We examined the cerebrospinal fluid findings, including the activity of the myelin-associated enzyme 2', 3'-cyclic nucleotide 3'-phosphohydrolase (CNP), and virus serology in patients with Bell's palsy. Eighty-nine of 164 patients showed hematological and/or serological evidence of an inflammatory reaction. Examinations of the CSF disclosed an elevated total protein level and pleocytosis in 33% and 10% of the 91 patients, respectively. The activity of CNP was significantly elevated in the patient group (15.5±3.8nmol/h/ml) as compared with the control (13.2±1.3nmol/h/ml) (p < 0.01), suggesting the presence of intrathecal myelin breakdown. Furthermore, there was an association between antibodies against herpes simplex virus (HSV) and elevated CNP activity. The inflammatory alterations and accompanying spinal fluid abnormalities, together with the high frequency of HSV antibodies suggest that HSV-associated demyelination may play a pathogenetic role in Bell's palsy.
(Internal Medicine 31 : 1250-1256, 1992)

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Patients with progressive spastic paraparesis (PSP), commonly middle-aged women, are distributed throughout the country of Chile. During the three years from 1987 to 1990, we collected 83 cases of PSP from among 225 patients with various neurological diseases. The clinical picture was of a bilateral pyramidal syndrome, with sensory deficits in only 15.5% of the cases, and a slow illness progression in the majority of them. In patients with PSP, antibody to human T-cell leukemia virus type I (HTL V-I) was analyzed by enzyme linked immunosorbent assay (ELISA) and confirmed by western blot analysis. Forty-five (54.2%) patients were anti- HTLV-I antibody positive in cerebrospinal fluid (CSF) and peripheral blood. Among them, 2 patients had leukemia/lymphoma and one had Sjögren syndrome. In the laboratory study of seropositive PSP, mononuclear pleocytosis was found in 35.7% ; there was an abnormal increase of the IgG index in 66.6% and an increase in CD2 in blood and CSF, and CD4in blood. A delayed latency of somatosensory evoked potentials was observed in 90.9%. The neuropsychological study revealed a WAIS with a mean verbal IQ of 80.7 and a mean performance IQ of 84.8. The most impaired items were digit symbol and digit span. Seven subjects (18.9%) with anti-HTL V-I antibody were found among 37 relatives from 19 anti-HTL V-I positive cases of PSP.
(Internal Medicine 31 : 1257-1261, 1992)

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We report a case of hypereosinophilic syndrome (HES) with marked eosinophilia (59.7%) and mononeuritis multiplex (upper limbs and buttocks). Necrosis of the finger tips was the primary manifestation which simultaneously occurred on both sides. These clinical manifestations were improved dramatically by subsequent steroid therapy. Interestingly, an elevation of serum tumor necrosis factor (TNF) was observed. These findings suggest that TNF may play a role in the etiology of necrosis of the finger tips.
(Internal Medicine 31 : 1262-1266, 1992)

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A 61-year-old man, who suffered from Charcot-Marie-Tooth disease (CMT) for 44 years, was evaluated for the respiratory disorder. He had diaphragmatic dysfunction induced by phrenic nerve disturbance. In this patient, central type apnea and hypopnea related to diaphragmatic weakness occurred during REM sleep, which induced accessory inspiratory muscle inhibition. Respiratory muscle dysfunction had not been generally recognized in CMT until recently, but its significance should be emphasized.
(Internal Medicine 31 : 1267-1270, 1992)

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A 14-year-old girl diagnosed as Crohn's disease had lymphoid hyperplasia throughout the large bowel. Biopsy specimens from the lymphoid hyperplasia demonstrated non-caseous granulomas within the lymph follicles. An immuno-histochemical study of biopsy specimens showed that HLA-DR antigens were expressed on epithelium close to the lymph follicle, which was observed in all specimens containing the lymph follicle. This case provides evidence for the importance of the lymph follicle in the etiopathogenesis of Crohn's disease by demonstration of non-caseous granulomas within the lymph follicle and expression of HLA-DR antigens on epithelium close to the lymph follicle.
(Internal Medicine 31 : 1271-1276, 1992)

