Internal Medicine

Hereditary Distal Dominant Amyotrophy Followed by Spastic Paraplegia

Nobuhiko SUNOHARA, Hideaki TOMI, Jun KISHIBAYASHI, Masakuni MUKOYAMA

1993 Volume 32 Issue 11 Pages 825-831
Published: 1993
Released on J-STAGE: March 27, 2006

DOIhttps://doi.org/10.2169/internalmedicine.32.825

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Clinical, neurophysiological and neuropathological investigations were performed on five patients from two families with autosomal dominant distal amyotrophy followed by spastic paraplegia and with a positive history in two generations of these two families. All cases in the two families had a benign clinical course, although two mothers could not walk without support at around 60 years old. Neurophysiological studies revealed normal maximum conduction velocities of peripheral sensory and motor nerves, and the central spinal sensory pathway. Distribution of motor nerve conduction velocities in the ulnar nerve had a normal pattern except for one patient who had severe deformities of the cervical vertebrae. The biopsied sural nerve disclosed no distinct abnormalities in any cases. From these results, we confirmed preservation of the myelinated nerve fibers of motor and sensory peripheral nerves.
(Internal Medicine 32: 825-831, 1993)

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Quality of Life Measurements Using a Linear Analog Scale for Elderly Patients with Chronic Lung Disease

Tomoko HIRATSUKA, Kozui KIDA

1993 Volume 32 Issue 11 Pages 832-836
Published: 1993
Released on J-STAGE: March 27, 2006

DOIhttps://doi.org/10.2169/internalmedicine.32.832

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We developed a linear analogue scale (QOL Scale) to measure the quality of life (QOL) in elderly patients with chronic lung disease. In this study, the validity of the QOL Scale was assessed and QOL Scale scores were compared with the results of other conventional questionnaires. A total of 76 subjects, aged 65 years or older, divided into three groups according to disease severity, were tested by the QOL Scale and two additional questionnaires. The QOL Scale had the advantages of comprehensibility, acceptability and reproducibility. QOL Scale scores differed among the groups, while the other questionnaires showed no significant differences according to disease severity. QOL Scale scores correlated with the tendency toward neurosis shown by another index. We conclude that the QOL Scale is a practical and useful indicator of QOL in elderly patients with chronic lung disease.
(Internal Medicine 32: 832-836, 1993)

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Assessment of Emaciation in Relation to Threat to Life in Anorexia Nervosa

Kenjiro OKABE

1993 Volume 32 Issue 11 Pages 837-842
Published: 1993
Released on J-STAGE: March 27, 2006

DOIhttps://doi.org/10.2169/internalmedicine.32.837

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Twenty patients with anorexia nervosa and a body weight below 60% of the standard weight were studied. One died of starvation; the others survived. Four patients, including the deceased, had such severe weakness that they could not sit up without support, and another five could sit up only from a lateral position. Serum albumin or hemoglobin levels at the beginning of therapy could not be used for nutritional assessment because of dehydration, while increased blood urea nitrogen was associated with acute illness. The present results together with data from previous studies of fatal anorexia indicate that the risk of mortality may be quite low when body weight is above 60% of the standard. We suggest that gross muscle weakness in addition to body weight for height can be a valuable indicator to assess the criticalness in anorexia nervosa.
(Internal Medicine 32: 837-842, 1993)

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Evaluation of Antibody against AR142, a Synthetic Peptide Derived from the Core Sequence of Hepatitis C Virus, in the Diagnosis of Non-A, Non-B Chronic Liver Disease

Lina DU, Hiroyuki SHIMOMURA, Hiroshi NAKAGAWA, Toshimi HASUI, Hideyuki ...

