Spinal intradural pseudocyst formation in central nervous system superficial siderosis.

The figure shows tissue samples taken from three previous cases, revealing the cause of hemosiderin deposition in the central nervous system because of superficial siderosis.

Progressive conus medullaris lesions are suggestive of intravascular large B-cell lymphoma.

BACKGROUND AND PURPOSE: Spinal cord lesions are observed in 40% of all central nervous system lesions in intravascular large B-cell lymphoma (IVLBCL). However, because IVLBCL is a very rare disease, its clinical features are not well defined, which may delay appropriate diagnosis and treatment, whilst the acute to subacute course of brain lesions in patients with IVLBCL is well established. Therefore, this study aimed to clarify the clinical features of spinal cord lesions in patients with IVLBCL. METHODS: The medical records of patients with IVLBCL admitted to our hospital between 2010 and 2020 were searched. The inclusion criteria were preceding neurological symptoms without non-neurological symptoms and pathologically confirmed IVLBCL in various organs. Clinical features of spinal cord involvement in patients with IVLBCL were assessed and distinguished from those of brain involvement. RESULTS: Sixteen consecutive patients with IVLBCL were divided into two groups: six patients with spinal involvement (spinal cord type) and 10 patients with brain involvement (brain type). In the spinal cord type, four patients had chronic progression and two had subacute progression. Acute progression (0% vs. 80.0%) and sudden onset (0% vs. 50.0%) occurred significantly less frequently in the spinal cord than in the brain. All spinal cord lesions involved the conus medullaris. CONCLUSIONS: Spinal cord involvement in IVLBCL has a predominantly chronic progressive course that is exclusive to brain involvement. Conus medullaris lesions are suggestive of IVLBCL and are useful for early and accurate diagnosis and treatment.

[A case of neuralgic amyotrophy with extension disturbance of fingers after Cushing's syndrome remission].

We describe a 57-year-old female patient who experienced hypercortisolemia caused by adrenal Cushing's syndrome. Two months post-adrenalectomy, she developed acute severe bilateral pain starting in her fingers and spreading up her arms. In the subsequent two weeks, the patient presented upper extremity patchy paralysis with extension disturbance of fingers. In the following two months, she experienced atrophy of the muscles in the hands and joint contracture. Consequently, we diagnosed her with neuralgic amyotrophy. Nerve conduction studies showed low compound muscle action potential of all the peripheral nerves in the forearms, suggesting motor neuron axonopathy. Gadolinium-enhanced MRI and ultrasound studies did not reveal any abnormalities in the brachial plexus and peripheral nerves of the forearms. The patient tested positive for anti-GalNAc-GD1a-IgM antibodies and received intravenous immunoglobulin 6 months after the onset of symptoms, which resulted in reduction of pain, muscle weakness, and contractures. This rare case of potentially immune-mediated bilateral patchy paralysis may have important implications in the understanding of clinical and pathological heterogenicity of neuralgic amyotrophy.

A Case of Perilymphatic Fistula with Inner Ear Anomaly Diagnosed Preoperatively by the Cochlin-Tomoprotein Detection Test.

We present a case of perilymphatic fistula (PLF) with inner ear anomalies having sudden, progressive sensorineural hearing loss and describe the fistula repair surgeries. We focus on the diagnosis methods of PLF and clinical course of PLF with inner ear anomaly. The cochlin-tomoprotein (CTP) detection test is very useful for the surgeons to encourage the earlier operation to sudden hearing loss cases. It is also helpful to define the diagnosis of PLF after operation. We could not get the good result as to hearing from the fistula repair surgery mainly because surgery was held 1 month after the onset. The results of the case, as well as recommendations of other reports, suggest that patients with sudden sensorineural hearing loss and PLF may need repair surgery within at most 2 weeks from the onset. We describe how to diagnose PLF more accurately using CTP detection combined with intraoperative findings.

