe method that improves quality of life and aids ambulation in patients with dystonia resistant to medical therapy.