Inflammatory progressive multifocal leukoencephalopathy with human T-cell lymphotropic virus-1 coinfection.

A middle-aged man with progressive multifocal leukoencephalopathy (PML) in a human T-cell lymphotropic virus type-1 (HTLV-1) carrier on haemodialysis presented with mild dysarthria and ataxia. Brain MRI revealed asymmetric T2-hyperintense lesions in the cerebral white matter, cerebellum and brainstem. A small amount of JC virus (JCV) genome in cerebrospinal fluid was detected by PCR and cerebellar biopsy demonstrated JCV-DNA presence. Pathological findings showed demyelinating lesions and glial cells with mildly enlarged nuclei, accompanied by T-lymphocytes, neutrophils and plasma cell infiltration. The CD4+/CD8+ratio was 0.83. High-dose corticosteroid therapy was effective for inflammatory PML lesions, and the administration of mefloquine combined with mirtazapine led to favourable outcome. The encephalitis in this case is considered to have occurred secondarily to JCV infection in the presence of HTLV-1 infection. Therefore, it is crucial to investigate the presence of HTLV-1 in order to understand the aetiology of this brain inflammation.

Low left atrial volume is an independent predictor of persistent hypotension after carotid artery stenting.

BACKGROUND: Persistent hypotension (PH) after carotid artery stenting (CAS) is a relatively common complication; however, it is unclear which patients are more likely to experience this phenomenon. Recently, lower left atrial (LA) volume was associated with vasovagal syncope, which has a similar neurological mechanism to hypotension after CAS. This study aimed to investigate whether LA volume can predict PH after CAS. METHODS: This single center retrospective analysis used data from 316 patients who had undergone CAS between March 2013 and February 2021. After the exclusion of urgent CAS, 212 procedures (202 patients) with transthoracic echocardiograms were included. The procedures were divided among two groups according to the presence or absence of PH for more than 1 hour after CAS. RESULTS: The mean age of the patients was 73.0±7.5 years. PH was observed during 52 (24.5%) procedures. The PH group exhibited a lower LA volume index (LAVI) than the no-PH group (29.7±9.1 vs 37.7±12.5 mL/m2, respectively; p<0.001). The area under the receiver operating characteristic curve was 0.716. The optimal cut-off value was 33.5 mL/m2 (sensitivity 0.750, specificity 0.625). Multiple logistic regression analysis showed that LAVI <33.5 mL/m2 was an independent predictor for PH after CAS (OR 4.950, 95% CI 2.190 to 11.200; p<0.001). Preoperative hydration was negatively associated with PH (OR 0.235, 95% CI, 0.070 to 0.794; p=0.020). CONCLUSIONS: A lower LA volume can predict PH after CAS, and preoperative hydration may prevent PH after CAS.

Clinical Impact of Large Vessel Occlusion Achieved First Pass Effect with Stent Retriever Alone: A Single-Center Retrospective Analysis.

Objective: The first pass effect (FPE), which means the achievement of complete or near-complete reperfusion of large vessel occlusion (LVO) in the first pass, is one of the goals of mechanical thrombectomy (MT). However, the impact of FPE on the prognosis has not been assessed for Japanese patients with various degrees of independence before the onset of LVO. The purpose of this study was to investigate the prognostic effects of FPE in a comprehensive stroke center in Japan, which includes patients in a variety of self-independence states with different comorbidities before stroke onset. Methods: Between April 2017 and March 2020, 151 patients who underwent MT with a stent retriever (SR) alone as initial strategy for anterior circulation (internal carotid artery terminal, M1, M2) LVO at our hospital and finally achieved modified treatment in cerebral infarction (mTICI) 2b-3 were analyzed. Forty-eight patients in whom first pass mTICI 2c-3 was achieved were classified into the FPE+ group, and the other 103 patients were classified into the FPE- group. We compared the characteristics and clinical outcomes between patients with and without FPE, and estimated the odds ratio for outcomes after adjusting for confounders. Results: The puncture-reperfusion time was shorter (20 vs. 35 minutes; p <0.01), and cardiogenic embolism was more common (81.3 vs. 60.2%; p = 0.01) in the FPE+ group. The FPE was significantly associated with good neurological outcome after 3 months (p <0.01; adjusted odds ratio [aOR], 3.87; 95% confidence interval [CI], 1.69-9.38), reduction in all intracranial hemorrhage (p <0.01; aOR, 0.24; 95% CI, 0.10-0.54), and symptomatic intracranial hemorrhage (p = 0.04; aOR, 0.16; 95% CI, 0.01-0.98). Conclusion: The FPE with an SR alone improved the neurological prognosis in a Japanese patient group.

