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We discuss an extremely rare case of low-grade Schwann cell leptomeningeal neoplasm with no evident intradural primary, presenting with rapid neurological decline leading to death reflecting the aggressive biological behaviour of this entity despite its low-grade morphology. Notwithstanding extensive investigations, the diagnosis was only established on autopsy as clinical presentation is non-specific making diagnosis challenging. This condition could be considered in patients presenting with leptomeningeal disease if initial workup of more common causes is non-revealing.

We present a case of a 51-year-old immunosuppressed man with underlying chronic lymphoproliferative leukaemia (CLL), who presented to us in emergency with breathlessness, hydrophobia, anxiety and restlessness. He had a history of category 3 dog bite 2 months ago and had received a full course of rabies immunoglobulin and antirabies vaccine (ARV) as per the national schedule. As there were frank clinical reports of rabies, the patient was managed according to Milwaukee regimen. The patients died within a week of the appearance of symptoms. The brain autopsy revealed Negri bodies conforming the mortality due to rabies.Immunosuppressed patients, like our patient who had CLL have low antibody formation after rabies prophylaxis. Antibody titres in immunosuppressed patients need to be measured after the 2–4 weeks of the last injection of ARV to decide whether a booster of ARV needs to be administered or not.

Introduction Desmoplastic malignant pleural mesothelioma (DMPM) is a sarcoma type mesothelioma, comprising about 5% of malignant pleural mesotheliomas. Although effusion cytology is commonly used as the primary diagnostic approach for mesothelioma, this may not be useful for DMPM due to its desmoplastic nature and bland cellular atypia. We hereby report a case of DMPM diagnosed through autopsy along with its cytological features that have not been described previously. Case presentation A male in his 60s with a history of occupational asbestos exposure was referred to our hospital with right chest pain. Chest computed tomography scan showed right pleural effusion. Thirteen months later, the patient died of respiratory failure. In autopsy, the scrape-imprint smear and the pleural effusions cytology were performed. The scrape-imprint smear samples exhibited spindle cells with mild nuclear atypia and grooves with fibrous stroma. In the pleural effusion cytology, spindle cells having mild nuclear atypia and grooves with loose epithelial connections were observed. Histological examination of the right pleura showed spindle cells proliferating with dense collagen fibers, as seen in cytological samples, thus rendering the diagnosis of DMPM. Diagnosis was confirmed by fluorescence in situ hybridization. Conclusion Cytological procedures, such as pleural effusion cytology and scrape-imprinting method, may be useful as an ancillary tool in the diagnosis of rare tumors such as DMPM.

A 60-year-old man was referred to our hospital because of chest tightness. CT scans showed no specific findings except a right ventricular (RV) mass. Echocardiogram revealed that the large cardiac mass was compressing the right ventricular outflow tract, and the patient thus underwent an emergency operation. As the tumour on the inlet side of the right ventricle was tightly adhered to the entire tricuspid chordae, a complete resection was impossible. Pathological findings revealed that the tumour was metastatic squamous cell carcinoma. Four cycles of chemotherapy were administered. Further investigations were conducted to identify the primary cancer focus, but there were no specific findings. Eight months after the operation, the patient returned to the hospital. Echocardiogram showed a severely occupying mass once again, and the patient died as a result. Autopsy revealed no findings relating to the primary origin of the cardiac metastases.

A 57-year-old woman presented with optic neuritis with repeated clinical symptoms of focal demyelination of the cerebral white matter and brain stem for 14 years. At the end of the patient's course, the clinical signs mimicked secondary progressive multiple sclerosis, but whether it was caused by interferon administration or neuromyelitis optica spectrum disorders (NMOSD) - or a combination of both or others - was unclear. Histopathological findings indicated the etiology to be NMOSD, with no apparent plaque in spinal cord specimens. This case suggests that an accurate clinical diagnosis requires serum anti-aquaporin 4 antibody measurements as well as an autopsy examination.

We herein report a case of Behçet's disease with renal infarction due to mucormycosis. A 76-year-old man with entero-Behçet's disease had been treated with glucocorticoid and tumor necrosis factor (TNF) inhibitors. His entero-Behçet's disease was refractory to these treatments, and ileocecal resection was performed. After the operation, renal infarction that was unresponsive to anticoagulation therapy developed. He ultimately died of renal failure due to renal infarction. At the autopsy, histopathology of abundant hyphae in the renal vessel wall revealed mucormycosis. Renal mucormycosis is an important cause of renal failure with renal infarction in immunocompromised patients.

A 61-year-old woman was admitted to our hospital with a fever, nausea, diarrhea, and back pain. Her condition rapidly deteriorated, and she was transferred to the intensive-care unit for mechanical circulatory support and antibiotics, but she died 40 h after admission. Autopsy findings showed necrotic and suppurative myocardial changes due to group B Streptococcus (GBS). To date, only one case of bacterial myocarditis caused by GBS has been reported. We herein report a case of GBS myocarditis, the etiology of which is poorly understood due to the limited number of cases. Bacterial myocarditis should be considered in patients with sepsis and myocardial dysfunction.

The quadricuspid aortic valve (QAV) is a rare congenital anomaly that typically is unrelated to other cardiac anomalies. It usually is discovered incidentally through imaging modalities such as echocardiogram or computed tomography angiogram, during surgery, or autopsy. Some patients with QAV develop aortic regurgitation and, rarely, other cardiovascular complications like aortic aneurysm. Due to its rarity, it is difficult to characterize these patients or standardize management. However, review of case reports can be very useful in rare medical conditions such as QAV. In this manuscript, in addition to presenting two cases of QAV, the authors reviewed 149 cases of QAV from 2010 to 2020 from published case studies and series in order to gain a better insight into the characteristics of the patient population with QAV and its management.

Amelanotic melanoma is a rare type of melanoma that shows little or no melanin pigmentation. When tumor lesions are not detected in cutaneous sites, the presence of melanin is the hallmark sign of malignant melanoma. We herein report a case of amelanotic melanoma with a BRAF V600E mutation mimicking primary lung cancer that was finally diagnosed on an autopsy. The current case suggests important caveats for the differential diagnosis of patients with BRAF V600E mutation-positive poorly differentiated lung tumors. In terms of the pathological diagnosis, routine immunohistochemical staining may be useful, especially in patients with a poorly differentiated lung tumor without TTF-1 expression.

Disseminated carcinomatosis of the bone marrow (DCBM) is often accompanied by disseminated intravascular coagulation (DIC) and has a poor prognosis. DCBM develops most frequently in gastric cancer and is rarely associated with intrahepatic cholangiocarcinoma. A 41-year-old man was incidentally found to have DIC on his regular visit for ulcerative colitis and was diagnosed with DCBM with intrahepatic cholangiocarcinoma. He received intensive care, including chemotherapy, but died suddenly from hyperkalemia, possibly due to tumor lysis syndrome (TLS). The autopsy showed the periductal infiltrating type of intrahepatic cholangiocarcinoma and tumor necrosis, possibly due to chemotherapy, indicating the effectiveness of chemotherapy for DCBM with intrahepatic cholangiocarcinoma.

