Autonomic dysfunction in different subtypes of multiple system atrophy

Human immunodeficiency virus (HIV) infection is associated with autonomic neuropathy. The resultant autonomic dysfunction impairs quality of life and can have fatal consequences. Our aim was to clearly define the symptoms of autonomic dysfunction in African HIV-positive patients and determine whether these symptoms were related with (a) autonomic reflex responses (b) the degree of immunosupression. Thirty-one HIV-positive treatment-naïve African patients (mean CD4 cell count 269.5 +/- 253.4/mm3) and 12 healthy controls completed a detailed questionnaire (Autonomic System Profile, Mayo Clinic, Rochester, MN) relating to specific symptoms of autonomic dysfunction. After completion of the questionnaire, subjects underwent a standard battery of autonomic reflex tests. The autonomic symptom score was higher in the male HIV-positive patients (26.7 +/- 14.7 points) and female patients with CD4 <200/mm3 (24.7 +/- 18.0) than sex-matched controls (male controls, 9.9 +/- 6.8, P < 0.05; female controls, 8.8 +/- 10.1; P < 0.05). Six patients had scores indicative of severe autonomic dysfunction (>43.8 points). The most common autonomic symptoms were: orthostatic intolerance, secretomotor and gastrointestinal dysfunction. There was no relationship between CD4 cell counts and autonomic symptom scores. The blood pressure response to sustained handgrip was blunted, but all other cardiovascular reflex tests were within the normal range or borderline. African HIV-positive patients report symptoms of autonomic dysfunction, despite normal or borderline autonomic reflex responses.

BackgroundThe long-term clinical and physiological consequences of COVID-19 infection remain unclear. While fatigue has emerged as a common symptom following infection, little is known about its links with autonomic dysfunction. SARS-CoV-2 is known to infect endothelial cells in acute infection, resulting in autonomic dysfunction. Here we set out to test the hypothesis that this results in persistent autonomic dysfunction and is associated with post-COVID fatigue in convalescent patients.MethodsWe recruited 20 fatigued and 20 non-fatigued post-COVID patients (median age 44.5 years, 36/40 (90%) female, median time to follow up 166.5 days). Fatigue was assessed using the Chalder Fatigue Scale. These underwent the Ewing’s autonomic function test battery, including deep breathing, active standing, Valsalva manoeuvre and cold-pressor testing, with continuous electrocardiogram and blood pressure monitoring, as well as near-infrared spectroscopy-based cerebral oxygenation. 24-hour ambulatory blood pressure monitoring was also conducted, and patients completed the generalised anxiety disorder-7 questionnaire. We assessed between-group differences in autonomic function test results and used unadjusted and adjusted linear regression to investigate the relationship between fatigue, anxiety, and autonomic test results.ResultsWe found no pathological differences between fatigued and non-fatigued patients on autonomic testing or on 24-hour blood pressure monitoring. Symptoms of orthostatic intolerance were reported by 70% of the fatigued cohort at the time of active standing, with no associated physiological abnormality detected. Fatigue was strongly associated with increased anxiety (p <0.001), with no patients having a pre-existing diagnosis of anxiety.ConclusionsThese results demonstrate the significant burden of fatigue, symptoms of autonomic dysfunction and anxiety in the aftermath of COVID-19 infection, but reassuringly do not demonstrate pathological findings on autonomic testing.

Background/Aims: Dysphagia, a symptom of multiple system atrophy (MSA), is a major clinical concern. In this study, we investigate the characteristics of early oropharyngeal dysphagia (OD) in patients with MSA, and the differences between MSA subtypes. Methods: Patients enrolled in the study had previously been diagnosed with MSA at the clinic of the Department of Neurology, and had been referred for a videofluoroscopic swallowing study (VFSS), between 2005 and 2014, to check for dysphagia. The clinical characteristics and VFSS findings were analyzed and compared between the MSA subtypes. Results: This study enrolled 59 patients with MSA (24 men; 31 with MSA-P, 21 with MSA-C, and 7 with MSA-PC). Dysphagia symptoms were mostly limited to aspiration symptoms (90.48%) in patients with MSA-C, while difficulty in swallowing, increased mealtime, and drooling were frequent in those with MSA-P. The most common VFSS finding amongst patients was vallecular residue (n = 53, 89.8%), followed by penetration/aspiration (n = 40, 67.8%), and coating of the pharyngeal wall (n = 39, 66.1%). Comparison analysis between subtypes showed that apraxia and vallecular residue were more frequent and severe in MSA-P than in MSA-C (p = 0.033 and p = 0.010, respectively). Conclusion: Understanding early OD characteristics in patients with MSA and the differences between MSA subtypes could be helpful in managing dysphagia in patients with MSA. Several dysphagia symptoms similar to those of Parkinson disease were frequently observed in MSA-P, but not in MSA-C. A follow-up study is needed to elucidate the natural course of OD in MSA patients and the difference between MSA subtypes.

