Clinical characteristics of patients with Parkinson's disease with reduced 123I-metaiodobenzylguanidine uptake in the major salivary glands and heart.

BACKGROUND: Parkinson's disease (PD) shows cardiac sympathetic denervation (SD) in 123I-metaiodobezylguanidine (MIBG) scintigraphy. Recently, SD in the major salivary glands (MSG-SD) was introduced as a possible radiological feature of PD. OBJECTIVE: To identify the clinical characteristics of patients with PD with reduced MSG and cardiac MIBG uptake (dual-SD) compared with those with reduced MSG or cardiac MIBG uptake only (single-SD). METHODS: We recruited 90 patients with PD and 30 controls and evaluated their non-motor (e.g., hyposmia, autonomic dysfunction) and motor (e.g., Movement Disorder Society-Unified Parkinson's Disease Rating Scale) features. We also assessed MIBG uptake in the MSG and heart using a quantitative semi-automatic method, and compared MIBG uptakes between PD and controls. We set cut-off values for optimal sensitivity and specificity, and compared the clinical characteristics of patients with PD between dual- and single-SD groups. RESULTS: MSG and cardiac MIBG uptakes were significantly reduced in PD. Sixty-one patients had dual-SD, 25 had single-SD, and four had non-SD. In patients with PD with normal cardiac SD, 76.5% (13/17) of whom showed abnormalities only in MSG-SD. When clinical characteristics were compared between the dual-SD and single-/non-SD groups, patients in the dual-SD group were older and had more severe hyposmia and autonomic dysfunction, except motor features. Multiple logistic regression analysis identified age as an important confounder. CONCLUSIONS: Patients with PD with dual-SD have more severe non-motor features than other patients. Autonomic dysfunction might progress independently from dopaminergic degeneration. Furthermore, our findings indicate that aging is a crucial factor in PD progression.

Aberrant morphometric networks in Alzheimer's disease have hemispheric asymmetry and age dependence.

Alzheimer's disease (AD) is associated with abnormal accumulations of hyperphosphorylated tau and amyloid-ß proteins, resulting in unique patterns of atrophy in the brain. We aimed to elucidate some characteristics of the AD's morphometric networks constructed by associating different morphometric features among brain areas and evaluating their relationship to Mini-Mental State Examination total score and age. Three-dimensional T1-weighted (3DT1) image data scanned by the same 1.5T magnetic resonance imaging (MRI) were obtained from 62 AD patients and 41 healthy controls (HCs) and were analysed by using FreeSurfer. The associations of the extracted six morphometric features between regions were estimated by correlation coefficients. The global and local graph theoretical measures for this network were evaluated. Associations between graph theoretical measures and age, sex and cognition were evaluated by multiple regression analysis in each group. Global measures of integration: global efficiency and mean information centrality were significantly higher in AD patients. Local measures of integration: node global efficiency and information centrality were significantly higher in the entorhinal cortex, fusiform gyrus and posterior cingulate cortex of AD patients but only in the left hemisphere. All global measures were correlated with age in AD patients but not in HCs. The information centrality was associated with age in AD's broad brain regions. Our results showed that altered morphometric networks due to AD are left-hemisphere dominant, suggesting that AD pathogenesis has a left-right asymmetry. Ageing has a unique impact on the morphometric networks in AD patients. The information centrality is a sensitive graph theoretical measure to detect this association.

Efficacy of Adjunctive Therapy with Zonisamide Versus Increased Dose of Levodopa for Motor Symptoms in Patients with Dementia with Lewy Bodies: The Randomized, Controlled, Non-Inferiority DUEL Study.

