The occurrence of myelitis with atopic diathesis (atopic myelitis) affecting young adults has recently been noted in Japan. The disease preferentially affects the posterior column of the cervical spinal cord, as shown clinically and by MRI. It is characterized by hyperlgEaemia and the presence of mite antigen-specific IgE. The spinal cord lesions have been shown to be eosinophilic inflammation on biopsy and thus an allergic mechanism is thought to be operative in this condition. In addition, we also found that Hirayama disease, juvenile muscular atrophy of the distal upper extremity, is also associated with airway allergy such as allergic rhinitis and atopic asthma. In children, poliomyelitis-like illness after acute asthma attacks is well known as Hopkins syndrome. Moreover, by the prospective study of the history of allergic disorders in common neurologic diseases, an association between spinal progressive muscular atrophy (SPMA) and asthma as well as between myelitis and atopic dermatitis has been demonstrated. These observations strongly suggest a link between atopic diathesis and spinal cord damage. Central nervous system damage associated with atopic diathesis may be classified into two types; eosinophilic myelitis preferentially affecting the cervical spinal cord and lower motor neuron damage, such as Hopkins syndrome, Hirayama disease and SPMA. The former is typically associated with atopic dermatitis while the latter, with airway allergy.
(Internal Medicine 41:169-174, 2002)

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Not all patients who fulfill the minimal criteria for the diagnosis of multiple myeloma should be treated. If a patient is younger than 70 years, autologous peripheral blood stem cell transplantation should be seriously considered. Major challenges for stem cell transplantation are: 1) the inability to eradicate multiple myeloma from the patient, and 2) removal of myeloma cells and their precursors from the reinfused stem cells. Allogeneic transplantation cannot be recommended at present because of the excessive mortality. Nonmyeloablative approaches are promising. There is no evidence that combinations of alkylating agents are superior to melphalan and prednisone. The use of thalidomide and intermittently administered prednisone for maintenance is being explored. New agents include the immunomodulatory drugs, inhibitors of the ubiquitin proteasone pathway such as PS-341, antiangiogenesis drugs including 2-methoxy-estradiol, and farnesyl transferase inhibitors. Management of skeletal complications, hypercalcemia, anemia, infection, spinal cord compression, and renal insufficiency is discussed.
(Internal Medicine 41: 175-180, 2002)

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Objective There is clinical and pathological evidence of thrombosis in pulmonary vessels of patients with chronic obstructive pulmonary disease (COPD). The purpose of this study was to investigate the presence of hypercoagulability and determine the extent of this abnormality in COPD patients.
Patients and Methods We measured plasma levels of thrombin antithrombin III complex (TAT), fibrinopeptide A (FPA), tissue plasminogen activator-plasminogen activator inhibitor (tPA-PAI): markers of coagulation-fibrinolysis-system, and also β-thromboglobulin (β-TG): a marker of platelet activation, in 40 COPD patients and in 20 control subjects. Measurements were also repeated 12 months after entry in all patients.
Results TAT, FPA, tPA-PAI, and β-TG concentrations were significantly higher in COPD than in control subjects. At 12 months follow-up, ΔA-aDO₂ and Δ%FEV₁ were significantly higher in patients with high TAT or tPA-PAI levels than in patients with low levels and TAT, FPA and tPA-PAI levels remained elevated, although β-TG levels decreased after domiciliary O₂ therapy.
Conclusion Our results showed an enhanced prothrombotic process in COPD patients, which could potentially account for the increased thrombosis in pulmonary vessels in these patients.
(Internal Medicine 41:181-185, 2002)

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Objective Aceruloplasminemia is an iron metabolic disorder caused by mutations in the ceruloplasmin gene. It is characterized by progressive neurodegeneration in association with iron accumulation. Excess iron functions as a potent catalyst of biologic oxidation. Previously we showed that an increased iron concentration is associated with the products of lipid peroxidation in the serum, cerebrospinal fluid, and brain tissues. To clarify the free radical-mediated tissue injury caused by intracellular iron accumulation through mitochondrial dysfunction.
Patients and Methods We have measure brain oxygen and glucose metabolisms using positron emission tomography (PET) and examined brains at autopsy for iron contents and activities of the mitochondrial respiratory chain in two affected patients who had different truncation mutations of the ceruloplasmin gene.
Results PET showed a marked decrease in glucose and oxygen consumption in the entire brain of aceruloplasminemia patients, with a preponderance of metabolic reduction in basal ganglia. Enzyme activities in the mitochondrial respiratory chain of the basal ganglia were reduced to approximately 45% and 42% respectively for complexes I and IV. An inverse relationship was shown between the amounts of iron accumulated and the levels of mitochondrial enzyme activities in all the brain regions examined.
Conclusion Iron-mediated free radicals may contribute to the impairment of mitochondrial energy metabolism in aceruloplasminemia.
(Internal Medicine 41: 186-190, 2002)