The cardiomyopathic and neuropathic phenotype of hereditary transthyretin amyloidosis are well recognized. Cardiovascular autonomic dysfunction is less systematically and objectively assessed. Autonomic and clinical features, quantitative cardiovascular autonomic function, and potential autonomic prognostic markers of disease progression were recorded in a cohort of individuals with hereditary transthyretin amyloidosis and in asymptomatic carriers of TTR variants at disease onset (T0) and at the time of the first quantitative autonomic assessment (T1). The severity of peripheral neuropathy and its progression was stratified with the polyneuropathy disability score. A total of 124 individuals were included (111 with a confirmed diagnosis of hereditary transthyretin amyloidosis, and 13 asymptomatic carriers of TTR variants). Symptoms of autonomic dysfunction were reported by 27% individuals at T0. Disease duration was 4.5 ± 4.0years [mean ± standard deviation (SD)] at autonomic testing (T1). Symptoms of autonomic dysfunction were reported by 78% individuals at T1. Cardiovascular autonomic failure was detected by functional testing in 75% individuals and in 64% of TTR carriers. Progression rate from polyneuropathy disability stages I/II to III/IV seemed to be shorter for individuals with autonomic symptoms at onset [2.33 ± 0.56 versus 4.00 ± 0.69years (mean ± SD)]. Cardiovascular autonomic dysfunction occurs early and frequently in individuals with hereditary transthyretin amyloidosis within 4.5years from disease onset. Cardiovascular autonomic failure can be subclinical in individuals and asymptomatic carriers, and only detected with autonomic function testing, which should be considered a potential biomarker for early diagnosis and disease progression.

The prognostic impact of dysphagia in multiple system atrophy (MSA) remains controversial. This study aimed to investigate the relationship between dysphagia severity and survival in MSA and to elucidate whether this impact differs between MSA-cerebellar ataxia (MSA-C) and MSA-parkinsonism (MSA-P). This retrospective study included 297 patients with MSA: 251 met criteria for clinically established MSA and 46 for clinically probable MSA. Among them, 171 had MSA-C and 126 had MSA-P. We evaluated symptomatic dysphagia within 3years of onset and quantified dysphagia severity using the Hyodo score (0 to 12) through fibreoptic endoscopic evaluation of swallowing (FEES) and clinical features, including autonomic dysfunction and vocal cord paralysis. Patients were followed up until death or tracheostomy, and survival factors were analysed using the log-rank test and multivariate Cox proportional hazards model. Ninety patients developed symptomatic dysphagia within 3years of onset, and 75 were evaluated for dysphagia severity using FEES. Survival from onset was shorter in patients with dysphagia within 3years compared to those without (median: 4.2years vs. 7.3years; p < 0.001). Symptomatic dysphagia within 3years of onset was an independent predictor of shorter survival in the multivariate Cox analysis. While the Hyodo score was higher in MSA-P than in MSA-C patients (p = 0.048), the Hyodo score was associated with survival in both MSA-C and MSA-P patients (log-rank p < 0.001 and p = 0.046, respectively). Symptomatic dysphagia within 3years of onset predicts shorter survival in MSA-C and MSA-P patients.