Neurological Manifestations and Long-term Sequelae in Hospitalized Patients with COVID-19.

Objective Various neurological manifestations have been increasingly reported in coronavirus disease 2019 (COVID-19). We determined the neurological features and long-term sequelae in hospitalized COVID-19 patients. Methods We retrospectively studied 95 consecutive hospitalized patients with COVID-19 between March 1 and May 13, 2020. Acute neurological presentations (within two weeks of the symptom onset of COVID-19) were compared between 60 non-severe and 35 severely infected patients who required high-flow oxygen. In the 12 ventilated patients (the most severe group), we evaluated neurological complications during admission, subacute neurological presentations, and neurological sequelae (51 and 137 days from the onset [median], respectively). Results Of the 95 patients (mean age 53 years old; 40% women), 63% had acute neurological presentations, with an increased prevalence in cases of severe infections (83% vs. 52%, p<0.001). Impaired consciousness and limb weakness were more frequent in severe patients than in non-severe ones (0% vs. 49%; p<0.001, and 0% vs. 54%; p<0.001, respectively). In the most severe group (mean age 72 years old; 42% women), 83% of patients had neurological complications [cerebrovascular disease (17%), encephalopathy (82%), and neuropathy (55%)], and 92% had subacute neurological presentations [impaired consciousness (17%), higher brain dysfunction (82%), limb weakness (75%), and tremor (58%)]. Neurological sequelae were found in 83% of cases, including higher brain dysfunction (73%), limb weakness (50%), and tremor (58%). Conclusions Neurological manifestations are common in COVID-19, with the possibility of long-lasting sequelae.

Tumor Embolism Through Right-to-Left Shunt Due to Venous Invasion of Esophageal Carcinoma.

A 69-year-old man was admitted to the hospital with right hemiparesis and global aphasia. Perfusion computed tomography imaging revealed ischemic penumbra in the middle cerebral artery territory. Angiography showed left middle cerebral artery occlusion. Mechanical thrombectomy with one pass was performed, and successful recanalization was obtained. Embolic material was retrieved; it contained tumor fragments with atypical keratinizing squamous cell carcinoma. Contrast computed tomography imaging indicated tumor invasion into the superior vena cava, and contrast transcranial Doppler indicated the presence of a right-to-left shunt after the Valsalva maneuver. We diagnosed the patient with acute ischemic stroke of large vessel occlusion due to venous invasion of esophageal carcinoma via a right-to-left shunt. To the best of our knowledge, this is the first case of embolic occlusion resulting from an extracardiac tumor via a right-to-left shunt. Contrast transcranial Doppler potentially detects right-to-left shunts in patients who cannot undergo transesophageal echocardiography.

Aseptic Meningitis as an Initial Manifestation of Anti-myelin Oligodendrocyte Glycoprotein Antibody-associated Disease.

Antibody against myelin oligodendrocyte glycoprotein (MOG-IgG) associated encephalitis is an important syndrome associated with MOG-IgG. However, there have been no reports of MOG-IgG-associated optic neuritis or demyelination following meningitis without encephalitic symptoms. A 55-year-old woman presented to our hospital with headache, nausea, fever, and nuchal rigidity that had persisted for more than a month. She was hospitalized due to aseptic meningitis and recovered with conservative therapy. However, she was re-admitted due to left optic neuritis and demyelinating lesions. We diagnosed MOG-IgG-associated neuromyelitis optica spectrum disorder (NMOSD). She responded to treatment with intravenous methylprednisolone and oral prednisolone. Aseptic meningitis may be an initial manifestation of MOG-IgG-positive NMOSD.

Erythrocyte-Rich Thrombus Is Associated with Reduced Number of Maneuvers and Procedure Time in Patients with Acute Ischemic Stroke Undergoing Mechanical Thrombectomy.