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A 52-year-old woman was admitted to our hospital with thrombophlebitis of the internal jugular vein. Abdominal ultrasonography demonstrated a high echogenic mass measuring 4.5 cm in diameter in the liver, and abdominal CT revealed another liver tumor and an isodensity mass in the" spleen. Abdominal MRI and angiography were performed and we presumed the tumors to be two hepatic angiomyolipoma and a splenic hamartoma. As an abdominal CT 21 months later revealed that all tumors were growing, these tomors were surgically resected. The histological diagnoses were hepatic angiomyolipoma and splenic hamartoma.
(Internal Medicine 41: 191-198, 2002)

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Swallowing is a rare cause of syncope. A 76-year-old woman was admitted to a hospital due to postprandial loss of consciousness. Although no remarkable cardiogenic problem was proven, upper gastrointestinal barium examination revealed a huge hiatal hernia. Both echocardiography and MRI presented the collapsed left atrium due to the herniated stomach. Water pouring examination successfully represented lightheadedness, and Nissen's fundoplication was carried out. After this procedure, she never suffered from syncopal attack.
(Internal Medicine 41:199-201, 2002)

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A complete response was obtained by interferon (IFN) therapy for hepatitis C virus (HCV)-related advanced chronic hepatitis after curative treatment of initial and recurrent hepatocellular carcinoma (HCC). The patient is alive 73 months after diagnosis of initial HCC and is in a tumor-free state 53 months after diagnosis of the last HCC. This case suggest that it is possible to suppress recurrence and to prolong the life of patients with HCV-related advanced chronic hepatitis, if a complete response is obtained by means of IFN therapy after the curative treatment of the recurrent HCC.
(Internal Medicine 41: 202-206, 2002)

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A 69-year-old woman was admitted to our hospital for the examination of syncope. When she ate solid food, she had dizziness or loss of consciousness. The ambulatory ECG suggested sino-atrial block during swallowing with a maximum sinus pause of 6 seconds. An electrophysiologic study revealed pre-existing sinus node dysfunction, which was exaggerated by the balloon inflation in the esophagus. Atropine counteracted the slowing of the basal sinus rate induced by esophageal pressure, but it did not block the effect on the maximum sinus node recovery time. This observation suggested that the syncope was mediated partly by a non-vagal mechanism.
(Internal Medicine 41: 207-210, 2002)

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A 40-year-old woman was referred to our hospital with severe hypovolemic shock and anasarca. The laboratory findings showed marked hemoconcentration and a decrease in total serum protein with the presence of monoclonal IgG-λ. She had had a similar episode of generalized edema 2 years previously. We diagnosed the patient as having typical systemic capillary leak syndrome (SCLS) and she improved gradually after infusion of albumin-containing fluid. SCLS is a very rare condition caused by unexplained episodic capillary hyperpermeability. Its treatment has remained largely supportive and the prognosis is generally poor. Awareness of SCLS is necessary for improvement of the outcome.
(Internal Medicine 41: 211-215, 2002)

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A case of microvascular angina accompanied by coronary artery spasm is described. A 54-year-old woman had anginal pain at rest and during exercise. Both exercise testing and rapid atrial pacing caused significant ST depression in the inferior and all precordial leads. Exercise thallium myocardial scintigraphy was negative despite similar ST depressions. Coronary angiography revealed insignificant stenoses of the left anterior descending coronary artery after the injection of nitrate. Intracoronary acetylcholine provoked diffuse spasm of the artery with concurrent myocardial lactate production. Coronary flow reserve assessed with papaverine was 2.75. The combined use of amlodipine and high-dose nicorandil was effective for the treatment of angina.
(Internal Medicine 41: 216-220, 2002)

