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A case of Friedreich's ataxia was followed for 47 years, beginning in 1930; this patient had an abnormal electrocardiogram (flat or inverted T waves in leads II and III with prolonged QT interval) from the very beginning of the onset of neurological symptoms. Cardiac and neurological disturbances progressed slowly but steadily, and the patient died suddenly at the age of 67. The autopsy revealed typical findings of Friedreich's ataxia and hypertrophic cardiomyopathy with thickened left ventricular wall and myocardial fiber disarray. To the authors' knowledge, this is the longest continuous follow-up study of Friedreich's ataxia, and it will provide invaluable information on the natural history and development of the cardiac and neurological disorders in this condition.

Our patient was first diagnosed with auditory agnosia following his second cerebral vascular accident (CVA) in 1975 when he was 37 years old. Comprehensive follow-up examinations of auditory function were periodically conducted until his sudden death 15 years later. His brain was studied postmortem for neuropathology. Initial pure-tone audiometry revealed moderate sensorineural hearing loss in the right ear and mild sensorineural hearing loss in the left ear. However, repeated pure-tone audiometry revealed that thresholds became progressively poorer over time, bilaterally. Speech audiometry of both ears consistently revealed that the patient was unable to discriminate any monosyllabic words (i.e. speech intelligibility scores were 0%, bilaterally). In general, speech and hearing tests demonstrated that he could not comprehend spoken words, but could comprehend written commands and gestures. Postmortem neuropathological study of the left hemisphere revealed total defect and neuronal loss of the superior temporal gyrus, including Heschl's gyrus, and total gliosis of the medial geniculate body. In the right hemisphere, examination revealed subcortical necrosis, gliosis in the centre of the superior temporal gyrus and partial gliosis of the medial geniculate body. The pathological examination supports clinical results in which the patient's imperception of speech sounds, music and environmental sounds could be caused by progressive degeneration of bilateral medial geniculate bodies.

A 76-year-old HTLV-I-positive male with acute cerebellar ataxia was suffering from dyspnea on exertion. Chest CT suggested interstitial pneumonitis. Methylprednisolone pulse therapy improved his symptoms and chest CT findings. Twelve months after discharge, when the prednisolone dose was tapered to 5 mg every other day, his lung lesion recurred. The lesion responded initially to steroid therapy. However, hypoxemia intractable to steroid pulse therapy developed and the patient died of respiratory failure. The autopsy revealed diffuse alveolar hemorrhage with no finding of vasculitis. This is the first case report of diffuse alveolar hemorrhage in an HTLV-I carrier.

A rare case of finger-print-like zymogen granules shown by electron microscopy is reported. The patient was a 75-year-old man who was histologically and ultrastructurally confirmed to have acinar cell carcinoma of the pancreas. Frozen section and postmortem examination revealed that the tumor was made up of solid nests of cells resembling the appearance of normal pancreatic acini, showing polygonal cells which had round or oval nuclei, and rare mitotic figures. Zymogen-like granules, shown by eosinophilic granular staining, were abundant in the cytoplasm. Electron microscopy showed that the tumor cells were closely packed, occasionally forming small intercellular spaces resembling pancreatic acini (microtubules). The cytoplasm contained characteristic zymogen granules with dark-to-medium electron density, measuring 660 nm +/-213 SD in diameter. The granules of medium density were large, and showed finger-print-like patterns. Investigation of more cases is necessary to identify whether these finger-print-like patterns are an important factor in the genesis of acinar cell carcinoma.

The temporal bone pathology of a 74-year-old female affected by vestibular schwannoma was compared with findings of auditory brainstem response and electrocochleography. At age 71, she complained of hearing loss in the left ear in which pure tone audiometry revealed threshold elevation in the middle- and high-frequency range. Temporal bone CT scanning revealed a medium-sized cerebellopontine angle tumour in the left ear. ABR showed no response in the left ear, but the electrocochleography showed clear compound action potentials. Three years later, at age 74, she died of metastatic lung cancer and sepsis. The left temporal bone pathology consisted primarily of a large vestibular schwannoma occupying the internal auditory meatus. The organ of Corti was well preserved in each turn. In the modiolus, the numbers of spiral ganglion cells and cochlear nerve fibres in each turn were decreased. These histological findings suggest that clear compound action potentials were recorded from the distal portion of the cochlear nerve in spite of the presence of the vestibular schwannoma, but ABR could not be detected because of the blockade of the proximal portion of the cochlear nerve by the vestibular schwannoma.