Objectives We report the clinical, radiological, laboratory, and neuropathological findings in support of the first diagnosis of lethal, small-vessel cerebral vasculitis triggered by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) in a pediatric patient. Patient description A previously healthy, 8-year-old Hispanic female presented with subacute onset left-sided weakness two weeks after a mild febrile illness. SARS-CoV-2 nasopharyngeal swab was positive. Magnetic resonance imaging (MRI) revealed an enhancing right frontal lobe lesion with significant vasogenic edema. Two brain biopsies of the lesion showed perivascular and intraluminal lymphohistiocytic inflammatory infiltrate consistent with vasculitis. Despite extensive treatment with immunomodulatory therapies targeting primary angiitis of the central nervous system, she experienced neurologic decline and died 93 days after presentation. SARS-CoV-2 testing revealed positive serum IgG and positive cerebrospinal fluid IgM. Comprehensive infectious, rheumatologic, hematologic/oncologic, and genetic evaluation did not identify an alternative etiology. Post-mortem brain autopsy remained consistent with vasculitis. Discussion This is the first pediatric presentation to suggest SARS-CoV-2 can lead to a fatal, post-infectious, inflammatory small-vessel cerebral vasculitis. Our case is unique in including supportive cerebrospinal fluid and post-mortem tissue analysis. While most children recover well from neurologic complications of SARS-CoV-2, we emphasize the potential mortality in a child with no risk factors for severe disease.

Background: Parkinson’s disease (PD) is a chronic, disabling neurodegenerative disorder. Objective: To predict a future diagnosis of PD using questionnaires and simple non-invasive clinical tests. Methods: Participants in the prospective Kuakini Honolulu-Asia Aging Study (HAAS) were evaluated biannually between 1995–2017 by PD experts using standard diagnostic criteria. Autopsies were sought on all deaths. We input simple clinical and risk factor variables into an ensemble-tree based machine learning algorithm and derived models to predict the probability of developing PD. We also investigated relationships of predictive models and neuropathologic features such as nigral neuron density. Results: The study sample included 292 subjects, 25 of whom developed PD within 3 years and 41 by 5 years. 116 (46%) of 251 subjects not diagnosed with PD underwent autopsy. Light Gradient Boosting Machine modeling of 12 predictors correctly classified a high proportion of individuals who developed PD within 3 years (area under the curve (AUC) 0.82, 95%CI 0.76–0.89) or 5 years (AUC 0.77, 95%CI 0.71–0.84). A large proportion of controls who were misclassified as PD had Lewy pathology at autopsy, including 79%of those who died within 3 years. PD probability estimates correlated inversely with nigral neuron density and were strongest in autopsies conducted within 3 years of index date (r = –0.57, p

OBJECTIVE Despite advancement of thrombectomy technologies for large-vessel occlusion (LVO) stroke and increased user experience, complete recanalization rates linger around 50%, and one-third of patients who have undergone successful recanalization still experience poor neurological outcomes. To enhance the understanding of the biomechanics and failure modes, the authors conducted an experimental analysis of the interaction of emboli/artery/devices in the first human brain test platform for LVO stroke described to date. METHODS In 12 fresh human brains, 105 LVOs were recreated by embolizing engineered emboli analogs and recanalization was attempted using aspiration catheters and/or stent retrievers. The complex mechanical interaction between diverse emboli (elastic, stiff, and fragment prone), arteries (anterior and posterior circulation), and thrombectomy devices were observed, analyzed, and categorized. The authors systematically evaluated the recanalization process through failure modes and effects analysis, and they identified where and how thrombectomy devices fail and the impact of device failure. RESULTS The first-pass effect (34%), successful (71%), and complete (60%) recanalization rates in this model were consistent with those in the literature. Failure mode analysis of 184 passes with thrombectomy devices revealed the following. 1) Devices loaded the emboli with tensile forces leading to elongation and intravascular fragmentation. 2) In the presence of anterograde flow, small fragments embolize to the microcirculation and large fragments result in recurrent vessel occlusion. 3) Multiple passes are required due to recurrent (15%) and residual (73%) occlusions, or both (12%). 4) Residual emboli remained in small branching and perforating arteries in cases of alleged complete recanalization (28%). 5) Vacuum caused arterial collapse at physiological pressures (27%). 6) Device withdrawal caused arterial traction (41%), and severe traction provoked avulsion of perforating and small branching arteries. CONCLUSIONS Biomechanically superior thrombectomy technologies should prevent unrestrained tensional load on emboli, minimize intraluminal embolus fragmentation and release, improve device/embolus integration, recanalize small branching and perforating arteries, prevent arterial collapse, and minimize traction.

La enfermedad por coronavirus de 2019 (COVID-19) es una emergencia sanitaria pública global con numerosas facetas clínicas que incluyen renopatía aguda y enfermedad cerebrovascular aguda. Es necesario un conocimiento adicional de su mecanismo patogénico. Los trastornos de coagulación están claramente incluidos en dichos mecanismos. La gammapatía monoclonal se caracteriza por la sobreproducción de inmunoglobulina monoclonal causada por proliferación clonal. Utilizando un estudio postmortem de biopsias básicas percutáneas ecoguiadas, el objetivo de este informe es presentar nuestras observaciones sobre la patología del síndrome respiratorio agudo severo por infección de coronavirus 2 (SARS-CoV2) con gammapatía monoclonal. La presentación clínica fue insuficiencia renal aguda. Los hallazgos patológicos revelaron nefropatía por depósito de cadenas ligeras kappa. La inmunohistoquímica de SARS-CoV-2 fue positiva en ciertas células tubulares renales. La presencia de megacariocitos alveolares (alta densidad) fue un hallazgo notable, que explica probablemente el resultado final del paciente (enfermedad cerebrovascular aguda). El estudio inmunohistoquímico frente a SARS-CoV-2 no verifica el efecto patogénico del virus y, por tanto, su contribución a la nefropatía aguda.

Pulmonary carcinoid tumors are considered as low-grade neoplasms, seen as centrally located endobronchial masses or as peripheral circumscribed nodules. Calcification or ossification is a known phenomenon, but presentation as large bony mass is extremely uncommon. Herein, we report a case of ossifying bronchial carcinoid along with nodular Hashimoto's thyroiditis as incidental autopsy findings in a 32-year-old patient with a prior recent excision of pituitary macroadenoma. This association suggests the possibility of multiple endocrine neoplasia in this young male.

Herpes simplex virus type 2 (HSV-2) infection is the leading cause of genital lesions. Infrequently HSV-2 primary infection can spread and involve other tissue and organs, however in immunocompetent individuals extra-genital complications are rare findings. In this report we present a fatal case of fulminant myocarditis and terminal liver involvement, caused by HSV-2 infection, with a unique presentation. Diagnosis was made only post-mortem.