1993 Volume 32 Issue 11 Pages 843-848
Published: 1993
Released on J-STAGE: March 27, 2006

DOIhttps://doi.org/10.2169/internalmedicine.32.843

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We evaluated serum anti-hepatitis C virus (HCV) using a synthetic peptide (AR142) which includes an epitope in the core region of HCV. The incidence of anti-AR142 in 98 patients with type non-A, non-B chronic liver diseases (NANB-CLD)was 89.8%, while all the 28 patients with nontype C chronic liver diseases were negative for anti-AR142. Among 98 NANB-CLD patients, 74 were positive for both anti-AR142 and anti-C100-3, 23 showed discordant results, and one was positive for neither. Eighty-one NANB-CLD patients underwent reverse transcription-polymerase chain reaction assay to detect viremia and 76 (93.8%) had a detectable level of HCV-RNA. Titers of anti-AR142 were not different among groups of different disease activities, genotypes of HCV, nor amount of serum HCV-RNA. These observations suggest that anti-AR142 could be a useful marker for chronic HCV infection.
(Internal Medicine 32: 843-848, 1993)

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Histological Abnormalities in a Biopsy Specimen Obtained from the Left Ventricle without Reduced Function in a Patient with Right Ventricular Dysplasia

Kazuyoshi HINA, Shozo KUSACHI, Hideaki KANAI, Kunio NOGAMI, Kohichiro ...

1993 Volume 32 Issue 11 Pages 849-853
Published: 1993
Released on J-STAGE: March 27, 2006

DOIhttps://doi.org/10.2169/internalmedicine.32.849

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Left ventricular histological examination was performed in a patient with right ventricular (RV) dysplasia. Although the left ventricle (LV) appeared to have a normal shape and function, the LV biopsy specimen revealed apparent myocyte hypertrophy and substantial fibrotic changes without fatty infiltration. It was not clear whether these considerable histological changes were part of right ventricular dysplasia or not. The present case indicates that even with normal LV shape and function in patients with right ventricular dysplasia, hitological examination of LV may provide additional information on its pathogenesis and prognosis.
(Internal Medicine 32: 849-853, 1993)

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Disseminated Herpes Zoster with Multifocal Neurologic Involvement in an HTLV-1 Carrier

Naoki FUJII, Youichi ITOH, Hiroyuki TOMODA

1993 Volume 32 Issue 11 Pages 854-856
Published: 1993
Released on J-STAGE: March 27, 2006

DOIhttps://doi.org/10.2169/internalmedicine.32.854

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A 56-year-old previously healthy man suffered from an attack of disseminated herpes zoster, followed by multifocal neurologic involvement. The patient was a human T-cell lymphotropic virus type 1 (HTLV-1) carrier. HTLV-1 infection, even though normally asymptomatic, has been suggested to be one of the risk factors for severe herpes virus infection and its associated neurologic disorders.
(Internal Medicine 32: 854-856, 1993)

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Benign, Non-Parasitic Hepatic Cyst Causing Obstructive Jaundice

Nobuo TERADA, Toshiki SHIMIZU, Yasuharu IMAI, Takeshi KOBAYASHI, Masuo ...

1993 Volume 32 Issue 11 Pages 857-860
Published: 1993
Released on J-STAGE: March 27, 2006

DOIhttps://doi.org/10.2169/internalmedicine.32.857

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A 71-year-old woman with an hepatic cyst that led to obstructive jaundice is reported.Percutaneous intracystic fluid aspiration was useful in transiently decreasing the jaundice. Thereafter, the cyst was extirpated, and was found to be large (nearly 10 cm), benign and nonparasitic.This patient and 15 similar cases reported in the literature, indicate that the risk of jaundice induced by a simple hepatic cyst is related to its size (larger than 10 cm) and location near the common hepatic duct.
(Internal Medicine 32: 857-860, 1993)

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Unusual Inferior Vena Cava Obstruction Causing Extensive Thrombosis: An Intravascular Endoscopic Observation

Masato ARAO, Hiromichi OGURA, Takeshi INO, Kazuo MUNAKATA, Hiroshi KIS ...

