To confirm the clinical utility of selegiline (L-deprenyl), a selective inhibitor of monoamine oxidase B, as an anti-Parkinson's disease (PD) agent, the first Japanese multi-center, double-blind comparative study of this drug was conducted. The subjects were patients who had responded poorly or suffered with other problems related to L-dopa treatment. A total of 112 patients in two groups, one given selegiline at a dose of 7.5 mg/day (Group D, n=60) and another given a placebo (Group P, n=52), were compared over an 8-week treatment period. The percentage patients showing "moderate improvement" or better was 34.5% in Group D, while that in Group P was 11.5 % (P<0.01). In the assessment of overall safety, 66.7 % in Group D showed no adverse reactions, which was not significantly different from the result of 78.9% for Group P.
(Internal Medicine 33: 517-524, 1994)

View full abstract

It has been suggested that accentuated pulmonary hypertension is a contributing factor in the development of high-altitude pulmonary edema (HAPE). The purpose of this study was to evaluate the chest radiographic features associated with pulmonary hemodynamic changes in HAPE. We studied 16 patients with HAPE using posteroanterior chest roentgenograms taken in the standing position both on admission and following recovery. The cardiothoracic ratio (CTR) as well as the area and volume of the main pulmonary artery were measured. During HAPE, we found slight enlargement of the CTR, especially the right ventricle, and prominence of the main pulmonary artery. These abnormalities significantly improved in the recovery state. The changes in area and volume of the main pulmonary artery were closely correlated with a decrease in CTR during recovery from HAPE, and correlated with the pulmonary hemodynamic changes observed by right heart catheterization (n=4). Radiographic changes reflect the alterations in pulmonary hemodynamics in patients with HAPE.
(Internal Medicine 33: 525-528, 1994)

View full abstract

We examined the eosinophil viability-enhancing activity (EVEA) of peripheral blood mononuclear cells (PBMNCs) obtained from 6 patients with mite-sensitive bronchial asthma (BA) and 9 normal control subjects. Mite concentrations of 1μg/ml and 10 μg/ml significantly increased EVEA in PBMNC culture supernatants from BA patients compared with PBMNCs from normal control subjects (76.1±11.0% at 10 μg/ml and 56.3±16.0% at 1 μg/ml vs 20.6±012.6% at 10 μg/ml and 7.4±2.3% at 1 μg/ml; p<0.05). The level of IFN-γ in PBMNC culture supernatants in BA patients was 2.3±0.9 IU/ml and in normal control subjects 0.7±0.3 lU/ml. A combination of mAbs (anti-IL3, anti-IL-5 and anti-GM-CSF, with or without anti-IFN-γ) neutralized the EVEA (p<0.001, p<0.001, respectively). Dexamethasone (10^-8 M to 10^-5 M), cyclosporin A (10^-7 M to 10^-5 M) and FK506 (10^-8 M to 10^-6 M) significantly inhibited EVEA in BA patients (p<0.05 to p<0.001). The release of eosinophil cationic protein (ECP) from eosinophils in the presence of mite-stimulated PBMNC culture supernatants was higher in patients with bronchial asthma (569±147 μg/l) than in normal control subjects (203±99 μg/1; p<0.05).
(Internal Medicine 33: 529-535, 1994)

View full abstract

We describe two cases of pulmonary infection due to Mycobacterium xenopi (M. xenopi). Both cases were men, ages 61 and 54 yr. In the first patient, lung infection due to M. xenopi occurred after gastrectomy. The second patient had an inactive M. tuberculosis infection. Both had pulmonary symptoms including cough, sputum and fever. Each chest X-ray showed an infiltrative shadow with a cavity in a unilateral, upper lobe. Isolates from both patients were studied not only by microbiological characteristics but also by DNA-DNA hybridization. All isolates were susceptible to streptomycin and kanamycin. In the first case, the patient had initially received rifampicin, isoniazid and ethambutol despite in vitro susceptibility patterns, however, there was no response and a new infiltrative shadow appeared in the contralateral lobe. With a multiple drug regimen based on in vitro susceptibility, clinical and roentgenographic improvements were achieved. The second patient showed a favorable response to the initial chemotherapy. Pulmonary infection due to M. xenopi can generally be successfully treated with drugs to which the organisms show in vitro sensitivity. We also reviewed the other two cases reported in Japan.
(Internal Medicine 33: 536-539, 1994)

View full abstract

An autopsied case of systemic lupus erythematosus with pulmonary hypertension is reported. A 29-year-old woman with a seven-year history of polyarthralgia, butterfly rash, nephrotic syndrome and Raynaud's phenomenon was admitted because of progressive dyspnea on exertion. Tests for antinuclear antibody, anti-cardiolipin antibody and lupus anticoagulant were positive. Echocardiographic examination revealed right ventricular hypertrophy and a moderate pericardial effusion. Estimated systolic pulmonary arterial pressure was 53 mmHg. Despite treatment with corticosteroids including pulse methylprednisolone therapy, lipo-PGE₁ and warfarin, she died of progressive congestive heart failure. Postmortem examination of the pulmonary vasculature revealed findings consistent with plexogenic pulmonary arteriopathy, without evidence of vasculitis, fibrinoid necrosis, or thromboemboli.
(Internal Medicine 33: 540-542, 1994)

View full abstract

A 50-year-old woman with subacute dementia and brain atrophy on CT showed periodic synchronous discharge (PSD) on electroencephalogram (EEG) and myoclonus. She was initially suspected of suffering from Creutzfeldt-Jakob disease (CJD), but dramatically recovered over 5 months. Based on further investigations, the final diagnosis was mitochondria! encephalomyopathy with an A-to-G substitution at nucleotide position 3243 in mitochondrial DNA (mtDNA), commonly seen in patients with mitochondrial myopathy, encephalopathy, lactic acidosis, and strokelike episodes (MELAS). This case suggests that patients suspected of suffering from CJD should be evaluated for mitochondrial encephalomyopathy.
(Internal Medicine 33: 543-546, 1994)