We describe a three-year-old girl whose growing congenital brain tumor may have been responsible for her perinatal intracranial hemorrhage. The patient, born after an uneventful pregnancy and delivery, had an intracranial hemorrhage in the right frontal area, including the basal ganglia, as a newborn. Her only symptoms at that time were vomiting and fever. She improved both, clinically and neuroradiologically during the following weeks, and except for mild left hemiparesis, the patient developed with no other apparent neurological deficits. However, a follow-up brain CT scan at 3 years of age, disclosed a heterogeneous tumor with a 5 cm diameter in the same right frontal area as the neonatal hemorrhage. The tumor was surgically removed, and diagnosed as a benign mixed tumor composed of differentiated astrocytoma and meningioma elements. It is conceivable that the brain tumor may have been growing rather slowly since an early fetal stage. This case indicates the need of being aware that neonatal intracranial hemorrhages may be caused by bleeding of congenital brain tumors.

We studied the pathological changes in the temporal bone and brainstem, with particular attention to the autolysis of red blood cells in and around the blood vessels, in six cases of brainstem death determined by auditory brainstem responses (ABRs). Our findings showed that the cochlea, the visceral organs and the spinal cord below a certain level of the cervical segments continued to live after brainstem death. Red blood cells in the vessels of the brainstem and cerebellum exhibited severe autolysis, whereas most red blood cells in the cochlea were preserved. Our findings of autolytic changes in red blood cells in the brainstem, and the preservation of red blood cells in the cochlea, imply initial loss of brainstem function and delayed loss of cochlear function after prolonged absence of ABRs.

Left atrial and left ventricular infarction with various atrial arrhythmias, multiple systemic arterial thromboembolism, and a large mural thrombus over the left atrial infarction is reported. In ischemic heart disease, systemic arterial thromboembolism may develop due to atrial infarction which is often overlooked in antemortem as well as in postmortem examinations. When a clinical diagnosis of atrial infarction is made, the possibility of a Stokes-Adams syndrome, thromboembolism, and rupture of the atrium should also be investigated.

An autopsy case of a 9-year-old Japanese girl revealed a carcinoid tumor originating in the duodenum and hyperplasia of the multiple endocrine organs as manifested by ectopic ACTH syndrome, carcinoid syndrome and giantism. The tumor cells were positive for histochemical argyrophile reaction and two types of secretory granules were identified by electron microscopy. Biochemical assay revealed the production of ACTH and beta-MSH by the tumor cells. Other changes of multiple endocrine organs included acidophil dominant hyperplasia of the pituitary, diffuse hyperplasia of the thyroid, chief cell hyperplasia of the parathyroid, hyperplasia of the islets of Langerhans and the adrenal cortex. This case was considered to be a type of multiple endocrine adenomatosis including carcinoid tumor. The relationship between the carcinoid tumor and multiple endocrine adenomatosis was discussed.

The high incidence of metastatic bone disease in urological cancer makes it necessary for clinicians to look for a valid experimental model to investigate the basic interactions between cancer cells and bone in order to improve the treatment. A new model of bone resorption was defined, namely the subcutaneous injection of tumor cell suspensions of serially transplanted renal cell carcinoma in nude mice after disruption of the periosteum of the calvaria. The tumor induced osteolysis associated with osteoclast proliferation with reactive bone formation. The X-p film from nude mice calvaria showed the same multiple osteolytic punched-out lesions as those of the patient's skull.

A painful intraperiosteal osteoid osteoma of the femur occurred in a 36-year-old man. Histologically, the tumor was encapsulated by the periosteum. The pain was relieved by removal of the tumor. Thirty-four months after operation, there has been no recurrence. Osteoid osteoma in this location does not appear to have been reported previously.

Benign pedunculated intraluminal tumor of the esophagus is extremely rare. Although the correct diagnosis of this tumor is difficult, it should be extirpated early because, if untreated, it may result in death of the patient by progressive emaciation, bleeding from erosions, or acute airway obstruction. We present a case rescued from suffocation caused by regurgitation of a giant esophageal tumor. In the discussion on the difficulty of diagnosis, the necessity of repeated examination is emphasized. The classification of benign esophageal tumors is also discussed.

A 12-year-old Japanese girl with a primary intracranial choriocarcinoma arising from the pituitary fossa is reported. There was a markedly high level of serum human chorionic gonadotropin (HCG) throughout the clinical course. Postmortem examination revealed metastatic lesions found in the cerebellum, dura mater, skull (clivus, sphenoidal bone), and lungs. Immunohistochemical characteristics of the primary intracranial tumor and lung lesions disclosed positive stainings for HCG and HCG-beta in the neoplastic syncytiotrophoblastic cells.

A rare case of astroblastoma of the pure type occurring in the left occipital lobe of a 54-year-old female is reported. The predominant histologic feature was that of perivascular pseudorosettes. The tumor tissues were not stained by the Mallory's phosphotungstic acid-hematoxylin, but the perikarya of some tumor cells were positively stained for the glial fibrillary acidic protein.

