Quality of life assessment when considering the introduction of device-assisted therapies in advanced Parkinson's disease: A retrospective observational cross-sectional study.

INTRODUCTION: Device-aided therapy (DAT) is an established treatment for improving the quality of life (QOL) in individuals with advanced Parkinson's disease (APD). Criteria for starting DAT, including motor and non-motor symptoms, have been proposed. However, it remains unclear whether QOL differences among patients with APD influence DAT introduction. Therefore, we aimed to investigate QOL differences between patients with and without DAT introduction. METHODS: This retrospective observational cross-sectional study included 245 patients with PD who were followed up between January 1, 2020, and June 30, 2022. We defined cases that underwent DAT introduction after evaluation as "planned-DAT" and those that did not as "not-planned-DAT." We performed between-group comparisons of the PD questionnaire-39 (PDQ-39) summary index (SI) in patients with APD who met the 5-2-1 criteria (≥5 times the oral levodopa dose/day, ≥2 h of "off" symptoms/day, and ≥ 1 h of troublesome dyskinesia/day). RESULTS: Seventy-nine patients met the inclusion criteria for APD (median age: 68 [61.0-73.0] years; 62.8% [N = 52] women). The PDQ-39 SI scores were higher in the planned-DAT group (N = 12) than in the not-planned-DAT group (N = 67) (29.2 [22.1-33.6] vs. 19.0 [10.3-49.6] points, P < 0.05). After propensity-score matching according to age and sex, the PDQ-39 SI scores remained higher in the planned-DAT (N = 9) than in the not-planned-DAT group (N = 18) (40.0 [25.4-60.0] vs. 18.5 [7.9-46.8] points, P < 0.05). CONCLUSIONS: Our results suggest that QOL assessment using PDQ-39 can be used to identify patients eligible for DAT.

Thin-slice Two-dimensional T2-weighted Imaging with Deep Learning-based Reconstruction: Improved Lesion Detection in the Brain of Patients with Multiple Sclerosis.

PURPOSE: Brain MRI with high spatial resolution allows for a more detailed delineation of multiple sclerosis (MS) lesions. The recently developed deep learning-based reconstruction (DLR) technique enables image denoising with sharp edges and reduced artifacts, which improves the image quality of thin-slice 2D MRI. We, therefore, assessed the diagnostic value of 1 mm-slice-thickness 2D T2-weighted imaging (T2WI) with DLR (1 mm T2WI with DLR) compared with conventional MRI for identifying MS lesions. METHODS: Conventional MRI (5 mm T2WI, 2D and 3D fluid-attenuated inversion recovery) and 1 mm T2WI with DLR (imaging time: 7 minutes) were performed in 42 MS patients. For lesion detection, two neuroradiologists counted the MS lesions in two reading sessions (conventional MRI interpretation with 5 mm T2WI and MRI interpretations with 1 mm T2WI with DLR). The numbers of lesions per region category (cerebral hemisphere, basal ganglia, brain stem, cerebellar hemisphere) were then compared between the two reading sessions. RESULTS: For the detection of MS lesions by 2 neuroradiologists, the total number of detected MS lesions was significantly higher for MRI interpretation with 1 mm T2WI with DLR than for conventional MRI interpretation with 5 mm T2WI (765 lesions vs. 870 lesions at radiologist A, < 0.05). In particular, of the 33 lesions in the brain stem, radiologist A detected 21 (63.6%) additional lesions by 1 mm T2WI with DLR. CONCLUSION: Using the DLR technique, whole-brain 1 mm T2WI can be performed in about 7 minutes, which is feasible for routine clinical practice. MRI with 1 mm T2WI with DLR enabled increased MS lesion detection, particularly in the brain stem.

Slow vital capacity as a useful indicator of the prognosis after percutaneous endoscopic gastrostomy in patients with amyotrophic lateral sclerosis.