View full abstract

Marked elevation of tumor markers in the peripheral blood was initially a sole manifestation of meningeal carcinomatosis in a man with gastric carcinoma. In addition to extensive meningeal carcinomatosis, no metastatic lesions were found at autopsy other than microscopic infiltration into a tiny paraaortic lymphnode. Elevation of these markers in the peripheral blood is best explained by meningeal carcinomatosis. When elevation of these markers is otherwise unexplainable, meningeal carcinomatosis should be considered as a diagnostic possibility even in the absence of neurological symptoms.
a(Internal Medicine 33: 547-549, 1994)

View full abstract

We report a case of pulmonary infiltration with eosinophilia (PIE), associated with increased serum levels of squamous cell carcinoma-related antigen (SCC) and neuron specific enolase (NSE). The diagnosis of PIE was confirmed by examination of bronchoalveolar lavage fluid and specimens of transbronchial lung biopsy. It was suggested that PIE was probably induced by a course of amoxicillin for a sore throat. Corticosteroid therapy resulted in clinical improvement of symptoms, resolution of pulmonary infiltrates on chest roentgenogram and reduction in serum levels of SCC and NSE.
(Internal Medicine 33: 550-553, 1994)

View full abstract

We report an interesting case of vasculitis in which the inflammatory lesion was limited to the peritracheobronchus. This case showed positive antineutrophil cytoplasmic antibodies, diffuse peritracheobronchial swelling, and vasculitis in its histology. Steroid therapy was effective for both roentgenological and serological findings. Although the biopsy specimen shows only inflammation and does not satisfy the WHO criteria of Wegener's granulomatosis (WG), a possible diagnosis of WG should not be disregarded.
(Internal Medicine 33: 554-556, 1994)

View full abstract

We describe a 68-year-old man who developed primary cerebral malignant lymphoma 5 years after the onset of progressive systemic sclerosis (PSS). There was no association with Sjögren's syndrome. This is the first report of PSS complicated with primary cerebral malignant lymphoma.
(Internal Medicine 33: 557-559, 1994)

View full abstract

We evaluated insulin resistance and assessed the effect of gliclazide on insulin resistance in a patient with diabetes mellitus associated with Turner's syndrome. Insulin-induced glucose metabolism markedly decreased compared with 12 healthy subjects. The insulin dose-response curve of this patient shifted to the right and down, and recovered somewhat after the administration of gliclazide. This patient had exhibited marked insulin resistance, which seemed to be caused by a defect at the receptor and/or post-receptor levels. Gliclazide reduced her insulin resistance, which suggests that this agent is suitable for treating the insulin resistance in diabetic patients with Turner's syndrome.
(Internal Medicine 33: 560-563, 1994)

View full abstract

HTLV-I carriers or patients with HTLV-I associated myelopathy (HAM) are prone to immune-mediated inflammatory disorders. We present a 44-year-old female with HAM who developed Graves' disease. She developed severe Graves' ophthalmopathy shortly after 131I therapy, concurrently with a remarkable increase in TSH-receptor antibody titer. Ophthalmopathy was aggravated in spite of prednisolone therapy and euthyroidism being maintained by thyroxine replacement. Uveitis also developed after 131I therapy and iridocyclitis finally required trabeculotomy. This case suggests that HAM patients may have a higher risk of immune-mediated Graves' ophthalmopathy after ¹³¹I therapy.
(Internal Medicine 33: 564-568, 1994)

View full abstract

A 49-year-old man was admitted because of general fatigue, cough and hematuria. During the hospital course, acute renal failure, hemoptysis and dyspnea developed. A percutaneous renal biopsy revealed a diffuse crescentic glomerulonephritis, and direct immunofluorescence showed a linear pattern of IgG along the glomerular basement membrane. Although serum anti-glomerular basement membrane (anti-GBM) antibody was not detected, Goodpasture's-like syndrome was suspected, and methylprednisolone pulse therapy and plasmapheresis were administered. Concomitantly, extracorporeal membrane oxygenation (ECMO) was instituted because of deterioration in respiratory status due to a severe pulmonary hemorrhage despite maximal ventilatory support. Temporarily, the patient improved and ECMO was discontinued. ECMO may be a useful therapeutic support for hypoxia resulting from pulmonary hemorrhage in Goodpasture's syndrome (GPS) and Goodpasture's-like syndrome.
(Internal Medicine 33: 569-573, 1994)

View full abstract

A 31-year-old woman presented with hypertrophy of the left upper extremity and thrombocytopenia. Physical examination revealed splenomegaly, and laboratory investigation revealed thrombocytopenia, elevation of cross-linked fibrin degradation products (XDP), and thrombin-antithrombin III complex (TAT). A diagnosis of Klippel-Trenaunay-Weber (K-T-W) syndrome was established by the dermatologic findings and angiography of the extremities. A splenic cavernous lymphangioma was diagnosed by ultrasonography and angiography, and was confirmed by pathology following splenectomy. Post-operatively, the platelet count increased, and hemostatic parameters normalized. Cavernous lymphangioma is a rare complication of Klippel-Trenaunay-Weber syndrome. Splenectomy proved to be an effective therapy for both cavernous lymphangioma and consumptive coagulopathy in Klippel-Trenaunay-Weber syndrome.
(Internal Medicine 33: 574-577, 1994)

View full abstract