[Case report of acute encephalopathy caused by enterohemorrhagic Escherichia coli infection in a 24-year-old woman].

Hemolytic uremic syndrome (HUS) and acute encephalopathy caused by enterohemorrhagic Escherichia coli infection occur commonly in children, whereas adult-onset disease is rare. Here we report the case of a 24-year-old woman who developed acute encephalopathy and recovered without sequelae. She initially developed abdominal pain and diarrhea. On day 6, O-157 Shiga toxin was detected in her stool and she developed HUS. On day 11, acute encephalopathy developed and she required artificial ventilation. She was treated with steroid pulse therapy and plasma exchange (PE) and then discharged on day 53 without any sequelae. Globotriaosylceramide, a Shiga toxin receptor, is more frequently present on the cellular membranes of women than on those of men. Therefore, it is conceivable that adult women are at a higher risk of developing acute encephalopathy than men. Steroid pulse therapy and PE may effectively treat acute encephalopathy by reducing inflammatory cytokine levels in the blood; therefore, these treatments should be proactively considered.

Magnetic resonance imaging of bone marrow for TAFRO syndrome.

We report two cases of TAFRO syndrome, which is characterized by thrombocytopenia, anasarca, fever, renal insufficiency, and organomegaly. Magnetic resonance imaging (MRI) of the spine showed a dark medullary pattern in the bone marrow on the T1- and T2-weighted images of both patients. One patient showed complete resolution after treatment. Serial MRIs of the improved patient revealed a transition to a normal marrow pattern on both images, which might represent resolution of the disease.

Evaluation of tinnitus retraining therapy for patients with normal audiograms versus patients with hearing loss.

OBJECTIVE: A few chronic tinnitus patients show normal hearing thresholds in the pure tone audiometry from 125Hz to 8000Hz (≤20dB). We report the characteristics of the course of those patients underwent tinnitus retraining therapy (TRT) compared with other patients suffering from chronic and severe tinnitus. METHODS: We identified 13 patients with normal hearing thresholds among 242 patients suffering over 3 months, Tinnitus Handicap Inventory (THI) ≥16/100, and follow up period is over 6 months. We divided into two groups - tinnitus with normal audiometry and with hearing loss - and contrasted these patients with age, gender, tinnitus duration, instruments for TRT, loudness and pitch of the tinnitus, THI and Visual Analogue Scale (VAS) scores. RESULTS: The pitch-match of the tinnitus was higher and tinnitus duration was shorter in normal audiometry. The age is younger and the tinnitus loudness was smaller in normal hearing group significantly. THI of normal audiogram group showed significant improvement on 18 months treatment, though it once got worse on 12 months. THI of hearing loss group showed significant decreases in first 3 months and decreased slightly until 48 months treatment. The VAS scores of annoyance also showed a large decrease in first 3 months and decreased slightly until 24 months. Both THI after 48 months and VAS scores after 24 months treatment showed almost stable until 72 months in hearing loss group. CONCLUSION: Chronic tinnitus with normal audiometry and with hearing loss both showed adaptation with TRT. Normal audiometry group with chronic tinnitus may have damage in high frequency though there were not significant differences between two groups as to tinnitus pitch-match. They also need at least 18 months TRT to become adaptation, while 48 months treatment is enough and first 3 months treatment is very important for hearing loss with chronic tinnitus.

[Case report of effective intravenous immunoglobulin for lower limbs pain in steroid-resistant eosinophilic granulomatosis with polyangiitis].