BACKGROUND: In patients with dementia with Lewy bodies (DLB), it is unknown whether adjunct zonisamide is as effective and safe as increasing levodopa dose when levodopa has inadequate efficacy on parkinsonism. OBJECTIVE: To compare adjunct zonisamide 25 mg/day versus an increased levodopa dose (increased by 100 mg/day) in patients with DLB treated with levodopa ≤300 mg/day for parkinsonism. METHODS: The DUEL study was a multicenter, randomized, controlled, open-label, parallel-group, interventional, non-inferiority trial. During the observation period, levodopa was administered at ≤300 mg/day for 4 weeks. Subsequently, patients were randomized to receive adjunct zonisamide 25 mg/day or levodopa increased by 100 mg/day. RESULTS: Respective adjusted mean changes in MDS-UPDRS Part III total score at 16 and 24 weeks (primary endpoint) were -6.3 and -4.4 in the zonisamide add-on and -0.8 and 2.0 in the levodopa increase groups. The adjusted mean difference at 24 weeks was -6.4 (95% confidence interval [CI] -13.5, 0.7); the upper limit of the 95% CI (0.7) was lower than the non-inferiority margin (3.0). No significant between-group differences were observed in total scores of the MDS-UPDRS Part II, Eating Questionnaire, EuroQol-5 dimension-5 level, Zarit Caregiver Burden Interview, or other secondary endpoints. No notable between-group differences were observed in adverse event incidences. CONCLUSION: Adjunct zonisamide 25 mg/day may yield moderate improvement in motor symptoms in patients with DLB when the levodopa effect is insufficient, but it could not be verified that the zonisamide 25 mg/day was as effective as levodopa 100 mg/day because levodopa showed no sufficient efficacy as assumed.

Simultaneous Four Supratentorial Lesions Predict Tube Dependency Due to an Impaired Anticipatory Phase of Ingestion.

This study aimed to identify the neuroanatomical predictors of oropharyngeal dysphagia and tube dependency in patients with supratentorial or infratentorial ischemic strokes. Patients with acute ischemic stroke were enrolled and were classified into 3 groups: right supratentorial (n = 61), left supratentorial (n = 89), and infratentorial stroke (n = 50). Dysphagia was evaluated by a modified water swallowing test and the Food Intake LEVEL Scale to evaluate oropharyngeal dysphagia and tube dependency, respectively. As two dysphagia parameters, we evaluated the durations from onset of stroke to (1) success in the modified water swallowing test and to (2) rating 7 points or above on the Food Intake LEVEL Scale: patients regained sufficient oral intake and were not tube-dependent. Voxel-based lesion-symptom mapping analysis was performed for a spatially normalized lesion map of magnetic resonance imaging to explore the anatomies that are associated with the two dysphagia parameters for each stroke group. The right precentral gyrus and parts of the internal capsule are associated with oropharyngeal dysphagia. The four supratentorial areas are associated with tube dependency. The dorsal upper medulla is associated with both oropharyngeal dysphagia and tube dependency. These results suggest that supratentorial stroke patients can be tube-dependent due to an impaired anticipatory phase of ingestion. The simultaneous damage in the four supratentorial areas: the inferior part of the precentral gyrus, lenticular nucleus, caudate head, and anterior insular cortex, predicts tube dependency. In contrast, infratentorial stroke patients can be tube-dependent due to oropharyngeal dysphagia caused by lesions in the dorsal upper medulla, damaging the swallowing-related nucleus.

Reduced 123I-MIBG uptake in the parotid and submandibular glands in patients with Parkinson's disease identified using a quantitative semi-automatic method.

OBJECTIVES: To analyze 123I-metaiodobenzylguanidine (MIBG) uptake in the parotid and submandibular glands in patients with Parkinson's disease (PD) in comparison with controls, and to compare MIBG uptake between those glands and the myocardium. Furthermore, we aimed to identify the relationships between clinical features and MIBG uptake. METHODS: We recruited 77 patients with PD and 21 age-matched controls. We assessed MIBG scintigraphy in the major salivary glands and myocardium. We calculated the MIBG uptake ratio in the parotid glands/mediastinum (P/M), submandibular glands/mediastinum (S/M), and heart/mediastinum (H/M) using a quantitative semi-automatic method. We investigated the correlations between MIBG uptake and clinical features. RESULTS: The P/M and H/M ratios in the early and delayed phases were significantly reduced in PD patients compared to controls, while the delayed phase S/M ratio was reduced in PD patients compared to controls. The P/M ratio correlated with the S/M ratio, while neither the P/M nor S/M ratio correlated with the H/M ratio. Between PD patients and controls, sensitivity and specificity were 54.8% and 59.1% for the delayed phase P/M ratio, while sensitivity and specificity were 59.5% and 61.0% for the delayed phase S/M ratio, respectively. Furthermore, sensitivity and specificity for the delayed phase H/M ratio were 85.7% and 79.2, respectively. CONCLUSION: MIBG uptake in the parotid and submandibular glands was reduced in patients with PD. Furthermore, sympathetic denervation in the major salivary glands and myocardium might progress independently. Our findings suggest a new aspect of the pathological distribution of PD.