The aim of the study was to identify the features of variations in the localization of monoamines (catecholamines, serotonin)in the cell structures of autopsy material of lungs affected by tuberculosis. Material and methods . 165 cases of secondary pulmonary tuberculosis were studied. We studied 2500 histological preparations of patients aged 30 to 55 years, divided into 5 groups, corresponding to the forms of secondary tuberculosis (group 1-acute focal tuberculosis, group 2-fibro-focal tuberculosis, group 3-infiltrative tuberculosis, group 4-acute cavernous tuberculosis, group 5 - fibro-cavernous tuberculosis. To selectively detect neuroamine-containing structures of the lungs and adrenergic nerve fibers, the Falk-Hillarp luminescence-histochemical method was used in the modification of E. M. Krokhina, based on the reaction of neuroamine condensation with formaldehyde. Results. When using the Falk-Hillarp luminescence-histochemical method, mycobacteria of tuberculosis containing catecholamines and serotonin were detected in autopsy material of lungs affected by a specific inflammatory process, as well as neuroamine-containing granular luminescent cells and mast cells. Affected and unaffected areas of the lungs contain different amounts of the studied monoamines. The results of the study revealed the dependence of the number of cells and the content of the studied bioamines in them on the form of secondary tuberculosis. It was found that in small forms of secondary tuberculosis (acute focal, fibrotic-focal), the number of cells and quantitative parameters of the studied neurotransmitters (catecholamines, serotonin) in granular luminescent cells and mast cells change in the direction of increase. In destructive processes in the lungs (acute cavernous and fibrous-cavernous tuberculosis), the content of catecholamines in the studied structures in the affected area prevails over the control. The concentration of serotonin in the studied cells of the area of lung tissue affected by the tuberculosis process is reduced. The number of neuroamine-containing cells is significantly reduced. The maximum luminescence of catecholamines and serotonin in granular luminescent cells was determined in the infiltrative form of tuberculosis. In mast cells, the highest content of catecholamines was found in the group of acute cavernous tuberculosis, and serotonin in the group of the infiltrative form of the disease. In stained sections of the lungs in this form of tuberculosis, a luminescent pathway of lymphocytes was found. The nerve fibers detected at the sites of mycobacteria were also changed. They did not have a clear luminescence, looked "swollen", in places they did not have varicose veins. Conclusion. Comparing the results of the study with the literature data, it was found that pulmonary tuberculosis occurs as a delayed reaction, and Mycobacterium tuberculosis manifests itself as a corpuscular antigen.

Background Histologic analyses of deep vein thrombi (DVTs) have used autopsy samples and animal models. To the best of our knowledge, no previous study has reported on thrombus composition after percutaneous mechanical extraction. Because elements of chronicity and organization render thrombus resistant to anticoagulation and thrombolysis, a better understanding of clot evolution could inform therapy. Methods We performed a histologic evaluation of DVTs from consecutive patients who had undergone mechanical thrombectomy for extensive iliofemoral DVTs using the Clottriever/Flowtriever device (Inari Medical, Irvine, Calif). The DVTs were scored using a semiquantitative method according to the degree of fibrosis (collagen deposition on trichrome staining) and organization (endothelial growth with capillaries and fibroblastic penetration). Results Twenty-three specimens were available for analysis, with 20 presenting as acute DVT (≤14 days from symptom onset). Of the 23 patients, 11 (48%) had had >5% fibrosis (ie, collagen deposition) and 14 (61%) had had >5% organization (ie, endothelial growth, capillaries, fibroblasts). Four patients with acute DVT had had ≥25% organized thrombus and two had had ≥25% collagen deposition. Of the 20 patients with acute DVT, 40% had had >5% fibrosis and 55% had had >5% organization. The acuity of DVT did not correlate with the amount of fibrosis or organizing scores. Conclusions A large proportion of patients with acute DVT will have histologic elements of chronicity and fibrosis. A better understanding of the relationship between such elements and the response to anticoagulant agents and fibrinolytic drugs could inform our approach to therapy.

A partir de la aparición del SARS-CoV-2 en el mundo se desató la pandemia de COVID-19 que afectó a 185 países aparecieron numerosas publicaciones científicas presentando datos epidemiológicos, clínicos, estadísticos y microbiológicos. La elevada mortalidad ha implicado la necesidad de contar con datos cada vez más precisos en relación al virus y a su comportamiento en el medio y, por otro lado, enfrentó a los equipos de salud con un grave problema en relación al manejo seguro de cadáveres y a los riesgos de transmisión viral que ello implica. El objetivo del presente estudio ha sido detectar la presencia de ARN del SARS-CoV-2 en cadáveres remitidos a la Morgue Judicial que no estuvieran comprendidos en la definición de caso sospechoso propuesta por el Ministerio de Salud de la República Argentina. De un total de 153 cadáveres ingresados entre el 16 y el 24 de abril del 2020 en la Morgue Judicial se realizaron hisopados nasofaríngeos y orofaríngeos en 50 cadáveres elegidos basándose en criterios de inclusión previamente establecidos para efectuar una detección de ARN viral mediante técnica RT PCR. De todos éstos, a 2 de los cadáveres (4%) se les detectó ARN viral, los mismos no estaban clasificados como casos sospechosos de COVID-19. En el caso de 28 cadáveres remitidos como casos sospechosos de COVID-19, en el mismo período, se observó una prevalencia similar, 3,6%. Following the emergence of SARS-CoV-2 in the world, the COVID-19 pandemic broke out, affecting 185 countries. There have been numerous scientific publications presenting epidemiological, clinical, statistical, and microbiological data. The high mortality has implied the need for increasingly accurate data regarding the virus and its behaviour in the environment and, on the other hand, faced health teams with a serious problem in relation to the safe handling of corpses and the risks of transmission that this implies. The objective of this study was to detect the presence of SARS-CoV-2 RNA in corpses sent to the National Judicial Morgue that were not included in the definition of a suspected case submitted by the Argentine Ministry of Health. Nasopharyngeal and oropharyngeal swabs were taken from 101 cadavers chosen randomly and based on inclusion criteria for detection of viral RNA using the RT-PCR technique. Of the cadavers included in the study, 16.8%, not classified as suspected cases of COVID-19, were tested for the presence of viral RNA in the samples collected. Fil: D'Addario, Adriana Claudia. Morgue Judicial de la Nación; Argentina Fil: Bustos, Cristina A.. Morgue Judicial de la Nación; Argentina Fil: Cohen, Roberto V.. Morgue Judicial de la Nación; Argentina Fil: Rullan Corna, Alejandro Félix. Morgue Judicial de la Nación; Argentina Fil: Gómez, Patricia Elizabeth. Morgue Judicial de la Nación; Argentina Fil: Torrisi, Rubén H.. Morgue Judicial de la Nación; Argentina Fil: Di Salvo, Héctor. Morgue Judicial de la Nación; Argentina Fil: Herbstein, Jorge A.. Morgue Judicial de la Nación; Argentina Fil: Remes Lenicov, Federico. Universidad de Buenos Aires; Argentina. Consejo Nacional de Investigaciones Científicas y Técnicas. Oficina de Coordinación Administrativa Houssay. Instituto de Investigaciones Biomédicas en Retrovirus y Sida. Universidad de Buenos Aires. Facultad de Medicina. Instituto de Investigaciones Biomédicas en Retrovirus y Sida; Argentina Fil: Maffia Bizzozero, Santiago. Morgue Judicial de la Nación; Argentina

A 75-year-old woman developed redness and swelling on her truncal skin, spreading from the lower abdomen to left thigh, 2 months before being admitted to our hospital. She was urgently hospitalised because of her worsening respiratory condition. On admission, she had reticular telangiectasia, diffuse skin induration on the lower abdomen and panhypopituitarism. She was diagnosed with intravascular large B-cell lymphoma (IVLBCL) by the third random abdominal skin biopsy. After histopathological examination at autopsy, we made a final diagnosis of IVLBCL causing respiratory failure and panhypopituitarism. This is the rare case of IVLBCL-induced panhypopituitarism showing visible skin lesions.