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A 65-year-old male patient developed acquired chronic pure red cell aplasia (PRCA) associated with hypergammaglobulinemia and positive Coombs' test during the treatment of eosinophilic pneumonia with prednisolone (PSL). His PRCA was treated with oral PSL at a dose of 60mg/day for 3 weeks, but anemia further progressed. Immediately after high-dose intravenous pulse methylprednisolone therapy (1g/day for 3 days) however, reticulocyte crisis ocurred and his anemia rapidly improved. He has been in complete remission under a maintenance dose of PSL for more than 2 years. This patient indicates that high-dose intravenous methylprednisolone therapy is one of the useful treatments, not only for constitutional PRCA, but also for acquired chronic PRCA.
(Internal Medicine 31 : 1277-1280, 1992)

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A 32-year-old man was admitted to our hospital because of lumbago and an abdominal mass revealed by abdominal ultrasonography. Abdominal CT scan and MRI revealed multiple para-aortic lymph node swelling involving several arteries and veins. As there was no superficial lymph node swelling, percutaneous lymph node biopsy was performed under ultrasonographic guide. Although non-Hodgkin's lymphoma, diffuse, small cell type was suspected by light microscopic study, the monoclonality of the lymphocytes in the obtained specimen was not clear by the immunohistochemical study. Southern blot hybridization analysis of the biopsy specimen revealed the rearrangement of IgH and IgL (λ) chain gene, indicating the existence of monoclonal proliferation of lymphoma cells. The DNA analysis appears useful for the differential diagnosis of lympho-proliferative diseases.
(Internal Medicine 31 : 1281-1285, 1992)

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A 60-year-old man complaining of diplopia and vertigo showed bilateral medial longitudinal fasciculus (MLF) syndrome. The CT scan revealed a space-occupying lesion with watery fluid in the left cranial fossa, which was divided into two parts by a thin septum. Surgical trepanation was performed followed by 4 weeks of prednisolone therapy. He was completely cured 5 months later. The plausible causes of MLF syndrome relevant to preexisting space-occupying lesions are discussed.
(Internal Medicine 31: 1286-1290, 1992)

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A 20-year-old woman with a primary angiosarcoma of the right atrium is reported. The patient had a cardiorespiratory arrest due to cardiac tamponade with bloody pericardial effusion. Magnetic resonance imaging (MRI) revealed a tumor, which was corroborated by selective coronary angiography. Open-heart surgery was performed. The tumor relapsed however, and she died four months after operation. The tumor was undetectable by echocardiography, but MRI demonstrated a heterogeneous mass with focal areas of high- and low-signal intensity in the right atrium, suggesting that MRI may allow characterization of cardiac tumors.
(Internal Medicine 31 : 1291-1297, 1992)

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After 9 years of treatment for Parkinson's disease, a 68-year-old woman developed the complications of neuroleptic malignant syndrome (NMS) and disseminated intravascular coagulation (DIC) while she was still receiving levodopa, bromocriptine and amantadine hydrochloride. The patient displayed a high fever (40°C), impaired consciousness, marked systemic muscle rigidity, tremor and bloody stools. The diagnosis of NMS and DIC was made on the basis of the symptoms and the results of blood serological tests. The antiparkinsonian drugs that had been administered until her admission to our hospital were continued unchanged, while the NMS was treated with dantrolene sodium and the DIC, with nafamostat mesilate. Both of the above-mentioned therapies were effective. The present case is rare in that the patient developed NMS and DIC during treatment and not after the discontinuation of the antiparkinsonian drugs.
(Internal Medicine 31 : 1298-1302, 1992)

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A 14-year-old female developed systemic lupus erythematosus (SLE)-like symptoms, rash, fever, leukopenia and positive anti-nuclear antibody (ANA) two weeks after administration of carbamazepine (CBZ ; Tegretol^®) used against benign Rolandic epilepsy. Clinical symptoms and leukopenia normalized after discontinuation of CBZ and administration of prednisolone at 40mg. The cases of CBZ-induced SLE reported in the literature were reviewed.
(Internal Medicine 31 : 1303-1305, 1992)

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A 56-year-old woman presented with severe iron-deficiency anemia and memory distur-bance. She had been in a state of severe iron deficiency for many years due to an unbalanced diet. Aerobic exercise test revealed an abnormal elevation of lactate and pyruvate reflecting mitochondrial dysfunction. After iron replacement therapy, WAIS verbal IQ score improved from 63 to 83, and levels of lactate and pyruvate on aerobic exercise test were normalized. Weraise the possibility that severe and long-term iron deficiency anemia may cause memory disturbance due to mitochondrial dysfunction.
(Internal Medicine 31 : 1306-1309, 1992)