Correlation of callosal angle at the splenium with gait and cognition in normal pressure hydrocephalus.

OBJECTIVE: Idiopathic normal pressure hydrocephalus (iNPH) is characterized by ventricular enlargement that deforms the corpus callosum, making the callosal angle (CA) small. The authors aimed to evaluate the clinical usefulness of the CA in different planes in iNPH. METHODS: Forty patients with iNPH were included in the study. As a control group, 241 patients with other neurological diseases and 50 healthy controls were included. The subjects had been seen at the authors' institutions from 2010 to 2020. The Timed Up and Go (TUG) test total time and Mini-Mental State Examination (MMSE) total score were evaluated. CAs were measured in the axial plane at the splenium and genu and in the coronal plane at the anterior commissure and posterior commissure by using 3-dimensional T1-weighted MR images. As other hydrocephalus parameters, the Evans index, frontal-occipital horn ratio, and third ventricular width were also measured in patients with iNPH. Associations between each CA or hydrocephalus parameter and clinical parameters were evaluated. The classification efficacy of each CA in differentiating between iNPH and other neurological diseases and healthy controls was evaluated. RESULTS: The CA at the splenium, but no other hydrocephalus parameters, was correlated with TUG total time or MMSE total score in patients with iNPH. Receiver operating characteristic analysis showed that a CA of 71.1° at the splenium has 90.0% sensitivity and 89.0% specificity in discriminating iNPH from other neurological diseases and healthy controls. Probabilistic tractography analysis showed that neuronal fibers via the splenium connect the superior parietal lobules, temporal lobes, and occipital lobes. CONCLUSIONS: The study results suggest that interhemispheric disconnections at the splenium are, at least in part, responsible for gait and cognitive disturbance in iNPH. The CA at the splenium is a unique morphological feature that correlates with gait and cognition in iNPH, and it is useful for discriminating iNPH from other neurological diseases and healthy controls.

Synuclein Motor Dysfunction Composite Scale for the Discrimination of Dementia With Lewy Bodies From Alzheimer's Disease.

Background: An abnormal increase of α-synuclein in the brain is the hallmark of dementia with Lewy bodies (DLB). However, the diagnostic power of plasma α-synuclein in DLB is not yet confirmed. Parkinsonism is highly associated with and is one of the core clinical features of DLB. We studied plasma α-synuclein and developed a novel tool that combined plasma α-synuclein level and Motor Dysfunction Questionnaire (MDQ), namely Synuclein Motor Dysfunction Composite Scale (SMDCS), for the clinical discrimination of DLB from Alzheimer's disease (AD). Methods: This cross-sectional study analyzed participants' demographical data, plasma α-synuclein level, MDQ, structured clinical history questionnaire, neuropsychological and motor function tests, and neuroimaging studies. The power of plasma α-synuclein level, MDQ, and SMDCS for discriminating DLB from non-demented controls (NC) or AD were compared. Results: Overall, 121 participants diagnosed as 58 DLB, 31 AD, and 31 NC were enrolled. Patients with DLB had significantly higher mean plasma α-synuclein level (0.24 ± 0.32 pg/ml) compared to the NC group (0.08 ± 0.05 pg/ml) and the AD group (0.08 ± 0.05 pg/ml). The DLB group demonstrated higher MDQ (2.95 ± 1.60) compared to the NC (0.42 ± 0.98) or AD (0.44 ± 0.99) groups. The sensitivity/specificity of plasma α-synuclein level, MDQ, and SMDCS for differentiating DLB from non-DLB were 0.80/0.64, 0.83/0.89, and 0.88/0.93, respectively. Conclusion: Both plasma α-synuclein and MDQ were significantly higher in patients with DLB compared to the NC or AD groups. The novel SMDCS, significantly improved accuracy for the clinical differentiation of DLB from AD or NC.

Foot Trajectory Features in Gait of Parkinson's Disease Patients.

