Ayaka Numao

Nocturnal disturbances and restlessness in Parkinson's disease: Using the Japanese version of the Parkinson's disease sleep scale-2

OBJECTIVE The aim of this study was to assess the validity and the reliability of the Japanese version of the Parkinsons disease sleep scale (PDSS)-2 and to use this scale to identify nocturnal symptoms and their impact on patients quality of life. METHODS A cross-sectional, case-controlled study was conducted consisting of 93 patients with Parkinsons disease (PD) and 93 age- and gender-matched control subjects. The Japanese version of the PDSS-2 was used for the evaluation of nocturnal disturbances. The patients quality of life was evaluated with the Parkinsons Disease Quality of Life questionnaire (PDQ-39) and their depressive symptoms were assessed with the Beck Depression Inventory-II (BDI-II), respectively. In addition, the Pittsburgh Sleep Quality Index (PSQI), Epworth Sleepiness Scale (ESS) and Parkinson Fatigue Scale (PFS) were administered. RESULTS As assessed using the PDSS-2, PD patients had significantly impaired scores compared with control subjects (15.0±9.7 vs. 9.1±6.6, p<0.001). The ESS, BDI-II and PFS scores were significantly impaired in PD patients compared with controls. A satisfactory internal consistency and test-retest reliability score were obtained for the PDSS-2 total score (Cronbachs alpha=0.86). The PDSS-2 was correlated with the PSQI, ESS, BDI-II, PFS, PDQ-39 summary index, all of the PDQ-39 domains and Unified Parkinsons Disease Rating Scale part III. The frequency of restless legs syndrome (RLS) was not significantly different between PD patients and controls (5.5% vs. 2.2%), but nocturnal restlessness was significantly more frequent in PD patients than controls. Stepwise linear regression analyses revealed the PDQ-39 summary index and the PSQI global score as significant predictors for the PDSS-2 total score. CONCLUSIONS Our study confirmed the usefulness of the Japanese version of the PDSS-2 that enables the comprehensive assessment of nocturnal disturbances in PD. The association between RLS and nocturnal restlessness in PD requires further study.

Clinical correlates of serum insulin-like growth factor-1 in patients with Parkinson's disease, multiple system atrophy and progressive supranuclear palsy

BACKGROUND Recently, increased serum insulin-like growth factor-1 (IGF-1) levels have been reported in patients with Parkinsons disease (PD) and multiple system atrophy (MSA). OBJECTIVE To assess a correlation between the serum IGF-1 levels and clinical background factors in patients with PD and related disorders such as MSA and progressive supranuclear palsy (PSP). METHODS A total of 79 PD patients, 25 MSA patients, 16 PSP patients and 52 healthy controls were included in this study. The serum IGF-1 and growth hormone (GH) levels were measured in a fasting state. Unified PD Rating Scale (UPDRS) part III was used to evaluate motor function. Unified MSA Rating Scale (UMSARS) part II was also employed for the MSA patients. RESULTS The serum IGF-1 levels were significantly increased in the MSA patients compared with the PD patients and controls. No significant differences were observed in the serum GH levels among the patients and controls. The serum IGF-1 levels of PD patients with Hoehn and Yahr stage 2 were significantly higher than those of patients with Hoehn and Yahr stages 3-5. In patients with PD and PSP, the serum IGF-1 levels were negatively correlated with UPDRS part III. In contrast, patients with MSA showed a positive correlation of the serum IGF-1 levels with disease duration, UPDRS part III and UMSARS part II. CONCLUSION The difference in the serum IGF-1 level and its correlation with clinical variables among these disorders may reflect different ongoing disease processes in each disorder.