BACKGROUND: Only few studies have investigated the relationship between the histopathology of retrieved thrombi and clinical outcomes. This study aimed to evaluate thrombus composition and its association with clinical, laboratory, and neurointerventional findings in patients treated by mechanical thrombectomy due to acute large vessel occlusion. METHODS: At our institution, 79 patients were treated by mechanical thrombectomy using a stent retriever and/or aspiration catheter between August 2015 and August 2016. The retrieved thrombi were quantitatively analyzed to quantify red blood cells, white blood cells, and fibrin by area. We divided the patients into two groups - a fibrin-rich group and an erythrocyte-rich group - based on the predominant composition in the thrombus. The groups were compared for imaging, clinical, and neurointerventional data. RESULTS: The retrieved thrombi from 43 patients with acute stroke from internal carotid artery, middle cerebral artery, or basilar artery occlusion were histologically analyzed. Erythrocyte-rich thrombi were present in 18 cases, while fibrin-rich thrombi were present in 25 cases. A cardioembolic etiology was significantly more prevalent among the patients with fibrin-rich thrombi than among those with erythrocyte-rich thrombi. Attenuation of thrombus density as shown on computed tomography images was greater in patients with erythrocyte-rich thrombi than in those with fibrin-rich thrombi. All other clinical and laboratory characteristics remained the same. Patients with erythrocyte-rich thrombi had a smaller number of recanalization maneuvers, shorter procedure times, a shorter time interval between arrival and recanalization, and a higher percentage of stent retrievers in the final recanalization procedure. The occluded vessels did not differ significantly. CONCLUSIONS: In this study, erythrocyte-rich thrombus was associated with noncardioembolic etiology, higher thrombus density, and reduced procedure time.

Multiple Cerebral Infarctions Due to Patent Foramen Ovale in a Patient with Eosinophilic Granulomatosis with Polyangiitis.

A 51-year-old man was diagnosed with eosinophilic granulomatosis with polyangiitis 6 years ago due to asthma, sinusitis, hypereosinophilia, and peripheral neuropathy based on the diagnostic criteria of American College of Rheumatology, and corticosteroid therapy achieved a remission. One year ago, he was hospitalized due to deep venous thrombosis (DVT) and pulmonary embolism, and rivaroxaban was administrated. He was admitted to our hospital for acute onset of diplopia and right hemiparesis. Peripheral blood examinations disclosed leukocytosis with hypereosinophilia. Perinuclear anti-neutrophil cytoplasmic antibodies were positive. Diffusion-weighted imaging showed multiple fresh ischemic lesions. Chronic ischemic lesions were seen in subcortical cerebral region. No stenosis or occlusion was shown in extracranial and intracranial arteries on magnetic resonance angiography. Ultrasonography of leg vein showed DVT. Right-to-left shunt through patent foramen ovale after Valsalva maneuver was seen on transesophageal echocardiography. Treatment with corticosteroid and cyclophosphamide alleviated clinical deterioration. Rivaroxaban was changed to warfarin. Diplopia and muscle strength of right limbs were improved. This is a first case of multiple cerebral infarction caused by paradoxical embolism due to patent foramen ovale with DVT based on hypercoagulable state of hypereosinophilia. Overall this case illustrates that eosinophilic granulomatosis with polyangiitis can be a risk factor for multiple cerebral infarction in the systemic phase and that transesophageal echocardiography and ultrasonography of leg vein should be conducted in stroke patient with eosinophilic granulomatosis with polyangiitis.

Cholesterol Crystals in Embolic Debris are Associated with Postoperative Cerebral Embolism after Carotid Artery Stenting.

OBJECT: We investigated possible associations among the presence of cholesterol crystals in embolic debris, the proportions of debris components, and postoperative cerebral embolism in patients undergoing carotid artery stenting (CAS). METHODS: Sixty-seven consecutive procedures were performed for internal carotid artery stenosis with CAS at our hospital between November 2015 and February 2018. Procedures for emergency CAS for stroke in evolution or crescendo transient ischemic attack were excluded (n = 12). The embolic debris from remaining procedures (n = 55) was stained with hematoxylin-eosin and the red blood cells, white blood cells, and fibrin were quantified by color-based segmentation. Cholesterol crystals and calcification were examined histopathologically. Diffusion-weighted imaging (DWI) was performed 1-3 days after CAS, and the images were used to classify procedures according to the presence of new lesions. RESULTS: Of the 55 CAS procedures, new DWI lesions were identified after 32. One patient had symptomatic cerebral embolism. Higher proportions of patients with cholesterol crystals in embolic debris (17 vs. 78%, p < 0.001) and higher proportion of white blood cells (mean 2.3 [0-9.9] vs. 4.2% [0-29.9%], p < 0.01) were observed in the embolic debris of procedures with and without new DWI lesions. CONCLUSIONS: Cholesterol crystals were common in the embolic debris from patients with postoperative ischemic lesions after CAS. These results suggest that inflammatory destabilization of the intraplaque lipid component is related to postprocedural DWI lesions.