Parkinson's disease (PD) is a progressive neurological disorder characterized by movement disorders, such as gait instability. This study investigated whether certain spatial features of foot trajectory are characteristic of patients with PD. The foot trajectory of patients with mild and advanced PD in on-state and healthy older and young individuals was estimated from acceleration and angular velocity measured by inertial measurement units placed on the subject's shanks, just above the ankles. We selected six spatial variables in the foot trajectory: forward and vertical displacements from heel strike to toe-off, maximum clearance, and change in supporting leg (F1 to F3 and V1 to V3, respectively). Healthy young individuals had the greatest F2 and F3 values, followed by healthy older individuals, and then mild PD patients. Conversely, the vertical displacements of mild PD patients were larger than the healthy older individuals. Still, those of healthy older individuals were smaller than the healthy young individuals except for V3. All six displacements of the advanced PD patients were smaller than the mild PD patients. To investigate features in foot trajectories in detail, a principal components analysis and soft-margin kernel support vector machine was used in machine learning. The accuracy in distinguishing between mild PD patients and healthy older individuals and between mild and advanced PD patients was 96.3 and 84.2%, respectively. The vertical and forward displacements in the foot trajectory was the main contributor. These results reveal that large vertical displacements and small forward ones characterize mild and advanced PD patients, respectively.

Mother and son cases of Bickerstaff's brainstem encephalitis and fisher syndrome with serum anti-GQ1b IgG antibodies: a case report.

BACKGROUND: Bickerstaff's brainstem encephalitis (BBE) and Fisher syndrome (FS) are immune-mediated diseases associated with anti-ganglioside antibodies, specifically the anti-GQ1b IgG antibody. These two diseases potentially lie on a continuous spectrum with Guillain-Barré Syndrome (GBS). There are some reports of family cases of GBS and fewer of FS. However, there are no reports of family cases of BBE and FS. CASE PRESENTATION: We report a familial case of an 18-year-old son who had BBE and his 52-year-old mother diagnosed with FS within 10 days. The son showed impaired consciousness 1 week after presenting with upper respiratory symptoms and was brought to our hospital by his mother. He showed decreased tendon reflexes, limb ataxia, albuminocytologic dissociation in his spinal fluid, and positive serum anti-GQ1b antibodies. Haemophilus influenzae was cultured from his sputum. He was diagnosed with BBE and treated with intravenous immunoglobulin (IVIg) therapy, which led to an improvement in symptoms. The mother presented with upper respiratory symptoms 3 days after her son was hospitalized. Seven days later, she was admitted to the hospital with diplopia due to limited abduction of the left eye. She showed mild ataxia and decreased tendon reflexes. Her blood was positive for anti-GQ1b antibodies. She was diagnosed with FS and treated with IVIg, which also led to symptomatic improvement. CONCLUSIONS: There are no previous reports of familial cases of BBE and FS; therefore, this valuable case may contribute to the elucidation of the relationship between genetic predisposition and the pathogenesis of BBE and FS.

Oculo-dento-digital Dysplasia Presenting as Spastic Paraparesis Which Was Successfully Treated by Intrathecal Baclofen Therapy.

A 42-year-old man with a history of migraine and bilateral syndactyly presented with numbness of the extremities and shaking legs, which thus prevented him from working as a carpenter. A neurological examination revealed spastic paraparesis with pathological reflexes on all four extremities. Oculo-dento-digital dysplasia (ODDD) was suspected based on his medical history and characteristic facial appearance including small eye slits, thin mouth, and pinched nose with anteverted nostrils. Genetic tests revealed a gap junction alpha 1 (GJA1) gene mutation and confirmed the diagnosis of ODDD. His spastic paraparesis was resistant to oral antispastic medication, however, his symptoms successfully improved after the initiation of intrathecal baclofen therapy, which thus allowed him to return to work.

Japanese Familial Cases of Frontotemporal Dementia and Parkinsonism with N279K Tau Gene Mutation.

