Muneto Tatsumoto

Intractable chronic inflammatory demyelinating polyneuropathy treated successfully with ciclosporin

Background: Chronic inflammatory demyelinating polyneuropathy (CIDP) is a heterogeneous disorder and both clinical course and response to treatment vary widely. Because of the propensity for relapse, CIDP requires maintenance therapy after the initial response to treatment. There is no consensus regarding this in the published literature. Present report: A patient with CIDP was treated with oral prednisolone and cyclophosphamide pulse therapy but required repeated plasma exchange and intravenous immunoglobulin (IVIg). Treatment with ciclosporin freed the patient from repeated IVIg administration. Therapeutic responses in 14 subsequent cases including three patients who showed improvement with ciclosporin are also presented along with an algorithm of the authors’ suggested protocol for treatment. Conclusion: Ciclosporin should be considered for patients with intractable CIDP who require repeated IVIg.

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.

Spectrum of neurological diseases associated with antibodies to minor gangliosides GM1b and GalNAc-GD1a

The authors reported the neurological disease spectrum associated with autoantibodies against minor gangliosides GM1b and GalNAc-GD1a. IgG and IgM antibody reactivity against gangliosides GM1, GM2, GM1b, GD1a, GalNAc-GD1a and GQ1b was investigated in sera from 7000 consecutive patients who had various neurological conditions. The clinical diagnoses for 456 anti-GM1b-positive patients were Guillain-Barré syndrome (GBS, 71%), atypical GBS with preserved deep tendon reflexes (12%), Fisher syndrome (10%), Bickerstaffs brainstem encephalitis (2%), ataxic GBS (2%) and acute ophthalmoparesis (1%). For 193 anti-GalNAc-GD1a-positive patients, the diagnoses were GBS (70%), atypical GBS (16%), Fisher syndrome (10%) and Bickerstaffs brainstem encephalitis (3%). Of the patients with GBS or atypical GBS, 28% of 381 anti-GM1b-positive and 31% of 166 anti-GalNAc-GD1a-positive patients had neither anti-GM1 nor anti-GD1a antibodies. Of those patients with Fisher syndrome, Bickerstaffs brainstem encephalitis, ataxic GBS or acute ophthalmoparesis, 33% of 67 anti-GM1b-positive, and 52% of 25 anti-GalNAc-GD1a-positive patients had no anti-GQ1b antibodies. Autoantibodies against GM1b and GalNAc-GD1a are associated with GBS, Fisher syndrome and related conditions. These antibodies should provide useful serological markers for identifying patients who have atypical GBS with preserved deep tendon reflexes, ataxic GBS, Bickerstaffs brainstem encephalitis or acute ophthalmoparesis, especially for those who have no antibodies to GM1, GD1a or GQ1b. A method to prepare GM1b was developed.

Prevalence of typical migraine aura without headache in Japanese ophthalmology clinics

The aim of this study was to investigate the prevalence and characteristics of typical aura without headache (TAWH) in Japan. We distributed a self-report questionnaire comprising seven items. Of 1914 cases, the number of patients who provided valid answers was 1063. They included 1063 out-patients with 81 of these patients positive in the ID migraine screener Japanese version. TAWH was diagnosed in 35 patients (3.2%), aged 23–87 years, and included 12 males and 23 females. The age of patients with TAWH showed a biphasic distribution (20–39 years and 60–69 years), similar to the age distribution of all patients. Migraine with aura was diagnosed in 67 patients (6.3%) and showed a monophasic age distribution (40–49 years). These data suggest that TAWH is not a rare headache type in clinics especially in a setting of general ophthalmology clinics, and some patients of migraine with aura may transform to TAWH with ageing.

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.