Cell proliferation in the brain of spontaneously hypertensive rats (SHR) and control Wistar rats of various ages was autoradiographically investigated using [3H]thymidine. The brain of SHR showed an increase in labeled cells. The increase of labeled cells was seen in the early stage of the development of hypertension when there were still no definite morphological changes in the cerebral vessel walls or brain parenchyma. The labeled cells tended to increase in number with the age of the animals. The distribution of these cells corresponded with areas of increased vascular permeability and cerebrovascular lesions, that is, water-shed regions. The labeled cells consisted of endothelial and adventitial cells of the intracerebral arterioles and pial arteries as well as glial cells. Arachnoid cells, subarachnoid cells, medial smooth muscle cells of the pial arteries were also labeled, though less intensely. The significance of labeled endothelial cells in the development of cerebrovascular changes and sequential parenchymal changes is discussed.

Systemic pathological alterations were studied in thirty-seven autopsied patients with Kawasaki disease. Systemic vasculitis was the most characteristic pathological finding and was present in all the patients. In addition to the vasculitis, there was a high incidence of inflammatory lesions in various organs and tissues: in the heart, endocarditis, myocarditis, and pericarditis; in the digestive system, stomatitis, sialoduct-adenitis, catarrhal enteritis, hepatitis, cholangitis, pancreatitis, and pancreas ductitis; in the respiratory system, bronchitis and segmental interstitial pneumonia; in the urinary system, focal interstitial nephritis, cystitis, and prostatitis; in the nervous system, aseptic leptomeningitis, choriomeningitis, gangliontis, and neuritis; in the hematopoietic system, lymphadenitis, splenitis, and thymitis. Dermatitis, panniculitis or myositis were also observed in some patients. Therefore, Kawasaki disease is a systemic inflammatory disease which mainly affects the cardiovascular system. These systemic inflammatory lesions are considered to correspond to the variegated clinical manifestaitions. The relationship between Kawasaki disease and infantile polyarteritis nodosa (IPN) were discussed, based on the clinicopathological characteristics.

Three types of renal hypertension in the rat have been compared with respect to blood pressure increase, activity of the RAS, and secretion of aldosterone and corticosterone: type I - unilateral stenosis of the renal artery in the presence of an intact contralateral kidney; type II - unilateral stenosis of the renal artery after contralateral nephrectomy; type III - bilateral stenosis of the renal arteries. Blood pressure rose more rapidly and reached higher values in type II and type III hypertension than in type I hypertension. In the latter group, the activity of the RAS was more stimulated than in types II and III. The marked stimulation of the RAS in type I hypertension is ascribed to the negative fluid and sodium balance, which is the consequence of a pressure-induced diuresis of the unclamped contralateral kidney. Suppression of the activity of the RAS by a 4-week pretreatment with DOC-TMA and saline or by the administration of DOCA and saline as from the induction of renal artery stenosis did not prevent the development of hypertension caused by the clamping of one renal artery (type I). In spontaneously hypertensive rats of the stroke-prone substrain, high dietary salt intake caused higher blood pressure values and a higher incidence of cerebral lesions than normal dietary salt intake. Low salt intake was followed by a marked stimulation of the RAS, but blood pressure rose only slightly and no symptoms of cerebrovascular lesions were observed. It is concluded that neither in hypertension induced by renal artery stenosis nor in spontaneously hypertensive rats, the RAS contributes significantly to the increase in blood pressure nor does it play a major part in the pathogenesis of vascular lesions. These seem to be related to the retention of sodium, which may be obtained by renal artery stenosis, by excessive salt intake, or by the administration of a mineralocorticoid and salt.

Cerebrovascular permeability in stroke-prone spontaneously hypertensive rats (SHR) at various ages was histologically studied using horseradish peroxidase as a tracer and such was related to the cerebrovascular lesions in the animals. An increase in permeability was demonstrated in the brain of SHR, particularly in those animals with an extremely high blood pressure. Increased cerebrovascular permeability occurred in some animals without any organic vascular change or severe parenchymal changes, although edema was present. Histologically, the SHR brain with an increase in permeability showed mild focal edema, rarefaction of tissue and necrosis with cyst formation. Thus a transitional progress was evident. Localization of the increase in permeability corresponded well with the predilection sites of cerebrovascular lesions in SHR. Constrictions and dilatations of intracerebral arterioles and small arteries were also demonstrated by the peroxidase method, and the dilated arterial walls did reveal a darker staining. From these results it is strongly suggested that certain cerebrovascular lesions, especially necrosis with cyst formation in SHR are sequelae of the increased cerebrovascular permeability caused by a chronic hypertensive state.