BACKGROUND: Mutations in the tau gene linked to chromosome 17 cause frontotemporal dementia and parkinsonism (FTDP-17). OBJECTIVE: This study presents 3 Japanese familial cases diagnosed with N279K tau gene mutation, including 1 autopsy-confirmed case. METHODS: We compared the clinical presentations, cognitive functions, and images between the 3 familial cases diagnosed with N279K mutation. RESULTS: All 3 patients presented symptoms in their early 40s. One patient showed severe cognitive dysfunction and died in his sixth year after onset. The remaining 2 cases presented with parkinsonism-dominant clinical features. Among the 2 cases, 1 presented the characteristic symptoms of progressive supranuclear palsy. The pathological features of the dementia-dominant case showed frontal and temporal lobe-dominant neuronal loss and gliosis. Tau-positive neuronal and glial inclusions were found throughout. Further, tufted astrocytes and globose tangles were present whereas there were no Pick bodies and astrocytic plaques, compatible with pathology-confirmed frontotemporal lobar degeneration (FTLD) -tau subtypes. CONCLUSIONS: Patients with FTDP-17 can be classified into the following 2 major groups: dementia and parkinsonism-plus predominant phenotypes. Among our 3 cases, 1 showed dementia predominance whereas the other 2 showed parkinsonism predominance. Mutations in the microtubule-associated protein tau (MAPT) present with several pathological features. Clinically, our case presented a behavioral variant frontotemporal dementia (bvFTD). However, morphologically, the observed glial and neuronal pathology met the criteria for progressive supranuclear palsy (PSP). This study highlights the clinical heterogeneity within and between families with same MAPT mutation. Few pathologically confirmed PSP cases have been reported with mutations in MAPT.

A Study for Expanding Application Sites for Rotigotine Transdermal Patch.

The rotigotine transdermal patch (RTP) is a dopamine agonist used to treat Parkinson's disease (PD). Some PD patients cannot continue RTP treatment due to application site reactions. We explored sites for RTP where application site reactions are less severe than those in the six approved application sites. Thirty PD patients (12 men, mean age = 76 years) who underwent RTP at the approved sites and had some application site reactions were enrolled in this study. When applying the RTP to the approved application sites for more than four weeks (pre-RTP) and then on the shin for the following four weeks (post-RTP), skin reactions, itching evaluated using the skin irritation score, motor symptoms, clinical global impressions scale, and plasma rotigotine concentration were examined. The mean visual analogue scale and skin irritation score in the post-RTP group were significantly lower than those in the pre-RTP group. The mean Movement Disorder Society-Unified Parkinson's Disease Rating Scale part III score in the post-RTP group was slightly but significantly lower than that in the pre-RTP group. Plasma rotigotine concentration in the post-RTP group was slightly but significantly lower than that in the pre-RTP group. These results indicate that the shin can be a useful application site for RTP.

A case of neuronal intranuclear inclusion disease with recurrent vomiting and without apparent DWI abnormality for the first seven years.

Neuronal intranuclear inclusion disease (NIID) is a rare, neurodegenerative disorder characterized by the presence of eosinophilic hyaline intranuclear inclusions, which are ubiquitin-positive and p62-positive, in neuronal and somatic cells; this can be observed on skin biopsy. Although patients with NIID present with a variety of symptoms that often make the diagnosis difficult, characteristic high-signal intensity of the corticomedullary junction on diffusion-weighted imaging (DWI) often provides a clue to the diagnosis of NIID. We present a case of NIID in a 57-year-old woman who only had recurrent vomiting for four years, which is uncommon as the presenting symptom; moreover, DWI showed no apparent abnormality until a slightly abnormal intensity lesion appeared at the right frontal corticomedullary junction seven years after the first episode of recurrent vomiting. Skin biopsies revealed multiple p62-positive nuclear inclusions, and genetic test showed GGC repeat expansion in NOTCH2NLC; this may form the genetic basis for NIID. Retrospectively, we found that abnormal cerebellar signals besides the vermis in the fluid attenuation inversion recovery (FLAIR) images were detected early-on in the disease. Periodic vomiting may be the only symptom of NIID in the early stages of the disease, and cerebellar abnormalities in FLAIR may serve as an important finding in the diagnosis of NIID, even in the absence of characteristic clinical symptoms or abnormal DWI signals at the cerebral corticomedullary junction.

Chemical Meningitis after a Golf Swing-induced Dermoid Cyst Rupture.