OBJECTIVES: Forced vital capacity (FVC) is recommended as a respiratory function test in patients with amyotrophic lateral sclerosis (ALS). However, in ALS associated with orofacial palsy, FVC may be an unreliable test. Slow vital capacity (SVC) is an easier and more reliable test even in cases with bulbar symptoms. However, it remains unclear whether respiratory function tests using SVC and FVC are associated with prognosis after percutaneous endoscopic gastrostomy (PEG) placement. This study aimed to confirm whether both SVC and FVC are related to prognosis after PEG placement in patients with ALS. MATERIALS AND METHODS: We conducted this retrospective observational cohort study of 69 consecutive patients diagnosed with sporadic ALS who underwent PEG placement between July 2007 and February 2020. We analyzed the association with mortality 6 months after PEG placement and evaluated long-term prognosis. RESULTS: Forty-four patients met the inclusion criteria. In cases with decreased SVC (p < .01) and FVC (p < .01), a significant difference was observed in mortality 6 months after PEG placement, with an optimal cut-off of SVC ≤57.4% (sensitivity, 0.828; specificity, 0.867) and FVC ≤57.3% (sensitivity, 0.828; specificity, 0.867). Multivariate analysis showed that onset age ≥ 65 years (p < .05), SVC ≤57.4% (p < .01), and FVC ≤57.3% (p < .01) were associated with survival after PEG placement. CONCLUSIONS: SVC, like FVC, is an important prognostic factor after PEG placement in patients with ALS, and there is a possibility that evaluation using SVC can complement respiratory function testing even in cases where the evaluation of FVC is limited.

Assessing the relationship between non-motor symptoms and health-related quality of life in Parkinson's disease: a retrospective observational cohort study.

OBJECTIVES: Non-motor symptoms (NMSs) negatively impact the health-related quality of life (HrQOL) of patients with Parkinson's disease (PD). The Movement Disorder Society-Sponsored Revision of the Unified Parkinson's Disease Rating Scale (MDS-UPDRS) is a comprehensive scale for evaluating PD. It remains unclear whether the NMSs evaluated with MDS-UPDRS are predictive of HrQOL. This study aimed to investigate whether NMSs, as evaluated with the MDS-UPDRS, could predict the HrQOL of patients with PD. MATERIALS AND METHODS: We conducted a 2-year retrospective observational cohort study assessing 108 patients with PD who were recruited from a single tertiary center between January 2015 and December 2017. MDS-UPDRS was used to assess NMSs and motor symptoms and Parkinson's Disease Questionnaire-39 (PDQ-39) to measure patients' HrQOL. RESULTS: The median age of patients was 69 years, and 65.7% were female. The median MDS-UPDRS part I, part II, part III, and PDQ-39-summary index scores were 8, 10, 22, and 25, respectively. The final stepwise multiple linear regression model showed that female sex (standard partial regression coefficient ß = 0.131, P < 0.05) and baseline MDS-UPDRS part I (ß = 0.272, P < 0.01) and part II (ß = 0.571, P < 0.01) scores significantly predicted the PDQ-39-SI scores at the 2-year follow-up. CONCLUSIONS: In addition to motor symptoms, NMSs at the 2-year follow-up may be useful for predicting the HrQOL of patients with PD. In clinical practice, MDS-UPDRS-guided assessment and treatment of motor symptoms and NMSs may contribute to improving HrQOL in patients with PD.

Helicopter Transport for Patients with Cerebral Infarction in Rural Japan.

OBJECTIVES: Physician-staffed helicopter transport is faster than ground transport and allows for prompt medical care of patients in rural areas. In this study, we evaluated the relationship between helicopter transport and the prognosis of patients with acute cerebral infarction in rural Japan. METHODS: This retrospective, observational study included 546 patients with acute cerebral infarction attending Aomori Prefectural Central Hospital, which serves a rural region of Japan. Patients were separated into 2 transport groups: physician-staffed helicopter emergency medical services and ground emergency medical services. Patients were assessed for stroke severity, treatment, and prognosis. RESULTS: Of the 546 patients, 11.2% were transported by physician-staffed helicopter emergency medical services and 88.8% by ground emergency medical services. Although the distance transported was significantly longer in the physician-staffed helicopter emergency medical services group, the time from onset to reaching our hospital was similar between the groups. National Institutes of Health Stroke Scale on admission and final prognosis were significantly worse with physician-staffed helicopter emergency medical services than with ground emergency medical services. Multivariate analysis showed no association between transport system and prognosis. CONCLUSIONS: In this study, patients transported by physician-staffed helicopter emergency medical services had more severe stroke symptoms and poorer functional outcomes than those transported by ground emergency medical services. However, the transport time was shorter for physician-staffed helicopter emergency medical services; thus, physician-staffed helicopter emergency medical services may be useful for reducing transport time for patients in rural Japan.