We report a 58-year-old woman with bronchial asthma. The onset of the disease was marked by numbness in the right lower extremity, for which she was hospitalized 10 days later. The patient presented with sensory impairment and muscle weakness in the distal regions of both lower limbs, acute pain, purpura, and a leukocyte count of 2.4 × 10(4)/µl (59.2% eosinophils). Nerve conduction tests revealed a decrease in the amplitude of the compound muscle action potential in all 4 extremities. Skin biopsy results led to the diagnosis of eosinophilic granulomatosis with polyangiitis (EGPA). Steroid pulse therapy and oral steroid therapy were initiated but did not resolve the acute pain or numbness. However, intravenous immunoglobulin (IVIg) was administered at day 28 after the beginning of the steroid treatment, and the pain started to improve immediately afterward. In some cases, IVIg can be effective in the treatment of intense pain in peripheral neuropathy associated with steroid-resistant EGPA.

Impact of continuous deterioration of kidney function 6 to 8 months after percutaneous coronary intervention for acute coronary syndrome.

Preprocedural chronic kidney disease and contrast-induced acute kidney injury are predictors of in-hospital death and long-term mortality. However, neither the time course of kidney function after percutaneous coronary intervention (PCI) nor the relation between the time course of kidney function and prognosis has been adequately studied. We studied 531 patients who underwent PCI for acute coronary syndrome. The continuous deterioration of kidney function (CDKF) was defined as a >25% increase in serum creatinine level or serum creatinine >0.5 mg/dl above baseline at 6 to 8 months after PCI. CDKF was observed in 87 patients (16.4%). Independent risk factors for CDKF were contrast-induced acute kidney injury, preprocedural hemoglobin level, and proteinuria. Patients with CDKF exhibited significant higher 5-year mortality rate than patients without CDKF (25% vs 9.4%, log-rank p = 0.0006). Independent risk factors for 5-year mortality were age >75 year, anemia, New York Heart Association class III or IV, low ejection fraction, and CDKF. CDKF is associated with an increased risk of all-cause mortality of 5 years in patients with acute coronary syndrome undergoing PCI.

The importance of medical interview with CKD patient in diagnoses of a family with Fabry disease.

A 47-year-old Japanese man was admitted to our hospital for evaluation of proteinuria, which was detected when he was 37 years of age. His creatinine clearance levels had fallen to 76.3 mL/min/1.73 m2. A kidney biopsy was conducted, and the patient's low plasma α-galactosidase A levels suggested Fabry disease. After genetic counseling, GLA analysis revealed a novel mutation p.L387P. Interview with the patient revealed that both his younger brother and mother suffered from cardiomyopathy and were undergoing cardiological treatment. They also were positive for proteinuria. About 30 years ago, the patient's cousin (aged 25) was diagnosed with Fabry disease. He underwent hemodialysis for 9 years until his death at 42. At that time, the patient and his brother had not been investigated for Fabry disease so their cousin could not act as a proband for the brothers. Eventually, the patient, his mother, and his brother were put on enzyme replacement therapy with agalsidase beta. As this series of cases shows, medical interviews to collate both medical and family history were essential for the discovery of Fabry disease in these patients. In addition, being a treatable genetic disorder, Fabry disease should be listed in the standard differential diagnoses of systemic and familial diseases, including unknown cause of nephropathy or cardiomyopathy, for early detection of the disorder.

Dramatic response to low-dose rivaroxaban in an Asian patient with deep vein thrombosis and pulmonary embolism.

We report a case of deep venous thrombosis and pulmonary embolism treated with rivaroxaban due to warfarin allergy. The patient responded well to a low dose of 15 mg/day. There has been a report about treating patients with atrial fibrillation using a low dose of rivaroxaban in Japan, but no previous reports about deep vein thrombosis/pulmonary embolism. This case suggests that rivaroxaban could be an alternative to warfarin for the treatment of deep vein thrombosis and pulmonary embolism in Japanese patients with warfarin allergy. .

Relation of coronary plaque composition determined by 64-slice multidetector computed tomography in patients with suspected coronary heart disease.