Raymond de Vieussens was an exemplary anatomist who made seminal contributions in the field of cardiology. During initial part of his academic career, he adopted human dissection based experiments as medium of his research. This was in accordance with prevailing trend among anatomists during 17th century. He discovered the presence of tiny venous tributaries communicating between cardiac veins and chambers of heart (ducti carnosi/venae cordis minimae). He reported the existence of a collateral circulatory pathway between right and left coronary arterial systems (Vieussens arterial ring). He was the first to note the valve at the junction of great cardiac vein and coronary sinus (valve of Vieussens) and the prominent oval margin of the fossa ovalis (Vieussens Annulus). All his findings were associated with considerable clinical significance as evidenced in literature that followed. Vieussens accurately demonstrated the three-layered orientation of myocardium and gave a precise description of coronary arteries and their branches. At the onset of 18th century, buoyed by royal patronage from King Louis XIV of France, the second half of Vieussens illustrious career was defined by pathologic anatomy (autopsy based) and anatomo clinical correlations. This was a new trend initiated by Vieussens in anatomical research and was later adopted as a signature method by anatomists of 18th century. As a true connoisseur of cardiologic anatomy, Vieussens accurately charted the anatomo clinical correlations of cardiac tamponade, mitral stenosis and aortic regurgitation. His contributions were pivotal elements in metamorphosis of cardiology as a robust discipline of medicine in modern times.

The present study is a retrospective study of medico-legal autopsies conducted in the Department of Forensic Medicine and Toxicology MMIMSR Mullana, Ambala, India, from Jan 2016 to December 2020. During this period, 793 cases of medico-legal autopsies were done in the mortuary of MMIMSR, Mullana, Ambala. The majority of victims were male 670 (84.48 %) cases and female 123 (15.52%) cases. A maximum number of post mortem done in the age group 20-40 years was 385(48.56 %) cases and minimum in age group 0-10 year were 18 (02.26%) cases. In our study, 601 (75.78%) were married, 169 (21.31%) unmarried, and unknown 23 (02.99%) cases. Hindus were 660 (83.22%), Muslim 50(06.30%), and Sikh 83 (10.48%) cases. The total number of deaths from rural areas was 406(51.20%) cases, which is slightly higher than urban area 387 (48.80%) cases. Manners of death due to natural causes were 38 (04.97%) cases and unnatural 755 (95.20%) cases. In unnatural, (accidents 551 (69.44%), Suicides 186 (23.44%) and homicides 18 (02.24%) cases). A maximum number of death due to Head injury were 235 (29.64%), shock and hemorrhage 188 (23.70%), poisoning 124 (15.64%), others-skeleton and visceral injuries 81 (10.22%), hanging 44 (05.54%), heart attack 38(04.78%), Drowning 23(02.90%), Electrocution 14 (01.76%), strangulation 13(01.64%), burns 11(1.38%), alcoholic 10(01.26%), septicemia 10(01.26%) and snake bite 2(00.26%)cases. A maximum number of deaths occurring between 0-6 hrs of injury was 395(49.82%) cases, and a minimum number of deaths in 24-48 hrs of injury was 22(02.77%) cases.

In 2021, three encephalitis cases due to the Borna disease virus 1 (BoDV-1) were diagnosed in the north and east of Germany. The patients were from the states of Thuringia, Saxony-Anhalt, and Lower Saxony. All were residents of known endemic areas for animal Borna disease but without prior diagnosed human cases. Except for one recently detected case in the state of Brandenburg, all >30 notified cases had occurred in, or were linked to, the southern state of Bavaria. Of the three detected cases described here, two infections were acute, while one infection was diagnosed retrospectively from archived brain autopsy tissue samples. One of the acute cases survived, but is permanently disabled. The cases were diagnosed by various techniques (serology, molecular assays, and immunohistology) following a validated testing scheme and adhering to a proposed case definition. Two cases were classified as confirmed BoDV-1 encephalitis, while one case was a probable infection with positive serology and typical brain magnetic resonance imaging, but without molecular confirmation. Of the three cases, one full virus genome sequence could be recovered. Our report highlights the need for awareness of a BoDV-1 etiology in cryptic encephalitis cases in all areas with known animal Borna disease endemicity in Europe, including virus-endemic regions in Austria, Liechtenstein, and Switzerland. BoDV-1 should be actively tested for in acute encephalitis cases with residence or rural exposure history in known Borna disease-endemic areas.

Background: Understanding Alzheimer’s disease (AD) in terms of its various pathophysiological pathways is essential to unravel the complex nature of the disease process and identify potential therapeutic targets. The renin-angiotensin system (RAS) has been implicated in several brain diseases, including traumatic brain injury, ischemic stroke, and AD. Objective: This study was designed to evaluate the protein expression levels of RAS components in postmortem cortical and hippocampal brain samples obtained from AD versus non-AD individuals. Methods: We analyzed RAS components in the cortex and hippocampus of postmortem human brain samples by western blotting and immunohistochemical techniques in comparison with age-matched non-demented controls. Results: The expression of AT1R increased in the hippocampus, whereas AT2R expression remained almost unchanged in the cortical and hippocampal regions of AD compared to non-AD brains. The Mas receptor was downregulated in the hippocampus. We also detected slight reductions in ACE-1 protein levels in both the cortex and hippocampus of AD brains, with minor elevations in ACE-2 in the cortex. We did not find remarkable differences in the protein levels of angiotensinogen and Ang II in either the cortex or hippocampus of AD brains, whereas we observed a considerable increase in the expression of brain-derived neurotrophic factor in the hippocampus. Conclusion: The current findings support the significant contribution of RAS components in AD pathogenesis, further suggesting that strategies focusing on the AT1R and AT2R pathways may lead to novel therapies for the management of AD.

Background: Knowledge of the anatomy of the circulus arteriosus cerebri (CAC) is important in understanding its role as an arterial anastomotic structure involved in collateral perfusion and equalization of pressure, and may explain observed variations in neurovascular disease prevalences across populations. This study was aimed at understanding the anatomical configuration and morphometric properties of the CAC in Malawian population. Materials and methods: Brains were collected from 24 recently-deceased black Malawian human cadavers during medico-legal autopsies. Photographs of the CACs were taken using a camera placed at a 30 cm height from the base of the brain. Whole-circle properties and segmental vessel parameters were analyzed using the OSIRIS computer program, paying attention to completeness, typicality, symmetry, and segmental vessel diameters and lengths. Results: 69.57 % of the CACs exhibited the complete-circle configuration. Of these, 37.5 % were typical, representing an overall typicality prevalence of 26.09 %. Vessel asymmetry was observed in 30.43 % of cases. There were 7 cases of vessel aplasia and 12 cases of vessel hypoplasia. The posterior communicating artery (PcoA) was the most variable (with 12 variations), widest (7.67 mm) and longest (27.7 mm) vessel while the anterior communicating artery (AcoA) was the shortest (0.78mm). Both the AcoA and the PcoA were the narrowest vessels (0.67 mm) in this study. CAC variations in Malawian populations appeared to be similar to those observed in diverse populations. Conclusions: Anatomical variations of the circulus arteriosus cerebri exist in Malawian population and should be taken into consideration in clinical practice.