1993 Volume 32 Issue 11 Pages 861-864
Published: 1993
Released on J-STAGE: March 27, 2006

DOIhttps://doi.org/10.2169/internalmedicine.32.861

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A 28-year-old, previously healthy man was referred to our hospital three months after the appearance of lumbago, leg pains and fever. Routine examinations were normal. Phlebography demonstrated occlusion of the common femoral veins by thrombosis. Inferior vena cavography and magnetic resonance image showed that the inferior vena cava (IVC) became gradually narrower from the level of 11th thoracic vertebra. An intravascular endoscopic study gave the same morphological findings; the endothelial surface was found to be intact. We concluded that the stenosis of the IVC was the primary lesion and was the essential pathological condition. The massive thrombosis appeared to have occurred secondarily.
(Internal Medicine 32: 861-864, 1993)

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β⁰-Thalassemia Trait (IVS-I-1 G→T) in a Japanese Family

Akihiro YOKOYAMA, Tsuyoshi NAKAMAKI, Kazunari YAMADA, Michiaki KOIKE, ...

1993 Volume 32 Issue 11 Pages 865-868
Published: 1993
Released on J-STAGE: March 27, 2006

DOIhttps://doi.org/10.2169/internalmedicine.32.865

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Three subjects in a family with microcytic and hypochromic anemia were studied; red blood cell morphology indicated aniso-poikilocytosis and hypochromasia. Target and tear-drop cells were also noted. In all three cases evaluated, there was an increase in HbA₂ levels and a decline in the β/α synthesis ratio. Direct cloning and DNA sequencing identified a point mutation (G→T) at position 1 of intervening sequence I. The resulting reduction of β-globin chain synthesis is considered to give rise to β⁰-thalassemia phenotype. This point mutation is to our knowledge, the first case in Japan.
(Internal Medicine 32: 865-868, 1993)

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Leukemoid Reaction Associated with Diabetic Ketoacidosis- With Measurement of Plasma Levels of Granulocyte Colony-Stimulating Factor

Tohru KAYASHIMA, Kohei YAMAGUCHI, Tatsujiro AKIYOSHI, Hiroshi NANIMATS ...

1993 Volume 32 Issue 11 Pages 869-871
Published: 1993
Released on J-STAGE: March 27, 2006

DOIhttps://doi.org/10.2169/internalmedicine.32.869

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A 40-year-old man with diabetic ketoacidosis showed marked leukocytosis reaching 60, 970/mm³ which subsided within 5 days to a level of4, 140/mm³. He had no evidence of infection. During the entire clinical course, no elevation of granulocyte colony-stimulating factor (G-CSF) was observed. No case of leukemoid reaction associated with diabetic ketoacidosis has been reported with the determination of plasma G-CSF levels.
(Internal Medicine 32: 869-871, 1993)

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Pasteurella ureae Endocarditis

Keiji YAMAMOTO, Uichi IKEAD, Chuhei OGAWA, Hiroshi FUKAZAWA, Masato ET ...

1993 Volume 32 Issue 11 Pages 872-874
Published: 1993
Released on J-STAGE: March 27, 2006

DOIhttps://doi.org/10.2169/internalmedicine.32.872

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Pasteurella ureae is found in the normal human respiratory flora. We encountered a case of endocarditis caused by Pasteurella ureae. The patient was a 59-year-old man with a history of Staphylococcus aureus endocarditis. After treatment with antibiotics, blood cultures became negative, and the patient recovered completely. The incidence of endocarditis due to this organism is very rare; this case is the second clinically diagnosed case report.
(Internal Medicine 32: 872-874, 1993)

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Malignant Exophthalmos Associated with Multiple Myeloma

Akira SEYA, Takashi TERANO, Yoko HATTORI, Takuya TOMIZUKA, Hiroshi MOR ...

