This disease is characterized by initially progressive muscular weakness and wasting of the distal upper limb(s) in young people predominantly in men, followed by a spontaneous arrest within several years. This disease has been thought to be separate from motor neuron diseases, yet some authors still consider the illness a variant of motor neuron disease. However, the pathological evidence of ischemic changes in the lower cervical anterior horn should facilitate differentiation of the disorder from degenerative motor neuron disease. Recent radiological investigations proved compressive flattening of the lower cervical cord due to forward displacement of the cervical dural sac and spinal cord induced by neck flexion. These findings suggest that sustained or repeated neck flexion may cause ischemic changes in the cervical anterior horn. Application of a cervical collar to minimize neck flexion prevents progressive muscular weakness in an early stage of the disease.
(Internal Medicine 39: 283-290, 2000)

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Objective Although tumor marker levels in blood become elevated after hemodialysis as a result of the amount of fluid removed, serum squamous cell carcinoma-related antigen (SCC) levels have not been always reported to increase after hemodialysis. The purpose of this report is to determine whether there is a difference between the change of serum SCC levels before and after hemodialysis according to the model of dialyzer employed.
Patients and Methods In ninety-four patients on hemodialysis (50 cases of diabetic nephropathy and 44 cases of glomerulonephritis), we examined serum SCC levels before and after hemodialysis.
Results There was no overall difference between SCC levels before and after hemodialysis (3.2±1.5 ng/ml vs 3.3±1.7 ng/ml, p=0.2381). In patients treated with a cellulosic type membrane dialyzer (n=73), SCC levels after hemodialysis were higher than those before hemodialysis (3.7±1.7 ng/ml vs 3.5±1.5 ng/ml, p=0.0495). In patients treated with a synthetic type membrane (n=21), SCC levels after hemodialysis decreased when compared to those before hemodialysis (1.9±0.7 ng/ml vs 2.2±0.8 ng/ml, p=0.0018) and in all patients, the SCC levels after hemodialysis were lower than, or equal to, those before hemodialysis.
Conclusion The results suggest that the decline in serum SCC levels during hemodialysis treated with synthetic type membrane of dialyzers, concealed the increased SCC levels in hemoconcentration.
(Internal Medicine 39: 291-295, 2000)

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Objective To clarify the clinical features of MS patients with hyperprolactinemia.
Subjects and Methods The serum prolactin level was measured in 67 Japanese patients (19 men and 48 women) with multiple sclerosis (MS) and in 16 patients (4 men and 12 women) with HTLV-I-associated myelopathy/tropical spastic paraparesis (HAM/TSP) using a two-site immunoradiometric assay.
Results In the MS patients, 32 were classified as having Asian type MS showing a selective involvement of the optic nerves and spinal cord, while the other 35 were classified as having Western type MS which displayed disseminated central nervous system involvement. In women, the serum prolactin level was found to be significantly higher only in Asian type MS (mean=23.1 ng/ml, n=25) than in HAM/TSP (mean=6.9 ng/ml, n=12) (p=0.0297), while it did not differ significantly in men among the three groups. Hyperprolactinemia was significantly associated with acute relapse involving the optic nerves. All MS patients with hyperprolactinemia (7 women with Asian type MS and 2 women with Western type MS) showed recurrent opticomyelitis either throughout or in the early course of the disease, and also had a higher age of onset, a higher Expanded Disability Status Scale score, a greater visual impairment, and higher cell counts and protein contents in the cerebrospinal fluid than did the normoprolactinemic patients.
Conclusion Hyperprolactinemia may be one of the characteristic features of Asian patients with MS who preferentially show the optic nerve involvement.
(Internal Medicine 39: 296-299, 2000)

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Recently, telomerase activity has been demonstrated in a large number of malignant tumors whereas its activity is not detected in most normal somatic cells suggesting its role in the immortalization process. Here we report the first investigation of telomerase activity in a case of gastric mucosa-associated lymphoid tissue (MALT) lymphoma. Elevated telomerase activity was detected in biopsy specimens of the lymphoma. After eradication of Helicobacter pylori, the level of telomerase activity returned to normal with histological regression of the lymphoma. The telomerase activity was associated with the disease activity of the gastric MALT lymphoma after eradication therapy in the present case.
(Internal Medicine 39: 300-304, 2000)

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We herein describe a 70-year-old male patient who developed colonic necrosis following severe acute pancreatitis. He was referred to our hospital with a diagnosis of acute pancreatitis. In the course of the disease, he developed sudden and massive hematochezia and died. The autopsy findings revealed large bowel ischemia with transmural infarction. The possible pathogenic mechanisms of colonic ischemia are also discussed.
(Internal Medicine 39: 305-308, 2000)

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We describe a rare case of pulmonary mycobacteriosis infected with rifampicin (RFP)-resistant Mycobacterium szulgai that was successfully eradicated with clarithromycin (CAM) treatment. An 80-year-old man was admitted to our hospital with a 4-week history of high fever, productive cough and malaise. Chest roentgenogram showed an infiltrative shadow in the left lower lung field. Isolated bacteria from sputum were acid-fast bacilli and identified as M. szulgai by the DNA-DNA hybridization method. Drug susceptibility tests showed resistance to RFP (MIC>100 μg/ml). Combined treatment with ethionamide, streptomycin and isoniazid based on the results of drug susceptibility tests resulted in clinical and radiologic improvement within two years. Additional treatment with CAM for another year resulted in complete eradication of the mycobacterium.
(Internal Medicine 39: 309-312, 2000)

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The MRI findings of amyloid arthropathy associated with primary amyloidosis are presented here possibly for the first time in the literature. Two types of lesions are noted: (1) capsular and tendon lesions; these regions are thickened, hypointense and enhanced by gadolinium (Gd) on Tl weighted imaging (Tl WI), and hyperintense on T2 weighted imaging (T2WI), and (2) periarticular and osseous lesions; these regions appear to be tumor-forming and hypointense on both T1WI and T2WI and are not enhanced by Gd. It is necessary to differentiate these findings from other diseases such as chondrosarcoma, rhabdomyosarcoma and chronic inflammatory lesions such as tuberculosis.
(Internal Medicine 39: 313-319, 2000)

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Systemic lupus erythematosus (SLE) is sometimes reported to complicate fatal pulmonary hypertension. A 46-year-old woman, with a ten-year history of SLE and pulmonary hypertension, was admitted to our hospital complaining of dyspnea and chest pain. She suffered pulmonary hemorrhage and after steroid pulse therapy, she underwent continuous intravenous infusion of epoprostenol (prostaglandin I₂) with corticosteroid for four weeks, which reduced the pulmonary artery pressure and resistance. Following the successful treatment, beraprost sodium, an oral PGI₂ analogue, was given and it maintained pulmonary hypertension remittance for four years.
(Internal Medicine 39: 320-323, 2000)

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