[Liver function of the most elderly patients].

To determine the decision level of liver function in the most elderly patients, we compared serum albumin, aspartate aminotransferase(AST), alanine aminotransferase(ALT) and alkaline phosphatase(ALP) values of the most elderly patients > 85 years with those of healthy young adults. Two hundred fifty five elderly people, aged 88-106 years and average 96.6 years(171 women, 84 men), were included in this study. Elderly people were divided into four groups according to their activities of daily living(ADL), 114 Rank-J: free living, 62 Rank-A: unable to go outside without help, 39 Rank-B: bedridden but able to sit up in bed and 40 Rank-C: completely bedridden. Serum albumin values for the most elderly patients in Rank-J were 4.2 +/- 0.3 g/dl for women and 4.0 +/- 0.3 g/dl for men, showing marked decrease from those of young healthy adults aged 19-59 years(p < 0.0001). In 22.2% of elderly women and 44.2% of elderly men, albumin values deviated from the reference interval of young adults. ALT value for the most elderly patients also showed a decrease in both sexes and AST and ALP values for the most elderly patients showed an increase in women compared with young adults. However, these were minor deviations from the reference interval for young adults. In ADL-stratified groups of the most elderly patients, serum albumin values showed marked decrease with decline in ADL, whereas AST, ALT and ALP values remained constant in both sexes regardless of ADL.

[Serum globulin levels and activities of daily living in centenarians].

Serum globulin levels in 101 centenarians (77 women, 24 men) were analyzed by zonal electrophoresis and immunoglobulin assays. Six subjects showed an M band in electrophoretograms. Five M bands were immunoglobulin G (IgG) type and one was IgA. One subject was suspected to suffer from IgG-type multiple myeloma, and the others were thought to have essential (benign) monoclonal gammopathy. The relationship between serum globulin levels and activities of daily living (ADL) was studied in remaining 95 subjects. There were 21 rank-J (free-living), 25 rank-A (unable to go outside without help), 23 rank-B (bedridden but able to sit on the bed), and 26 rank-C (completely bedridden) centenarians. The mean serum alpha 1-, alpha 2-, and beta-globulin level in the rank-J centenarians was at the upper limit of the normal adult range. The mean alpha 1-globulin levels in rank-B (3.3 +/- 0.4%) and C (3.4 +/- 0.5%) subjects were significantly higher than those in rank-J (2.9 +/- 0.3%) centenarians. The mean serum gamma-globulin level of rank-J centenarians (16.9 +/- 3.7%) was within the normal range. The mean gamma-globulin levels in rank-B (21.0 +/- 2.7%) and C (22.8 +/- 4.9%) subjects were significantly higher than that in rank-J centenarians. The mean immunoglobulin G concentrations in rank-B (1.620 +/- 304 mg/dl) and C (1.720 +/- 392 mg/dl) subjects were significantly higher than those in rank-J centenarians (1.320 +/- 361 mg/dl) and in normal adults (1.150 +/- 235 mg/dl). IgA concentrations in all centenarians were higher than in normal controls. There was no significant difference in IgA or IgM concentration between rank-J subjects and those in other ranks. Serum globulin levels measured with electrophoresis, and the immunoglobulin concentrations, can be valuable indicators of the ability to perform activities of daily living, and may be useful predictors of subclinical diseases or morbidity in centenarians.

[Giant cell arteritis with bilateral obstruction of the internal carotid artery--report of an autopsy case].

Internal carotid artery involvement and dementia occur infrequently in patients with giant cell (temporal) arteritis. A 75-year-old woman admitted with progressive cognitive decline, drowsiness and headache was diagnosed as having giant cell arteritis by temporal artery biopsy (TAB). High dose corticosteroid improved inflammatory reaction but did not improve his cognitive function. Cerebral angiograms showed obstruction of both internal carotid arteries at the siphon. Brain MRI showed only small cerebral infarcts in the basal ganglia and corona radiata bilaterally. However, brain SPECT disclosed reduced cerebral blood flow in the frontal lobe bilaterally. A postmortem examination revealed bilateral parietal infarcts and isolated giant cell arteritis involving the both internal carotid arteries at the siphon. We speculated that perfusion insufficiency and multiple cerebral infarction due to bilateral internal carotid artery occlusion had caused this neurologic deterioration.

