In this issue of Internal Medicine, Chen et al. (1) documented the case of a 30-year-old man who presented with an 11-month history of abnormal nocturnal behavior related to vivid dreams. Brain magnetic resonance images revealed lesions involving the pontomesencephalic junction and the upper pons, and polysomnography (PSG) findings confirmed a diagnosis of rapid eye movement (REM) sleep behavior disorder (RBD). A brainstem biopsy revealed diffuse large B-cell lymphoma. Notably, chemotherapy for lymphoma resulted in the disappearance of the lesions and reduced the frequency of RBD symptoms in the patient. RBD is a parasomnia characterized by dream-enacting behaviors and a loss of normal muscle atonia during REM sleep. The estimated prevalence of this disorder was reported to be 0.5% in a survey of approximately 4,900 subjects between the ages of 15 and 100 years (2). The dreams of patients with RBD usually include unpleasant and aggressive content, and the patient’s abnormal behavior is typically aggressive and violent, often resulting in serious injury to the patient or their bed partner (3). Based on the International Classification of Sleep Disorders, second edition (4), the use of PSG is essential for diagnosing RBD. The following criteria should be met: A, the presence of REM sleep without atonia, including excessive amounts of sustained or intermittent elevations of submental electromyographic (EMG) tone or excessive phasic submental or limb EMG twitching; B, abnormal REM sleep behavior based on the patient’s history and/or PSG findings; C, the absence of electroencephalogram (EEG) epileptiform activity during REM sleep; and D, the sleep disturbance is not better explained by medication use or another sleep, medical, neurological, mental or substance use disorder. In almost 90% of patients with RBD, the administration of clonazepam (0.5 to 1.5 mg) at bedtime is effective. Additionally, melatonin, pramipexole and Yi-Gan San alone or in conjunction with clonazepam may effectively treat RBD. The anatomic substrate for REM sleep control in humans includes a “REM-off” region, which consists of the ventrolateral part of the periaqueductal gray matter and lateral pontine tegmentum, and a “REM-on” region, which consists of the precoeruleus, sublaterodorsal nucleus, extended portion of the ventrolateral preoptic nucleus, locus coeruleus, laterodorsal tegmental nucleus, pedunculopontine nucleus and raphe nucleus (5). Motor behaviors in patients with RBD may reflect brainstem impairment, while the frightening dreams of RBD may reflect amygdala dysfunction (6). In humans, RBD can be caused secondarily by brain lesions including the brainstem nuclei, which regulate REM sleep, and the supratentorial structures, including the posterior hypothalamus, anterior thalamus and limbic system, which connect with the brainstem nuclei. Brain tumors, demyelinating plaque, strokes and limbic encephalitis have been reported to cause RBD (6), and narcolepsy is associated with RBD. Several medications can cause RBD, including antidepressants such as tricyclics, selective serotonin reuptake inhibitors and serotonin-norepinephrine reuptake inhibitors (3). In 1996, Schenck et al. (7) first reported the delayed emergence of Parkinsonian disorders in 38% of 29 older men initially diagnosed with idiopathic RBD, with a mean interval of 3.7 years after RBD diagnosis and a mean interval of 12.7 years after RBD onset. After an additional 7year follow-up, the conversion rate from idiopathic RBD to neurodegenerative diseases increased to 65%. Additionally, Iranzo et al. (8) reported that 45% of 44 patients with idiopathic RBD developed neurodegenerative diseases (n=9, Parkinson’s disease (PD); n=6, dementia with Lewy bodies (DLB); n=4, mild cognitive impairment; n=1, multiple system atrophy) at a mean follow-up of 5.1 years and a mean of 11.5 years after RBD onset. Postuma et al. (9) reported that 28% of 93 patients with idiopathic RBD developed neurodegenerative diseases (n=14, PD; n=7, DLB; n=4, Al-
The basal ganglia, which have been shown to be a significant multisensory hub, are disordered in Parkinson’s disease (PD). This study was to investigate the audiovisual integration of peripheral stimuli in PD patients with/without sleep disturbances. Thirty-six age-matched normal controls (NC) and 30 PD patients were recruited for an auditory/visual discrimination experiment. The mean response times for each participant were analyzed using repeated measures ANOVA and race model. The results showed that the response to all stimuli was significantly delayed for PD compared to NC (allp<0.01). The response to audiovisual stimuli was significantly faster than that to unimodal stimuli in both NC and PD (p<0.001). Additionally, audiovisual integration was absent in PD; however, it did occur in NC. Further analysis showed that there was no significant audiovisual integration in PD with/without cognitive impairment or in PD with/without sleep disturbances. Furthermore, audiovisual facilitation was not associated with Hoehn and Yahr stage, disease duration, or the presence of sleep disturbances (allp>0.05). The current results showed that audiovisual multisensory integration for peripheral stimuli is absent in PD regardless of sleep disturbances and further suggested the abnormal audiovisual integration might be a potential early manifestation of PD.
A 68 year-old-woman presented with a 20-year history of progressive difficulty in walking.A neurological examination showed hatchet face, impaired extraocular movement and facial and limb weakness with percussion and grip myotonia.Brain computed tomography (Picture A, C) and T1weighted magnetic resonance imaging (Picture B, D) showed the symmetrical thickening of the inner table of the frontal bone, "hyperostosis frontalis interna" (HFI).Her serum creatine kinase level was within the normal range (65 U/L).Her serum calcium level was normal (9.3 mg/dL; normal range, 8.6-10.6),but her phosphorus level was low (2.0 mg/dL; normal range, 2.5-4.5).Large cytosine-thymine-
e16097 Background: Degarelix directly blocks GnRH receptors, therefore, it is believed to produce a rapid decline of serum testosterone level without an initial surge in leutenizing hormone (LH), follicule stimulating hormone (FSH) and testosterone(T) seen in LHRH agonists. However, hormonal change caused by Degarelix in ultra-acute phase is unknown. We investigated the change of serum levels of LH, FSH and testosterone within 24hours of administration of Degarelix. Methods: Eleven patients (median age 74 years old; range 63-74), with a histologic diagnosis of advanced prostate cancer and without pretreatment by androgen deprivation therapy, were involved. Serum levels of LH, FSH and T were measured at 0, 1, 3, 6, 9, 12, 24 hours and1 month after injection of Degarelix. Serum prostate-specific antigen (PSA) level was also measured at baseline and 1 month after treatment. Results: The median serum PSA level before treatment was 378ng/ml (15.9-7187). The median baseline serum levels of LH, FSH and T were 7.6mIU/ml (2.9-17), 10.6mIU/ml (2.4-32) and 390ng/dL (206-697), respectively. Those at 24hr were 0.7mIU/ml (0.01-2), 6.6mIU/ml (1.4-18.6) and 50ng/ml (8-95). Although, serum levels of LH and T declined rapidly, serum levels of FSH declined more slowly. In 5 patients, serum levels of T at 24hr were below the castration level (<50ng/dL). T surge (an elevation of serum level of T within first 3hr) was observed in 6 patients. In 9 patients, hormonal levels at 1 month after treatment were available. In 7 of 9 patients, serum levels of T were below the castration level. Four patients showed T surge and 5 did not show this phenomenon. In four of 5 patients without T surge, serum levels of PSA declined to 96% in average at 1 month. In contrast, only one of 4 patients with T surge showed 92% reduction of serum levels of PSA, in the rest of 3 patients PSA reduction was only 67% in average. Conclusions: Degarelix suppresses serum levels of and Testosterone rapidly. Testosterone surge could be a possible predictor of effectiveness of Degarelix on prostate cancer.
