Right-sided Herpes Zoster Ophthalmicus Complicated by Bilateral Third, Fourth, and Sixth Cranial Nerve Palsies and Syndrome of Inappropriate Antidiuretic Hormone Secretion: A Case Report.

Cases of herpes zoster ophthalmicus (HZO) complicated by bilateral ophthalmoplegia are rare, and no cases of bilateral third, fourth, or sixth cranial nerve palsies have been reported. Syndrome of inappropriate antidiuretic hormone secretion (SIADH) is a rare complication of HZO. We herein report an 80-year-old Japanese woman with right-sided HZO complicated by meningoencephalitis and discuss the pathogenesis of this condition. She developed bilateral third, fourth, and sixth cranial nerve palsies and SIADH almost simultaneously during treatment for HZO. The bilateral cranial palsy spontaneously resolved within a few months.

Streamlined workflow including nurse recognition of conjugate gaze deviation for reduced door-to-puncture time in endovascular thrombectomy: A retrospective study.

BACKGROUND: Endovascular thrombectomy is recognized as a pivotal treatment for acute ischemic stroke due to large vessel occlusion. Prolonged door-to-puncture time correlates with decreased patient independence after acute ischemic stroke. This study aimed to assess whether a streamlined workflow, including nurse recognition of conjugate gaze deviation, could reduce door-to-puncture time in endovascular thrombectomy. METHODS: This study retrospectively reviewed patients with acute ischemic stroke who underwent endovascular thrombectomy between March 2017 and March 2022 and compared a previous workflow with a streamlined workflow implemented in April 2019. In the streamlined workflow, nurses recognized conjugate gaze deviation to identify patients with large vessel occlusions and played a more active role in reducing the door-to-puncture time. We compared time metrics and outcomes, including recanalization status, parenchymal hemorrhage type 2, and favorable outcomes (modified Rankin Scale score 0-2) at three months between the previous and streamlined workflow groups. RESULTS: After the application of the streamlined workflow, the door-to-puncture time was reduced from 76 min to 68 min (p = 0.014), and the number of patients with a door-to-puncture time of less than 60 min increased (15% vs. 36%, p = 0.002). Outcomes including modified thrombolysis in cerebral infarction ≥ 2b (73% vs. 71%, p = 1.000), parenchymal hemorrhage type 2 (7% vs. 2%, p = 0.281), and favorable outcome (33% vs. 34%, p = 1.000) were comparable between the two groups. CONCLUSION: Nurse recognition of conjugate gaze deviation contributed to an 8-minute reduction in the door-to-puncture time, demonstrating the potential benefits of an organized workflow in acute ischemic stroke.

Impact of width of susceptibility vessel sign on recanalization following endovascular therapy.

BACKGROUND AND PURPOSE: We aimed to investigate the relationship between arterial recanalization following endovascular therapy and the susceptibility vessel sign (SVS) length and width on susceptibility-weighted imaging. METHODS: We retrospectively evaluated consecutive patients with anterior circulation ischemic stroke who underwent magnetic resonance imaging preceded endovascular therapy, and measured the SVS length and width. Successful recanalization was defined as expanded thrombolysis in cerebral infarction grade of 2b to 3. Logistic regression analysis was executed to determine the independent predictors of successful recanalization and first-pass reperfusion (FPR) after endovascular therapy. RESULTS: Among 100 patients, successful recanalization and FPR were observed in 77 and 34 patients, respectively. The median SVS length and width were 10.3 mm (interquartile range, 6.8-14.1 mm) and 4.2 mm (interquartile range, 3.1-5.2 mm), respectively. In multivariate logistic regression analysis, SVS width was associated with successful recanalization (odds ratio, 1.88; 95% confidence interval, 1.14-3.07; p = 0.005) and FPR (odds ratio, 1.38; 95% confidence interval, 1.01-1.89; p = 0.039). The optimal cutoff value for the SVS width to predict successful recanalization and FPR were 4.2 mm and 4.0 mm, respectively. CONCLUSIONS: Larger SVS width may predict successful recanalization and FPR following endovascular therapy.

Perfusion abnormality in neuronal intranuclear inclusion disease with stroke-like episode: A case report.

Neuronal intranuclear inclusion disease (NIID) is a slowly progressive neurodegenerative disease. Some patients with NIID occasionally present with acute symptoms. However, its mechanism remains unclear. We report a patient with NIID who presented with a stroke-like episode. Arterial spin labeling magnetic resonance imaging revealed hypoperfusion in the focal cerebral region at the onset while no apparent arterial occlusion was observed. The abnormal perfusion area was normalized 6 days after admission. Therefore, the perfusion abnormality was likely the main cause of acute neurologic deficits in NIID. NIID should be considered in the differential diagnosis of stroke mimics.