Subacute Sensory Ataxic Neuronopathy With Thymoma Presenting Marked Improvement After Steroid Therapy.

Subacute sensory ataxic neuronopathy is a well-known form of paraneoplastic syndrome. Most sensory neuronopathies are associated with small cell lung cancer and anti-Hu antibodies, and usually show only slight improvement with immunotherapy. To date, there have been few reports of neuropathy associated with thymoma and no treatment strategy has been established for thymoma-related neuropathy. Here, we provide the first report of a case of sensory ataxic neuronopathy with thymoma that showed marked improvement after steroid therapy, even though preceding intravenous immunoglobulin treatments and tumor resection were less effective. A 57-year-old Japanese man was referred to our hospital with a 6-week history of distal paresthesia in his four limbs and an unsteady gait. He presented with left-dominant ataxia in his four limbs due to reduced sensation in his extremities. He also complained of constipation, difficulty urinating, and erectile dysfunction. Upon investigation, including electrodiagnostic studies, the patient was diagnosed as having sensory ataxic neuronopathy with invasive thymoma. A first round of intravenous immunoglobulin therapy, a following thymectomy, and a second round of intravenous immunoglobulin therapy after the surgery were not effective in treating his neurological symptoms. Subsequently, oral steroid therapy was started, which brought about a remarkable improvement; 6 weeks after the beginning of the steroid therapy, his neurological symptoms were resolved, except for slight distal paresthesia in his feet. Although rarely reported, thymoma can underlie sensory neuronopathy, and the response of thymoma-associated sensory neuronopathy to immunotherapy might be better than that of anti-Hu antibody-related neuropathies. Even if the first immunotherapy is not effective in treating neuropathy with thymoma, further immunomodulatory treatment should be tried after treating the tumor.

Evaluation of cerebral blood flow in older patients with status epilepticus using arterial spin labeling.

INTRODUCTION: Although older patients with status epilepticus (SE) have a high mortality rate and poor outcome, it is difficult to perform emergent electroencephalography (EEG) to diagnose SE in community hospitals. Arterial spin labeling (ASL) is a non-invasive magnetic resonance imaging (MRI) technique that can rapidly assess cerebral blood flow (CBF). Further, ASL can detect increased CBF in the ictal period. Therefore, ASL may be a useful tool for diagnosing SE in older patients. However, its effectiveness in this population is unknown. METHODS: We retrospectively investigated differences in CBF abnormalities between older patients (≥70 years) and non-older patients (<70 years) with SE using ASL. Participants were diagnosed with convulsive status epilepticus (CSE) or non-convulsive status epilepticus (NCSE) based on symptoms, brain MRI, and EEG. RESULTS: ASL detected CBF abnormalities in 40% of older patients with CSE or NCSE. Rates of CBF abnormalities in older patients were not significantly different compared with that in non-older patients. CONCLUSIONS: ASL did not detect a higher rate of CBF abnormalities in older patients, but may help physicians diagnose SE in older patients in a community hospital setting if emergent EEG cannot be immediately performed.

Clinical diagnostic utility of contrast-enhanced three-dimensional fluid-attenuated inversion recovery for selection of brain biopsy sites in neurosarcoidosis: A case report.

Neurosarcoidosis is difficult to diagnose, because definite diagnosis requires detailed histology of the central nervous system. Three-dimensional contrast-enhanced fluid-attenuated inversion recovery (CE-FLAIR) is more useful for detecting leptomeningeal lesions compared with 3D CE-T1 weighted imaging. However, the clinical diagnostic utility of 3D CE-FLAIR for neurosarcoidosis is unclear. We describe a case of a 46-year-old Japanese woman who was admitted to our department due to chronic headache with fever and diplopia. Using 3D CE-FLAIR, we performed brain biopsy from right cerebellar lesion. The histological examination revealed typical non-caseating granulomas, indicating neurosarcoidosis. Our findings suggest that 3D CE-FLAIR may detect leptomeningeal lesions that are candidates for biopsy in chronic meningitis undetermined etiology.

Association of survival and hyperthermia after rt-PA for ischemic stroke.