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It has been reported that microangiopathic hemolytic anemia occasionally occurs in patients with severe diabetic microangiopathy. We report a case of insulin-dependent diabetes mellitus in whom microangiopathic hemolytic anemia and chronic disseminated intravascular coagulation (DIC) were thought to be complicated. The patient showed fragmentation hemolytic anemia and progressive diabetic microangiopathy, together with a mild elevation of serum fibrin (ogen) degradation products. Considering the state of chronic DIC, heparin therapy was started, but mild hemolysis persisted. It is possible that microangiopathic hemolytic anemia and chronic DIC cause a vicious cycle in patients with severe diabetic microangiopathy, leading to rapid progression of diabetic microangiopathy.
(Internal Medicine 31 : 1310-1312, 1992)

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We report two aged patients with Guillain-Barré syndrome (GBS), in whom the rapid progression necessiated the use of a respirator. The first case, a 72-year-old woman, needed a respirator on the 5th day after onset of illness and the second case, a 74-year-old woman, needed a respirator on the 23rd day. We treated the first patient with plasmapheresis ; the second patient underwent plasmapheresis as well as a large dose of intravenous methylprednisolone. Both patients showed remarkable recovery and did not need the respirator from the early stages. It was suggested that plasmapheresis is beneficial for treatment of aged patients with severe GBS, who necessiate the support of a respirator, because it prevents the decline in functional status by shortening the period of hospitalization.
(Internal Medicine 31 : 1313-1316, 1992)

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A 26-year-old female patient developed high fever, hepatosplenomegaly and subcutaneous nodules. Atypical lymphocytes were present in the peripheral blood. Skin biopsy revealed lobular panniculitis. Bone marrow examination showed prominent phagocytosis by benign histiocytes. The diagnosis of cytophagic histiocytic panniculitis was made, and the disease has been well controlled with oral prednisolone. This is the first report of cytophagic histiocytic panniculitis with atypical lymphocytes in the peripheral blood, which are frequently seen in virus-associated hemophagocytic syndrome. This observation suggests that underlying viral infection may be one factor in the development of cytophagic histiocytic panniculitis.
(Internal Medicine 31 : 1317-1321, 1992)

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Ganglioneuroblastoma is an extremely rare neoplasm of adults. We present a case of adrenal ganglioneuroblastoma in a 47-year-old female with disseminated bone marrow involvement. Bone marrow involvement was the major manifestation which determined the mortality of this patient. This case suggests that the diagnosis of bone marrow involvement should be carefully considered even in adult-onset ganglioneuroblastoma.
(Internal Medicine 31 : 1322-1324, 1992)

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A case of a 6Q-year-old Japanese woman with small cell lung cancer involving skin and stomach is reported. She was diagnosed as primary small cell lung cancer accompanied by extensive cutaneous metastases. Three months after the last chemotherapy, she complained of nausea and vomiting. Brain CT scan showed no evidence of central nervous system involvement. Upper gastrointestinal study and upper gastrointestinal fiberscopy revealed multiple metastatic gastric tumors. Skin and stomach are uncommon metastatic sites for any malignancy. Furthermore, only a few cases with gastric metastasis could be diagnosed during their lifetime.
(Internal Medicine 31 : 1325-1328, 1992)

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A 23-year-old male patient revealed hypercortisolism with stigmata of Cushing's syndrome, and post-operative pathological examination demonstrated primary adrenal nodular dysplasia. Because of the presence of cardiac myxomas and skin pigmentation, the diagnosis of Carney's complex was given. After the control of hypercortisolism by adrenalectomy, the patient experienced iridocyclitis and bilateral hilar lymphadenopathy with elevated levels of serum angiotensin-converting enzyme and lysozyme, all of which indicated the presence of sarcoidosis. Despite the numerous recent descriptions concerning Carney's complex, an association with sarcoidosis has not yet been documented. Moreover, the sequential occurrence of sarcoidosis after adrenalectomy suggests an etiological link between these two rare disorders.
(Internal Medicine 31 : 1329- 1334, 1992)

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