We investigated the relationship between the Trp64Arg mutation in the β₃-adrenergic receptor gene and insulin sensitivity, which was evaluated by the euglycemic-hyperinsulinemic-clamp technique, in 54 patients with impaired glucose tolerance (IGT) or non-insulin dependent diabetes mellitus (NIDDM) who were not receiving insulin therapy. The frequencies of Trp/Trp, Trp/Arg, and Arg/Arg genotypes in the patients were 63.0, 33.3, and 3.7%, respectively, which did not differ significantly from those of the 227 controls (67.0, 31.3, and 1.8 %, respectively). The mean glucose infusion rate of the 34 patients with Trp/Trp did not differ from that of the 18 patients with Trp/Arg (4.3 ± 2.2 and 5.3 ± 2.4 mg/kg/min, respectively); while that of the 2 patients with Arg/Arg was 11.5 mg/kg/min. There were no differences in the BMI or fat distribution in the abdomen between each genotype of patients, although the frequency of the Arg64 allele tended to increase with body mass index (BMI) in the control subjects under 60 years of age, which suggests that the mutation is involved in weight gain.
(Internal Medicine 36: 603-606, 1997)

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We studied relationships between anti-ganglioside antibodies and the clinical characteristics of Guillain-Barré syndrome (GBS) using multivariate analysis. Serum anti-ganglioside antibodies were measured by enzyme-linked immunosorbent assay (ELISA) in 42 GBS patients and 47 controls. Relationships between antibodies and 15 clinical characteristics were analyzed using a logistic model. Anti-GM1 antibodies were significantly infrequent in patients with objective sensory disturbance (immunoglobulin G (IgG), p < 0.05, odds ratio = 0.094; immunoglobulin M (IgM), p < 0.01, odds ratio = 0.032) and frequent in patients with prodromal diarrhea (IgG, p < 0.05, odds ratio = 5.759; IgM, p < 0.05, odds ratio = 16.28). The combination of anti-GM1 and anti-GD1b antibodies was frequent in patients with prodromal diarrhea (IgG, p < 0.01, odds ratio = 9.667; IgM, p < 0.01, odds ratio = 14.50). IgG anti-GQ1b antibodies were extremely frequent in patients with ophthalmoplegia (p < 0.01, odds ratio = 102.3), and infrequent in patients with objective sensory disturbance (p < 0.05, odds ratio = 0.023).
(Internal Medicine 36: 607-612, 1997)

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We report a case of acute myocardial infarction due to coronary embolism in a patient with echocardiographically documented hypertrophic cardiomyopathy. Emergency coronary arteriography revealed embolic occlusion of the proximal left circumflex coronary artery and the first diagonal branch. Intracoronary thrombolysis with urokinase and subsequent balloon angioplasty was successful. Transesophageal echocardiography revealed thrombus in the left atrial appendage. Coronary arteriography performed on the 46th hospital day revealed a patent left circumflex coronary artery and diagonal branch. The patient was discharged uneventfully.
(Internal Medicine 36: 613-617, 1997)

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We treated a 23-year-old male with neuroflbromatosis with acute myocardial infarction. Cardiac catheterization revealed severe organic stenosis in the left anterior descending artery, an ectasic left circumflex artery and a small right coronary artery. Percutaneous transluminal coronary angioplasty (PTCA) was performed on the stenosis. In follow-up catheterizations, intracoronary administration of ergometrine induced vasospasm of the left coronary artery including at the PTCA site. An I 123 metaiodobenzylguanidine (MIBG) scintigram showed defects in the septum, inferior wall and apex. These findings suggest abnormality of the cardiac sympathetic nerve in neurofibromatosis.
(Internal Medicine 36: 618-623, 1997)

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A 69-year-old male diabetic patient was hospitalized with pneumonia in February 1996. Abdominal ultrasonography performed as a routine examination on admission revealed marked dilatation of gallbladder with a fasting maximal size of 25.7 cm² compared with 8.8±2.9 cm² evaluated in 30 male healthy controls aged 67±8 years old. Four months after recovery from pneumonia, abnormal gallbladder dilatation remained unchanged (25.0 cm²), while dilatation had not been observed on an examination performed 6 years earlier (9.8 cm²). Because of accompanying neurological defects, we suppose that Cholecystoparesis may be caused by progression of autonomic neuropathy in this patient with diabetes mellitus.
(Internal Medicine 36: 624-627, 1997)

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A 48-year-old man with bilateral adrenal incidentalomas was studied. Although the serum cortisol level was normal, autonomous cortisol secretion was shown by the loss of diurnal rhythm, no suppressibility by dexamethasone, and complete suppression of plasma adrenocorticotropin levels. Imaging analyses revealed bilateral adrenal masses, showing isotope uptake. He was diagnosed as preclinical Cushing's syndrome due to adrenocorticotropin-independent bilateral adrenocortical macronodular hyperplasia, confirmed histologically. Despite the undetectable plasma adrenocorticotropin level and renin activity, serum mineralocorticoids levels were not suppressed, which were decreased after subtotal adrenalectomy. It was suggested that hyperplastic adrenal glands could produce both gluco- and mineralocorticoids concomitantly.
(Internal Medicine 36: 628-632, 1997)

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We describe a patient with noninsulin-dependent diabetes mellitus presenting with an amnestic disorder, personality change, and autonomic seizure. Magnetic resonance images of the brain showed T2-high signal lesion in the hippocampi bilaterally and nonspecific white matter disease, while single photon emission computed tomography revealed a diffuse reduction of cerebral blood flow. Sensory and auditory evoked potentials revealed delayed impulse conduction velocities in the central nervous system. Degenerative changes caused by a microvascular angiopathy related to noninsulin-dependent diabetes mellitus may underlie the central nervous system manifestations in our patient.
(Internal Medicine 36: 633-636, 1997)