Probable rapid eye movement sleep behavior disorder, nocturnal disturbances and quality of life in patients with Parkinson's disease: a case-controlled study using the rapid eye movement sleep behavior disorder screening questionnaire

BackgroundIncreasing evidence provides a clear association between rapid eye movement sleep behavior disorders (RBD) and Parkinson’s disease (PD), but the clinical features that determine the co-morbidity of RBD and PD are not yet fully understood.MethodsWe evaluated the characteristics of nocturnal disturbances and other motor and non-motor features related to RBD in patients with PD and the impact of RBD on their quality of life. Probable RBD (pRBD) was evaluated using the Japanese version of the RBD screening questionnaire (RBDSQ-J).ResultsA significantly higher frequency of pRBD was observed in PD patients than in the controls (RBDSQ-J ≥ 5 or ≥ 6: 29.0% vs. 8.6%; 17.2% vs. 2.2%, respectively). After excluding restless legs syndrome and snorers in the PD patients, the pRBD group (RBDSQ-J≥5) showed higher scores compared with the non-pRBD group on the Parkinson’s disease sleep scale-2 (PDSS-2) total and three-domain scores. Early morning dystonia was more frequent in the pRBD group. The Parkinson’s Disease Questionnaire (PDQ-39) domain scores for cognition and emotional well-being were higher in the patients with pRBD than in the patients without pRBD. There were no differences between these two groups with respect to the clinical subtype, disease severity or motor function. When using a cut-off of RBDSQ-J = 6, a similar trend was observed for the PDSS-2 and PDQ-39 scores. Patients with PD and pRBD had frequent sleep onset insomnia, distressing dreams and hallucinations. The stepwise linear regression analysis showed that the PDSS-2 domain “motor symptoms at night”, particularly the PDSS sub-item 6 “distressing dreams”, was the only predictor of RBDSQ-J in PD.ConclusionOur results indicate a significant impact of RBD co-morbidity on night-time disturbances and quality of life in PD, particularly on cognition and emotional well-being. RBDSQ may be a useful tool for not only screening RBD in PD patients but also predicting diffuse and complex clinical PD phenotypes associated with RBD, cognitive impairment and hallucinations.

Evaluation of cutoff scores for the Parkinson's disease sleep scale-2

The Parkinsons Disease Sleep Scale (PDSS)‐2 is a recently developed tool for evaluating disease‐related nocturnal disturbances in patients with Parkinsons disease (PD). However, its cutoff score has not been clinically assessed. We determined the optimal cutoff score of the Japanese version of the PDSS‐2.

Usefulness of Cardiac MIBG Scintigraphy, Olfactory Testing and Substantia Nigra Hyperechogenicity as Additional Diagnostic Markers for Distinguishing between Parkinson’s Disease and Atypical Parkinsonian Syndromes

Background We aimed to evaluate the utility of the combined use of cardiac 123I-metaiodobenzylguanidine (MIBG) scintigraphy, olfactory testing, and substantia nigra (SN) hyperechogenicity on transcranial sonography (TCS) in differentiating Parkinson’s disease (PD) from atypical parkinsonian syndromes (APSs), such as multiple system atrophy (MSA) and progressive supranuclear palsy (PSP). Methods Cardiac MIBG scintigraphy, card-type odor identification testing (Open Essence (OE), Wako, Japan), and TCS were performed with 101 patients with PD and 38 patients with APSs (MSA and PSP). Receiver operating characteristic (ROC) curve analysis was used to assess the sensitivity and specificity of these batteries for diagnosing PD from APSs. The diagnostic accuracy of the three tests was also assessed among patients at the early disease stage (drug-naïve patients with a disease duration of 3 years or less). Results In differentiating PD from APSs, the area under the ROC curve was 0.74 (95% CI, 0.65–0.83), 0.8 (95% CI, 0.73–0.87), and 0.75 (95% CI, 0.67–0.82) for TCS, cardiac MIBG scintigraphy, and olfactory testing, respectively. The diagnostic sensitivity and specificity were 53.1% and 91.7%, respectively, for TCS, 70.3% and 86.8%, respectively, for cardiac MIBG scintigraphy, 58.4% and 76.3%, respectively, for OE. Among early-stage patients, sensitivity and specificity were 50.0% and 93.8%, respectively, for TCS, 57.1% and 87.5%, respectively, for cardiac MIBG scintigraphy, and 54.8% and 79.2%, respectively, for OE. At least one positive result from 3 tests improved sensitivity (86.1%) but decreased specificity (63.2%). In contrast, at least 2 positive results from 3 tests had good discrimination for both early-stage patients (50.0% sensitivity and 93.8% specificity) and patients overall (57.8% sensitivity and 95.8% specificity). Positive results for all 3 tests yielded 100% specificity but low sensitivity (25%). Conclusions At least 2 positive results from among TCS, cardiac MIBG scintigraphy, and olfactory testing can support clinical diagnosis in distinguishing PD from APSs.

