Idiopathic REM Sleep Behavior Disorder: Implications for the Pathogenesis of Lewy Body Diseases

Objectives. Both results of the odor identification and cardiac 123I-metaiodobenzylguanidine accumulation have been investigated for their potential to enhance the detection of pathogenesis resembling that of Lewy body-related α-synucleinopathies in patients clinically diagnosed as having idiopathic REM sleep behavior disorder. Methods. We performed both the Odor Stick Identification Test for Japanese and 123I-metaiodobenzylguanidine scintigraphy in 30 patients with idiopathic REM sleep behavior disorder, 38 patients with Parkinson's disease, and 20 control subjects. Results. In idiopathic REM sleep behavior disorder, reduced odor identification score and an early or delayed heart to mediastinum ratio on 123I-metaiodobenzylguanidine were almost as severe as in Parkinson's disease patients. Delayed cardiac 123I-metaiodobenzylguanidine uptake was even more severe in the idiopathic REM sleep behavior disorder group than in the Parkinson's disease group. Conclusions. Reduced cardiac 123I-metaiodobenzylguanidine uptake, which is independent of parkinsonism, may be more closely associated with idiopathic REM sleep behavior disorder than olfactory impairment

パーキンソン カンレン シッカン ニオケル ケイズガイ チョウオンパ ケンサ ニヨル チュウノウ コクシツ ノ コウキド ヘンカ ノ ケントウ

目 的:パーキンソン病 (Parkinson disease:PD),多系統萎縮症 (multiple system atrophy:MSA),進行性核上性麻痺 (progressive supranuclear palsy:PSP) の患者において経頭蓋超音波検査 (transcranial sonography:TCS) よる中脳黒質の高輝度変化を検討した.方 法:パーキンソン関連疾患連続110 例 (PD 86 例,MSA 12 例,PSP 12 例) と健常者34 例に対しTCSを施行した.中脳黒質を観察しえたPD 47 例,MSA 10 例,PSP 6 例,健常者32 例を解析対象として中脳黒質高輝度所見を評価した.定性評価は高輝度の程度によって視察的にI:none or faint,II:equivocal,III:definite,IV:marked の4 段階に分類した.定量評価は中脳黒質で高輝度変化の面積が0.20 cm2 以上のとき,病的な黒質高輝度変化と定義した.結 果:定性評価では,高輝度範囲が視察的に病的と判定されるIII+IVの割合は,PD 72.4%,MSA 10.0%,PSP 66.7%,健常者3.1%であった.定量評価では,PD 63.8%,MSA 20.0%,PSP 66.7%,健常者9.4%で病的な高輝度変化をみとめた.PD,PSP で病的な高輝度変化の割合が多かった.PSP をPSP-parkinsonism( PSPP)とRichardson\u27s syndrome の2 群に分けた場合,前者では病的な高輝度変化を3 例中3 例 (100%), 後者では3 例中1 例( 33.3%) に認められ,PSP-P で割合が高かった.MSA では10 例中2 例( 20%) に病的な高輝度を認め,いずれもパーキンソン病型の多系統萎縮症であった.結 論:パーキンソン関連疾患における病的な中脳黒質高輝度変化は,疾患特異性というよりも,パーキンソニズムの症候と関連し,ドパミン神経細胞の脆弱性を示す所見と推察された.Objective:We investigated substantia nigra (SN) hyperechogenicitydetermined by transcranial sonography(TCS) to detect abnormalities, and compare findings withthose from Parkinson disease (PD), multiple system atrophy(MSA), progressive supranuclear palsy (PSP) or controlsubjects.Method:In this study, echogenicity of SN was examinedin consecutive 110 parkisonian disorders patients with PD86, MSA12, PSP 11, and 34 control subjects. A sufficientbone window for TCS was available in 47 of 86( 71.2%) inthe PD group, 10 of 12( 86.3 %) in the MSA group, 6 of 11(54.5%) in the PSP group and 32 of 34( 94.1%) in the controlgroup. SN hyperechogenicity was scored using a fourpointscale as follows:I=none or faint, II=equivocal, III=definite, IV=marked. In accordance with previously reportedcut-off values, areas of echogenicity £ 0.19 cm2 wereclassified as normal and areas of echogenicity £ 0.20 cm2were classified as pathological SN hyperechogenicity.Results:The frequency of SN hyperechogenicity, assessedas III and IV scales, was significantly increased in PDpatients, and observed in 72 . 4 % of assessable SN(34/47);qui-squire;p=0.001, vs. controls). The meansize of the SN hyperechogenic area in the PD group, MSAgroup and PSP group was 0.26 cm2±0.13, 0.11 cm2±0.11and 0.23 cm2±0.04, respectively, compared with 0.07 cm2±0.06 in the control group.We have identified two clinical phenotypes, such as Richardson\u27ssyndrome (RS) and PSP-parkinsonism (PSP-P).All of three PSP-P (100%) patients showed a pathologicalSN hyperechogenicity.Conclusion:SN hyperechogenicity was associated with asymptom of parkinsonism rather than disease specificity,and suggested a vulnerability marker of the dopaminergicneuron

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

<p>Abstract</p> <p>Background</p> <p>Increasing 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.</p> <p>Methods</p> <p>We 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).</p> <p>Results</p> <p>A 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.</p> <p>Conclusion</p> <p>Our 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.</p