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.

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.

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.

[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.

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.

[Patient with encephalitis presenting with olanzapine-responsive malignant catatonia].

We report the case of a 29-year-old man, who could not remember some words of Kanji and showed emotional instability. Magnetic resonance imaging (MRI) scan of his brain appeared normal. Cerebrospinal fluid (CSF) analysis showed lymphocytic pleocytosis. An electroencephalogram (EEG) showed slow activities in both frontal regions of the brain. He was diagnosed as acute encephalitis. On his fourth hospital day, he was found to be catatonic and showed mutism, akinetism, and catalepsy. On the ninth day, he showed hyperpyrexia, muscle rigidity, difficulty in swallowing, respiratory insufficiency, and rhabdomyolysis (creatine phosphokinase (CK), 3038 IU/l). He was diagnosed as malignant catatonia. Intravenous administration of acyclovir, high-dose methylprednisolone, antibiotics, diazepam, and dantrolene sodium was not effective. After initiating oral administration of olanzapine, his condition improved.

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.

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.

Thymidine phosphorylase mRNA level predicts survival of patients with advanced oropharyngeal cancer.

CONCLUSION: The present study suggested that a high level of thymidine phosphorylase (TP) gene expression is significantly associated with favorable prognosis of patients treated with 5-fluorouracil (5-FU)-based chemotherapy. Further studies consisting of large series should be performed to confirm our present results. BACKGROUND: Expression levels of the thymidylate synthase (TS) and 5-FU metabolic enzymes including dihydropyrimidine dehydrogenase (DPD), TP, and orotate phosphoribosyl transferase (OPRT), are reported to be associated with sensitivity to 5-FU-based chemotherapy in several cancers. PATIENTS AND METHODS: Intratumoral mRNA expression levels of TS, DPD, TP, and OPRT in pretreatment biopsy specimens were quantified in 27 patients with advanced oropharyngeal squamous cell carcinomas. Association of these expression levels with response to platinum and 5-FU-based chemotherapy and survival were analyzed statistically. RESULTS: By Spearman's correlation analysis, significant correlation was observed between TS and TP (gamma=0.51, p=0.018). While no correlation was observed between the tumor regression and expression of any of the genes investigated, significant association was observed between prognosis and mRNA expression levels of TS and TP. Multivariate Cox regression analysis revealed that TP mRNA expression level is a significant factor predicting prognosis (hazard ratio (HR) = - 0.204, p=0.043).

Comparison of coronary arterial finding by intravascular ultrasound in patients with "transient no-reflow" versus "reflow" during percutaneous coronary intervention in acute coronary syndrome.

Previous studies have shown that transient no-reflow during coronary intervention but with Thrombosis in Myocardial Infarction (TIMI) grade 3 flow at the completion of the procedure is associated with increased in-hospital and 6-month mortality. We hypothesized that the use of intravascular ultrasound before intervention could identify morphologic features that were predictive of transient no-reflow in patients who had acute coronary syndrome (ACS). We analyzed 220 patients with ACS who had suitable intravascular ultrasound images that were acquired before intervention. We defined "transient no-reflow" as TIMI grade 0, 1, or 2 flow during the procedure and TIMI grade 3 flow at the completion of the procedure. We defined "reflow" as good coronary flow (TIMI grade 3 flow) during and after the procedure. Patients were categorized to a transient no-reflow group (n = 20) or a reflow group (n = 200). In the transient no-reflow group, vessel area and amount of plaque burden in the culprit lesion were significantly greater than in the reflow group (vessel 20.8 +/- 5.4 vs 16.4 +/- 6.2 mm(2), p < 0.01; plaque burden 0.90 +/- 0.03 vs 0.83 +/- 0.08, p < 0.001). The presence of ruptured plaque, lipid pool-like images, and thrombus formation were significantly higher in the transient no-reflow group than in the reflow group. Multivariate analysis identified the presence of thrombus formation (odds ratio 4.53, 95% confidence interval 1.03 to 20.0, p = 0.04) and larger plaque burden (odds ratio 1.79, 95% confidence interval 1.01 to 3.23, p = 0.05) as independent predictors of transient no-reflow. In conclusion, lesion morphologies are different for transient no-reflow and reflow. These findings suggest that the presence of thrombus formation and large plaque burden increase the risk for developing transient no-reflow during coronary intervention for ACS.