Delayed facial weakness in Guillain-Barré and miller fisher syndromes

Introduction: Dr. C. Miller Fisher described the appearance of unilateral facial palsy after resolution of ataxia in a patient with the eponymic Miller Fisher syndrome (MFS). However, there have been very few reports of delayed appearance of facial weakness in Guillain‐Barré syndrome (GBS) and MFS when the other neurological signs reached nadir or started improving. Methods: In this study we reviewed the clinical and laboratory findings of consecutive patients with GBS (n = 195) and MFS (n = 68). Results: Delayed facial weakness occurred in 12 (6%) GBS and 4 (6%) MFS patients and was unilateral in 5 (42%) GBS and 2 (50%) MFS patients. In those patients with delayed facial weakness, neither limb weakness nor ataxia progressed, and facial weakness disappeared without immunotherapy. Conclusions: Because facial weakness can lead to further morbidity, it would be prudent for clinicians to warn patients of this possibility, although additional immunotherapy is usually not required. Muscle Nerve 51: 811–814, 2015

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.

Normal peripheral circadian phase in the old-old with abnormal circadian behavior

Almost all organisms maintain a circadian clock from birth to death to synchronize their own physiology and behavior with the earths rotation. However, extensive studies based on animal experiments have showed that aging results in circadian dysfunction. Human studies have also indicated age‐associated abnormal phase, reduced amplitude and enhanced fragmentation in circadian physiology and behavior, thereby strongly implying age‐related dysfunction of the clock machinery. Here, we carried out functional assessment of the circadian clock machinery in elderly patients aged 83–94 with severe dementia who showed abnormal circadian behavior. To investigate whether or not the systemic pathway from the circadian input to peripheral clocks functioned normally, the circadian phase in peripheral clock gene expression rhythms was evaluated using plucked hair tissues. Unexpectedly, the phase in all volunteer patients was within a range similar to that of healthy subjects. The circadian pathway from external inputs to peripheral clocks may therefore function normally, even in the old‐old with severe dementia.

Characteristics of migraines during the postpartum period: A long-term prospective study