Background Infection by SARS‐CoV‐2 produces significant pulmonary pathology including endothelial damage with resultant thrombotic events. While pathologic features were described, there are limited data on the relationship of these changes to the inflammatory response and the production of thromboses. Objective To investigate pathology of COVID‐19‐related immunothrombosis. Patients/Methods Tissue samples from lung, kidney, brain and heart that were collected from 45 patients who died of COVID‐19. Histopathological examination was performed after H&E and Picro‐Mallory staining in combination with (immuno)fluorescence to visualize neutrophil extracellular traps. Ultrastructural alterations in lungs were studied with scanning and transmission electron microscopy. Results Inflammatory changes and thrombosis were substantially more pronounced in the lung than in the kidney, heart, and brain. The most common pathologic finding was diffuse alveolar damage. In addition, most lung samples showed thrombi in vessels. The cause of death in single cases was massive pulmonary embolism. Ultrastructural examination revealed neutrophils attached to endothelium, perhaps as a step towards transendothelial migration. In addition, platelets were identified in the midst of fibrin as individual procoagulant balloon‐like cells. Ultrastructural examination demonstrated numerous virion‐like particles. Conclusions Studying (ultra)structural features of the autopsy lung samples from patients with COVID‐19 has provided evidence for a pathogenic link between inflammation and thrombosis. The major features in the lungs of COVID‐19 patients comprised primary inflammatory thrombosis associated with diffuse alveolar damage. The lungs had pronounced circulatory changes with inflammation‐dependent intravascular blood clotting, whereas heart, brain, and kidneys had predominantly degenerative changes that were distinct from the lung pathology.

Objectives The clinical manifestations of COVID-19 associated cardiac complications are heterogeneous, ranging from asymptomatic to severe symptoms, including arrhythmias and cardiogenic shock. For COVID-19 patients with cardiac sequela, only a small subset of patients have myocarditis; the pathogenesis of cardiac sequela caused by SARS-CoV-2 other than microthrombi associated sequela remains to be determined. Methods Retrospective analysis of 71 heart autopsy specimens from COVID-19 and putative COVID-19 in the NIH COVID Digital Pathology Repository. Results The most consistent observation was localized myocardial cell death not associated with either myocarditis or microthrombi. Red blood cells were typically absent from capillaries but, when observed, were predominately in linear clusters (stacks) of adjacent cells. Conclusions Based on our retrospective analysis, we propose that localized ischemia and subsequent cell death by anoxia contributes to the cardiac pathogenesis in some COVID-19 patients. We propose two new models predicting vasoconstriction of cardiac pericyte cells induced by elevated histamine from hyper-activated mast cells or direct infection. We propose that impeded blood flow and cell death by anoxia are initial steps in the development of SARS-CoV-2 induced cardiac injury in COVID-19 patients independent of microthrombi or myocarditis.

OBJECTIVE Heterotaxy or isomerism of the atrial appendages is a congenital disorder with variable presentation, associated with both cardiac and non-cardiac anomalies, which may have a serious impact on fetal outcome. The aim of this exploratory study was to assess the value of fetal magnetic resonance imaging (MRI), as a complementary tool to ultrasound, for describing the morphological spectrum encountered in heterotaxy. METHODS This retrospective study included 27 fetuses that underwent fetal MRI following prenatal suspicion of heterotaxy on ultrasound from 1998 to 2019 in a tertiary referral center. Heterotaxy was classified as left atrial isomerism (LAI) or right atrial isomerism (RAI) based on fetal echocardiography (FE) examination. In addition to routine prenatal ultrasound, fetal MRI was offered routinely to enhance the diagnosis of non-cardiac anomalies, which might have been missed on ultrasound. Prenatal findings on ultrasound, FE and MRI were reviewed systematically and compared with those of postnatal imaging and autopsy reports. RESULTS Twenty-seven fetuses with heterotaxy and cardiovascular pathology, of which 19 (70%) had LAI and eight (30%) had RAI, were included. Seven (7/19 (37%)) fetuses with LAI had normal intracardiac anatomy, whereas all fetuses with RAI had a cardiac malformation. All 27 fetuses had non-cardiac anomalies on fetal MRI, including situs and splenic anomalies. In 12/19 (63%) fetuses with LAI, a specific abnormal configuration of the liver was observed on MRI. In three fetuses, fetal MRI revealed signs of total anomalous pulmonary venous connection obstruction. An abnormal bronchial tree pattern was suspected on prenatal MRI in 6/19 (32%) fetuses with LAI and 3/8 (38%) fetuses with RAI. CONCLUSIONS Visualization on MRI of non-cardiac anomalies in fetuses with suspected heterotaxy is feasible and can assist the complex diagnosis of this condition, despite its limitations. This modality potentially enables differentiation of less severe cases from more complex ones, which may have a poorer prognosis. Fetal MRI can assist in prenatal counseling and planning postnatal management. © 2021 The Authors. Ultrasound in Obstetrics & Gynecology published by John Wiley & Sons Ltd on behalf of International Society of Ultrasound in Obstetrics and Gynecology.

A 57-year-old woman with a sudden-onset seizure was hospitalized. Brain magnetic resonance imaging findings led to a suspicion of leptomeningeal carcinomatosis (LMC) without a brain parenchymal tumor, and abdominal computed tomography showed a tumor in the pancreatic tail. Endoscopic ultrasonography-guided fine needle aspiration of the pancreatic mass revealed adenocarcinoma. Therefore, LMC from pancreatic ductal adenocarcinoma was strongly suspected. She received three courses of nab-paclitaxel plus gemcitabine and whole-brain radiation. Shortly thereafter, she developed a severe consciousness impediment and died. A pathological autopsy showed adenocarcinoma in a wide area of the leptomeninges.

Fabry disease (FD) is an inherited X-linked lysosomal storage disorder, with hemizygous males being more severely affected than heterozygous females. Herein, we report a rare case of FD in a heterozygous female with a severe phenotype. The patient had obesity and hyperlipidemia and had her first cerebral infarction at the age of 33 years. She underwent renal biopsy and was diagnosed with FD with morphological features of focal segmental glomerulosclerosis nephropathy at the age of 34 years. Her leukocyte alpha-galactosidase A activity was 2.3 Agal/U (normal: >20 Agal/U), and genetic analysis revealed the presence of the classical phenotype. Enzyme replacement therapy (ERT) was initiated at the age of 35 years; however, peritoneal dialysis owing to end-stage renal failure occurred at the age of 37 years. The patient died of a cerebral hemorrhage at the age of 44 years. Her Mainz Severity Score Index at the time of death was 48/76, suggestive of the severe phenotype. Autopsy findings revealed remarkable globotriaosylceramide accumulation on electron microscopy, particularly in the major organs and their vascular smooth muscle cells. Regarding the vertebral arteries which sourced the cerebral hemorrhage, the effects of FD-induced vascular thickening and long-term renal failure-induced atherosclerosis were confirmed. Furthermore, the patient’s vascular sclerosis was modified with acquired factors such as lifestyle-related disease associated with obesity. We recommend intensified treatment for metabolic factors simultaneous with ERT to help in delaying the progression of FD.

A 9-year-old boy collapsed shortly after complaining of shortness of breath. Despite immediate resuscitation measures, the boy died. A few weeks earlier, he had received antibiotic treatment for respiratory infection. However, the post-mortem examination revealed an advanced tumor mass of the mediastinum with infiltration of vital structures, which was identified as a small blue round neoplasm with aspects of an extramedullary Ewing-like sarcoma by supplementary histological and immunohistochemical examinations.This dramatic clinical course of events shows that the possible presence of serious diseases should always be considered behind harmless symptoms, even in children.

