Search

A 29-year-old man presented with a high-grade fever, headache, and urinary retention, in addition to meningeal irritation and myoclonus in his upper extremities. A cerebrospinal fluid (CSF) examination showed pleocytosis and high adenosine deaminase (ADA) levels with no evidence of bacterial infection, including Mycobacterium tuberculosis. T2-weighted brain magnetic resonance imaging showed transient hyper-intensity lesions at the splenium of the corpus callosum (SCC), bilateral putamen, and pons during the course of the disease. The CSF was positive for anti-glial fibrillary acidic protein (GFAP) antibodies. He was diagnosed with autoimmune GFAP astrocytopathy. The present case shows that the combination of an elevated ADA level in the CSF and reversible T2-weighted hyper-intensity on the SCC supports the diagnosis of autoimmune GFAP encephalopathy.

Objective To clarify the associations between serum omega-6 (n-6) and omega-3 (n-3) polyunsaturated fatty acid (PUFA) levels and obesity-related metabolic abnormalities in patients with type 2 diabetes. Methods and Materials Data from 225 Japanese patients with type 2 diabetes were cross-sectionally analyzed. The serum levels of n-6 PUFAs [dihomo-γ-linolenic acid (DGLA) and arachidonic acid (AA)] and n-3 PUFAs (eicosapentaenoic acid and docosahexaenoic acid) were measured, and the estimated Δ-5 desaturase (D5D) activity was calculated based on the AA to DGLA ratio. The associations between the composition of PUFAs and obesity-related parameters, including the body mass index (BMI), waist circumference, alanine amino transferase (ALT) level, homeostatic model assessment of insulin resistance (HOMA-IR), and body fat percentage, as measured by a bioelectrical impedance analysis, were analyzed. Results Among the PUFAs, the DGLA level had the strongest correlations with BMI (p<0.001), waist circumference (p<0.001), ALT level (p<0.001), HOMA-IR (p<0.001), and body fat percentage (p<0.01). AA was positively correlated and D5D was negatively correlated with several obesity-related parameters, while n-3 PUFAs did not have a constant correlation. A multivariate regression analysis revealed that the DGLA level was an independent determinant for HOMA-IR (β=0.195, p=0.0066) after adjusting for sex, age, BMI, and the ALT, triglyceride, and HbA1c levels. Conclusion A high serum DGLA level was associated with obesity, body fat accumulation, a high ALT level, and insulin resistance in patients with type 2 diabetes. The measurement of the serum PUFA levels may be useful for evaluating metabolic abnormalities and estimating the dietary habits of patients.

A 33-year-old man was admitted to our hospital to undergo an evaluation to determine the cause of secondary hypertension. Computerized tomography angiography (CTA) showed bilateral multiple renal arteries with significant stenosis of the right extra-renal artery due to fibromuscular dysplasia and segmental impairment of renal perfusion. Although the plasma aldosterone concentration and plasma renin activity were within the normal ranges, percutaneous balloon dilatation of the stenotic lesion resolved his hypertension, leading to a diagnosis of renovascular hypertension caused by segmental renal ischemia due to extra-renal artery stenosis. CTA should be considered during the examination of patients with early-age hypertension, even if the plasma renin activity is not sufficiently elevated.

We describe a case of amiodarone-induced thyrotoxicosis (AIT) with cardiopulmonary arrest (CPA) in a 49-year-old woman. The patient had been treated with amiodarone for non-sustained ventricular tachycardia. Two weeks prior to her admission, she developed thyrotoxicosis and prednisolone (PSL, 30 mg daily) was administered with the continuation of amiodarone. However, she was admitted to our hospital for CPA. We performed total thyroidectomy to control her thyrotoxicosis and the pathological findings were consistent with type 2 AIT. She gradually improved and was discharged on day 84. This case demonstrates the importance of considering immediate total thyroidectomy for patients with uncontrollable AIT.

Lipoprotein lipase (LPL) and hepatic triglyceride lipase (HTGL) enhance the hydrolysis of triglycerides (TG) transported by chylomicron (CM) and very-low-density lipoprotein (VLDL). We report a case of severe hyperchylomicronemia with high levels of remnant lipoprotein and total cholesterol (T-Chol) in a 15-year-old boy. Precise examination of the lipid profile showed decreased activities of both LPL and HTGL, although the protein mass for LPL and HTGL were maintained. In addition, bezafibrate treatment effectively ameliorated hypertriglyceridemia in this case. This is the first case of hyperchylomicronemia with decreased activities and unaffected protein masses for both LPL and HTGL, without overt immuno-dysfunction.

Two young man patients with refractory post-bulbar duodenal ulcer (post-bulbar ulcer) were encountered. They had a single punched-out ulcer in the absence of an underlying disease. Patient 1 was Helicobacter pylori (Hp)-positive, and did not respond to Hp eradication therapy. The ulcer scarred after the long-term administration of a proton pump inhibitor (PPI), but recurred after a reduction in the dose. Patient 2 was Hp-negative. His ulcer did not scar even after long-term PPI administration, but it formed a fistula into the gallbladder, and the fistula was surgically closed. In both patients, laboratory and imaging studies excluded Zollinger-Ellison syndrome, but suggested a hyperacidic tendency. Unlike duodenal bulb ulcer (bulbar ulcer), the post-bulbar ulcer in Patient 1 did not heal with Hp eradication therapy, suggesting that post-bulbar ulcer differs etiologically from bulbar ulcer. We speculate that the possible causes of the refractoriness to treatment in both patients were ulcer penetration, callosity formation, and insufficient inhibition of gastric acid secretion due to the impaired passage of PPI into the deep portion of the duodenum as a result of luminal narrowing.

A 68-year-old man with a 28-year history of non-insulin dependent diabetes mellitus (NIDDM) was admitted to our hospital because of foot gangrene. He had previously suffered from cerebral infarction resulting in right hemiplegia and his right foot was amputated because of right femoral lesion presenting diabetic foot gangrene 5 years previously. The diabetic foot gangrene gradually became worse, although he had received various medications. Then, we attempted to treat the patient with low density lipoprotein (LDL)-apheresis ten times a month. The foot gangrene itself and the local circulation around the gangrene lesion were remarkably improved after treatment with LDL-apheresis. We present here the first case of diabetic foot gangrene improved by LDL-apheresis. LDL-apheresis therapy is anticipated to be a new therapeutic approach for treatment of fatal foot gangrene associated with diabetes mellitus.