BACKGROUND: Hyperthermia in patients with acute ischemic stroke is associated with poor outcome. Although previous studies have shown a negative effect on functional outcome, even in patients treated with intravenous recombinant tissue plasminogen activator (rt-PA), the effect on survival remains unclear. AIMS OF THE STUDY: The aim of this study was to evaluate the association between the functional and survival prognosis and hyperthermia in patients with acute ischemic stroke treated with rt-PA. METHODS: We studied 120 patients treated with rt-PA from 2306 consecutive Japanese patients with acute cerebral infarction at Aomori Prefectural Central Hospital between December 2009 and March 2017. We defined hyperthermia as ≥38°C within 72 hours after rt-PA administration. Propensity score matching was used to compare 34 non-hyperthermia and hyperthermia patient pairs. RESULTS: Final modified Rankin Scale scores were higher in the hyperthermia group than in the non-hyperthermia group. In addition, the Kaplan-Meier model showed that the non-hyperthermia group had significantly better survival rates than the hyperthermia group (hazard ratio, 5.3; 95% confidence intervals, 1.2-24.8). CONCLUSIONS: Hyperthermia within 3 days after rt-PA is associated with poor functional prognosis and survival outcome in patients with acute cerebral infarction.

The factors associated with impulse control behaviors in Parkinson's disease: A 2-year longitudinal retrospective cohort study.

INTRODUCTION: Impulse control behaviors (ICBs) are impulsive-compulsive behaviors often associated with dopamine replacement therapy in Parkinson's disease (PD). Although remission can occur in ICB, only four reports on the ratio of remission and the persistence of ICB have been published, and the associated factors with ICB remission or persistence have been little known. Therefore, we conducted a longitudinal assessment of the remission, persistence, and development of ICB and those associated factors in patients with PD. METHODS: We retrospectively investigated a PD database at Aomori Prefectural Central Hospital, Japan. One hundred and forty-eight patients with PD who could be followed up for 2 years were enrolled. ICB was assessed using the Questionnaire for Impulsive-Compulsive Disorders in Parkinson's disease. Motor severity (Hoehn and Yahr scale and United Parkinson's Disease Rating Scale), cognitive function (Mini-Mental State Examination), and other clinical variables (sex, age, onset age, disease duration, olfactory dysfunction, and dyskinesia) and medications used to treat PD were assessed. Univariate analyses were performed. RESULTS: Seven patients were excluded because of the exclusion criteria, and 141 patients were analyzed. Thirty patients (21.3%) had ICB at baseline, and these patients also had significantly higher use of pergolide. The ICB remission rate was 60%, the ICB persistence ratio was 40%, and the ICB development ratio was 12.6% over 2 years. Statistically, younger age and pergolide use were associated with ICB persistence. Being male, having dyskinesia, and rotigotine, entacapone, zonisamide, and istradefylline use were associated with ICB development. CONCLUSION: This study suggests that younger age and pergolide use may be the new associated factors with ICB persistence and that entacapone, zonisamide, and istradefylline use may be associated with the development of ICB. Drug profiles and medication practices in Japan may explain the association of these factors with ICB.

Progressive multifocal leukoencephalopathy associated with thymoma with immunodeficiency: a case report and literature review.

BACKGROUND: The development of progressive multifocal leukoencephalopathy (PML) is associated with severe cellular immunosuppression. Good's syndrome (GS) is a rare immunodeficiency syndrome related to thymoma, with the development of humoral as well as cellular immunosuppression; however, there are few reports of PML due to GS. One report suggested that the neurological symptoms of PML related to thymoma may be improved by a reduction of immunosuppressive therapy for myasthenia gravis (MG). It is therefore necessary to identify the cause of immunodeficiency in patients with PML to enable an appropriate treatment strategy to be adopted. CASE PRESENTATION: A 47-year-old Japanese woman was admitted with aphasia and gait difficulty. She had an invasive thymoma that had been treated with repeated chemotherapy, including cyclophosphamide. She had also previously been diagnosed with MG (Myasthenia Gravis Foundation of America clinical classification IIa), but her ptosis and limb weakness had completely recovered. On admission, neurological examination revealed motor aphasia and central facial weakness on the right side. Laboratory studies showed severe lymphopenia, decreased CD4+ and CD8+ T cell and CD19+ B cell counts, and reduced levels of all subclasses of immunoglobulins, suggesting GS. Serology for human immunodeficiency virus (HIV) infection was negative. Brain magnetic resonance imaging showed asymmetric multifocal white matter lesions without contrast enhancement. Cerebrospinal fluid real-time polymerase chain reaction for JC virus was positive, showing 6,283,000 copies/mL. We made a diagnosis of non-HIV-related PML complicated with GS and probable chemotherapy-induced immunodeficiency. She then received intravenous immunoglobulin therapy, mirtazapine, and mefloquine, but died of sepsis 46 days after admission. CONCLUSIONS: It is necessary to consider the possibility of immunodeficiency due to GS in patients with PML related to thymoma. Neurologists should keep in mind the risk of PML in MG patients with thymoma, even if the MG symptoms are in remission, and should thus evaluate the immunological status of the patient accordingly.