Sixty-four-slice multidetector row computed tomography is a noninvasive method of assessing coronary artery stenosis and plaque composition. The aim of this study was to clarify the relation between plaque composition and coronary heart disease. Three hundred sixty consecutive patients and 1,085 plaques were evaluated using 64-slice multidetector row computed tomography. On axial or cross-sectional multiplanar reconstruction images, 3 regions of interest were randomly selected within each plaque. Soft plaques and calcified plaques were defined as having computed tomographic densities <50 and >130 Hounsfield units, respectively. The association between coronary risk factors and plaque composition was analyzed. The number of plaques and the mean computed tomographic density of plaques were significantly higher in men than in women (p = 0.002 and p = 0.04, respectively). Coronary plaques were more frequent in patients with stroke, diabetes, hypertension, and dyslipidemia than in patients without these conditions (all p values <0.001). Calcified plaques were more frequent in patients with hypertension (p = 0.02), and patients with calcified plaques also had significantly lower low-density lipoprotein cholesterol levels (p <0.001). Soft plaques were more frequent in patients with dyslipidemia (p <0.001). Patients with soft plaques had significantly higher low-density lipoprotein cholesterol levels (p = 0.02) and lower high-density lipoprotein cholesterol levels (p <0.001) than those without soft plaques. In conclusion, 64-slice multidetector row computed tomography is a useful noninvasive method for quantifying coronary plaques.

Heart rate recovery after exercise is a predictor of silent myocardial ischemia in patients with type 2 diabetes.

OBJECTIVE: Slow heart rate recovery (HRR) predicts all-cause mortality. This study investigated the relationship between silent myocardial ischemia (SMI) and HRR in type 2 diabetes. RESEARCH DESIGN AND METHODS: The study enrolled 87 consecutive patients with type 2 diabetes and no chest symptoms. They underwent treadmill exercise testing and single-photon emission computed tomography imaging with thallium scintigraphy. Patients with abnormal myocardial perfusion images also underwent coronary angiography. RESULTS: SMI was diagnosed in 41 patients (47%). The SMI group showed slower HRR than the non-SMI group (18 ± 6 vs. 30 ± 12 bpm; P < 0.0001). HRR was significantly associated with SMI (odds ratio 0.83 [95% CI 0.75-0.92]; P = 0.0006), even after adjustment for maximal exercise workload, resting heart rate, maximum heart rate, rate pressure product, HbA(1c), use of sulfonamides, and a history of cardiovascular disease. CONCLUSIONS: HRR can predict SMI in patients with type 2 diabetes.

Higher body mass index at the time of acute myocardial infarction is associated with a favorable long-term prognosis (8-year follow-up).

Obesity is an important public health problem, especially among patients with cardiovascular disease. However, little is known about the impact of obesity on the long-term prognoses of patients with acute myocardial infarction (AMI). Major adverse cardiac and cerebrovascular events (MACCE) consist of all causes of death, stroke, target lesion revascularization, target vessel revascularization, non-fatal myocardial infarction, and hospitalization. From January 2001 to March 2005, we analyzed 121 patients who survived >30 days after suffering their first AMI of the left anterior descending artery for MACCE. The mean follow-up period for this study was 59 ± 26 months. Seventy-five patients presented with normal weight (BMI <25 kg/m(2)) and 46 were obese (BMI >25 kg/m(2)). During the follow-up period, 56 patients presented MACCE, including 18 deaths, 11 strokes, and seven non-fatal myocardial infarctions. Normal weight was significantly associated with the occurrence of MACCE (p = 0.012). Grouping of the patients by BMI and homeostasis model assessment ratio (HOMA-R) indicated that the combination of a higher BMI (>25) and lower insulin resistance (HOMA-R < 2.0) provided the best prognosis (p = 0.0006). Kaplan-Meier curves stratified to the four groups, sorted by diabetes mellitus and BMI at admission, showed that the normal weight patients with diabetes mellitus presented the highest risk of MACCE (p < 0.0001). Patients with higher BMI and no insulin resistance or diabetes mellitus present better long-term outcomes following anterior AMI.