[Serum albumin level and activities of daily living in centenarians].

The relationship between serum albumin level and activities of daily living was studied in 95 centenarians. There were 73 women (12 rank J: free-living, 18 rank A: unable to go outside without help, 20 rank B: bedridden but able to sit on the bed, 23 rank C: completely bedridden) and 22 men (9 rank J, 7 rank A, 6 rank B or C). The serum albumin level (mean +/- S.D. 4.0 +/- 0.4 g/dl) of the rank J women was at the lower limit of normal for young adults. The albumin levels of rank A, rank B, and rank C were 3.7 +/- 0.4 g/dl, 3.5 +/- 0.3 g/dl, and 3.4 +/- 0.4 g/dl, respectively. The levels of rank B and rank C women were significantly lower than that of rank J women. The albumin level of rank J men (3.9 +/- 0.3 g/dl) was lower than that of young adults. The albumin level of ranks B and C men (3.1 +/- 0.3 g/dl) was significantly lower than that of rank J men. The A/G ratio or albumin fraction (%) measured by serum electrophoresis was similar to that of the serum albumin level of centenarians of both sexes. There were no significant differences in the serum protein level or in the peripheral hemoglobin level between rank J centenarians and those of other ranks, for both sexes. The serum albumin level is a valuable indicator of the ability to perform activities of daily living and may be a useful prognostic index in centenarians.

Effect of extracranial carotid artery stenosis and other risk factors for stroke on periventricular hyperintensity.

BACKGROUND AND PURPOSE: The pathogenesis of periventricular hyperintensity (PVH) is still uncertain. We investigated the relationship between PVH and risk factors for cerebrovascular diseases, especially extracranial carotid artery stenosis (ECAS). METHODS: We studied PVH and ECAS in 323 subjects between 1991 and 1994. Using 1.5-T MRI scan images, we measured PVH quantitatively at eight points and evaluated cerebral infarction. Duplex carotid sonography was performed on the carotid arteries bilaterally and used to divide the severity of ECAS into five grades. Risk factors for cerebrovascular diseases and atherosclerotic complications were assessed from the clinical history. RESULTS: Age was significantly correlated with the size of frontal and whole PVH (P < .01). Frontal PVH was significantly more severe in subjects with hypertension (P < .05). Frontal, occipital, and whole PVH were significantly more severe in subjects with a history of cerebrovascular accident (P < .01). Other risk factors and atherosclerotic complications were not correlated with PVH. There were no significant differences in the severity of PVH among the five groups of ECAS. The severity of PVH in each region was not related to ECAS. There was no significant difference in the age of patients in relation to the five grades of ECAS. However, PVH was significantly more severe in subjects with lacunar infarction or infarction of the deep border zone (P < .05). There was no relationship between PVH and cortical infarction or infarction of the cortical border zone. CONCLUSIONS: PVH correlated with age, hypertension, and past history of cerebrovascular disease but not with ECAS. PVH was significantly more severe in lacunar infarction and infarction of the deep border zone. These results suggest that small-vessel disease may underlie the pathogenesis and development of PVH.

Aging and superoxide dismutase activity in cerebrospinal fluid.

We investigated superoxide dismutase (SOD) activity in human cerebrospinal fluid (CSF) as an index of the aging process in the central nervous system (CNS). The subjects were 61 individuals aged 21-77 years, comprising 24 men and 37 women without organic disorders of the nervous system. SOD activity in CSF was measured by the nitrite method modified by Oyanagui. The results showed that SOD activity in CSF gradually increased with age and that the values of SOD activity after the fifth decade were significantly higher than those in the third and fourth decades. It might suggest that the productivity of SOD in the CNS gradually increased with age due to stimulation of various types of oxidative stress which accumulated in vivo especially after the fifth decade.

Yolk sac tumor of the liver combined with hepatocellular carcinoma.