Serum uric acid levels in Parkinson's disease and related disorders

Serum uric acid (UA) levels are reported to be decreased in patients with Parkinsons disease (PD) and multiple system atrophy (MSA). However, clinical correlates of serum UA levels are still unclear in PD‐related disorders. We conducted a cross‐sectional study to evaluate the associations between serum UA levels and disease duration, disease severity, and motor function among PD, MSA, and progressive supranuclear palsy (PSP) patients.

The Prevalence and Characteristics of Primary Headache and Dream-Enacting Behaviour in Japanese Patients with Narcolepsy or Idiopathic Hypersomnia: A Multi-Centre Cross-Sectional Study.

Background Because the prevalence and characteristics of primary headache have yet to be thoroughly studied in patients with hypersomnia disorders, including narcolepsy and idiopathic hypersomnia, we examined these parameters in the Japanese population. Methods In a multicentre cross-sectional survey, among 576 consecutive outpatients with sleep disorders, 68 narcolepsy patients and 35 idiopathic hypersomnia patients were included. Additionally, 61 healthy control subjects participated. Semi-structured headache questionnaires were administered to all participants. Results The patients with narcolepsy (52.9%) and idiopathic hypersomnia (77.1%) more frequently experienced headache than the healthy controls (24.6%; p<0.0001). The prevalence rates were 23.5%, 41.2% and 4.9% for migraine (p<0.0001) and 16.2%, 23.5% and 14.8% (p = 0.58) for tension-type headache among the narcolepsy patients, the idiopathic hypersomnia patients and the control subjects, respectively. Those who experienced migraine more frequently experienced excessive daytime sleepiness, defined as an Epworth Sleepiness Scale score of ≥10, than those who did not experience headache among the patients with narcolepsy (93.8% vs. 65.6%, p = 0.040) and idiopathic hypersomnia (86.7% vs. 37.5%, p = 0.026). Dream-enacting behaviour (DEB), as evaluated by the rapid eye movement sleep disorders questionnaire, was more frequently observed in the narcolepsy patients than in the idiopathic hypersomnia patients and the control subjects. An increased DEB frequency was observed in the narcolepsy patients with migraines compared to those without headache. Conclusions Migraines were frequently observed in patients with narcolepsy and idiopathic hypersomnia. DEB is a characteristic of narcolepsy patients. Further studies are required to assess the factors that contribute to migraines in narcolepsy and idiopathic hypersomnia patients.

Characterizing restless legs syndrome and leg motor restlessness in patients with Parkinson's disease: A multicenter case-controlled study

BACKGROUND We investigated the prevalence and impact of restless legs syndrome (RLS) and leg motor restlessness (LMR) in patients with Parkinsons disease (PD) in a multicenter study. METHODS A total of 436 PD patients and 401 age- and sex-matched controls were included in this study. RLS was diagnosed based on four essential features. LMR was diagnosed when a participant exhibited the urge to move his or her legs but did not meet the four essential features of RLS. RESULTS The RLS prevalence did not differ between PD patients and controls (3.4% vs. 2.7%), while LMR prevalence was significantly higher in PD patients than in controls (12.8% vs. 4.5%). PD patients with RLS or LMR had a higher prevalence of excessive daytime sleepiness (EDS) (50.7%, vs. 6.9%), probable REM sleep behavior disorder (38.0% vs. 3.4%) and PD-related sleep problems (49.3% vs. 20.7%) than controls with RLS or LMR. RLS/LMR preceding PD onset was related to an older age of PD onset. CONCLUSION Our study revealed an increased prevalence of LMR but not RLS in PD patients. LMR could be an early manifestation of PD; however, whether LMR is within the range of RLS or whether LMR and RLS constitute different entities in PD requires further studies.

