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Cortical superficial siderosis (cSS) is a rare condition that is regarded as a potential magnetic resonance marker of cerebral amyloid angiopathy (CAA). We describe the case of a 68-year-old man with cSS and Parkinson's disease (PD) who subsequently exhibited incidental microhemorrhages, which were only detected on magnetic resonance imaging (MRI), at one week after deep brain stimulation (DBS) surgery. cSS is now considered to be a significant risk factor for CAA and future bleeding. Therefore, because DBS surgery is invasive and may increase the risk of intracerebral hemorrhage, the procedure should be performed carefully when managing patients with PD and CAA.

Background: Some patients with Parkinson’s disease (PD) develop peri-lead brain edema after deep brain stimulation (DBS) surgery. The influence of edema on neurological function is not well characterized. We investigated the relationship of brain edema after DBS surgery with motor and cognitive function. Methods: Thirteen patients with PD (6 males and 7 females; mean age: 61.2 years) who underwent bilateral subthalamic nucleus (STN) DBS surgery were included. All patients underwent magnetic resonance imaging (MRI) examination on day 6 post-DBS surgery. The volume of edema was measured either in the frontal white matter or STN on fluid attenuated inversion recovery (FLAIR) images. We examined the relationship between these volumes and changes in cognitive and motor function. Results: Patients were divided into those with frontal subcortical edema (FE) ≥3,000 mm 3 (FE+ group; n = 7) and

A 74-year-old man was administered nivolumab to treat recurrent squamous cell carcinoma of the lungs. He developed fatigue, redness on the front of his neck, muscle weakness, and difficulty in swallowing after receiving the third course of nivolumab. Physical and neurological examinations showed proximal limb muscle weakness, periorbital erythema, and erythema of the front of his neck as well as fingers. Laboratory investigations revealed elevated serum CK and aldolase levels, and he was diagnosed with dermatomyositis. We initiated steroid pulse therapy and intravenous immunoglobulin therapy; however, he died of advanced lung cancer. Immune checkpoint inhibitor-induced neuromuscular disease is increasingly being observed in clinical practice. We report a rare case of dermatomyositis with squamous cell carcinoma of the lungs secondary to nivolumab treatment.

Purpose Istradefylline is useful in treating the wearing-off state in Parkinson’s disease (PD). We investigated the effectiveness of istradefylline (ISD) in improving arousal, sleep, and gait deficits in patients with PD. Methods We examined 14 patients with PD treated with ISD. We assessed the patients using the Unified Parkinson's Disease Rating Scale, Parkinson's Disease Questionnaire, Timed Up-and-Go test (TUG), Freezing of Gait Questionnaire (FOG-Q), Epworth Sleepiness Scale (ESS), and Parkinson’s Disease Sleep Scale (PDSS) before and 1 month after ISD use. Results ESS scores were significantly lower 1 month after the start of ISD treatment (6.79 ± 6.50) than before the intervention (8.14 ± 6.15, Wilcoxon signed-rank test, p = 0.0033). PDSS scores were not significantly different 1 month after beginning the treatment (112 ± 23 mm) when compared to those before the intervention (110 ± 27 mm, Wilcoxon signed-rank test, p = 0.40). TUG scores were not changed after 1 month of ISD use (14.9 ± 8.3 s) when compared to those before the intervention (21.3 ± 30.0 s, Wilcoxon signed-rank test, p = 0.59). Although these measures were not significantly affected by ISD treatment, some patients remarkably improved after the treatment. FOG-Q scores were significantly lower 1 month after the beginning of treatment (9.79 ± 7.16) than those before the intervention (12.14 ± 5.82, Wilcoxon signed-rank test, p = 0.030). Conclusions ISD may improve daytime sleepiness and FOG in patients with PD.

The present study aims to determine the gynecologic health status of asymptomatic women at a unique Japanese Health Check-up Institute, Ningen Dock. Medical records of Japanese women, who underwent gynecological medical (health) examinations between January 2011 and December 2016, were retrospectively reviewed. Of the cervical smears from 8927 women aged 18 - 85 years, 50 (0.6%) were classified as dysplastic and malignant changes: 18 of low-grade squamous intraepithelial lesion, 10 high-grade squamous intraepithelial lesion, 21 atypical squamous cells of undetermined significance and 1 cervical squamous cell carcinoma. No case of cervical adenocarcinoma was found. Ultrasonographic examination detected uterus enlargements and ovary tumors in 2.0% and 0.9% of cases, respectively. Most of participants (95.6%) revealed no gynecologic abnormalities. The present study based on the records of Ningen Dock, where asymptomatic participants undergo a medical examination at their own expense, showed very low incidence of abnormal cytologic and/or ultrasonographic findings.

Palmar fasciitis and polyarthritis (PFPA) is an uncommon disorder clinically characterized by rapidly developing bilateral arthritis of the hands and fasciitis of the palms. This cancer-associated syndrome, primarily linked to ovarian cancer, has also been associated with multiple different malignancies. PFPA symptoms usually precede the detection of ovarian cancer. In all patients from case series and single case reports described, ovarian cancer is already present at an advanced stage. Therefore, the authors aimed to present a case and systematically review available evidence on the association between PFPA and ovarian cancer, as gynecological oncologists. Awareness and recognition of PFPA may allow for earlier diagnosis and treatment of an occult ovarian cancer. The commitment of the attending gynecologists to become familiar with and identify rare diseases may lead to the detection at an early and curative stage and potentially life-saving therapeutic interventions.