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A 39-year-old man had been suffering from periodic fever since childhood. He was started on hemodialysis due to secondary amyloidosis on December 2000. The patient was believed to have Familial Mediterranean fever (FMF) because of recurrent fever with peritonitis, arthritis and inflammatory changes and secondary amyloidosis in his kidneys, heart and colon. No other family member had recurrent fever. IL-6, TNF, and dopamine β-hydroxylase were not increased in the febril phase. The patient was homozygous for the M694I mutation. We report the first Japanese case of FMF associated with amyloidosis and confirmed by a gene mutation.
(Internal Medicine 41: 221-224, 2002)

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A case of gastrointestinal cancer-associated antigen (CA19-9)-positive bronchioloalveolar carcinoma accompanied by bronchorrhea and respiratory failure successfully treated with corticosteroids is reported. The patient was treated with pulse methylprednisolone at a dose of 1, 000 mg/day for three days, followed by oral prednisolone (60 mg/day). Within 2 days, the sputum volume decreased from >100 ml/day to 20 ml/day and it was finally controlled to 0-10 ml/day. The reduction in the sputum volume was associated with alleviation of dyspnea and hypoxemia. The levels of CA19-9 in the serum and the sputum were extremely high and an immunocytochemical study showed that the tumor cells were stained by CA19-9 antibody. This case demonstrates the therapeutic value of corticosteroids in the treatment of bronchorrhea in subjects with bronchioloalveolar carcinoma.
(Internal Medicine 41: 225-228, 2002)

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This is a report of hemolytic anemia and respiratory failure due to Mycoplasma pneumoniae pneumonia. His chest CT scans showed bilateral diffuse thickened bronchovascular bundles and emphysema tons changes. The pulmonary function test supported the diagnosis of chronic obstructive pulmonary disease (COPD). He was diagnosed as coldagglunitin-associated hemolytic anemia and M. pneumoniae pneumonia in inapparent COPD. Corticosteroid administration was remarkablly effective for hemolytic anemia and beneficial for acute exacerbation of COPD.
(Internal Medicine 41: 229-232, 2002)

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A 45-year-old woman was hospitalized because of systemic edema and peripheral nerve impairment. The patient had complications of organomegaly, endocrinopathy, and monoclonal gammmopathy, and was diagnosed with PO-EMS syndrome based on these characteristic signs and symptoms. Interleukin-6 (IL-6) and vascular endothelial growth factor (VEGF) levels in the serum and ascitic fluid were high. Many of the patient's symptoms were ameliorated, and IL-6 and VEGF levels in the serum and ascitic fluid decreased slightly during chemotherapy, but she died of respiratory failure. Autopsy revealed severe systemic edema and macroscopic hemorrhage in many organs, but VEGF and IL-6 producing cells were not found.
(Internal Medicine 41: 233-236, 2002)

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A 27-year-old man developed gait disturbance over a three-year period. Histochemical analysis suggested nemaline bodies, type I fiber atrophy and type II fiber hypertrophy. Conventional magnetic resonance imaging (MRI) showed a severe degree of homogenous hyperintensity in the soleus muscle. Fat-suppression MRI exhibited marked diffuse hypointensity in the soleus muscle. MRIs demonstrated unusual fatty proliferation of the soleus muscle. Fat-suppression MRI of skeletal muscles is beneficial in evaluating the accurate topography and degree of fatty infiltration. Radiological patterns of muscle damage are variable in patients with congenital nemaline myopathy, similar to the heterogeneous clinical aspects.
(Internal Medicine 41: 237-240, 2002)

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A 62-year-old man was admitted to hospital with general malaise, arthralgia, edema, and high-grade fever. He was diagnosed as dermatomv ositis because of typical skin rashes and muscle weakness. His symptoms were improved by high-doses of prednisolone and cyclosporin A, with the exception of the skin rash over the back. High-grade fever developed again when tapering prednisolone. His condition deteriorated gradually. Paresthesia, hypothyroidism, metabolic acidosis, and disseminated intravascular coagulopathy occurred, and he died eight months after the first admission. Postmortem examination revealed intravascular proliferation of atypical mononuclear cells in the lumens of small vessels in all organs. Intravascular lymphomatosis (B cell type) was diagnosed.
(Internal Medicine 41: 241-244, 2002)

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