A 70-year-old man with multicentric Castleman disease (MCD) was admitted to our hospital with jaundice and ascites. Elevations in his bilirubin and interleukin-6 levels were noted, and computed tomography revealed hepatic atrophy and portal vein and bile duct disorders. Steroid therapy was started for MCD, but he died of hepatic failure. An autopsy revealed that the MCD activity was mild, but advanced fibrosis and cholestasis were observed in the liver. Mild infiltration of interleukin-6-positive plasma cells was noted in the highly fibrotic area of the liver. Although rare, liver and biliary tract damage may be also considered organ disorders of MCD.

Fusarium spp. can cause invasive infection with fatal outcomes in immunocompromised patients. Therefore, invasive fusariosis is rare after solid organ transplantation. For this reason, experience and management are limited to single published case reports.We report a 65-year-old female patient with disseminated brain abscesses caused by Fusarium after liver transplantation (LT). The patient underwent LT for secondary sclerosing cholangitis after acute respiratory distress syndrome (ARDS). After a complicated course with aneurysm and thrombosis of the hepatic artery, re-transplantation was performed after one month. Due to inadequate awakening response, cerebral imaging was performed, which showed multiple abscesses. The patient died shortly thereafter, and an autopsy showed fusariosis.Fusarium spp. führen bei immungeschwächten Patienten zu einer invasiven Infektion mit meist tödlichem Ausgang. Eine invasive Fusariose wird nach der Transplantation solider Organe jedoch seltener berichtet. Aus diesem Grund ist die Erfahrung und das Management dieses Infektes auf einzelne Fallberichte beschränkt.Wir berichten von einer 65-jährige Patientin, die nach einer Lebertransplantation (LT) disseminierte Hirnabszesse verursacht durch Fusarien entwickelte. Die Patientin unterzog sich einer LT aufgrund einer sekundär sklerosierender Cholangitis nach akutem Lungenversagen (ARDS). Nach einem komplizierten Verlauf mit Aneurysma und Thrombose der Leberarterie erfolgte nach einem Monat eine Re-Transplantation. Aufgrund der unzureichenden Aufwachreaktion wurde eine zerebrale Bildgebung durchgeführt, die multiple Abszesse zeigte. Die Patientin verstarb kurz darauf, in der Autopsie wurden Fusarien nachgewiesen.

Two rock hyraxes ( Procavia capensis), from the Chattanooga Zoo, were submitted separately for autopsy at the University of Tennessee Veterinary Medical Center. The first was a 4-y-old intact female that died without premonitory signs and the second was a 10-y-old intact male that was euthanized because of severe renal disease. Microscopically, the lungs of both hyraxes had multifocal-to-coalescing

Összefoglaló. Bevezetés: A kórboncolás hozzájárul a súlyos akut légzőszervi szindrómát okozó koronavírus-2 (SARS-CoV-2-) fertőzés klinikopatológiai vonatkozásainak megismeréséhez. Célkitűzés: A SARS-CoV-2-fertőzöttek boncolása során gyűjtött tapasztalatok bemutatása. Módszer: Egymást követően boncolt, védőoltásban nem részesült, SARS-CoV-2-fertőzött elhunytak klinikai adatait, makro- és mikroszkópos észleleteit összegeztük; a tüdőkimetszéseket SARS-CoV-2-nukleokapszid-immunfestéssel vizsgáltuk. Eredmények: A boncolást a halálok megállapítására (n = 14), tumorgyanú (n = 9), illetve törvényi kötelezettség (n = 3) miatt végeztük. A fertőzést a klinikai észlelés vagy a boncolás során (n = 4) végzett SARS-CoV-2-nukleinsav-teszt igazolta. A tünetes betegség átlagos hossza 12,9 nap volt. 21 betegnél (medián életkor 69 év; 18 férfi) állt fenn COVID–19-pneumonia, mely 16 esetben önmagában, 4 esetben bakteriális pneumoniával vagy álhártyás colitisszel szövődve okozott halált; 1 antikoagulált pneumoniás beteg heveny retroperitonealis vérzésben halt meg. 3 betegnél a halált disszeminálódott malignus tumor, 1 betegnél coronariathrombosis, 1 mentálisan retardált betegnél pedig pulmonalis emboliás szövődmény okozta. A COVID–19-pneumoniás tüdők nehezek, tömöttek és vörösen foltozottak voltak. Szövettanilag a betegség időtartamától függően diffúz alveolaris károsodás korai exsudativ vagy későbbi proliferativ fázisa látszott atípusos pneumocytákkal; gyakori volt a microthrombosis (n = 7), a macrothrombosis (n = 5), illetve a pulmonalis embolia (n = 4). A SARS-CoV-2-immunfestés pozitívnak bizonyult az esetek 38,5%-ában, dominálóan az exsudativ fázisban. Minden elhunyt társbetegség(ek)ben szenvedett, így magasvérnyomás-betegségben (n = 17), érelmeszesedésben (n = 14), 2-es típusú diabetesben (n = 8), rosszindulatú daganatban (n = 6), krónikus obstruktív tüdőbetegségben (n = 4), elhízásban (n = 3), vesetranszplantáció utáni immunszuppresszióban (n = 3). Következtetés: Az irodalmi adatokkal összhangban, halálos COVID–19-pneumonia túlnyomóan idős, társbetegség(ek)től sújtott férfiakban alakult ki. A boncolási gyakorlatban a SARS-CoV-2-nukleokapszid-immunfestéstől a diffúz alveolaris károsodás korai fázisában várható pozitivitás. Orv Hetil. 2021; 162(45): 1791–1802. Summary. Introduction: Autopsy is an important tool for the evaluation of severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) infection. Objectice: The aim of this study was to present our experience with autopsies of patients diagnosed with SARS-CoV-2 infection. Method: Clinical data, macroscopic and microscopic findings of consecutive postmortems of non-vaccinated SARS-CoV-2 patients are summarized. Lung samples were evaluated with SARS-CoV-2 nucleocapsid immunohistochemistry. Results: Autopsies were performed to determine the cause of death (n = 14), suspected tumours (n = 9) or due to legal obligation (n = 3). SARS-CoV-2 infection was verified by ante mortem (n = 22) and post mortem (n = 4) polymerase chain reaction. The mean duration of symptomatic disease was 12.9 days. Of 21 patients with COVID-19 pneumonia, 16 died of respiratory failure, 4 had additional bacterial pneumonia or Clostridioides difficile infection, and 1 developed hemorrhagic complication (n = 1). Other causes of death included disseminated malignancies (n = 3), coronary thrombosis (n = 1) and pulmonary embolism (n = 1). The affected lungs were heavy and had patchy red appearance. Exudative or proliferative phases of diffuse alveolar damage (DAD) were detected with atypical pneumocytes. Microthrombosis (n = 7), macrothrombosis (n = 5) and pulmonary embolism (n = 4) were frequent. The SARS-CoV-2 immunohistochemical reaction was positive in 38.5% of cases. All patients had co-morbidities, namely, hypertension (n = 17), atherosclerosis (n = 14), diabetes (n = 8), malignancies (n = 6), chronic obstructive pulmonary diseases (n = 4), obesity (n = 3) and immunosuppression after kidney transplantation (n = 3). Conclusion: Fatal COVID-19 pneumonia occurred mostly in elderly males with co-morbidities. In the autopsy practice, the SARS-CoV-2 nucleocapsid immunohistochemical reaction may confirm the infectious etiology in the early phase of DAD. Orv Hetil. 2021; 162(45): 1791–1802.