A 51-year-old man developed a sudden headache during golf practice, followed by a high fever. He was admitted with suspected neutrophilic meningitis and was diagnosed with chemical meningitis caused by a dermoid cyst rupture based on the characteristic magnetic resonance imaging (MRI) findings, which showed multiple lipid droplets in his ventricle and cistern. His repetitive golf-swing motion was suggested to be the cause of his dermoid cyst rupture. On MRI, the lipid droplets appeared to have migrated by gravity because of the body position. Therefore, the body position should be considered to prevent obstructive hydrocephalus by lipid droplets after a dermoid cyst rupture.

Late-onset myasthenia gravis successfully treated with local resection of cervical ectopic thymoma.

A 78-year-old woman was admitted complaining progressive respiratory failure, neck weakness and gait disturbance. She was diagnosed as acetylcholine receptor antibody-positive myasthenia gravis crisis with ectopic cervical thymoma. After she recovered from crisis by plasmapheresis and administration of prednisone, we did not choose extended thymectomy but chose local resection of ectopic thymoma considering her age and complications. After the operation, she got minimal manifestations and no relapse of thymoma. Although international and Japanese guidelines recommend extended thymectomy for myasthenia gravis with thymoma, isolated local resection of ectopic thymoma may be enough for controlling myasthenia gravis especially in elderly patients.

Synchronized Tactile Stimulation on Upper Limbs Using a Wearable Robot for Gait Assistance in Patients With Parkinson's Disease.

This study aimed to investigate whether using a wearable robot applying interactive rhythmic stimulation on the upper limbs of patients with Parkinson's disease (PD) could affect their gait. The wearable robot presented tactile stimuli on the patients' upper limbs, which was mutually synchronized with the swing of their upper limbs. We conducted an evaluation experiment with PD patients (n = 30, Modified Hoehn-Yahr = 1-3, on-state) to investigate the assistance effect by the robot and the immediate after-effect of intervention. The participants were instructed to walk 30 m under four different conditions: (1) not wearing the robot before the intervention (Pre-condition), (2) wearing the robot without the rhythm assistance (RwoA condition), (3) wearing the robot with rhythm assistance (RwA condition), and (4) not wearing the robot immediately after the intervention (Post-condition). These conditions were conducted in this order over a single day. The third condition was performed three times and the others, once. The arm swing amplitude, stride length, and velocity were increased in the RwA condition compared to the RwoA condition. The coefficient of variance (CV) of the stride duration was decreased in the RwA condition compared to the RwoA condition. These results revealed that the assistance by the robot increased the gait performance of PD patients. In addition, the stride length and velocity were increased and the stride duration CV was decreased in the Post-condition compared to the Pre-condition. These results show that the effect of robot assistance on the patient's gait remained immediately after the intervention. These findings suggest that synchronized rhythmic stimulation on the upper limbs could influence the gait of PD patients and that the robot may assist with gait rehabilitation in these patients.

[Relationship between striatal 123I-FP-CIT uptake and cognitive functions in Parkinson's disease].

We studied whether 123I-FP-CIT uptake in the striatum correlates with cognitive performance in patients with Parkinson's disease (PD). Sixty-nine patients with PD (24 men and 45 women, average age = 71.3 years, disease duration = 5.2 years) underwent 123I-FP-CIT SPECT and neuropsychiatric measurements. Cognitive performance was assessed using the MMSE (Mini-mental state examination), TMT (Trail making test), MoCA-J (Japanese version of Montreal cognitive assessment), FAB (Frontal assessment battery), ACE-R (Addenbrooke's cognitive examination revised), BDI-II (Beck depression inventory-II), and RBMT (Rivermead behavioural memory test) in the ON state. The average specific binding ratio (SBR) and AI (Asymmetry index) of striatal 123I-FP-CIT uptake were semi-quantitatively measured using DaTView software. Clinical data and SBR were compared. In addition, participants were divided into four groups according to the visibility of the striatum on 123I-FP-CIT SPECT images, and results of the Eagle Wing (EW) group and the Egg shape (ES) group were compared. SBR was negatively correlated with age (P < 0.001), disease duration (P < 0.001), H-Y stage (P < 0.001), levodopa equivalent dose (P = 0.004), TMT-A (P = 0.001), and TMT-B (P < 0.001), and positively correlated with MMSE (P = 0.021) and FAB (P = 0.029), MoCA-J (P = 0.012)，RBMT (P = 0.021). Multiple regression analysis confirmed that age (P = 0.016) and FAB (P = 0.035) were independent predictors for SBR. Furthermore, in the ES group, Movement Disorder Society - Unified Parkinson's disease rating scale part III scores were significantly higher (P = 0.013) and the AI was lower (P < 0.001) than those in the EW group. Conversely, there were no differences in the findings of neuropsychiatric measures between the two groups. Our study results demonstrate that imaging with 123I-FP-CIT SPECT is sensitive for detecting dopaminergic deficits associated with frontal lobe functions in patients with PD.