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Diffuse parenchymal infiltrates in lower lung fields, right pleural effusion, multiple bronchial stenoses and hepatosplenomegaly were observed in a 30-year-old woman with iritis. Pleural effusion was a yellow exudate which was predominantly lymphocytic. A pulmonary function test showed an obstructive pattern, and multiple bronchial stenoses were observed on bronchoscopy. Both transbronchial lung biopsy (TBLB) and bronchial mucosal biopsy failed in demonstrating non-caseating granulomas, but liver biopsy revealed granulomas consistent with sarcoidosis. This is a very rare case, in which pleural effusion and bronchial stenoses occurred in the same patient.
(Internal Medicine 36: 637-639, 1997)

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We report a 64-year-old male with granulocytopenia and dermatitis due to cyanamide treatment. We administered cyanamide for alcoholism. After about one month he suffered from scaly erythema over his whole body and granulocytopenia (granulocyte; 140/μl) with maturation arrest in bone marrow. After cessation of cyanamide and the start of granulocyte colony-stimulating factor administration, the skin eruption ameliorated gradually, and the peripheral blood granulocyte counts increased. Cyanamide showed positive results in the drug lymphocyte stimulation test (198%) and the patch test led to the diagnosis of granulocytopenia and dermatitis induced by cyanamide. After restarting glibenclamide and diazepam administration, his granulocytopenia did not reoccur. To our knowledge, this is the first report of a case with granulocytopenia induced by cyanamide.
(Internal Medicine 36: 640-642, 1997)

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We report a case of idiopathic CD4₊ T-lymphocytopenia with malignant lymphoma (diffuse large, B-cell type) for which there was no evidence of human immunodeficiency virus type 1 or type 2 infection and no other known causes of immunodeficiency. She had never suffered from any opportunistic infection until the diagnosis of malignant lymphoma was made, and the CD4₊ T-lymphocytopenia persisted after complete remission of the lymphoma. As the clinical features and immune status of the patient differed from those associated with the acquired immunodeficiency syndrome (AIDS)-related syndrome, we conclude that immunodeficiency in this case did not contribute to the opportunistic infection but may have been associated with the genesis of malignant lymphoma.
(Internal Medicine 36: 643-646, 1997)

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A 47-year-old man with Hirayama's disease who developed cervical spondylotic amyotrophy (CSA) is presented. The patient had noted weakness and atrophy of hand and forearm muscles bilaterally at the age of 16. At the age of 40, he developed proximal muscle atrophy and weakness bilaterally after 20 years of a non-progressive state. Myelography and computed tomography (CT)-myelography revealed that ventral cord compression at multiple levels of C4-7 vertebral bodies was increased when the neck was extended. The clinical diagnosis was CSA associated with Hirayama's disease. To our knowledge, this is the first such case to be reported.
(Internal Medicine 36: 647-650, 1997)

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We report a patient with probable Parkinson's disease who experienced a temporary worsening of his symptoms and signs after cigarette smoking. Deterioration of tremor, rigidity, and writing disturbance began soon after the inhalation of cigarette smoke and persisted for 15 minutes. Chewing nicotine gum also induced a brief worsening of the patient's symptoms. Aggravation of parkinsonian symptoms after cigarette smoking appeared to be caused by nicotine alone, although the mechanism for this effect remains unclear.
(Internal Medicine 36: 651-653, 1997)

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We report a 26-year-old man with eosinophilic fasciitis who presented with progressive joint contractures of all four limbs. In this patient, the serum aldolase concentration was elevated while the serum creatinine kinase concentration was normal. Magnetic resonance imaging showed gadolinium enhancement of fascial structures in the lower limbs. With the clinical improvement by the treatment with oral corticosteroid, the aldolase concentration was decreased, and the fascial hyperintensity on magnetic resonance images was reduced. The phenomenon of increased an aldolase concentration accompanied by a normal creatinine kinase concentration may be characteristic of eosinophilic fasciitis. Serial magnetic resonance images and serum aldolase concentration are useful for monitoring the therapeutic response in this condition.
(Internal Medicine 36: 654-656, 1997)

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A 73-year-old man developed refractory multiple hemorrhagic gastric ulcers. Emergent gastrectomy was performed, and the diagnosis of polyarteritis nodosa was made by histologic evaluation of the resected stomach. Although gastric ulceration is a relatively rare complication of polyarteritis nodosa, it should be considered in the differential diagnosis of refractory hemorrhagic gastric ulcers. Emergent surgery may be indicated in such cases.
(Internal Medicine 36: 657-660, 1997)

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A 28-year-old woman with a history of a spontaneous abortion developed thrombocytopenia, Coombs-negative hemolytic anemia, and liver dysfunction at the sixteenth week of pregnancy. These findings were compatible with hemolysis, elevated liver enzymes, and low platelets (HELLP) syndrome (hemolysis, elevated liver enzymes, and low platelet counts). Moreover, serum antiphospholipid antibodies were positive, suggesting the association of anti-phospholipid antibody syndrome. An artificial abortion, anti-coagulation therapy, and plasma exchange were performed concomitantly with corticosteroid therapy. She responded to the therapy, a remission was obtained. Anti-phospholipid antibodies may play a role in the pathogenesis of HELLP syndrome.
(Internal Medicine 36: 661-666, 1997)

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