1993 Volume 32 Issue 11 Pages 875-878
Published: 1993
Released on J-STAGE: March 27, 2006

DOIhttps://doi.org/10.2169/internalmedicine.32.875

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We report a patient with malignant exophthalmos associated with multiple myeloma which showed no evidence of direct orbital involvement of plasma cells. This exophthalmos had similarities with Graves' ophthalmopathy, but the patient had no detectable autoimmune thyroid diseases. Plasmapheresis was effective not only for the treatment of heart and renal failure due to the myeloma kidney but also for the malignant exophthalmos. As the serum monoclonal IgG level was decreased by plasmapheresis, the improvement of proptosis, visual acuity, and hypertrophy of the extraocular muscle as measured by magnetic resonance imaging were observed. It is suggested that humoral factors removed by plasmapheresis might be involved in the pathogenesis of this nonendocrine exophthalmos.
(Internal Medicine 32: 875-878, 1993)

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Bleeding Tendency Caused by the Deposit of Amyloid Substance in the Perivascular Region

Yoshiaki HOSHINO, Kiyohiko HATAKE, Kazuo MUROI, Saburo TSUNODA, Toshiy ...

1993 Volume 32 Issue 11 Pages 879-881
Published: 1993
Released on J-STAGE: March 27, 2006

DOIhttps://doi.org/10.2169/internalmedicine.32.879

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A 55-year-old woman, who had systemic amyloidosis associated with multiple myeloma, had sudden development of hematomas of her lip and upper eye lid. There was no evidence of deterioration of multiple myeloma, thrombocytopenia nor deficiency of coagulation factors. Biopsy specimen showed the deposit of amyloid substance in the dermis and perivascular region. The bleeding tendency in this patient with myeloma was likely due to the deposit of amyloid substance in the vascular wall; improvement was achieved with administration of hemostatic agents.
(Internal Medicine 32: 879-881-1993)

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Aggressive Granular Lymphocyte Leukemia of Natural Killer Cell Type in an Elderly Patient

Naoaki ICHIKAWA, Kiyoshi KITANO, Makoto HIGUCHI, Hikaru KOBAYASHI, Nob ...

1993 Volume 32 Issue 11 Pages 882-885
Published: 1993
Released on J-STAGE: March 27, 2006

DOIhttps://doi.org/10.2169/internalmedicine.32.882

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A 75-year-old man was admitted to our hospital because of intermittent fever. His peripheral blood picture showed granular lymphocyte (GL) proliferation. The GLs were immunologically and functionally phenotyped as natural killer cells. Chromosomal analysis of peripheral lymphocytes with interleukin-2 stimulation revealed an inversion of chromosome 9 with an unusual breakpoint, showing abnormal monoclonal proliferation of the GLs. Progressive increase of GL count and hepatosplenomegaly necessitated the start of combined chemotherapy. His condition was complicated by icterus and renal failure, and he died finally of respiratory failure. Autopsy revealed disseminated intravascular coagulation and infiltration of GLs in the bone marrow, spleen, and liver.
(Internal Medicine 32: 882-885, 1993)

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Increased 1, 25-(OH)₂D₂ Concentration in a Patient with Malignancy-Associated Hypercalcemia Receiving Intravenous Hyperalimentation Inadvertently Supplemented with Vitamin D₂

Kanji SATO, Toshihiro IMAKI, Shozo TORAYA, Hiroshi DEMURA, Masato TANA ...

1993 Volume 32 Issue 11 Pages 886-890
Published: 1993
Released on J-STAGE: March 27, 2006

DOIhttps://doi.org/10.2169/internalmedicine.32.886

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A 55-year-old patient with hypercalcemic crisis due to gastric carcinoma with bone marrow metastasis was treated with bisphosphonate (pamidronate) and calcitonin. Urinary excretion of parathyroid hormone-related protein (PTHrP) was increased. When normocalcemia had been attained, intravenous hyperalimentation was started, in which 1, 000 U vitamin D₂ was inadvertently supplemented on days 5-18, On days 15-18, hypercalcemia rapidly recurred, accompanied by markedly increased serum levels of 25-OHD₂ (9.1 ng/dl) and 1, 25-(OH)₂D₂ (161 pg/ml). This clinical course suggests that PTHrP, like PTH, stimulated 1α-hydroxylase activity and produced excessive 1, 25-(OH)₂D₂. Vitamin D should not be administered to patients with malignancyassociated hypercalcemia, particularly that due to PTHrP-producing tumors.
(Internal Medicine 32: 886-890, 1993)

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