Does Pramipexole Treatment Improve Headache in Patients with Concomitant Migraine and Restless Legs Syndrome

Background Recent studies have suggested a strong link between migraines and restless legs syndrome (RLS). It is possible that these disorders share a dopaminergic dysfunction in the hypothalamic A11 nucleus that contributes to this association. However, there have been no clinical studies to evaluate the effect of dopaminergic treatment on migraine symptoms in patients with concomitant migraines and RLS. Methods We present an illustrative patient with concomitant RLS and migraine who showed improvement in her headache frequency and RLS symptoms following immediate-release pramipexole (P-IR) treatment and provide review results from the medical records of patients who experienced both migraines and RLS in our previous cross-sectional study. Results Ten patients (nine patients from the previously completed single-center study) received P-IR treatment were included in the study. RLS symptoms improved markedly in all of the subjects. Five out of the 10 patients (50%) reported improvement in migraine headaches. Of these five patients, four (80%) had reported morning headaches before P-IR treatment. Discussion Our results indicate that the identification of RLS in migraine patients is clinically significant and that dopaminergic treatment may improve both migraines, particularly morning headache (80% improvement in this study), and RLS symptoms. However, further clinical studies are warranted to verify our results.

Computer mouse-related dystonia: a novel presentation of task-specific dystonia

Task-specific dystonia (TSD) is a disabling movement disorder characterized by focal, involuntary, and excessive muscle contractions that develop in a single body part involved in highly skilled tasks [1]. In TSD, involvement of the hand (while writing, typing, or playing a musical instrument) is more common than the involvement of other parts of the body, but TSD related to the use of a computer mouse has never been described. A 68-year-old right-handed man presented to our outpatient clinic with involuntary flexion of his right thumb, which interfered with the use of a computer mouse. When the patient was 62 years old, he started composing music using a computer. Since then, he had used the computer mouse with his right hand for composition for approximately 5 h per day. At age 64, he developed abnormal flexion of his right thumb whenever he used the computer mouse; the flexion interfered with moving the mouse, but he could click the mouse button without any trouble. During the following year, abnormal flexion in the right thumb was observed even when the patient put his right hand on the table, and he had difficulty in extending and flexing his right thumb voluntarily. This forced him to use the computer mouse with his left hand instead. He had no symptoms in other parts of the body. The patient had played classical guitar since childhood. He had no previous medical history of psychiatric disease or a family history of dystonia. The patient had no history of alcohol use or smoking. The initial examination showed excessive flexion of his right thumb, and voluntary flexion and extension of his right thumb was impaired. This abnormal posture significantly worsened when using the computer mouse (see video, segment 1). However, he noticed that right-thumb flexion never occurred while eating (using chopsticks, spoon, or knife) or writing with a pen. The abnormal flexion of his right thumb never occurred when his right thumb and index finger touched. A neurological examination was unremarkable except for the abnormal flexion position of his right thumb. Laboratory data were normal. The finding of brain magnetic resonance images was normal. TSD was diagnosed based on the observation that involuntary and excessive flexion posture was prominent in specific situations. Oral medications such as levodopa, carbamazepine, and phenytoin were ineffective. The patient was then treated with clonazepam 0.5 mg, which modestly improved his symptoms. The combined use of clonazepam 1 mg and trihexyphenidyl 6 mg resulted in significant improvement of his symptoms (see video, segment 2). We report the first case of a patient in whom excessive flexion of the right thumb occurred initially only when using the computer mouse. Similar to other TSD patients [1], our patient’s symptoms later involved other tasks. As the patient had dystonic posturing of the right hand at rest at the examination, the patient was classified as having complex TSD rather than simple TSD [2]. His finger movements were preserved when his right thumb and index Electronic supplementary material The online version of this article (doi:10.1007/s00415-012-6519-1) contains supplementary material, which is available to authorized users.