Tsubasa Sada

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.

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.

Optimization of Intravenous Immunoglobulin in Chronic Inflammatory Demyelinating Polyneuropathy Evaluated by Grip Strength Measurement

Background: Optimal dose and timing of repeated intravenous immunoglobulin therapy (IVIg) for intractable chronic inflammatory demyelinating polyneuropathy (CIDP) patients have not been determined. The aim of this study was to optimize dose and timing of IVIg for CIDP patients who need frequent IVIg using daily grip strength measurement. Methods: Repeated IVIg were administered for two intractable CIDP patients. Grip strength was recorded at home every day to access the clinical change in symptoms, and dose and timing of IVIg were optimized based on the results. Results: The decrement on grip strength was a sensitive indicator of symptom exacerbation. 100 g of IVIg had a limited effect for each patient. In one patient, symptoms maintained after monthly 60 g of IVIg. In another, 100 g of IVIg every 7 weeks resulted in a marked improvement. After receiving 20 g of IVIg weekly, each patient showed further improvement. Conclusion: Optimal dose and timing possibly vary in each individual patient. Dose titration of IVIg is necessary to avoid over- and undertreatment. The daily self-monitoring of grip strength is a helpful tool for clinical assessment in CIDP.

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.

The ‘Mickey Mouse ears’ sign: a bilateral cerebral peduncular infarction

The literature rarely reports bilateral cerebral peduncular infarction. We report the unique magnetic resonance imaging (MRI) finding of a bilateral cerebral peduncular infarction that simulated a ‘Mickey Mouse ears’ sign. A 62-year-old man with a history of diabetes and hypertension was admitted to our hospital with acute onset vertigo and an unsteady gait. At admission, his blood pressure was 198/118 mmHg, and his pulse rate was 60 beats/min. A neurological examination revealed truncal and bilateral limb ataxia. A diffusionweighted MRI showed an acute infarction in the bilateral cerebellum and occipital lobes (Fig. 1a). On the third day of hospitalization, despite anticoagulation and antiplatelet therapies, consciousness disturbance developed. Although the patient’s extraocular movement was preserved, his light reflexes were sluggish bilaterally. Tetraparesis with bilateral Babinski’s sign was noted. A diffusion-weighted MRI identified an acute bilateral cerebral peduncular infarction that looked like a ‘Mickey Mouse ears’ (Fig. 1b). An MR angiography showed decreased flow signals of the proximal bilateral P1 segments compared with images taken at admission, which showed severe stenosis at the proximal basilar artery and the absence of flow signals from the bilateral P1–P2 junctions of the posterior cerebral artery (Fig. 1c and d). The patient’s neurological symptoms progressed, and the patient died 2 days later. Bilateral cerebral peduncular infarction is extremely rare, and is associated with locked-in syndrome and a persistent vegetative state [1–4]. Table 1 demonstrates previously reported brain computed tomographic (CT) scans, MRI-confirmed bilateral cerebral peduncular infarcts [2,4–7] and pathologically confirmed cases [3,8]. Out of nine patients, five individuals showed locked-in syndrome, and two patients initially had unilateral cerebral peduncular infarcts that progressed bilaterally. Typically, locked-in syndrome has been attributed to ventral pontine lesions [9]. Although additional pontine involvements are shown in Table 1, the reported cases of bilateral cerebral peduncular infarcts without the involvement of the pons or thalamus [6,8], or with small pontine infarcts [7], suggest that cerebral peduncular infarcts are the cause of locked-in syndrome. The bilateral cerebral peduncular lesions without the involvement of the ventral portion of the pons that result in locked-in syndrome are also found in patients with multiple sclerosis [10]. Cerebral peduncles are supplied with blood via the perforating branches of the posterior communicating, posterior cerebral and superior cerebellar arteries or the anterior choroidal artery that branches from the internal carotid artery. In our patient, a decrease in the flow signals of the proximal portions of the P1 segments on the MR angiography corresponded to the occurrence of a bilateral cerebral peduncular infarction. This result suggests that perforating branches of the P1 segments, such as the directly perforating branches and the short circumflex branches, play a major role in supplying the ventral portions of the midbrain. Although rare, the ‘Mickey Mouse ears’ sign might be a reproducible finding. Furthermore, it provides clinical significance for predicting a patient’s symptoms and prognosis.

Demyelinating hypertrophic inferior alveolar nerve mimicking a nerve tumor.

We herein report a patient with demyelinating inferior alveolar nerve hypertrophy, which was initially suspected to have a nerve tumor. A 39-year-old woman with childhood-onset polyneuropathy presented with tooth pain and visited a dental clinic. An X-ray examination of the mandible revealed enlargement of the mandibular canal, and a nerve tumor was suspected. CT scan and MRI showed hypertrophy of the inferior alveolar nerve along its entire length. We diagnosed the patient with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), which was supported by the spontaneous recovery reported in her childhood, the results from a nerve conduction study and MRI data. CIDP should be considered in the differential diagnosis of mandibular canal enlargement.

