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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.

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.

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.

BACKGROUND AND PURPOSE The aim was to clarify the features affecting cardiac sympathetic denervation in autopsy-confirmed dementia with Lewy bodies (DLB) patients. METHODS Fifty-four autopsy-confirmed DLB patients were enrolled. Tissue samples of the left ventricular anterior wall were immunostained with anti-tyrosine hydroxylase antibody to identify catecholaminergic nerve axons. Immunostained areas were quantified as residual cardiac sympathetic nerve (CSN) axons and the relationship between the degree of residual CSN axons and clinical and neuropathological features was examined. RESULTS Virtually all patients showed small amounts of residual CSN axons (0.87%, range 0.02%-9.98%), with 50 patients (92.6%) showing

Dementia greatly contributes to poor prognosis in patients with Parkinson's disease (PD). We previously reported that severe olfactory dysfunction may be a good predictor of Parkinson's disease dementia (PDD). In this trial, we investigated whether early administration of donepezil to patients with severe hyposmia can reduce the development of PDD.This was a multi-centre, randomized, double-blind, parallel group, placebo-controlled trial in patients with non-demented PD with severe hyposmia (The Donepezil Application for Severe Hyposmic Parkinson's Disease [DASH-PD] study). A total of 201 patients were randomly allocated to receive donepezil or placebo in addition to standard therapy for PD. Patients were followed up every 6 months until the onset of PDD or for a maximum of 4 years. The primary endpoint was the onset of dementia. The secondary endpoint was cognitive impairment measured by Addenbrooke's Cognitive Examination-Revised (ACE-R) and the Clinical Dementia Rating (CDR).(UMIN000009958: February 2013 to May 2019).A total of 201 hyposmic patients with PD were randomly assigned to a treatment: 103 to donepezil and 98 to placebo. Overall, 141 (70%) patients completed the 4-year intervention. During follow-up, 7 of 103 (6.8%) patients in the donepezil group and 12 of 98 (12.2%) patients in the placebo group developed PDD; however, the hazard ratio of PDD incidence was not statistically significant (hazard ratio (HR), 0.609; 95% confidence interval, 0.240 to 1.547;Administration of donepezil to PD patients with severe olfactory dysfunction for 4 years did not change the incidence of dementia but had a beneficial effect on neuropsychological function, with good tolerability.The Ministry of Health Labour and Welfare and the Japan Agency for Medical Research and Development provided funding for this study.

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.

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.

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.

Background and Purpose: High blood pressure increases bleeding risk during treatment with antithrombotic medication. The association between blood pressure levels and the risk of recurrent stroke during long-term secondary stroke prevention with thienopyridines (particularly prasugrel) has not been well studied. Methods: This was a post hoc analysis of the randomized, double-blind, multicenter PRASTRO-I trial (Comparison of Prasugrel and Clopidogrel in Japanese Patients With Ischemic Stroke-I). Patients with noncardioembolic stroke were randomly assigned (1:1) to receive prasugrel 3.75 mg/day or clopidogrel 75 mg/day for 96 to 104 weeks. Risks of any ischemic or hemorrhagic stroke, combined ischemic events, and combined bleeding events were determined based on the mean level and visit-to-visit variability, including successive variation, of systolic blood pressure (SBP) throughout the observational period. These risks were also compared between quartiles of mean SBP level and successive variation of SBP. Results: A total of 3747 patients (age 62.1±8.5 years, 797 women), with a median average SBP level during the observational period of 132.5 mm Hg, were studied. All the risks of any stroke (146 events; hazard ratio, 1.318 [95% CI, 1.094–1.583] per 10-mm Hg increase), ischemic stroke (133 events, 1.219 [1.010–1.466]), hemorrhagic stroke (13 events, 3.247 [1.660–6.296]), ischemic events (142 events, 1.219 [1.020–1.466]), and bleeding events (47 events, 1.629 [1.172–2.261]) correlated with increasing mean SBP overall. Similarly, an increased risk of these events correlated with increasing successive variation of SBP (hazard ratio, 3.078 [95% CI, 2.220–4.225] per 10-mm Hg increase; 3.051 [2.179–4.262]; 3.276 [1.172–9.092]; 2.865 [2.042–4.011]; 2.764 [1.524–5.016], respectively). Event rates did not differ between the clopidogrel and prasugrel groups within each quartile of SBP or successive variation of SBP. Conclusions: Both high mean SBP level and high visit-to-visit variability in SBP were significantly associated with the risk of recurrent stroke during long-term medication with either prasugrel or clopidogrel after stroke. Control of hypertension would be important regardless of the type of antiplatelet drugs. Registration: URL: https://www.clinicaltrials.jp ; Unique identifier: JapicCTI-111582.

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.