WCN 2013 No: 933 Topic: 8 — Headache Characteristics of migraines during the postpartum period: A long-term prospective study E. Hoshiyama, M. Tatsumoto, H. Iwanami, A. Saisu, H. Watanabe, N. Inaba, K. Hirata. Department of Neurology, Dokkyo Medical University, Mibu, Japan; Department of Obstetrics and Gynecology, Dokkyo Medical University, Mibu, Japan Background: Although there have been many reports regarding the course of migraines during pregnancy, the prevalence and characteristics of migraines during the postpartum period remain unclear. Objective: To investigate the characteristics of migraines over a long postpartum period. Patients and methods: We prospectively investigated the course of migraines experienced during the postpartum period by patients in a postnatal ward. The patients were surveyed during the first postpartum week and 1, 3, 6 and 12 months after delivery. The patients were provided a headache diary to assessmedication use andmigraine attack frequency, severity (the faces pain scale) and duration. Results: The migraine remission rate was 63%, 83% and 85% during the first, second and third trimesters, respectively. No patient experienced a worsening of headaches during pregnancy. Headache recurrence during the first month after delivery was more frequent in the patients N30 years of age than in those ≤30 years of age (p b 0.05). The percentage of women experiencing recurrence at 1, 3, 6 and 12 months after delivery was 63%, 75%, 78% (n = 60) and 87.5% (n = 40), respectively. In breastfeeding patients, the rates were 50%, 65.8%, 71.1% and 91.7% and in bottle feeding patients, the rates were 86.4%, 90.9%, 95.5% and 81.3%, respectively. Conclusion: In our study, we found that 85% of the patients with migraines experience remission during pregnancy and that more than 50% experience recurrence during the first month after delivery. Breastfeeding is associated with a lower recurrence rate than bottle feeding until six months after delivery. doi:10.1016/j.jns.2013.07.1804 Abstract — WCN 2013 No: 912 Topic: 8 — Headache Does pramipexole treatment improve headache in patients with concomitant migraine and restless legs syndrome? WCN 2013 No: 912 Topic: 8 — Headache Does pramipexole treatment improve headache in patients with concomitant migraine and restless legs syndrome? S. Suzuki, K. Suzuki, M. Miyamoto, T. Miyamoto, A. Numao, Y. Watanabe, R. Takashima, K. Hirata. Department of Neurology, Dokkyo Medical University, Mibu, Japan; Department of Neurology, Dokkyo Medical University Koshigaya Hospital, Koshigaya, Japan Background: Recent studies have suggested a link between migraines and restless legs syndrome (RLS). Although these disorders may share a dopaminergic dysfunction in the hypothalamic A11 nucleus that contributes to this association, there have been no clinical studies evaluating the effect of dopaminergic treatment on migraine symptoms in patients with concomitant migraines and RLS. Objective: To report an illustrative case and to investigate the effects of immediate-release pramipexole (P-IR) treatment onmigraine headaches. Patients and methods: We retrospectively reviewed the medical records of patients who experienced both migraines and RLS. Results: Ten patients (9 patients from the previously completed single-center study) received P-IR treatment were included in the study. Five out of the 10 patients (50%) reported subjective and objective improvement according to the Clinical Global Impression rating scale for migraine headaches. Of these 5 patients, 4 (80%) reported morning headaches. Conclusion: Our results suggest that the identification of RLS in migraine patients is important and that dopaminergic treatment may improve both migraines and RLS symptoms. doi:10.1016/j.jns.2013.07.1805 Abstract — WCN 2013 No: 753 Topic: 8 — Headache Cerebellar infarction due to vertebral artery dissection presented with isolated headache in a patient with migraine WCN 2013 No: 753 Topic: 8 — Headache Cerebellar infarction due to vertebral artery dissection presented with isolated headache in a patient with migraine H.S. Lee, H.Y. Park, H. Chang, K.H. Cho. Department of Neurology, School of Medicine, Wonkwang University, Iksan, Republic of Korea Background: Migraine is a known risk factor for ischemic stroke, especially in young cwoman. Also, cervical artery dissection is the most common single etiology of stroke in young adults. However the mechanism of migraine-related arterial dissection is still unknown. It is proposed that the repeated attack of migraine would make the involved artery more vulnerable to tearing and lead to dissection. Patients andmethods: (Case) A 34-year-old femalewas presentedwith sudden severe headache during 24 h. The patient complained severe headache with nausea and vomiting after awakening. She suffered from migraine for more than 10 years. The throbbing nature headache usually persisted with 1 or 2 days associated with nausea, vomiting, and photophobia. At emergency department, routine laboratory test and brain CT showed normal findings.We started conservativemanagement for decreasing pain. However, headache and vomiting persisted, and dizziness and imbalance were also noted later. After admission, the neurological examination showed dysmetria, gaze-evoked nystagmus, and ataxia. Brain MRI demonstrated a high signal on the diffusion weighted image and low on the apparent diffusion coefficient image in the right cerebellar hemisphere consisted with acute infarction. Cerebral CT angiography revealed the left vertebral artery dissection. The patient was reluctant to use an anticoagulant. Also, topiramate and beta blocker were given to patient for migraine prophylaxis. Conclusions: The exact mechanism of migraine-related intracranial artery dissection has yet to be proven, we propose that this may be caused by vessel wall edematous changes with repeated migraine attacks resulting in sudden or unusual stretching. doi:10.1016/j.jns.2013.07.1806 Abstract — WCN 2013 No: 786 Topic: 8 — Headache Study on mood and anxiety disorders in patients with tensin-type headache WCN 2013 No: 786 Topic: 8 — Headache Study on mood and anxiety disorders in patients with tensin-type headache V. Tudorica, C. Zaharia, D. Pirscoveanu, D. Stanca, O. Alexandru, F. Trifan, C. Albu. University of Medicine and Pharmacy, Craiova, Romania Abstracts / Journal of the Neurological Sciences 333 (2013) e481–e518 e511