Penjangkitan wabah Avian influenza menjadi persoalan kesehatan yang menimbulkan dampak serius di bidang yang lain, seperti ekonomi, sosial-budaya, politik, pertahanan, dan keamanan, baik di tingkat nasional maupun internasional, termasuk di Indonesia. Berbagai upaya telah dilakukan untuk mengantisipasi penyebaran wabah ini, salah satunya dengan tindakan karantina untuk menghambat penyakit tersebut dengan tetap mempertimbangkan kelancaran perjalanan, yang dilakukan di area pintu masuk, antara lain di bandar udara, pelabuhan laut, stasiun angkutan darat, sebagai tempat perbatasan antara suatu wilayah dan wilayah luar. Karena itu, tujuan penelitian ini adalah untuk mengetahui peran karantina sebagai alat pencegah untuk menghalangi penjangkitan Avian influenza dan efektivitas implementasinya di Bandara Internasional Soekarho-Hatta. Penelitian ini menggunakan metode deskriptif kualitatif. Lokasi penelitian ini adalah Kantor Kesehatan Pelabuhan (KKP) Kelas I Jakarta Bandara Soekarno Hatta. Data yang digunakan adalah data primer yang diperoleh melalui observasi dan wawancara dan data sekunder yang diperoleh lewat studi dokumenter. Data yang terkumpul dianalisis dengan teknik deskriptif kualitatif. Hasil penelitian ini menunjukkan bahwa di Indonesia, kegiatan karantina diterapkan terutama di Kantor Kesehatan Pelabuhan Kelas I Jakarta dengan tugas dan fungsi pokok mencegah masuknya penyakit karantina dan penyakit menular berpotensi wabah. Sementara itu, di Kantor Kesehatan Pelabuhan Kelas I Bandara Soekarno-Hatta, tugas dan fungsi pokoknya adalah melaksanakan karantina pada pintu masuk wilayah negara Republik Indonesia. Pelaksanaan tugas dan fungsi itu dipengaruhi oleh beberapa faktor antara lain: dasar legalitas (landasan hukum dan operasional) organisasi, sarana dan prasarana, sumber daya manusia, Standar Operasional Prosedur, Kerjasama Lintas-Sektor dalam penerapan teknologi informasi serta dukungan dana untuk pembiayaan operasional kegiatan. Efektivitas berbagai upaya tersebut sangat diperlukan untuk mendukung kokohnya ketahanan nasional dalam upaya penyempurnaan kegiatan karantina yang dilaksanakan KKP Kelas I Bandara Soekarno-Hatta. Pertama, revisi UU No. 2/1962 tentang Karantina perlu dilakukan agar lebih disesuaikan dengan Peraturan Kesehatan Internasional 2005. Kedua, pembenahan sarana dan prasarana perlu dilakukan untuk mendukung pelaksanaan pengawasan higienitas dan sanitasi pesawat sebagai alat angkut, pelayanan kesehatan berupa perbaikan sarana observasi penderita, laboratorium maupun kegiatan rujukan penderita. Ketiga, pengembangan sumber daya manusia, rekrutmen tenaga operasional sesuai dengan tugas melalui pendidikan dan pelatihan. Keempat, penyempurnaan Standar Operasional Prosedur, yang sesuai dengan karakteristik penyakit, dengan tetap mempertimbangkan kelancaran pelaku perjalanan. Kelima, mempermantap kerjasama lintas-sektor antar- dan inter-lembaga serta jaringan informasi pihak banda dan di luar negeri. Dan keenam, mengoptimalkan ketersediaan dana dalam upaya mendukung kegiatan karantina dengan menggali sumber dana dari pihak yang terkait. The outbreak of Avian influenza epidemics is a health problem resulting in serious impact in economic, socio-cultural, political, defence and security sectors at the national and international levels, including in Indonesia. Various efforts are done to anticipate the epidemic, one of which is to do the quarantine efforts of impeding its dissemination while considering smooth traveling in gateways, such as in airport, seaport and land transport station as main borders between territory and the outside. Therefore, purpose of this study is to find out the role of quarantine as the preventive instrument of inhibiting the outbreak of Avian influenza and its effectiveness of implementation in Soekarno-Hatta International Airport. The study used a descriptive qualitative method. The location of the study was the Airport Health Office of Class I Jakarta. Data used were both primary data obtained from interview and secondary data obtained through documentary study. Data collected were then analyzed by a descriptive qualitative technique. Result of the study indicates that the quarantine efforts in Indonesia were particularly done in Airport Health Officer of Class I (AHO-I) Jakarta with main task and function of inhibiting the dissemination of quarantined diseases and potential infectious disease epidemics. In AHO-I of Soekarno-Hatta International Airport, the main task and function was to do quarantine activity in gateways of the Republic of Indonesia. The performance of main tasks and functions were influenced by several factors, such as regulation, infrastructure, human resource, Standard Operating Procedures, cross-sector cooperation in the application of information technology, and fund to support operational finance. The effectiveness of various efforts is highly required to support the national security of completing the quarantine programs in AHO-I of Soekarno-Hatta International Airport. First, revision of Act No. 2/1962 on Quarantine should be done in order to be adapted to International Health Regulation of 2005. Second, the improvement of infrastructures is necessary to do for supporting the performance of hygienist and sanitation monitoring of plane as transport facility, health services by improving patient observation facility, laboratory, and patient reference guides. Third, human resource development and operational personnel recruitment appropriate with main tasks by education and training. Four, completing the Standard Operating Procedures adapted to the characteristics of disease while considering smooth traveling. Five, the establishment of intra- and inter-institutional cross-sector cooperation and the information networks of domestic and foreign airports. And six, optimizing fund sufficiency in the efforts of supporting the quarantine activity by exploring the source of fund from related parties.

