Noriyoshi Yamakita

Successful Treatment with Cyclosporin A in Tocilizumab-resistant TAFRO Syndrome.

Thrombocytopenia, anasarca, fever, reticulin fibrosis, and organomegaly (TAFRO) syndrome is a unique clinicopathologic variant of multicentric Castlemans disease that has recently been identified in Japan. Previous reports have shown that affected patients typically respond to immunosuppressive therapy, such as prednisolone and tocilizumab. However, the optimal treatment for refractory TAFRO syndrome, which can be fatal, remains unclear. We herein report a case of tocilizumab-resistant TAFRO syndrome successfully treated with cyclosporin A, indicating that cyclosporine A may be an alternative therapy for refractory TAFRO syndrome.

A case of Graves’ disease associated with polymyositis

A patient with Graves’ disease associated with severe muscle weakness who was finally diagnosed as polymyositis by pathological examination of the muscle is reported. A 28-year-old women was incidentally found to have hyperthyroidism when she consulted a hospital for the evaluation and treatment of anemia in 1979. She was treated with methimazole for approximately a month when she stopped the medication by herself. Approximately two yr later (Nov. 4, 1981) she consulted another hospital with complaints of palpitation and muscle weakness. Diagnosis of hyperthyroidism due to Graves’ disease and thyrotoxic myopathy were made, followed by the treatment with radioiodine (4 mCi of 131I). She was further treated with propylthiouracil (PTU). Four yr after the treatment, serum thyroid hormone concentration declined to the lower level than normal and serum TSH concentration increased. She was subsequently treated with synthetic I-T4. Despite the fact she became euthyroid with the treatment, muscle weakness as well as elevated concentrations of muscle enzymes were not improved. Muscle biopsy was made in July 1983, and she was diagnosed as immune polymyositis and treatment with prednisolone and cyclophosphamide in addition to PTU or I-T4, was started. With the treatment, serum LDH decreased to the normal range. However she still has muscle weakness and serum concentrations of CPK and aldolase are still in higher levels than normal range.

Measurement of plasma free steroids by direct radioimmunoassay of ultrafiltrate in association with the monitoring of free components with [14C]glucose

We describe a modified method of centrifugal ultrafiltration using the Grace MPS-3 device for the measurement of plasma free (unbound) steroids (cortisol, testosterone, estradiol, and prednisolone). Plasma was incubated with [14C]glucose to monitor the movement of free components, applied to the MPS-3 and centrifuged. Steroid concentration of ultrafiltrate was directly measured by radioimmunoassay, and multiplied by the ratio of [14C]glucose count (dpm) in plasma to [14C]glucose count (dpm) in ultrafiltrate. The data by this method correlated well with those obtained by equilibrium dialysis. Our results of free steroid in healthy volunteers and patients with various diseases were comparable with the previously reported values. This procedure showed the advantages of small sample volume, rapid separation and the ability to process a large number of samples in a single run.

Hyperglycemic Chorea-Ballism or Acute Exacerbation of Huntington's Chorea? Huntington's Disease Unmasked by Diabetic Ketoacidosis in Type 1 Diabetes Mellitus

CONTENT Hyperglycemic chorea-ballism is predominantly observed in older type 2 diabetic patients, and it is rare in type 1 diabetes and diabetic ketoacidosis (DKA). Huntingtons disease (HD) is one of several genetic syndromes associated with diabetes, although the reported prevalence of the association varies. There are few opportunities for most physicians to diagnose early-stage HD. OBJECTIVE We describe bilateral hyperglycemic chorea-ballism in a 40-yr-old female type 1 diabetes patient with DKA and HD. SETTING The study was conducted in a tertiary care referral hospital. RESULTS On admission, the patient exhibited severe involuntary movement of bilateral extremities with DKA, and hyperglycemic chorea-ballism was diagnosed. She recovered from chorea-ballism with admission of fluids and insulin, but mild choreatic movement persisted in the upper extremities. Brain magnetic resonance imaging and DNA analysis revealed HD. Although it has been considered that depletion of striatal γ-aminobutyric acid (GABA) content is rare in DKA, it is largely decreased in HD. Therefore, it is probable that hyperglycemic chorea-ballism or exacerbation of Huntingtons chorea resulted from transient depletion of GABA. CONCLUSION The present case provides important insights on the role of GABA in hyperglycemic chorea-ballism and on the clinical issues associated with HD diagnosis.

Dehydroepiandrosterone sulphate is increased and dehydroepiandrosterone‐response to corticotrophin‐releasing hormone is decreased in the hyperthyroid state compared with the euthyroid state

OBJECTIVE Dehydroepiandrosterone (DHEA) and DHEA‐sulphate (S) have been suggested to play protective roles in many pathological states, some of which are observed in hyperthyroidism. If DHEA and DHEA‐S levels change in hyperthyroidism, they might participate as a possible causative link with such pathophysiological changes in hyperthyroidism. However, the CRH–ACTH–DHEA system in hyperthyroidism has not been clearly defined. We examined plasma levels of DHEA and DHEA‐S together with ACTH and cortisol in both hyperthyroid (Hyper) and euthyroid states (Eu).