Motor end-plate analysis to diagnose immune-mediated myasthenia gravis in seronegative patients.

This study aimed to evaluate the diagnostic usefulness of motor end-plate (MEP) analysis along with clustered acetylcholine receptor (AChR) antibody (Ab) assays in patients with myasthenia-like symptoms but negative routine AChR and muscle-specific kinase (MuSK) Ab tests. MEP analysis of muscle biopsies of the biceps brachii was performed in 20 patients to try to differentiate between those with or without immune-mediated myasthenia gravis (MG). Using a quantitative method, complement C3 deposition and AChR densities in MEPs were examined. Independently, cell-based assays were used to detect serum clustered-AChR Abs. Only five of 20 patients had complement deposition at MEPs; four of these patients had reduced AChR densities similar to those in patients with typical AChR Ab positive MG, and distinct from those in the remaining 15 patients. Two of the four serum samples from these patients had clustered-AChR Abs. All complement-positive patients were considered as having immune-mediated MG and improved with appropriate treatments; although one patient presented with MG 3 years later, the remaining patients had other diagnoses during over 10 years of follow-up. These results suggest the usefulness of MEP analysis of muscle biopsies in diagnosing immune-mediated MG in seronegative patients with myasthenia-like symptoms but, due to the invasiveness of the muscle biopsy procedure, clustered AChR Abs should, if possible, be tested first.

[Progressive multifocal leukoencephalopathy during the treatment for mycosis fungoides].

A 58-year-old man was diagnosed with mycosis fungoides (MF) confirmed by skin biopsy for systemic erythema that appeared in 2006 and had been on psoralen plus ultraviolet A (PUVA) therapy and topical steroids. In September 2017, he had diffuse large B-cell lymphoma and received chemotherapy. Since March 2019, tumor stage MF with large cell transformation was observed, and chemotherapy containing brentuximab vedotin (BV) was performed, which yielded a remarkable response. During the preparation for allogeneic hematopoietic stem cell transplantation, bradykinesia, delayed response, and cognitive decline were observed. Head magnetic resonance imaging fluid-attenuated inversion recovery images showed hyperintensity in the deep white matter below the bilateral frontal cortex. The general cerebrospinal fluid test revealed no abnormalities and was below the sensitivity of JC virus (JCV) quantitative PCR. As progressive multifocal leukoencephalopathy (PML) was strongly suspected from clinical symptoms and radiographic signs, ultrasensitive JCV testing was performed. The test result was positive; hence, the patient was diagnosed with PML. Chemotherapy was discontinued, but his central nervous system symptoms worsened, and he died on the 135th day of illness. We considered that PML developed based on the underlying disease and immunodeficiency caused by chemotherapy such as BV.

A case of Lemierre's syndrome with double vision as the first symptom.

Lemierre's syndrome is a serious disease that typically causes oropharyngeal infection with internal jugular vein thrombosis, followed by distant infection focus, such as septic pulmonary embolism. The main causative organisms are anaerobic bacteria in the oral cavity, namely Fusobacterium necrophorum. We encountered an extremely rare case of Lemierre's syndrome, where double vision was found to be the first symptom. The patient's blood culture results showed the presence of F. nucleatum, which spread from the sphenoid sinus to the skull base because of chronic sinusitis; the patient presented with longus colli abscess, clivus osteomyelitis, venous thrombosis, and hematogenous infection. Antibiotic treatment with sulbactam/ampicillin was continued for 14 weeks, and no recurrence has been observed so far. Lemierre's syndrome can be complicated with atypical symptoms such as double vision if the cranial nerves are involved. It might be important to consider this disease in the differential diagnosis in the presence of cranial nerve symptoms of unknown origin with fever or inflammatory findings.

Allogeneic hematopoietic stem cell transplantation for adult T-cell leukemia/lymphoma with HTLV-1-associated myelopathy.

Allogeneic hematopoietic stem cell transplantation (allo-HSCT) can be curative for adult T-cell leukemia/lymphoma (ATL), but comorbidities increase transplant-related mortality. Here we report the outcome of allo-HSCT in a patient with ATL with human T-cell leukemia virus type I (HTLV-1)-associated myelopathy-tropical spastic paraparesis (HAM/TSP). A 48-year-old man was diagnosed with HAM/TSP and started prednisolone therapy. Ten years later, he developed lymphoma-type ATL. At the diagnosis of ATL, Osame's Motor Disability Score (OMDS) was 4. When prednisolone was gradually tapered and stopped following chemotherapy for ATL, HAM/TSP symptoms recurred (OMDS 7). Bone marrow transplantation from a human leukocyte antigen allele 8/8 matched unrelated donor was performed while ATL was in partial remission. Neutrophil engraftment with complete donor chimerism was achieved on day 19 after allo-HSCT. Mild gait improvement (OMDS 5) was observed on day 30. Although ATL relapsed on day 275, progression of HAM/TSP symptoms was not observed. Furthermore, there was no clear progression of HAM/TSP symptoms after donor lymphocyte infusions. The outcome of this case suggests that ATL patients with HAM/TSP tolerate allo-HSCT and donor lymphocyte infusions.