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., Antibody; Immune disorder; Immune response; Immunoglobulin; Immunology; Inflammation; Nervous system; Neurology; Neuroscience; Neuronal intranuclear inclusion disease; Recurrent vomiting; Diffusion-weighted imaging; Cerebellum; Leukoencephalopathy

Gait analysis is used widely in clinical practice to evaluate abnormal gait caused by disease. Conventionally, medical professionals use motion capture systems or make visual observations to evaluate a patient's gait. Recent biomedical engineering studies have proposed easy-to-use gait analysis methods employing wearable sensors with inertial measurement units (IMUs). IMUs placed on the shanks just above the ankles allow for long-term gait monitoring because the participant can walk with or without shoes during the analysis. To the knowledge of the authors, no IMU-based gait analysis method has been reported that estimates stride length, gait speed, stride duration, stance duration, and swing duration simultaneously. In the present study, we tested a proposed gait analysis method that uses IMUs attached on the shanks to estimate foot trajectory and temporal gait parameters. Our proposed method comprises two steps: stepwise dissociation of continuous gait data into multiple steps and three-dimensional trajectory estimation from data obtained from accelerometers and gyroscopes. We evaluated this proposed method by analyzing the gait of 19 able-bodied participants (mean age 23.9 years, 9 men and 10 women). Wearable sensors were attached on the participants' shanks, and we measured three-axis acceleration and three-axis angular velocity with the sensors to estimate foot trajectory during walking. We compared gait parameters estimated from the foot trajectory obtained with the proposed method and those measured with a motion capture system. Mean accuracy (± standard deviation) was 0.054 ± 0.031 m for stride length, 0.034 ± 0.039 m/s for gait speed, 0.002 ± 0.020 s for stride duration, 0.000 ± 0.017 s for stance duration, and 0.002 ± 0.024 s for swing duration. These results suggest that the proposed method is suitable for gait analysis, whereas there is a room for improvement of its accuracy and further development of this IMU-based gait analysis method will enable us to use such systems for clinical gait analysis.

Background and purposeWe previously reported the usefulness of iodine-123 metaiodobenzylguanidine (123I-MIBG) myocardial scintigraphy for differentiation of dementia with Lewy bodies (DLB) from Alzheimer’s disease (AD) in a cross-sectional multicentre study. The aim of this study was, by using reassessed diagnosis after 3-year follow-up, to evaluate the diagnostic accuracy of 123I-MIBG scintigraphy in differentiation of probable DLB from probable AD.MethodsWe undertook 3-year follow-up of 133 patients with probable or possible DLB or probable AD who had undergone 123I-MIBG myocardial scintigraphy at baseline. An independent consensus panel made final diagnosis at 3-year follow-up. Based on the final diagnosis, we re-evaluated the diagnostic accuracy of 123I-MIBG scintigraphy performed at baseline.ResultsSixty-five patients completed 3-year follow-up assessment. The final diagnoses were probable DLB (n=30), possible DLB (n=3) and probably AD (n=31), and depression (n=1). With a receiver operating characteristic curve analysis of heart-to-mediastinum (H/M) ratios for differentiating probable DLB from probable AD, the sensitivity/specificity were 0.77/0.94 for early images using 2.51 as the threshold of early H/M ratio, and 0.77/0.97 for delayed images using 2.20 as the threshold of delayed H/M ratio. Five of six patients who were diagnosed with possible DLB at baseline and with probable DLB at follow-up had low H/M ratio at baseline.ConclusionsOur follow-up study confirmed high correlation between abnormal cardiac sympathetic activity evaluated with 123I-MIBG myocardial scintigraphy at baseline and the clinical diagnosis of probable DLB at 3-year follow-up. Its diagnostic usefulness in early stage of DLB was suggested.Trial registration numberUMIN00003419.

New methods for the diagnosis and new treatments for Parkinson's disease (PD) were explained. As imaging tools, neuromelanin imaging using brain MRI, meta-iodobenzylguanidine (MIBG) cardiac scintigraphy, dopamine transporter scintigraphy, and transcranial sonography were introduced. Olfactory dysfunction and REM sleep behavior disorders (RBD), which are important non-motor symptoms, and the new Clinical Criteria for PD launched by Movement Disorder Society (MDS) were also described. Investigative new medications and new anti-PD medications, which recently became available in Japan, were introduced. I explained the rationale of early treatment, strategy of initial treatment, the significance of continuous dopaminergic stimulation, strategy of treatment for advanced PD, and deep brain stimulation as a surgical treatment together with promising new treatments including gene therapy and cell transplantation.

We studied whether

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.

Highlights • 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.

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.

Brain MRI is essential for differentiating Parkinson's disease (PD) from other parkinsonian syndromes (PS). The purpose of performing brain MRI is to exclude other PS. Recently, several new MRI techniques such as functional MRI and neuromelanin imaging have been introduced in the diagnosis of PD. MIBG cardiac scintigraphy is a sensitive imaging tool to differentiate PD from other PS. Dopamine transporter imaging is a sensitive tool to detect very early neurodegenerative parkinsonism but is difficult to differentiate PD from other neurodegenerative PS. Brain perfusion imaging is sometimes useful to diagnose PD. Transcranial sonography (TCS) of the substantia nigra is useful to differentiate PD from other PS. However the recording failure of TCS in aged, particularly female subjects, may limit the clinical use in Japan.