Hepatic yolk sac tumor is extremely rare, and only nine cases have been reported previously to our knowledge. We report the occurrence of a tumor combining hepatocellular carcinoma and yolk sac tumor. The clinical, pathologic, and immunohistochemical findings are presented. The patient was a 62-year-old Japanese man who died of hepatic failure and uncontrolled ascites. On autopsy, a large hepatic tumor was observed, which microscopically was composed of hepatocellular carcinoma and yolk sac tumor. The two components were intermingled, and transition zones were evident. Immunohistochemically, both components were positive for alpha-fetoprotein, but only the yolk sac tumor component was positive for carcinoembryonic antigen, epithelial membrane antigen, stage specific embryonic antigen-1, placental alkaline phosphatase, and keratin. To our knowledge, this is the first reported case of hepatic yolk sac tumor associated with hepatocellular carcinoma. The present tumor is best considered a variant of hepatocellular carcinoma showing yolk sac differentiation, and it provides another explanation for the histogenesis of extragonadal yolk sac tumor.

[Lymphomatoid granulomatosis of the brain with multiple lesions on MRI].

A 28-year-old man was admitted to our hospital, because of double vision, memory disturbance and dysarthria. These symptoms developed in November, 1994. His mental activity was gradually decreased and he became apathetic. A physical examination on admission was unremarkable. There were no lymphadenopathy, hepatosplenomegaly or skin rash. Neurological examination revealed disorientation, decreased mental activity and left gaze-evoked nystagmus. Neuropsychological tests demonstrated impairment of recent memory and performance but frontal lobe functions were maintained. Laboratory data showed hyponatremia, elevation of antinuclear antibodies and IgG-antibodies to Epstein-Barr virus capsid antigen and Epstein-Barr virus nuclear antigen. A brain MRI revealed multiple lesions in the cerebral subcortex, cerebellum and brainstem. These lesions showed high signal intensity on T2-weighted images and enhanced high signal intensity on T1-weighted images using Gd-DTPA injection. A cystic mass was seen between bilateral lateral ventricles and contacted with the fornix and thalamus. Lymphomatoid granulomatosis was diagnosed by an open brain biopsy, which showed diffuse infiltration of small lymphocytes, histiocytes and plasma cells, especially around the small vessels. Epithelioid granuloma or necrosis was not seen. An immunohistochemical study showed that nearly all lymphocytes were MT-1 positive T lymphocytes. The patient was treated by whole brain irradiation (30Gy) and pulse therapy of methylprednisolone. Multiple lesions in the brain responded to these modalities with complete resolution of the lesions and clinical improvement. In this case, impressive multiple and cystic lesions seen on brain MRI disappeared by whole brain irradiation and steroid. Since etiologies of multiple lesions on MRI includes many possibilities, histological diagnostic methods should be performed to obtain a definitive diagnosis. This is the first case of lymphomatoid granulomatosis that showed multiple and cystic lesions on brain MRI in Japan.

[Bacterial intracavernous carotid aneurysm presented as massive epistaxis].

A 55-year-old man was admitted with complaints of headache, diplopia, fever and swelling of the right eyelid. Physical examination revealed low grade fever of 37.6 degrees C, but no evidence of infectious focus was detected. The neurological examination disclosed right abducens nerve palsy. An initial routine blood count showed an increased white blood cell count of 11,800/microliters. The erythrocyte sedimentation rate was 93mm and the C-reactive protein level was 3.6mg/dl. Massive epistaxis and hypovolemic shock developed on the 14th day while the patient was receiving antibiotics (CEZ, CMZ) for a possible intracranial infection. The magnetic resonance angiography of the brain and the cerebral angiography demonstrated an aneurysm within the cavernous sinus, which had not been present on the admission. Since epistaxis was caused by a rupture of the aneurysm, internal carotid artery ligation was done. The signs of inflammation improved as well as patients' symptoms. The improvement while using antibiotics strongly implies that the aneurysm was a result of bacterial infection of the cavernous sinus. There have been no reports of ruptured bacterial intracavernous aneurysms presented with massive epistaxis as this case.