Neuromyelitis Optica Spectrum Disorder Complicated by Posterior Reversible Encephalopathy Syndrome as an Initial Manifestation.

A 25-year-old woman was admitted to our hospital due to tonic convulsion with severe headache after having experienced symptoms of nausea and vomiting for a month. Brain magnetic resonance imaging showed extensive symmetrical lesions in the cortical and subcortical areas of parieto-occipital lobes and basal ganglia, consistent with typical characteristics of posterior reversible encephalopathy syndrome (PRES). Furthermore, some residual lesions in the left side of dorsal medulla oblongata and central area of the cervical spinal cord along with the presence of serum anti-aquaporin-4 antibody yielded the diagnosis of neuromyelitis optica spectrum disorder (NMOSD). We herein discuss the mechanism by which PRES may occur together with NMOSD.

Biomarkers of Cardiac Dysfunction as Risk Factors in Cryptogenic Stroke.

BACKGROUND: It is unclear whether biomarkers of cardiac dysfunction are associated with cryptogenic stroke (CS). METHODS: We retrospectively evaluated consecutive ischemic stroke patients. Patients underwent transthoracic echocardiography to evaluate left atrial diameter and the peak transmitral filling velocity/mean mitral annular velocity during early diastole (E/e'). Patent foramen ovale (PFO) and left atrial appendage flow velocity were evaluated by transesophageal echocardiography. We compared clinical characteristics and biomarkers of cardiac dysfunction (brain natriuretic peptide [BNP], left atrial diameter, E/e', and left atrial appendage flow velocity) between CS or CS without large PFO and other causative stroke subtypes. RESULTS: Among 1,514 patients with ischemic stroke, 264 patients were classified as having CS. Of these, transesophageal echocardiography revealed 27/158 (17%) large PFOs. In comparison, for the noncardioembolic stroke group, which consisted of large artery and small vessel subtypes, patients with CS without large PFO had higher log10 BNP (adjusted OR 2.70; 95% CI 1.92-3.78; p < 0.001), higher log10 E/e' (3.41; 1.21-13.15; p = 0.019), and lower left atrial appendage flow velocity (0.98; 0.97-1.00; p = 0.031). Left atrial diameter was similar for noncardioembolic stroke and CS without large PFO (p = 0.380). Cutoff values of BNP, E/e', and left atrial appendage flow velocity capable of distinguishing CS without large PFO from noncardioembolic stroke were 65.0 pg/mL (sensitivity 55.3%; specificity 70.9%), 13.0 (45.5%; 68.0%), and 46.0 cm/s (37.1%; 87.5%), respectively. CONCLUSION: Patients with CS without large PFO could have biomarkers of cardiac dysfunction.

A score using left ventricular diastolic dysfunction to predict 90-day mortality in acute ischemic stroke: The DONE score.

PURPOSE: The aim of this study was to identify whether diastolic dysfunction predicts death at 90 days after acute ischemic stroke. METHODS: We retrospectively analyzed patients with ischemic stroke. All patients underwent transthoracic echocardiography to evaluate systolic function and diastolic function by means of assessing ejection fraction and septal E/e'. We evaluated the initial National Institute of Health Stroke Scale (NIHSS) score, arterial occlusion, and laboratory data. We used multivariate regression models to identify independent predictors of 90-day mortality. RESULTS: Among 1208 patients, the overall 90-day mortality rate was 8%. In multivariate logistic regression analysis, a higher initial NIHSS score, plasma D-dimer level and E/e', and occlusion of internal carotid artery or basilar artery were independent predictors of 90-day mortality. The DONE score derived from these valuables showed good discrimination with area under the curve (AUC) value of 0.82 (95% confidence interval [CI], 0.78-0.87) to predict 90-day mortality. The DONE score also predicted poor outcome (modified Rankin scale score, 4-6) at 90 days (AUC, 0.82; 95% CI 0.80-0.85). CONCLUSIONS: Higher E/e', indicating diastolic dysfunction, may be associated with 90-day mortality in patients with acute ischemic stroke. The DONE score could readily predict poor outcome after acute ischemic stroke.

[A Case of Pol III-related Leukodystrophy with Homozygous Mutation in POLR3A].