A 59-year-old man with a history of cerebral palsy and dextroscoliosis died in a group home. He required supplemental oxygen and had no bowel movement for weeks prior to death. At autopsy, the abdomen was markedly distended and there were flexion contractures of the legs. Postmortem computed tomography revealed a dilated digestive tract and fecal loading in the sigmoid and rectum, marked upwardly displaced diaphragm and scoliosis. On internal examination, the diaphragm was displaced rostrally and the rectosigmoid colon contained 2.5 kg of fecaloma with two rectal fecaliths. Severe scoliosis with marked reduction in volume of thoracic cavity was present. Microscopic examination revealed chronic aspiration pneumonia and chronic pulmonary hypertension. Overall, four factors led to respiratory failure: fecaloma; cerebral palsy; scoliosis; and chronic aspiration pneumonia. Based on clinicopathological correlation, the cause of death was determined to be a combination of these factors, and the key acute factor was the fecaloma.

This report describes the clinical context and autopsy findings in the first reported fatal case of acute disseminated encephalomyelitis (ADEM), developed after being vaccinated using the Oxford/AstraZeneca COVID-19 vaccine. ADEM is a rare autoimmune disease, causing demyelination in the brain and spinal cord. A wide variety of precipitating factors can trigger ADEM, and it has long been known to be a rare adverse event following some types of vaccinations. Recently, ADEM has also been associated with COVID-19 infection and (very rarely) with COVID-19 vaccination. The reports of the latter however all pertain to living patients. Our case demonstrates that ADEM should be considered in patients developing neurological symptoms post COVID-19 vaccination, although that this adverse reaction is likely to remain extremely rare. Our report further emphasizes the added value of comprehensive post mortem investigation to confirm ante mortem diagnosis and to determine vaccination safety. Supplementary Information The online version contains supplementary material available at 10.1007/s12024-021-00440-7.

We report the death of a 22-year-old woman, with a 3½ year history of cyclic vomiting and cannabis use since age 14, who developed torsades de pointes cardiac arrythmia while being treated in the emergency room for nausea and vomiting. Resuscitation restored spontaneous cardiac circulation, however, due to post-cardiac arrest anoxic brain injury, she never regained consciousness and was declared brain dead 4 days later. Postmortem examination confirmed hypoxic-ischemic encephalopathy, in keeping with the in-hospital diagnosis of brain death. The heart was anatomically normal but showed signs of acute post-cardiopulmonary arrest reperfusion injury. As a consequence of limited survival in hospital in a neuro-vegetative state, early bronchopneumonia and isolated pulmonary thromboemboli were seen. Toxicological studies confirmed cannabis use, in addition to the presence of haloperidol and ondansetron. Genetic studies were performed to rule out a possible channelopathy and revealed a mutation in the MYBPC3 and RYR2 genes. Death in this woman with cannabinoid hyperemesis syndrome was attributed to a fatal cardiac arrhythmia complicating vomiting-induced hypokalemia and treatment with QT interval prolonging and potentially arrhythmogenic medications, with the identified cardiac genetic mutations listed as contributing factors. The emphasis of this report is a) to raise awareness that death can occur due to cyclic vomiting, b) provide a brief but practical overview of cannabinoid hyperemesis syndrome, c) describe the findings from our postmortem examination and come to the most reasonable cause and mechanism of death, d) comment on the risk factors associated with torsades de pointes cardiac arrythmia, and e) conclude that a complete postmortem examination is needed to exclude an anatomical or toxicological cause of death in cannabinoid hyperemesis syndrome, a disabling but preventable disorder.

Background Pulmonary tumor thrombotic microangiopathy (PTTM), a rare manifestation of metastatic cancer with poor prognosis, is characterized by subacute/acute fatal pulmonary hypertension. The main cause of PTTM is gastric cancer, and cases of early gastric cancer confirmed using autopsy have been reported. Moreover, several cases of early gastric cancer that are undetectable on endoscopy or macroscopic postmortem examination have been reported. Case presentation A previously healthy 50-year-old man presented with progressive dyspnea and cough for 1 month. Echocardiography suggested pulmonary hypertension. Computed tomography revealed diffuse lymphadenopathy, whereas blood work revealed an elevation in several serum tumor marker levels. Despite normal upper endoscopic findings, a presumptive diagnosis of PTTM due to gastric cancer was made based on pathological findings of cervical lymph node biopsy, which indicated signet ring cell carcinoma. Imatinib and tegafur/gimeracil/oteracil plus oxaliplatin therapy were started on day 7. The patient’s condition was initially stable. However, his symptoms suddenly progressed, and the patient died on day 8. Macroscopic postmortem examination revealed no abnormal gastric wall findings. Microscopically, PTTM was confirmed, and multiple serial sections of the stomach revealed early gastric cancer. Conclusions Despite normal endoscopic findings, micro-occult gastric cancer can lead to PTTM. Physicians should be aware of this disease presentation. Taking prompt action is needed when PTTM is suspected, even if the patient appears stable.

BACKGROUND AND AIMS: This study aimed to investigate whether N-benzyl-N-methyl-2-[7,8-dihydro-7-(2-[18F]fluoroethyl)-8-oxo-2-phenyl-9H-purin-9-yl]acetamide (18F-FEDAC), a probe for translocator protein (TSPO), can visualize atherosclerotic lesions in rabbits and whether TSPO is localized in human coronary plaques. METHODS: 18F-FEDAC-PET of a rabbit model of atherosclerosis induced by a 0.5% cholesterol diet and balloon injury of the left carotid artery (n = 7) was performed eight weeks after the injury. The autoradiography intensity of 18F-FEDAC in carotid artery tissue sections was measured, and TSPO expression was evaluated immunohistochemically. TSPO expression was examined in human coronary arteries obtained from autopsy cases (n = 16), and in human coronary plaques (n = 12) aspirated from patients with acute myocardial infarction (AMI). RESULTS: 18F-FEDAC-PET visualized the atherosclerotic lesions in rabbits as high-uptake areas, and the standard uptake value was higher in injured arteries (0.574 ± 0.24) than in uninjured arteries (0.277 ± 0.13, p

Background Disseminated Mycobacterium avium complex infection is an important indicator of acquired immunodeficiency syndrome (AIDS) in patients with advanced human immunodeficiency virus (HIV) infection. Effective antiretroviral therapy has dramatically reduced the incidence of and mortality due to HIV infection, although drug resistance and poor medication adherence continue to increase the risk of disseminated M. avium complex infection. However, gastrointestinal lesions in cases of disseminated M. avium complex infection resulting in protein-losing enteropathy have been rarely discussed. Therefore, we present a case of protein-losing enteropathy caused by disseminated M. avium complex infection in a patient undergoing antiretroviral therapy. Case presentation A 29-year-old man was diagnosed with AIDS 4 years ago and was admitted for a 10-month history of refractory diarrhea and fever. Despite receiving antiretroviral therapy, the viral load remained elevated due to poor medication adherence. The patient was diagnosed with disseminated M. avium complex infection and started on antimycobacterial drugs 2 years before admission. However, the infection remained uncontrolled. The previous hospitalization 1 year before admission was due to hypoalbuminemia and refractory diarrhea. Upper gastrointestinal endoscopy revealed a diagnosis of protein-losing enteropathy caused by intestinal lymphangiectasia, and treatment with intravenous antimycobacterial drugs did not resolve his intestinal lymphangiectasia. The patient inevitably died of sepsis. Conclusions Clinical remission is difficult to achieve in patients with AIDS and protein-losing enteropathy caused by disseminated M. avium complex infection due to limited options of parenteral antiretroviral drugs. This report highlights the importance of identifying alternative treatments (such as an injectable formulation) for patients who do not respond to antiretroviral therapy due to protein-losing enteropathy with disseminated M. avium complex infection.