[A case of polymyositis with ventricular tachycardia].

We reported a case of polymyositis with ventricular tachycardia. She had been suffering from proximal dominant muscle weakness and palpitation for 11 months. Holter EKG demonstrated 23 beats ventricular tachycardia. The noninvasive cardiac examination (Thalium cardiac scintigraphy, echocardiography, radionuclide ventriculography) demonstrated no abnormal findings. After initiation of steroid therapy, muscle strength and CK improved and ventricular tachycardia disappeared. Ventricular tachycardia is one of the important complications of polymyositis.

[Visual hallucination in the hemianopic field caused by dural arteriovenous malformation].

We presented a 65-year-old man with the right occipital dural arteriovenous malformation (AVM) who first experienced photopsia in his left inferior quadrantanopic field and then developed formed hallucinations 8 days later. His visual hallucinations disappeared with saccadic eye movements. EEG showed no epileptic discharge. MRI revealed hyperintensity in the right medial occipital lobe on proton density image. 123I-IMP SPECT during the hallucinations revealed decrease of regional cerebral blood flow (r-CBF) not only in the right medial occipital lobe but also in the right lateral occipital and in the right medial temporal lobe. Both left inferior quadrantanopia and visual hallucinations disappeared following the treatment of the dural AVM by transarterial embolizations. Because the lesion on MRI improved after the treatment, we considered that the lesion was brain edema, not infarction. Additionally decreased r-CBF in the temporo-occipital lesion on SPECT improved after the treatment, we emphasized the role of the temporo-occipital lesion as the cause of visual hallucinations in the hemianopic field.

[Superior sagittal sinus thrombosis in paroxysmal nocturnal hemoglobinuria--an autopsied case].

A 43-year-old Japanese man was admitted to our hospital because of acute occipital headache and drowsiness. He had been diagnosed as paroxysmal nocturnal hemoglobinuria (PNH) with acute renal failure for 2 months prior to the admission. He had abdominal pain and fever of unknown origin for 2 weeks. On admission neurological examination revealed mild disturbance of consciousness, right papilledema and minimal nuchal rigidity. The urinalysis was normal and blood hemoglobin was 8.3 g/dl. Mild hemolysis was suspected because of decreased level of the serum haptoglobin. The magnetic resonance imaging (MRI) of the brain demonstrated the complete occlusion of the superior sagittal sinus by fresh thrombus and the cerebral hemorrhage in the right parieto-occipital subcortex. No other cause for cerebral sinus thrombosis was detected. The patient was treated medically using anti-edema agents, but on the twentieth hospital day he died because of brain herniation. The postmortem examination disclosed thrombosis in the superior sagittal sinus and transverse sinus on both side. There was a massive hematoma in the right parieto-occipital subcortex. The spleen and kidney also contained multiple venous thromboses. Twelve cases of cerebral sinus thrombosis with PNH have been reported in the literatures. Although cerebral sinus thrombosis is a common complication with PNH in Caucasian, but is rare in Asian including Japanese. This may probably depend upon a racial difference. To our knowledge, this is the first report of a patient with cerebral sinus thrombosis with PNH in Japan.

[A case of giant pigmented nevus with multiple meningiomas].

A 36-year-old female had a giant congenital pigmented nevus on her anterior trunk. She complained of muscle weakness of the right arm and leg in August 1988, and she was admitted to our hospital to take medical examinations in September 1988. Neurological examination revealed right hemiparesis and exaggerated deep tendon reflexes. Three small falx meningiomas were shown on CT scans and MR images, but they did not appear to be causes of her symptoms. CT-myelography and MR images revealed an intradural extramedullary tumor spreading from the foramen magnum to C2. Oncotomy and C1 laminectomy were performed. Histological finding was compatible with transitional meningioma. Neurological symptoms markedly improved after operation. This patient is considered as a case of neurocutaneous syndrome with central nervous system tumors. Considering that both giant pigmented nevus and meningioma originate from neural crest, they appear to be closely related each other. Such a combination of giant pigmented nevus and meningioma has not yet been reported.