Multiple system atrophy(MSA) is a rare adult-onset synucleinopathy that can be divided in two subtypes depending on whether the prevalence of its symptoms is more parkinsonian or cerebellar (MSA-P and MSA-C, respectively). The aim of this work is to investigate the structural MRI changes able to discriminate MSA phenotypes. The sample includes 31 MSA patients (15 MSA-C and 16 MSA-P) and 39 healthy controls. Participants underwent a comprehensive motor and neuropsychological battery. MRI data wereacquired with a 3T scanner (MAGNETOM Trio, Siemens, Germany). FreeSurfer was used to obtain volumetric and cortical thickness measures. A Support Vector Machine (SVM) algorithm was used to assess the classification between patients' group using cortical and subcortical structural data. After correction for multiple comparisons, MSA-C patients had greater atrophy than MSA-P in the left cerebellum, whereas MSA-P showed reduced volume bilaterally in the pallidum and putamen. Using deep gray matter volume ratios and mean cortical thickness as features, the SVM algorithm provided a consistent classification between MSA-C and MSA-P patients (balanced accuracy 74.2%, specificity 75.0%, and sensitivity 73.3%). The cerebellum, putamen, thalamus, ventral diencephalon, pallidum, and caudate were the most contributing features to the classification decision (z>3.28; p<.05 [false discovery rate]). MSA-C and MSA-P with similar disease severity and duration have a differential distribution of gray matter atrophy. Although cerebellar atrophy is a clear differentiator between groups, thalamic and basal ganglia structures are also relevant contributors to distinguishing MSA subtypes.

To evaluate the possibility that parkinsonian signs may be the only presenting feature of multiple system atrophy (MSA), parkinsonian patients were studied who had no atypical clinical signs and had no symptoms of autonomic dysfunction, but who reported that they had not experienced the anticipated good response to dopaminergic treatment. These stringent criteria identified 20 patients from a series of 298 consecutive parkinsonian outpatients. The following clinical pointers were analysed: (a) rate of disease progression; (b) symmetry of parkinsonian symptoms and signs; (c) occurrence of resting tremor during the first three years from onset. In addition, all patients underwent (d) acute and chronic challenge with dopaminergic drugs; (e) cardiovascular reflex autonomic function tests; (f) high field MRI. Rapid progression of disease was seen in 45% of patients, onset was symmetric in 25%, tremor was absent at onset in 70%, response to dopaminergic drug challenges was inadequate in 40%, abnormal cardiovascular reflexes occurred in 50%, and some abnormal MRI finding occurred in 35% of cases. Each of these features was equally weighted by giving to each patient a 0 to 6 point score corresponding to the number of abnormal findings. Fifteen patients scoring higher than 1 were considered at risk for having MSA: five of them were classified as clinically possible (score 2), six as clinically probable (score 3-4), and four patients were classified as clinically definite multiple system atrophy (score 5). The six pointers considered were variably combined in each patient, none of them being universally abnormal in patients with high scores. The patients were followed up for a mean 2.1 (SEM 0.65) years. All but one of the 10 patients prospectively classified as probable or definite MSA developed unequivocal clinical signs of fully symptomatic MSA. A receiver operator characteristic cure was plotted for the prospective score based on follow up diagnosis. The best compromise for trade off between sensitivity and specificity was a cut off value at a score of 3. The sensitivity and specificity of the individual pointers considered to predict fully symptomatic MSA varied considerably, and no single item could predict whether patients presenting with just parkinsonian signs went on during the two year follow up period to develop fully symptomatic MSA. Instead, the number of abnormalities offered a predictive value for the clinical prognosis of these parkinsonian patients.