The Safety of Carotid Artery Stenting for Patients in the Acute Poststroke Phase.

BACKGROUND: The efficacy of carotid artery stenting (CAS) for patients in the acute poststroke phase has not been established. We investigated the outcome of CAS for patients with symptomatic internal carotid artery (ICA) stenosis in the acute poststroke phase. METHODS: We performed a retrospective analysis of all patients who underwent CAS for symptomatic ICA stenosis in our institution. Patients in whom the time interval between neurological deterioration and the CAS procedure was less than 3 days were included in the early group, and the other patients were included in the delayed group. Perioperative complications including major adverse events (MAEs) were compared between the early and the delayed groups. RESULTS: One hundred five patients were included in the study. Forty patients were assigned to the early group and 65 patients were assigned to the delayed group. The overall MAE rate was 4.8%. There was no significant increase in the perioperative MAE in the early group compared with the delayed group (early group 2.5% versus delayed group 6.5%, P = .65). In the early group, 25 of 40 patients (62.5%) were functionally independent (modified Rankin scale [mRS] score of 0-2) at discharge. Significant differences between the independent patients and the disabled patients (mRS score of 3-6) included age (independent 72 versus disabled 79, P < .01) and prevalence of transient ischemic attack (36.0% versus .0%, P = .02). CONCLUSIONS: CAS performed within 3 days from the last ischemic event did not increase the risk of perioperative complication. Early CAS may be a useful option for the treatment of symptomatic carotid artery stenosis.

[ACUTE MYELOID LEUKEMIA COMPLICATED BY DISSEMINATED TUBERCULOSIS AT DIAGNOSIS].

A 58-year-old man was admitted to our hospital because of fever and night sweating. Laboratory examinations showed pancytopenia on admission. Examination of bone marrow smear specimens revealed many myeloblasts, thus the diagnosis of acute myeloid leukemia (AML) was made. In contrast, many central necrotic epithelioid granulomas were found in clot specimens prepared from the same bone marrow sample. Computed tomography showed small lymphadenopathies and hepatosplenomegaly. Mycobacterium tuberculosis was isolated only from the urine culture. These findings of the bone marrow and the urine culture led to the diagnosis of disseminated tuberculosis. Therefore, these results mentioned above led to the diagnosis of AML complicated with disseminated tuberculosis. After disseminated tuberculosis treatment with anti-tuberculosis drugs, induction chemotherapy for AML helped the patient to achieve complete remission (CR). During treatment and CR, he showed a paradoxical reaction with lymph node enlargement without worsening of disseminated tuberculosis. This is a rare case of AML complicated by disseminated tuberculosis.

X-linked agammaglobulinemia in a 10-year-old boy with a novel non-invariant splice-site mutation in Btk gene.

X-linked agammaglobulinemia (XLA) is a primary immunodeficiency disease caused by mutations in the gene coding for Bruton's tyrosine kinase (Btk). Most XLA patients have severely reduced or absent peripheral blood B cells and serum immunoglobulins, since the expression or function of Btk, critical for the maturation of B cell lineages at pro-B and pre-B cell stages, is deficient. Early and accurate diagnosis of XLA is important, since the affected patients suffer from severe and recurrent infections unless they receive intravenous immunoglobulin (IVIG) replacement therapy. However, the diagnosis of XLA is not always easy because some patients have detectable ( approximately 2%) B cells in the peripheral blood and have significant levels of serum immunoglobulins. In this study, we report on a patient who was diagnosed with XLA at the age of 10years. The diagnosis was delayed due to near-normal levels of serum immunoglobulins, although he presented with severe and recurrent bacterial infections since the age of 1year. He was demonstrated to have a novel non-invariant splice-site mutation in intron 10 (IVS10 -11C-->A) of the Btk gene, which was not detected by the standard PCR-based mutation analysis. This mutation resulted in no detectable Btk expression. This case suggests that patients suffering from severe or recurrent bacterial infection should be suspected to have XLA even though they may have significant levels of serum immunoglobulins. Furthermore, significant levels of serum immunoglobulins in XLA patients do not necessarily mean less severe phenotype.