Cardiac meta-iodobenzylguanidine (MIBG) uptake on (123)I-MIBG cardiac scintigraphy is reduced in patients with Lewy body disease such as Parkinson's disease (PD), dementia with Lewy bodies (DLB), and pure autonomic failure, and has been reported to be useful for differentiating PD from other parkinsonian syndromes, as well as DLB from Alzheimer disease (AD). Postmortem studies have shown that the number of tyrosine hydroxylase (TH)-immunoreactive nerve fibers of the heart was decreased in pathologically-confirmed Lewy body disease, supporting the findings of reduced cardiac MIBG uptake in Lewy body diseases. Now, reduced cardiac MIBG uptake can be a potential biomarker for the presence of Lewy bodies in the nervous system. (123)I-MIBG cardiac scintigraphy can allow us to determine the presence of Lewy bodies.

Neuropathological hallmarks of Lewy body disease including Parkinson's disease (PD) and dementia with Lewy bodies (DLB) are Lewy bodies in not only the central nervous system but also the peripheral autonomic nervous system. α-synuclein is a presynaptic protein, and is a major constituent of Lewy bodies. The peripheral autonomic nervous system innervates various organs such as the gastrointestinal tract, the heart, the bladder, and the skin. Severe autonomic dysfunction is one of the supportive clinical features of DLB, and reduced cardiac MIBG uptake is an indicative biomarkers of DLB according to the revised criteria for the clinical diagnosis of DLB in 2017. Recently, it was reported that α-synuclein deposits in various biopsied tissue samples, particularly in the skin, can act as a possible biomarker to diagnose PD. Above approach was conducted by using skin biopsies from patients with DLB. Phosphorylated α-synuclein deposits in the skin were found in all patients with DLB, but not in patients with non-synucleinopathy dementia or controls, suggesting that phosphorylated α-synuclein can potentially act as a diagnostic biomarker in DLB.

Objectives Reduced cardiac meta -iodobenzylguanidine (MIBG) uptake and loss of cardiac sympathetic axons, as its possible anatomical substrate, were both recognised in Lewy body disease (LBD), while their direct correlation has so far remained speculative. Increasing availability of autopsy-confirmed cases of LBD prompted us to quantify residual cardiac sympathetic axons to establish their relationship to cardiac MIBG uptake. Methods We collected cardiac tissue samples from 23 patients with autopsy-confirmed LBD and two non-LBD control patients who underwent 123 I-MIBG cardiac scintigraphy in life. Samples of the left ventricular anterior wall were stained with anti-tyrosine hydroxylase (TH) and anti-neurofilament (NF) antibodies as markers of cardiac nerve axons. We quantified the immunolabelled areas and assessed their correlation to standardised heart to mediastinum (H/M) ratios of 123 I-MIBG cardiac scintigraphy. Results Cardiac MIBG uptake in the early and delayed phases was reduced in 90.9% and 95.7% of patients with LBD, respectively. The area of TH-immunoreactive axons correlated significantly with the H/M ratio in the early (p=0.036) as well as in the delayed (p=0.018) phases. The area of NF-immunoreactive axons also correlated with the H/M ratio in the early (p=0.003) as well as in the delayed (p=0.001) phases. Conclusions Tight quantitative correlation between cardiac 123 I-MIBG uptake and corresponding loss of sympathetic axons in LBD, as established for the first time by this study, provides a scientific basis to confirm the reliability of MIBG cardiac scintigraphy as a powerful clinical tool to detect loss of these axons as a biomarker for the presence of Lewy body disease.

A 70-year-old man with a 28-year history of type 2 diabetes mellitus was admitted due to persistent vomiting and neurological abnormalities in Nov 2012. He had developed gait disturbance and diplopia for six months during antiplatelet therapy, which was initiated following the diagnosis of a cerebellar infarction in June 2012. He had nystagmus, truncal ataxia and an ocular motility disorder, and the MRI study showed increased FLAIR and DWI signals in the peri-third ventricle and periaqueductal region, in addition to the cerebellar vermis. Wernicke encephalopathy was suspected according to his symptoms, and thiamine administration dramatically improved his condition. He did not have a history of alcohol abuse or poor eating habits; however, various coexisting factors, including diabetes mellitus, pyloric stenosis and the use of antiulcer drugs and insulin, were considered to be responsible for Wernicke encephalopathy. This case demonstrates the importance of distinguishing Wernicke encephalopathy from cerebrovascular disease in elderly patients.

It is a well-established fact that the sympathetic, parasympathetic and enteric nervous systems are affected at early stages in Parkinson's disease (PD). However, it is not yet clarified whether the earliest pathological events preferentially occur in any of these three divisions of the autonomic nervous system (ANS). Significant involvement of the peripheral autonomic nervous system of the heart and gastrointestinal tract has been documented in PD. Accumulating evidence suggests that the PD pathology spreads centripetally from the peripheral to central nervous system through autonomic nerve fibers, implicating the ANS as a major culprit in PD pathogenesis and a potential target for therapy. This study begins with a brief overview of the structures of the central and peripheral autonomic nervous system and then outlines the major clinicopathological manifestations of cardiovascular and gastrointestinal disturbances in PD.