Involvement of the capsular genu in reward-related feeding

Dear Sirs, Food intake is regulated by homeostatic and reward-related pathways. The hypothalamus and brainstem integrate homeostatic signals with reward-related pathways, such as the dopaminergic neurons of the ventral tegmental area, nucleus accumbens, insular cortex, anterior cingulate cortex, and orbitofrontal cortex [1]. Eating disorders following brain lesions of the frontal and temporal lobe, hypothalamus, thalamus, and brainstem have all been described [2–4]. A 58-year-old right-handed woman suddenly presented with abnormal eating behavior and excessive daytime sleepiness in August 2009. The patient was seen consuming a large amount of salt-boiled, green edamame-type soybeans by a family member, and she slept throughout the day, except for when she was eating, on the day before her admission. The patient had no history of eating disorder. Neurological examination was unremarkable except for memory disturbance. She scored 26 points on the MiniMental State Examination (MMSE) scale (the impaired items being related to orientation and recall). No frontal release signs were observed. Brain magnetic resonance imaging (MRI) showed an acute ischemic lesion in the right capsular genu (Fig. 1a), with deep white matter lesions. Sixteen days after onset of symptoms, the patient was discharged with mild residual cognitive impairment and daytime sleepiness. After discharge, the patient could not resist the urge to eat and consumed three packs of edamame per day, resulting in weight gain of 5 kg over 3 months. The feeling of hunger was not necessary for this eating behavior to occur. The patient admitted that, before the onset of stroke, she had wanted to eat edamame whenever she saw her husband eating them while drinking beer but that she had previously restrained herself from doing so. She did not display increased eating behavior with other foods. Her appetite and eating behavior returned to normal by November 2009. In August 2010, she returned to our outpatient clinic with a 2-month history of the previously observed symptoms of daytime sleepiness and increased consumption of edamame. Brain MRI showed bilateral capsular genu infarcts (Fig. 1b). Brain single-photon emission computed tomography (SPECT) showed hypoperfusion in the bilateral frontal lobes (Fig. 1c, d). She scored 23 points on the MMSE scale (the impaired items being related to orientation, attention, calculation, and recall). We report a patient who developed right and left capsular genu infarcts on two separate occasions; both episodes were associated with identical clinical symptoms of increased reward-related feeding and daytime sleepiness. The patient displayed an irrational desire to consume edamame. Edamame is a nutritious green vegetable, also known as fresh green soybean, which is harvested at the peak of ripening just before it reaches a hardening stage. The stroke of each side of the capsular genu in the same patient on different occasions, which induced reproducible abnormal eating behavior related only to palatable food in the absence of frontal release signs, suggests the involvement of reward-related food pathways. This behavior is in contrast with binge eating, which is observed in patients with thalamic infarcts [2, 4]. However, because our patient K. Suzuki (&) T. Sada H. Takekawa K. Hirata Department of Neurology, Dokkyo Medical University, 880 Kitakobayashi, Mibu, Shimotsuga, Tochigi 321-0293, Japan e-mail: keisuke@dokkyomed.ac.jp

P2-61. Evaluation of verbal fluency in Parkinson’s disease patients with deep brain stimulation

Parkinson’s disease (PD) is a common disease of neurological disorders, and the motor symptoms of PD respond well to bilateral deep brain stimulation (DBS). Recent study, there are also reports of worsened verbal fluency, executive dysfunction, and processing speed with DBS. Whether subthalamic nuclei (STN) stimulation worsens there are under debate. The aim of this study was to explore the effects of STN stimulation verbal fluency as assessed with clinical neuropsychological tests. Eight patients treated with DBS were enrolled. Assessments were done both with the STN stimulation turned OFF and ON. In both test conditions, the following were assessed: speech, word fluency A, and B. The score of the word fluency test (WFT) of all patients have undergone DBS surgery significantly worsened as compared with before surgery. Five patients speech ware worsened, but three patients were improved when the STN stimulation was turned OFF. On the other hand, five patients were reduced the word fluency’s total score when the STN stimulation was turned OFF. In this sample, STN stimulation significantly worsened the result of the WFT. When the STN stimulation was turned OFF, it was reduced. These finding suggests that STN-DBS might be worse speech conditions and verbal fluency.

44. Electrodiagnostic features in Guillain–Barré syndrome after Campylobacter jejuni enteritis

ease (PD). Overall 9 MSA patients and 23 PD patients were investigated regarding presences of RBD symptom, REM sleep without atonia (RWA), and the proportion of RWA during PSG (%RWA) at two separate occasions. In MSA patients, %RWA significantly increased from the baseline to the follow up (16.0 ± 19.4% to 31.0 ± 27.7%) whereas numbers of patients with RWA and RBD symptoms unchanged. On the other hand, in PD patients, %RWA and numbers of patients with RWA and RBD symptoms did not change. In conclusion, RBD symptoms in MSA may decrease in association with the aggravation of MSA despite increase in %RWA. However, RBD symptoms in PD patients may not change with the clinical course of PD.

P12-9 Conduction block in acute motor axonal neuropathy

from acute onset to chronic. Weakness was focal in 1, while localized to both hands in another patient. All other patients had widespread mainly asymmetric weakness of the upper and lower limbs. Sensory symptoms were mild or inconspicuous. Motor and sensory nerve conductions were performed, including MNC studies of the forearm and elbow-axilla segments of the median and ulnar nerves. The results were evaluated using our reference values. Results: Six patients exhibited undetectable or small mixed nerve action potential amplitudes, while sensory nerve conductions showed no or only minimal abnormalities. These patients demonstrated demyelinating motor nerve conduction abnormalities along with multifocal CB and responded favorably to steroids, with an unsatisfactory or absent response to IVIg. The remaining patient with chronic focal weakness and motor CB responsive to IVIg displayed no demyelinating motor nerve conduction velocity slowing. Conclusion: MNC studies predict the therapeutic response to corticosteroids in chronic demyelinating neuropathy patients with multifocal CB, in a group of cases which otherwise would be classified as multifocal motor neuropathy or pure motor variant of the chronic inflammatory demyelinating neuropathy.