Pemphigus is an autoimmune blistering disease with two major subtypes, pemphigus vulgaris (PV) and pemphigus foliaceus (PF). Patients with pemphigus have circulating antidesmoglein (Dsg)1 and/or anti-Dsg3 IgG autoantibodies. We have previously developed enzyme-linked immunosorbent assays (ELISAs) using recombinant Dsg1 and Dsg3 expressed by baculovirus as a diagnostic tool for pemphigus. The purpose of this study was to evaluate the practical application of these ELISAs for clinical use with a large number of serum samples. We used 81 PV sera, 48 PF sera, 114 bullous pemphigoid (BP) sera, 124 collagen disease sera, nine sera of other non-pemphigus bullous diseases and 179 normal control sera. A cut-off value was determined by receiver-operating-characteristic plots. Forty-seven of 48 PF sera (97.9%) were positive in the Dsg1 ELISA and 79 of 81 PV sera (97.5%) were positive in the Dsg3 ELISA, while only two (1. 1%) and four (2.2%) of 179 normal sera were positive in Dsg1 and Dsg3 ELISAs, respectively. However, some disease control sera of BP and collagen diseases exceeded the cut-off value. Introduction of a grey zone helped to decrease the number of these false-positive sera. Furthermore, in three patients studied, the respective Dsg1 and Dsg3 ELISA scores showed parallel fluctuation with the disease activity along the time course. We conclude that Dsg1 and Dsg3 ELISAs provide a simple, sensitive and highly specific assay for the diagnosis of patients with PV and PF and that these ELISAs may be a valuable tool to monitor the disease activity. We also propose diagnostic criteria for pemphigus based on ELISA reactivity: if a serum is positive against Dsg3 it indicates a diagnosis of PV, regardless of reactivity against Dsg1; if a serum is negative for Dsg3 and positive for Dsg1, it indicates a diagnosis of PF.

Objective There are many reports regarding the course of migraines during pregnancy. However, the prevalence and characteristics of migraines during the postpartum period have not been adequately investigated. We prospectively investigated the patients suffering from migraines over a long postpartum period in an obstetrics department in Japan. Methods We 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 assess medication use and migraine 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 >30 years of age than in those ! 30 years of age (p<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 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. Until six months after delivery, breastfeeding is associated with a lower recurrence rate than bottle feeding.

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.

The Japanese Headache Society and the Japanese Society of Neurology has published the 2013 guidelines for the diagnosis and treatment of chronic headache. A new CQ has been set up in the guidelines on the topic of "How to treat chronic migraine." In the past, lomerizine was the only prophylactic medication of migraine that was eligible under insurance coverage. However, afterward in 2010, valproate was added to the list of antimigraine medications approved under insurance coverage, followed by propranolol, amitriptyline, and verapamil, with rapid advances in the treatment of migraine. Valproate, topiramate (not approved under insurance coverage until date), and amitriptyline could potentially be used in the treatment of chronic migraine in Japan; further, considering the clinical outcomes thus far, lomerizine could also be added to the list. As a drug for migraine prophylaxis, valproate is contraindicated in pregnant women and needs to be used with caution.

A 56-year-old woman had been experiencing episodic left eye pain followed within 3 days by double vision and adduction of the left eye since the age of 30. The episodes occurred once per month, and her symptoms spontaneously resolved within 3 days. The patient was diagnosed with ophthalmoplegic migraine (OM) with left abducens nerve palsy at the age of 53 years. In May 2011, she developed bilateral retro-orbital pain followed by double vision and limitation of abduction of the right eye. She recalled having a cold and high fever 10 days before the onset of the headache. MRI showed no thickening or enhancement of the right abducens nerve. Constructive interference in steady-state (CISS) MRI showed neurovascular contact between the right abducens nerve and anterior inferior cerebellar artery. Right abducens nerve palsy accompanied by OM was diagnosed after other diseases that can cause ophthalmoplegia were excluded. The patient's eye symptoms gradually improved following steroid treatment. There have been a few similar case reports of adult patients with OM showing left and right abducens nerve palsy at different time points. In this case report, we discuss the possible mechanisms related to OM.