Neuromelanin-sensitive MR imaging (NMI) is an increasingly powerful tool for the diagnosis of Parkinson's disease (PD). This study was undertaken to evaluate longitudinal changes on NMI in PD patients.We examined longitudinal changes on NMI in 14 PD patients. The area and contrast ratio (CR) of the substantia nigra pars compacta (SNc) were comparatively analyzed.The total area and CR of the SNc upon follow-up NMI were significantly smaller than those on initial NMI (from 33.5±18.9 pixels and 6.35±2.86% to 21.5±16.7 pixels and 4.19±2.11%; Wilcoxon signed-rank test, p0.001 and p=0.022, respectively). The area and CR of the dominant side SNc upon initial NMI were significantly greater than those on follow-up NMI (from 15.3±9.1 pixels and 6.5±2.7% to 7.9±8.5 pixels and 3.7±2.9%; Wilcoxon signed-rank test, p=0.002 and p=0.007, respectively). On a case-by-case basis, the area of the SNc invariably decreased upon follow-up NMI in all patients. We further demonstrated that the total area and CR of the SNc negatively correlated with disease duration (Pearson correlation coefficient, r=-0.63, p0.001 and r=-0.41, p=0.031, respectively). In area analyses, our results demonstrated very high intraclass correlation coefficients for both intra- and inter-rater reliability.NMI is a useful and reliable tool for detecting neuropathological changes over time in PD patients.

BACKGROUND Juvenile muscular atrophy of the distal upper extremity (Hirayama disease) is a sporadic disorder, but very rarely occurs in a familial pattern. OBJECTIVE To describe the clinical features of Hirayama disease in 2 brothers, especially the look-alike anatomic physiques and magnetic resonance imaging (MRI) findings. DESIGN Case report. PATIENTS Two physically look-alike young brothers with Hirayama disease. RESULTS Clinical symptoms of the present brothers were consistent with those of sporadic cases. Their lanky physique, clinical features, and MRI findings looked alike to each other. CONCLUSIONS The pathogenesis of familial Hirayama disease is not well understood. The present brothers looked alike in terms of their physical frame and clinical symptoms. These findings suggest that similar mechanical damage against the cervical spinal cord by look-alike anatomic frame of the neck could be an important factor of familial Hirayama disease in the present brothers.

Topographical disorientation is a condition in which a subject becomes disoriented in familiar surroundings [1]. The selective deficit of topographical disorientation is relatively rare and has been attributed to cerebrovascular disease, Alzheimer’s disease, and frontotemporal dementia [1–3]. Recently, cases of transient topographical amnesia (TTA) have been published [4–7]. These cases are characterized by a sudden onset and short-lasting disturbance in orientation without any loss of consciousness or episodic memory. The patients soon recover completely, and their prognosis seems good without progression to dementia or physical disability. We report a patient who had recurrent episodes of TTA, which gradually worsened over 16 years. The patient was a 61-year-old right-handed man, with 9 years of formal education. His birth and development were normal, and there was no history of neurological disease in his family. His first episode of topographical disorientation occurred at 45 years of age, when he was driving his car along familiar roads. The episode lasted for only several seconds, and immediately following he was reorientated. These episodes first occurred once a year, but gradually increased in frequency with time. The attacks also occurred while walking and occasionally their duration was close to 1 min. At age 61, he experienced these episodes a few times a month, and then visited our hospital. He described the episodes as follows: ‘‘I was driving my car to go home from the office. I suddenly became insensible even to familiar sights. I could not recognize the buildings and bridges for pedestrians while I should know them. Then, they became clear several seconds later.’’ During the episode, he was alert and well-oriented. He was aware of, and memorized his attack, and could describe it later in detail. Neuropsychological evaluation of memory and abstract logical abilities proved normal by means of standardized tests (Table 1). There were no abnormalities in his neurological examination, or in his complete blood count, serum biochemistry including thyroid function and vitamins, Holter electrocardiogram, repeated electroencephalogram (EEG), brain magnetic resonance imaging (MRI) and angiography, with the exception of [I]iodoamphetamine single photon emission computed tomography (SPECT) performed 2 weeks after the last attack, showing hypoperfusion in the inferolateral surface of bilateral occipital lobes (Fig. 1). Repeated EEGs revealed no abnormalities and carbamazepine medication failed to improve his symptoms, although epilepsy was suspected as the pathogenic mechanism. Transient topographical amnesia is a rare symptom, and previous literature reported that some episodes are caused by transient ischemic attack [8], migraine [9], normal pressure hydrocephalus [10], and epilepsy [7, 11]. The etiology of TTA remains unknown. Most patients with TTA are healthy middle-aged or elderly people, and A. Shindo (&) H. Tomimoto Department of Neurology, Mie Graduate School of Medicine, 2-174 Edobashi, Tsu, Mie 514-8507, Japan e-mail: a-shindo@clin.medic.mie-u.ac.jp