The Differential Diagnosis of Acute Onset Truncal Ataxia: The Importance of Dysgeusia in Miller Fisher Syndrome.

Miller Fisher syndrome (MFS) can be difficult to diagnose, particularly in mild cases where some of the standard triad of symptoms (external ophthalmoplegia, ataxia, and loss of deep tendon reflex) are absent. We herein report a case of the incomplete form of MFS diagnosed in a 54-year-old Japanese man who presented only with ataxia symptoms and was positive for the anti-GQ1b antibody. However, the patient also suffered from dysgeusia, a significant impairment of taste perception. We propose that dysgeusia in acute-onset ataxia cases may constitute an important clinical feature to aid in the diagnosis of the incomplete form of MFS.

Utility of nerve conduction studies for diagnosis of injury to the medial branch of the superficial radial nerve.

INTRODUCTION: The clinical utility of nerve conduction study (NCS) for the distal medial branch of the superficial radial nerve (SRN) has not yet been clarified. Therefore, we investigated the clinical utility of NCS in patients with suspected SRN injury and compared the results with those in healthy control subjects. METHODS: Bilateral NCS of the medial branch of the SRN was performed in two patients with suspected injury of the medial branch of the SRN, and in 20 healthy control subjects. A surface recording electrode was placed at the medial side of the metacarpophalangeal joint of the thumb. The SRN was then stimulated at a location 12 cm proximal from the recording electrode. RESULTS: The mean sensory nerve action potential in the two patients was significantly lower than that of the controls (6.75 ± 0.92 vs. 23.8 ± 8.2 µV, P < 0.05). The side-to-side differences in sensory nerve action potential in the two patients were significantly higher than in the controls (55 ± 7.1 vs. 11 ± 7.8%, P < 0.05). CONCLUSIONS: NCS may be useful for diagnosing injury of the medial branch of the SRN.

LSVT-BIG Improves UPDRS III Scores at 4 Weeks in Parkinson's Disease Patients with Wearing Off: A Prospective, Open-Label Study.

The efficacy of LSVT-BIG for advanced Parkinson's disease (PD) patients with wearing off remains to be determined. Therefore, we evaluated whether LSVT-BIG improves motor disability in eight PD patients with wearing off. Unified Parkinson's Disease Rating Scale (UPDRS) scores, daily off time, and mobility assessments were evaluated during the "on" time before and after the LSVT-BIG course. LSVT-BIG significantly improved UPDRS III scores at 4 weeks and UPDRS II scores in the "off" state at 12 weeks, with no changes in the other measures. The findings suggest that LSVT-BIG may be an effective therapy for advanced PD patients with wearing off.

Dermatomal Sensory Manifestations in Opalski Syndrome.

A 31-year-old Japanese woman presented with sudden-onset unstable gait followed by nuchal pain. A neurological examination revealed right-sided limb weakness and decreased pain and thermal sensation on the left side below the level of the L1 dermatome. A lower lateral medullary infarction with ipsilateral hemiplegia, known as Opalski syndrome, caused by spontaneous vertebral artery dissection was diagnosed by magnetic resonance imaging. The spinothalamic tract in the medulla oblongata has a topographic arrangement of sensory fibers, and the dermatomal sensory deficit in this case can be explained in relation to that. This is the first reported case of Opalski syndrome with dermatomal sensory manifestations. Opalski syndrome could be a differential diagnosis for dermatomal sensory manifestations.