Distribution and impact of lymph node metastases in oropharyngeal cancer.

CONCLUSIONS: In the treatment of oropharyngeal cancers, possible metastases to retropharyngeal lymph nodes (RPLNs) should be taken into account, especially in tumors arising in the lateral wall and/or posterior wall. Patients with multiple positive neck nodes must have intensified adjuvant therapy, especially when they have extracapsular spread (ECS). OBJECTIVE: To develop optimal treatment strategies for oropharyngeal cancers, we retrospectively analyzed the lymph node metastases of oropharyngeal squamous cell carcinoma. PATIENTS AND METHODS: Between 1988 and 2003, 77 patients with previously untreated oropharyngeal squamous cell carcinoma underwent neck dissections. RESULTS: Among the patients with tumor arising in the lateral wall or posterior wall, retropharyngeal nodes were involved in 29% (11/38), while RPLN metastasis was not observed in patients with tumors arising in the superior wall or anterior wall. The survival rate of patients with two or fewer positive lymph nodes was significantly better than that of patients with three or more positive lymph nodes (p < 0.05). The survival rate of the patients who had ECS was significantly worse than that of the patients who had lymph node metastases but not ECS (p < 0.05). There was no significant difference between the survival rates of the patients with and without RPLN metastases.

[Guillain-Barré syndrome associated Campylobacter jejuni serogroup Penner A showing antecedent fever and abdominal pain without diarrhea].

We reported a 28-year-old woman who had Guillain-Barré syndrome (GBS). Eight days after fever up and abdominal pain, she had weakness and dysesthesia of both hands and feet. Campylobacter jejuni (C. jejuni) was isolated from her stool culture without diarrhea. IgG anti-GM1 antibody was detected in her serum. Nerve conduction studies revealed a predominantly axonal neuropathy. After intravenous immunoglobulin therapy, her symptoms promptly improved. Penner 19 of C. jejuni was isolated more frequently from the GBS patients than from sporadic enteritis patients. In this case, C. jejuni with the serogroup Penner A was isolated. The serogroup is detected rarely in GBS.

Dementia and delirium in 4 patients with Machado-Joseph disease.

BACKGROUND: Machado-Joseph disease (MJD; spinocerebellar ataxia type 3) is a hereditary neurodegenerative disease caused by mutation of the MJD1 gene. Patients with MJD usually present with cerebellar ataxia, external ophthalmoplegia, pyramidal and extrapyramidal signs, and muscle wasting. However, it has been reported that these patients do not demonstrate dementia. CASE DESCRIPTION: We noticed symptoms of dementia and delirium in 4 patients with MJD. The symptoms included abnormal behavior, excitation, an uncooperative attitude, crying, disorientation, slow thought processes, hallucinations, and delusions. These symptoms were observed in patients with a relatively young onset age, and after a long clinical course. In these patients, the CAG repeat length in the MJD1 gene was much longer compared with the mean repeat length found in patients with MJD. On electroencephalographical examination, they showed slow background activity, but computed tomography and magnetic resonance imaging scans showed no cerebrocortical atrophy. Neuropathological findings in 2 patients revealed a normal cortical structure on conventional morphological examination, but at immunohistochemical examination, we found abnormal staining by an antipolyglutamine antibody in the cerebrocortical neuronal nuclei. CONCLUSIONS: Symptoms of dementia and delirium in patients with MJD could occur in the late stages, and they might be caused not by loss of cerebrocortical neurons, but by their dysfunction.

[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.