We describe a 27-year-old man with mental retardation, symptomatic epilepsy, myopia, and cerebellar ataxia without spontaneous puberty whose brain magnetic resonance imaging showed hypomyelination. He had child-like facial appearance, with thin facial hair. He had no underarm and pubic hairs, and his penis was small. Laboratory tests showed low levels of luteinizing hormone, follicle-stimulating hormone, and testosterone. Brain MRI showed diffuse hypomyelination, atrophy of the cerebellum and brainstem, and hypoplastic corpus callosum. Ictal N-isopropyl-p-(indone-123)-iodoamphetamine single photon emission computed tomography (123I-IMP SPECT) revealed hypoperfusion of bilateral frontal cingulate and temporal lobe and cerebellar hemispheres. Homozygous missense mutation c.2350G>A was found in POLR3A and the patient was diagnosed with Pol III-related leukodystrophy, which is a rare disease. We describe the present case in light of the characteristics of the past reports in Japan. (Received April 5, 2016: Accepted June 30, 2016; Published November 1, 2016).

A case of recurrent optic neuritis associated with cerebral and spinal cord lesions and autoantibodies against myelin oligodendrocyte glycoprotein relapsed after fingolimod therapy.

A previously healthy 16-year-old girl developed sudden eye pain and visual loss in her right eye. On day 7 from onset her right visual acuity had decreased to light perception, and she underwent 5 courses of intravenous methylprednisolone therapy (IVMP, 1 g/day for 3 consecutive days per week). Her eye pain and her visual acuity had improved immediately. Eleven months later, follow-up MRI revealed three T2-hyperintense plaques involving subcortical white matter in the left occipital lobe, right frontal lobe, right thalamus, and thoracic spinal cord. We suspected the diagnosis as multiple sclerosis and treated with fingolimod. She developed recurrent optic neuritis (ON) on day 19 from fingolimod therapy, and we stopped fingolimod. For two years from onset she was admitted five times due to recurrences of ON and appearance of white matter lesion and myelitis. At 22 months, anti-myelin oligodendrocyte glycoprotein (MOG) antibodies revealed to be positive in her sera from the onset to the present. Our case report suggests that fingolimod might not be effective in anti-MOG antibody-related disorders together with anti-aquaporin-4 (AQP4) antibody-positive group.

Antibodies to myelin oligodendrocyte glycoprotein in idiopathic optic neuritis.

OBJECTIVES: To investigate the differences of clinical features, cerebrospinal fluid (CSF), MRI findings and response to steroid therapies between patients with optic neuritis (ON) who have myelin oligodendrocyte glycoprotein (MOG) antibodies and those who have seronegative ON. SETTING: We recruited participants in the department of neurology and ophthalmology in our hospital in Japan. METHODS: We retrospectively evaluated the clinical features and response to steroid therapies of patients with ON. Sera from patients were tested for antibodies to MOG and aquaporin-4 (AQP4) with a cell-based assay. PARTICIPANTS: Between April 2009 and March 2014, we enrolled serial 57 patients with ON (27 males, 30 females; age range 16-84 years) who ophthalmologists had diagnosed as having or suspected to have ON with acute visual impairment and declined critical flicker frequency, abnormal findings of brain MRI, optical coherence tomography and fluorescein fundus angiography at their onset or recurrence. We excluded those patients who fulfilled the diagnostic criteria of neuromyelitis optica (NMO)/NMO spectrum disorders (NMOSD), MS McDonald's criteria, and so on. Finally we defined 29 patients with idiopathic ON (14 males, 15 females, age range 16-84 years). RESULTS: 27.6% (8/29) were positive for MOG antibodies and 3.4% (1/29) were positive for AQP4. Among the eight patients with MOG antibodies, five had optic pain (p=0.001) and three had prodromal infection (p=0.179). Three of the eight MOG-positive patients showed significantly high CSF levels of myelin basic protein (p=0.021) and none were positive for oligoclonal band in CSF. On MRIs, seven MOG-positive patients showed high signal intensity on optic nerve, three had a cerebral lesion and one had a spinal cord lesion. Seven of the eight MOG-positive patients had a good response to steroid therapy. CONCLUSIONS: Although not proving primary pathogenicity of anti-MOG antibodies, the present results indicate that the measurement of MOG antibodies is useful in diagnosing and treating ON.

[Increased rate of serum uric acid levels in Yusho sufferers].

We measured serum uric acid levels in Yusho sufferers annually from 2007 to 2012 in Nagasaki prefecture. We observed an increased rate of serum uric acid levels in 38.2% of the male and 5.5% of the female sufferers. There was no relation among serum uric acid value, Body Mass Index, liver function, blood polychlorinated biphenyls and hypersensitive C reactive protein. We conclude that it is unclear if blood polychlorinated biphenyls may play a role in the increase of serum uric acid levels in Yusho sufferers.