The aim of this study is to identify the prevalence of frontotemporal dementia (FTD)/corticobasal syndrome (CBS) in a large cohort of pathologically confirmed cases of multiple system atrophy (MSA) and to determine the α-synuclein seeding characteristics and electron cryo-microscopy (cryo-EM) filament structure in frontotemporal lobar degeneration with MSA-type α-synuclein pathology (FTLD-synuclein). The archives of the Queen Square Brain Bank (1989-2023) were searched for histologically confirmed MSA cases, and those with a clinical diagnosis of FTD/CBS were reviewed for pathological features of FTLD-synuclein. Phosphotungstic acid (PTA)-precipitated brain homogenates from FTLD-synuclein, dementia with Lewy bodies (DLB) and G51D SNCA synucleinopathy cases were used to seed aggregation in α-syn140*A53T-YFP HEK293T cells. The structure of α-synuclein filaments from an FTLD-synuclein case was determined by cryo-EM. We identified 283 cases of MSA. Four cases had a clinical diagnosis of CBS, one of which met pathological criteria for FTLD-synuclein. Genetic studies in this case were negative for SNCA variants, and PTA-precipitated brain homogenates seeded abundant cytoplasmic α-synuclein inclusions that were morphologically indistinguishable from those of typical MSA but distinct from those of G51D SNCA and DLB. MSA Type II α-synuclein filaments were identified by cryo-EM. FTD/CBS is rarely associated with MSA pathology. The cell seeding characteristics and cryo-EM findings support the classification of FTLD-synuclein as a subtype of MSA, differentiating it from genetic synucleinopathies, such as those with SNCA variants G51D and A53E, which have neuropathological features overlapping with MSA and Lewy body diseases. These cases expand the clinicopathological spectrum of MSA and FTLD and have implications for our understanding of selective neuronal vulnerability in MSA and the interpretation of α-synuclein biomarker studies.

The aim of this study was to demonstrate circadian blood pressure trends and to evaluate autonomic function in patients with spinocerebellar degeneration (SCD). We performed 24-h ambulatory blood pressure monitoring (ABPM) on 10 patients from seven Japanese families with spinocerebellar ataxia type 6 (SCA6), 10 patients with idiopathic cerebellar ataxia (ICA), eight patients with multiple system atrophy (MSA) suffering from cerebellar MSA (MSA-C), and 12 age- and gender-matched normal subjects. We also evaluated autonomic function in these patients. Three SCA6 patients (30%), six ICA patients (60%), eight MSA-C patients (100%) and four normal subjects (33%) were non-dippers. The nocturnal reduction rate of blood pressure in MSA patients was significantly less than those in normal subjects, SCA6 patients and ICA patients. There were no abnormal findings in any patient with SCA6 in autonomic function tests. All dipper patients exhibited normal findings on autonomic function tests. This is the first report of ABPM in patients with SCD. Blunted reduction of nocturnal blood pressure may be associated with autonomic dysfunction in patients with SCD.

Impaired autonomic function is common in acute ischemic stroke. Previous limited studies have suggested that atherosclerosis may affect the distensibility of the carotid sinus and then impair the cardiovascular autonomic function. This study sought to investigate cardiovascular autonomic function in patients with ischemic stroke with carotid stenosis. Eighty-five patients with ischemic stroke (58 ones without carotid stenosis and 27 ones with carotid stenosis, average 6 months after stroke onset) and 37 elderly controls were recruited. All performed Ewing's battery autonomic function tests. From Ewing's battery of autonomic function tests, atypical, definite, or severe autonomic dysfunction was identified in 69.0% patients without carotid stenosis and 88.9% with carotid stenosis, with significant difference between the two groups, and the prevalence of autonomic dysfunction in both groups was higher than that in controls (21.6%). Patients with carotid stenosis showed impairment of all parasympathetic tests (all P < 0.05) and one of the sympathetic tests [Mean fall in systolic blood pressure (BP) on standing: P = 0.051], and those without carotid stenosis only showed impairment in two parasympathetic tests (Valsalva ratio: P = 0.014; heart rate response to deep breathing: P < 0.001) in comparison with controls. Patients with carotid stenosis had significantly more impairment than those without carotid stenosis in some autonomic parameters (Valsalva ratio: P < 0.05; mean fall in systolic BP on standing: P < 0.05). Cardiovascular autonomic function is impaired in patients with ischemic stroke, but patients with carotid stenosis show more severely impaired parasympathetic and sympathetic functions.