Hypopituitarism and Diabetes Insipidus with Localized Hypertrophic Pachymeningitis (Tolosa-Hunt Syndrome) Associated with Hashimoto Thyroiditis

We report a 69-year-old woman with intracranial pachymeningitis showing hypopituitarism, diabetes insipidus, and Tolosa-Hunt syndrome associated with Hashimoto thyroiditis confirmed by autopsy. A large tumorous lesion of the hypothalamo-pituitary gland was revealed on magnetic resonance imaging, after the patient complained of gait and visual field disturbance. These symptoms subsided after thyroid hormone supplementation. Hypopituitarism and diabetes insipidus were diagnosed after cessation of the treatment by the patient herself. Multiple cranial nerve palsies and orbito-frontalgia appeared. Methylprednisolone pulse therapy improved the symptoms, but they recurred when the dose of glucocorticoid was decreased. The patient died of brain thrombosis. Autopsy revealed typical findings of Hashimoto thyroiditis and intracranial pachymeningitis involving the cranial base and pituitary gland. The high titer of rheumatoid factor and Hashimoto thyroiditis in this patient suggest an immunological role in the pathogenesis of pachymeningitis.

Serum albumin-adjusted glycated albumin is a better predictor of mortality in diabetic patients with end-stage renal disease on hemodialysis

AIMS Glycated albumin (GA) is a marker for monitoring glycemic control in diabetic patients with end-stage renal disease (ESRD). We evaluated whether serum albumin-adjusted GA (adjusted GA) could predict mortality in diabetic patients with ESRD on hemodialysis. METHODS Seventy-eight patients with type 2 diabetes treated with regular hemodialysis were enrolled and followed up for 5-years. The adjusted GA was calculated from the regression formula and mean GA. The cut-off values for GA and adjusted GA that predicted mortality risk were determined using receiver operating characteristic curve analysis. RESULTS During the follow-up period (median: 36months), 15 patients died. In the Kaplan-Meier analysis, there were no significant differences in the 5-year cumulative survival rate (58.3% [GA ≥19.8%] vs. 88.6% [GA <19.8%], P=0.075). Conversely, this rate was significantly higher in patients with adjusted GA <21.2% than adjusted GA ≥21.2% (86.4 vs. 49.5%, P=0.0068). After adjustment for other confounders, adjusted GA ≥21.2% was an independent predictor for mortality (hazard ratio 3.76, 95% confidence interval 1.12-17.44, P=0.031), but GA ≥19.8% was not (hazard ratio 2.63, 95% confidence interval 0.65-17.69, P=0.19). CONCLUSIONS Adjusted GA is a better predictor of mortality than GA in diabetic patients with ESRD on hemodialysis.

Pseudo-adrenal incidentaloma : magnetic resonance imaging in a patient with para-adrenal Castleman's disease

We report a case of para-adrenal angiofollicular lymph node hyperplasia (Castlemans disease) of the hyaline-vascular type. The mass could not be differentiated from an adrenal tumor by ultrasonography and computed axial tomography (CT). However, magnetic resonance imaging (MRI) suggested the possibility of an extra-adrenal origin of the mass. The intensity of the mass by MRI was homogeneous and of a higher intensity in the T2-weighted image than in the T1-weighted image, a finding similar to lymphadenopathy, lymphatic tumorous mass or metastatic tumor of the lymph node. Ultrasonography, CT and MRI may not be useful in characterizing Castlemans disease, but MRI was useful to distinguish asymptomatic para-adrenal masses from those of adrenal origin.

Delineation of adrenal in controls and nontumorous adrenal disorders by real-time ultrasonic-scanner

In 90 control subjects, 90% of the right adrenal and 38% of the left were delineated by sector US scanner. A longitudinal scan from an intercostal space in the mid-axillary line and a right-anterior-transverse scan from the intercostal space were useful in displaying the right adrenal, and an anterior-transverse scan from the epigastrium was also useful in showing the left adrenal. The latter was not as clear as the right. Moreover, a transverse scan from the left flank did not fully display the whole image of the left adrenal. The sizes of both adrenal images were somewhat smaller than those made by computerized tomography (CT), as shown in our previous study. Although the left adrenal in one case of congenital adrenogenital syndrome and those in three cases of Cushings disease could not be delineated, all of the enlarged right adrenals in these cases were demonstrated by ultrasonic scanning (US). These results suggest the clinical usefulness of US for the detection of affected, nontumorous right adrenals.

COMPARATIVE ASSESSMENT OF ULTRASONOGRAPHY AND COMPUTED TOMOGRAPHY IN ADRENAL DISORDERS

Comparative diagnostic assessment by ultrasonography (US) and computed tomography (CT) was performed in each of 17 patients with adrenal disorders. US is better than CT for demonstrating the intratumorous characteristics, the relationship between the tumor and the surrounding organs, and the organ from which large tumors arise, such as pheochromocytoma. On the other hand, CT is better able to detect small adrenal tumors in primary aldosteronism, although US with a sector or linear scanner can also detect small tumors in some cases. Adrenal hyperplasia in both Cushings disease and congenital adrenogenital syndrome was more easily demonstrated by CT than by US. Of the two US instruments, a sector scanner was more useful in the delineation of the enlarged right nontumorous adrenal by scanning from the right intercostal region, but both scanners were of no use in the delineation of the enlarged nontumorous left adrenal in patients with obesity.