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Esophageal intramural pseudodiverticulosis (EIPD) is a rare disorder characterized by an abnormal, cyst-like dilatation of the excretory ducts of esophageal submucosal glands. We aimed to elucidate the histopathological features and immunohistochemical properties of the epithelial lining of the cyst-like lesions in EIPD. We performed a histopathological and immunohistochemical study of 2 cases (one autopsy and one surgical) of EIPD. The ductal walls consisted of inner ductal cells, which were cytokeratin (CK) 7-positive and CK5/6-negative, and outer basal cells, which were CK7-negative and CK5/6-positive. The ductal epithelium frequently showed squamous metaplasia and rarely simulated a false diverticulum. Immunohistochemistry for CK7 was useful for distinction between the conditions because the surface epithelium was negative for CK7. We also confirmed that myoepithelial cells in the acinar portion of submucosal glands were well-preserved in EIPD, the finding that explained the periodic opening and closing movements of orifices of cyst-like lesions in this disorder. The immunohistochemical properties of the epithelial lining of cyst-like lesions in EIPD were essentially similar to those of the normal ducts of submucosal glands. [ABSTRACT FROM AUTHOR]

Genetic Creutzfeldt‐Jakob disease (gCJD) with a methionine to arginine substitution at codon 232 of the prion protein gene (gCJD‐M232R) is rare and has only been reported in Japan. We report an autopsy case of gCJD‐M232R showing alleles of codon 129 that were homozygous for methionine and the presence of multiple strains of the protease‐resistant, abnormal isoform of prion protein (PrPSc), M1 + M2C + M2T. The patient, a 54‐year‐old Japanese man, died after a clinical course of 21 months characterized by slowly progressive dementia and sleep disturbance. At autopsy, the neuropil of the cerebral neocortex showed a widespread and severe spongiform change. Grape‐like clusters of large confluent vacuoles were admixed with fine vacuoles. Neuronal loss was moderate, but reactive astrocytosis was mild. The dorsomedial nucleus of the thalamus and the inferior olivary nucleus showed moderate and severe neuronal loss, respectively. Many amyloid plaques were present in the cerebellar molecular layer. PrPSc deposition pattern was predominantly the synaptic type in the cerebrum and corresponded to the plaques in the cerebellum. Perivacuolar deposition was also seen. Western blot analysis of PrPSc revealed the predominance of type 2. Moreover, by employing Western blot analysis in combination with the protein misfolding cyclic amplification (PMCA) method, which selectively amplifies the minor M2T prion strain, we demonstrated the presence of M2T, in addition to M1 and M2C strains, in the brain of the patient. PMCA was a powerful method for demonstrating the presence of the M2T strain, although the amount is often small and the transmission is difficult. [ABSTRACT FROM AUTHOR]

An autopsy case of chronic Chagas disease, a debilitating disorder caused by persistent infection by protozoa, Trypanosoma cruzi (Tr. cruzi), is reported. The patient was a 73‐year‐old Brazilian woman of Japanese descent, who had emigrated to Japan at the age of about 40 years. She died of chronic cardiac insufficiency about 8 years after the onset of cardiac symptoms. At autopsy, the heart showed typical features of chronic Chagastic cardiomyopathy: chronic lymphocytic myocarditis with extensive fibrosis and the formation of an apical aneurysm. The pathogenic protozoa were not detected in the cardiac tissue. The kidney showed typical features of membranoproliferative glomerulonephritis (MPGN). On the basis of experimental data which suggested that chronic infection of Tr. cruzi could elicit immune complex‐mediated glomerulonephritis, we considered that the chronic persistent infection by Tr. cruzi contributed to the pathogenesis of MPGN in this patient. [ABSTRACT FROM AUTHOR]

Human herpes virus 6 (HHV6) has attracted attention in recent years as an important causative agent for limbic encephalitis after bone marrow transplantation (BMT). We report an autopsy case of HHV6-induced encephalomyelitis that developed after BMT. The patient was a 61-year-old man with acute myeloid leukemia, who developed disorientation and short-term memory disturbance 35 days after allogenic BMT. MRI demonstrated T1-weighted high-signal intensity lesions in the medial temporal lobe and thalamus, and PCR of the CSF disclosed an increase in the copy numbers of the HHV6 genome. The patient died after a clinical course of 6 months, and at autopsy the brain showed remarkable atrophy of the hippocampus. Histopathologically, neuronal loss with astrocytosis and patchy necrosis with infiltration of macrophages were found predominantly in the hippocampus, amygdala, mamillary body, claustrum, and thalamus. Perivascular and intraparenchymal lymphocytic infiltration was slight. Similar lesions were also scattered in the cerebral neocortex, midbrain, pontine base, cerebellar white matter, and lumbar cord. In some of these lesions, axons were relatively preserved in comparison with myelin sheaths. Significant increase in the copy numbers of the HHV6 genome was demonstrated in the postmortem brain tissue by PCR. Neuropathological features of the present case were similar to those described in previously reported cases, but the distribution of lesions was more widespread. Demyelination was supposed to be involved in the pathogenesis of some of the lesions. [ABSTRACT FROM AUTHOR]

An autopsy case of chromosome 16q22.1-linked autosomal dominant cerebellar ataxia is reported. The patient was a 77-year-old man who died after a clinical course of about 19 years characterized by pure cerebellar ataxia. Main neuropathological findings included moderate loss of Purkinje cells, variegated degenerative features of the remaining Purkinje cells, finely fibrillary material surrounding the perikarya of Purkinje cells, and ubiquitin-immunoreactive small dots in the molecular layer and cerebellar white matter. Neuritic hyperplasia surrounding the perikarya of Purkinje cells was also a prominent finding. Golgi impregnation study demonstrated poor dendritic arborization of some Purkinje cells. It is our assumption that the pathological processes leading to Purkinje cell death in this disorder are not singular because the intracellular functions of the protein coded by the mutant gene are manifold, and the multiplicity of the pathological processes is reflected in diverse cytomorphological changes seen in degenerating Purkinje cells. [ABSTRACT FROM AUTHOR]

An autopsy case of primary pulmonary choriocarcinoma that manifested as diffuse alveolar hemorrhage is reported. A 44-year-old nurse presented with fever, dry cough, hemoptysis, and progressive dyspnea, and died after a downhill course of 2 weeks. Chest radiographs showed diffuse parenchymal shadows throughout the entire lung and a nodular lesion in the right lower lobe. Findings suggestive of acute renal failure were not seen. The autopsy revealed primary pure choriocarcinoma of the right lower lobe and diffuse alveolar hemorrhage throughout the entire lung. Findings of small vessel vasculitis (''pulmonary alveolar capillaritis'') were not observed, and extensive neoplastic involvement of the pulmonary vasculature was considered the cause of the diffuse alveolar hemorrhage. Small metastatic foci were found in the liver, adrenal glands, pancreas, and ovaries. This case shows that primary pulmonary neoplasms, on rare occasions, can produce the clinical and pathologic features of diffuse alveolar hemorrhage, probably through elevated pulmonary venous pressure caused by extensive destruction of the vasculature. [ABSTRACT FROM AUTHOR]