MSA is a progressive neurodegenerative disorder with two main subtypes: MSA with predominant cerebellar ataxia (MSA-C) and MSA with predominant parkinsonism (MSA-P). The latest diagnostic criteria emphasize the importance of neuroimaging markers from MRI alongside clinical symptomatology assessment. This study investigates the relationship between visual MRI markers and multiple system atrophy (MSA) subtypes, clinical features, and cerebral glucose metabolism and striatal dopaminergic degeneration. Eighty-nine patients with MSA, 67 with predominant parkinsonism (MSA-P) and 22 with predominant cerebellar ataxia (MSA-C), underwent extensive clinical and neuropsychiatric evaluations and routine MRI scans to assess markers like the hot-cross bun (HCB) sign, putaminal iron deposition, midbrain to pons (M/P) ratio, and cerebellar atrophy. PET imaging with 18F-fluorodeoxyglucose (18F-FDG) and 11C-2β-carbomethoxy-3β-(4-fluorophenyl) tropane (11C-CFT) was performed to evaluate brain metabolism and striatal dopaminergic uptake abnormalities. Canonical correlation analysis revealed significant associations between clinical symptoms and MRI markers, particularly the HCB sign, M/P ratio, and putaminal iron deposition. The HCB sign and M/P ratio correlated with cerebellar dysfunction, while putaminal iron deposition correlated with parkinsonism severity, particularly in MSA-P. Cerebellar and putaminal metabolism negatively correlated with their respective structural changes. However, putaminal iron deposition showed no significant correlation with striatal dopaminergic uptake. Visual MRI markers are crucial for diagnosing MSA and delineating disease subtype and symptom severity. Supratentorial and infratentorial MRI markers reflect the severity of parkinsonism and cerebellar dysfunction, respectively. Putaminal iron deposition reflects the severity of parkinsonism, suggesting that iron deposition plays an important role in the pathophysiological mechanisms contributing to parkinsonism in MSA.

Neuronal cytoplasmic inclusions (NCIs) were demonstrated by means of silver staining (Gallyas staining) in the central nervous systems of 18 deceased patients with multiple system atrophy (MSA) - 6 with olivopontocerebellar atrophy (OPCA), 6 with striatonigral degeneration (SND) and 6 with Shy-Drager syndrome (SDS). We observed NCIs in the cerebral cortex, putamen, pons, medulla oblongata and spinal cord, and especially in the putamen and pons of all cases with MSA. No NCIs were observed in the cerebellum and midbrain. The findings were common to all 3 subtypes of MSA. NCIs were not present in patients with other neurodegenerative disorders and non-neurological disorders. Our findings indicate that NCIs represent a special neuronal alteration characteristic of MSA and support the theory that OPCA, SND and SDS represent manifestations of a single condition i.e. MSA.

Abnormalities of rate-corrected QT intervals in Parkinson's disease—a comparison with multiple system atrophy and progressive supranuclear palsy

Multiple system atrophy (MSA) is a neurodegenerative disorder that presents as parkinsonism, cerebellar ataxia, and autonomic dysfunction. Magnetic resonance imaging (MRI) findings of MSA are reported to be the atrophy of the putamen/pons/cerebellum, hot cross bun sign, and bright middle cerebellar peduncle (MCP) sign. Here, we assessed the sensitivity of detecting the bright MCP sign in patients with MSA cerebellar variant (MSA-C) using a double inversion recovery (DIR) procedure, comparing it to the sensitivity of detection by T2-weighted image (T2WI) and fluid-attenuated inversion recovery (FLAIR) sequences on conventional MRI. We evaluated 6 MSA-C patients and 6 control patients (multiple sclerosis, neuromyelitis optica, and spinocerebellar atrophy). Characteristics of all the patients were collected, and MRI was analyzed. Two neurologists independently evaluated the visualization of the bright MCP sign using a 4-point visual grade from Grade 0 to Grade 3. Differences in grade between DIR and T2WI or FLAIR were statistically analyzed. Also, as a quantitative analysis, the signal intensity of the MCP lesion was compared with that of the ipsilateral thalamus, and the MCP/thalamus ratio was evaluated. As a result, DIR more sensitively showed the bright MCP signs of MSA-C patients than T2WI or FLAIR. Also, the bright MCP sign deteriorated and expanded over time in the cases we followed with MRI. We also evaluated hot cross bun sign in the pons, but the hot cross bun sign in MSA-C patients was not significantly different between the DIR and conventional MRI sequences. The DIR procedure can be a more sensitive method for detecting the involvement of MCP lesions in MSA-C.