Portable Thermographic Screening for Detection of Acute Wallenberg's Syndrome.

Wallenberg's syndrome (WS) is a type of brainstem infarction. WS patients often show Horner's syndrome, dissociated sensory disturbance, truncal ataxia, and hoarseness. However, they rarely show tactile sensory disturbance and paralysis of the extremities. Additionally, acute brainstem infarction is often not apparent in magnetic resonance images. These symptomatic and imaging characteristics sometimes lead to misdiagnosis of WS as a non-stroke disease, including auditory vertigo. Although careful neurological examination is necessary to prevent misdiagnosis of WS, this type of examination may be difficult for non-neurologists to whom affected patients initially present. Lateral differences in body surface temperature (BST) constitute a recognized and widespread symptom of WS. We previously reported that most acute WS patients exhibit lateral differences in BST at multiple locations and that these lateral differences in BST could easily be detected by thermographic measurement. Here, we present the method for use of portable thermography to detect acute WS, using a simple, rapid, noninvasive, and cost-effective approach. To assess lateral differences in BST among patients with suspected WS, BST was measured as soon as possible in the examination room or in the patient's bedroom. Measurements were performed bilaterally at four locations where images could easily be acquired (face, palm of the hand, abdomen, and dorsum of the foot) using a portable thermal camera. When lateral differences in BST are observed macroscopically, especially in multiple locations on the same side, a diagnosis of WS should be suspected. Macroscopic assessment of BST laterality can be made within 2 min of the acquisition of thermographic images. This method may be useful in preventing misdiagnosis of acute WS as a non-stroke disease, especially when such patients initially present to non-neurologists.

Adult-onset Still's disease presenting with aseptic meningitis as the first symptom in an elderly patient.

•Neutrophilic meningitis is rarely observed in Adult onset Still's disease (AOSD).•AOSD-related meningitis has been observed in the AOSD course of young adults.•An elderly man showed neutrophilic meningitis as a first symptom of AOSD.•First-line therapy with steroid for bacterial meningitis complicated the diagnosis.•Hyperferritinemia led to the correct diagnosis of AOSD and AOSD-related meningitis.

Efficacy of istradefylline for gait disorders with freezing of gait in Parkinson's disease: A single-arm, open-label, prospective, multicenter study.

Background: Gait disorders are common in Parkinson's disease patients who respond poorly to dopaminergic treatment. Blockade of adenosine A2A receptors is expected to improve gait disorders. Istradefylline is a first-in-class selective adenosine A2A receptor antagonist with benefits for motor complications associated with Parkinson's disease. Research design and methods: This multicenter, open-label, single-group, prospective interventional study evaluated changes in total gait-related scores of the Part II/III Movement Disorder Society-Unified Parkinson's Disease Rating Scale (MDS-UPDRS) and Freezing of Gait Questionnaire (FOG-Q) in 31 Parkinson's disease patients treated with istradefylline. Gait analysis by portable gait rhythmogram was performed. Results: MDS-UPDRS Part III gait-related total scores significantly decreased at Weeks 4-12 from baseline with significant improvements in gait, freezing of gait, and postural stability. Significant decreases in MDS-UPDRS Part II total scores and individual item scores at Week 12 indicated improved daily living activities. At Week 12, there were significant improvements in FOG-Q, new FOG-Q, and overall movement per 48 h measured by portable gait rhythmogram. Adverse events occurred in 7/31 patients. Conclusions: Istradefylline improved gait disorders in Parkinson's disease patients complicated with freezing of gait, improving their quality of life. No unexpected adverse drug reactions were identified. Trial registration: UMIN-CTR (UMIN000020288).