Background/Purpose Ectopic cervical thymus (ECT) is a rare cause of neck swelling in children. Our aim was to evaluate the diagnostic and management challenges of this condition. Methods Systematic review of the literature (1997–2020) using PRISMA guidelines. Results A total of 192 cases of ECT were identified. Forty-two cases (4 studies) were excluded because of insufficient data resulting in 150 cases (7 bilateral) in a total of 143 patients. ECT presented as a solid mass in 71 cases (10 incidentally found at autopsy, 33 conservatively managed and 28 excised) and as a cystic mass in 79 cases (all excised). A total of 165 imaging studies in 123 patients were performed (65 ultrasound scans (USS), 51 magnetic resonance imaging (MRI) scans and 49 computer tomography (CT) scans). ECT was diagnosed by imaging in 18.7% (23/123) of patients. Fine needle aspiration cytology or incisional/needle biopsy were performed in 16% (23/140) cases; a correct diagnosis of ECT was obtained in 52% (12/23) of those cases. There were no reports of malignancy in 143 specimens analysed. A normal mediastinal thymus was absent in 2.5% (1/40) of patients investigated. Conclusions ECT is a benign and relatively rare condition in children that is seldom diagnosed on imaging before tissue sampling or surgical excision. ECT might be the only active thymus in 2.5% of cases and careful consideration should be taken prior to its excision in young children. We recommend that ECT should be considered in the differential diagnosis of neck masses in children. Conservative management might be appropriate in selected cases and the presence of a normal mediastinal thymus in the thorax should be investigated before planning surgical excision.

Heme oxygenase-1 (HO-1), an inducible stress-response protein, exerts anti-oxidant and anti-apoptotic effects. However, its significance in forensic diagnosis of acute ischemic heart diseases (AIHD) such as myocardial infarction (MI) is still unknown. We examined the immunohistochemical expression of HO-1 in the heart samples to discuss their forensic significance to determine acute cardiac ischemia. The heart samples were obtained from 23 AIHD cases and 33 non-AIHD cases as controls. HO-1 positive signals in cardiomyocyte nuclear were detected in 78.2% of AIHD cases, however, that were detected in only 24.2% control cases with statistical difference between AIHD and non-AIHD groups. In contrast to HO-1 protein expression, there was no significant difference in the appearance of myoglobin pallor regions and leukocyte infiltration in the hearts between AIHD and non-AIHD groups. From the viewpoints of forensic pathology, intracardiac HO-1 expression would be considered a valuable marker to diagnose AIHD as the cause of death.

The concurrent onset of chronic lymphocytic leukemia (CLL) and acute myeloid leukemia (AML) is rare, and no autopsy case has been reported. We report herein the first case of concurrent-onset CLL and AML with an atypical phenotype revealed by autopsy. Notably, the diagnosis of AML was quite difficult during the patient’s lifetime because of the atypical phenotype. However, autopsy revealed that the patient’s bone marrow, liver, and spleen were filled with myeloblasts. In addition, p53 stain and PCR of IgH rearrangement using the autopsy specimen suggested that CLL and AML might be different clones. In conclusion, our case highlights the importance of considering synchronous complications of AML in CLL patients, particularly in those with an atypical clinical course.

Background Certain types of enteroviruses, including coxsackieviruses, cause encephalitis, and other neurological complications. However, these pathogens rarely cause fatal infections, especially in immunocompetent infants. In this study, we present a rare case of acute encephalopathy caused by coxsackievirus A2 (CV-A2), which progressed rapidly in a previously healthy female child. Case presentation In June 2013, a 26-month-old female child from Kanagawa, Japan, was found unresponsive during sleep. She was healthy until that morning. Her temperature was 37 °C at 08:00. She was feeling fine and went to the nursery that same morning. However, her condition worsened around noon. Therefore, she went home and slept at around 13:00. Surprisingly, after 2 h, her parents checked on her and found that she was lying on her back and was not breathing. Hence, she was immediately taken to a hospital by ambulance, but she was declared dead on arrival at the hospital. Subsequently, pathological autopsy and pathogenetic analysis, including multiple pathogen detection real-time PCR, were conducted to investigate the cause of death. The examination results revealed that she had an infectious respiratory disease and acute encephalopathy due to a CV-A2 infection. Conclusions Based on our findings, we concluded that a previously healthy girl who had no immediate history of underlying medical condition were susceptible to death by acute encephalopathy due to CV-A2 infections. We proposed this conclusion because the patient’s condition progressed rapidly in less than 2 h and eventually led to her death. This is the first report on an acute encephalitis-dependent death in a child due to CV-A2 infection.

We herein report a rare case of cartilage-hair hypoplasia (CHH) complicated with liver cirrhosis. A 20-year-old Japanese man with CHH was found incidentally to have liver cirrhosis and an esophageal varix. This patient had been treated for infections due to immunodeficiency since early childhood. He ultimately died of liver failure at 31 years of age. An autopsy revealed an abnormality of the interlobular bile ducts and intrahepatic cholestasis. Liver cirrhosis was thought to have been caused by chronic intrahepatic cholestasis due to biliary duct hypoplasia and changes in the intestinal microbiome. Therefore, CHH may cause biliary cirrhosis due to multiple effects.

Cryptococcus gattii infection is a rare cause of severe pulmonary disease and meningoencephalitis that has only recently been detected in the southeastern United States. We describe an organ transplant-associated outbreak of C. gattii infection involving an HIV-negative immunosuppressed donor in this region who died following new-onset headache and seizure of unknown cause. Retrospective cryptococcal antigen (CrAg) testing of donor serum was positive. Two of the three transplant recipients developed severe C. gattii infection 11 and 12 weeks following transplantation. One recipient died from severe pulmonary infection, identified on autopsy, and the other ill recipient survived following treatment for cryptococcal meningitis. This outbreak underscores the importance of considering cryptococcosis in patients with clinical findings suggestive of subacute meningitis or other central nervous system (CNS) pathology, and the potential benefit of routine pre-transplant donor CrAg screening using lateral flow assay to guide recipient antifungal prophylaxis. The case also adds to emerging evidence that C. gattii is a potential threat in the southeastern United States.

A 69-year-old man with refractory lung adenocarcinoma was treated with gemcitabine and vinorelbine. Dyspnea and hypertension developed after the 17th cycle of chemotherapy. Laboratory findings revealed intravascular hemolysis and renal dysfunction. Thrombotic microangiopathy (TMA) was confirmed by renal biopsy. Antihypertensive and steroid therapies were ineffective. After plasmapheresis, intravascular hemolysis and renal dysfunction gradually improved. However, the disease progressed, and he died 6 months after TMA diagnosis. Autopsy revealed similar pathological findings to those of the renal biopsy. It is important to discontinue gemcitabine at the onset of TMA and consider TMA when using gemcitabine for long periods.