Paraneoplastic Cerebellar Degeneration and Lambert-Eaton Myasthenic Syndrome with SOX-1 Antibodies

A 69-year-old man was admitted to our hospital for progressive muscle weakness in both lower limbs and limb ataxia (day 0). Nerve conduction studies showed low compound muscle action potential amplitudes at rest and increased amplitudes after maximum voluntary contraction. Blood testing revealed SOX-1 antibodies. He was diagnosed with paraneoplastic cerebellar degeneration and Lambert-Eaton myasthenic syndrome (PCD-LEMS). He died from aspiration pneumonia on day 9. Small-cell lung carcinoma (SCLC), which had not been obvious on computed tomography, was found during the autopsy. Patients with PCD-LEMS who test positive for SOX-1 antibodies should be carefully evaluated for SCLC.     

Forensic medicine in Nepal: Past,present, and future

The practice of autopsy for medico-legal purposes in Nepal was started during the 1960s when jail doctors used to perform the autopsy. However, the medico-legal service in clinical forensic settings is less than three decades old. In Nepal, a police inquest is done for all the unnatural deaths who then subjects the body for medico-legal autopsy at the nearest government hospital. Except for a few hospitals where forensic medicine experts are available, the medico-legal work in Nepal is done by medical officers most of who have no forensic qualification. For a country with a population of nearly 30 million, there are less than 50 forensic medicine experts who are currently practicing. There are 21 medical colleges in Nepal each having a forensic medicine department engaged in teaching forensic medicine as a separate subject in the undergraduate medical curriculum. Currently, postgraduate courses are also offered in the country. However, there is a lack of uniformity in the curriculum set by different universities for both postgraduate and undergraduate education. Due to the lack of forensic medicine faculty members in some medical colleges, the undergraduate students of medicine have to undertake the exam being taught by some guest lectures and without witnessing a single medico-legal autopsy. To standardize the medico-legal services and forensic medicine education, the Medico-Legal Society of Nepal was established with an aim to conduct regular seminars, conferences, and CMEs and also various training programs for the non-forensic medicine experts who deal with medico-legal cases. This paper aims to provide a brief history of medico-legal practice in Nepal, the current situation, and future plans to improve the medico-legal service of the country.     

COVID-19 autopsies: conclusions from international studies

The rapid pace at which COVID-19 studies are being published is surpassed only by the spread of the virus and the destruction wreaked by the pandemic globally. Therefore, it is likely that, even in the few months prior to this article reaching print, the COVID-19 literature would have moved on. The authors of this article work at a centre for COVID autopsies in London, and the aim of the article is, using their first-hand experience of COVID-19 autopsies, to distil what in their judgement are the most valid and important findings of internationally published COVID-19 autopsy studies. The intention is to provide an illustrated summary of the pathology of the organ systems most often affected by COVID-19, which will be particularly useful to trainee histopathologists and to busy consultant surgical histopathologists who may not have encountered COVID-19 first hand. For the reader who wishes to probe further the question of pathogenesis, a few pertinent references are provided.     

Neoplastic lesions in CADASIL syndrome: report of an autopsied Japanese case

Cerebral autosomal dominant arteriopathy with subcortical infarcts and leucoencephalopathy (CADASIL) is one of the most common heritable causes of stroke and dementia in adults. The gene involved in the pathogenesis of CADASIL is Notch3; in which mutations affect the number of cysteine residues in its extracellular domain, causing its accumulation in small arteries and arterioles of the affected individuals. Besides the usual neurological and vascular findings that have been well-documented in CADASIL patients, this paper additionally reports multiple neoplastic lesions that were observed in an autopsy case of CADASIL patient; that could be related to Notch3 mutation. The patient was a 62 years old male, presented with a past history of neurological manifestations, including gait disturbance and frequent convulsive attacks. He was diagnosed as CADASIL syndrome with Notch3 Arg133Cys mutation. He eventually developed hemiplegia and died of systemic convulsions. Autopsy examination revealed-besides the vascular and neurological lesions characteristic of CADASIL- multiple neoplastic lesions in the body; carcinoid tumorlet and diffuse idiopathic pulmonary neuro-endocrine cell hyperplasia (DIPNECH) in the lungs, renal cell carcinoma (RCC), prostatic adenocarcinoma (ADC) and adenomatoid tumor of the epididymis. This report describes a spectrum of neoplastic lesions that were found in a case of CADASIL patient that could be related to Notch3 gene mutations.     

Typical or atypical progressive supranuclear palsy: a comparative clinicopathologic study of three Chinese cases

Progressive supranuclear palsy (PSP) is an atypical parkinsonism, which is the third most common geriatric neurodegenerative disease. We reported three pathology-confirmed Chinese PSP cases with special focus on the pathological accumulations of tau, a-synuclein and A-beta in the three PSP brains. Cases 1 and 2 initiated with extrapyramidal signs and gait disorders, while case 3 suffered behavioral abnormalities with cognitive decline at the beginning. In neuropathology, PSP-changes such as tau-positive tufed astrocytes, oligdendrocytes with the tau-positive coiled-body and threads and globose NFTs were widely seen in the basal ganglia, isocortex and allocortex, as well as in brainstem, cerebellum and spinal cord. In addition, numerous AGs were found in the hippocampus of cases 1 & 2, while Aβ amyloid depositions were found in hippocampus and leptomeningeal vessels of case 1 and in neocortex, entorhinal cortex, hippocampus, cingulate gyrus and amygdale of case 3. Vessel infarcts were observed in cases 1. Cortical laminar III necrosis in case 1 suggested the ischemic damage. Cervical spinal cords in cases 2 & 3 were obtained with tau-positive globose NFTs, tufted astrocytes and neuropil threads were respectively found in the neurons of anterior horn and surrounding white matters. In summary, pathological examination is crucial for the ambiguous cases to exclude other neurodegenerative diseases. Furthermore, cervical spinal cord should be routinely examined in the PSP autopsy.     

Stillbirths and early neonatal mortality in rural Northern Ghana

Objective To calculate perinatal mortality (stillbirth and early neonatal death: END) rates in the Upper East region of Ghana and characterize community‐based stillbirths and END in terms of timing, cause of death, and maternal and infant risk factors. Methods Birth outcomes were obtained from the Navrongo Health and Demographic Surveillance System over a 7‐year period. Results Twenty thousand four hundred and ninty seven pregnant women were registered in the study. The perinatal mortality rate was 39 deaths/1000 deliveries, stillbirth rate 23/1000 deliveries and END rates 16/1000 live births. Most stillbirths were 31 weeks gestation or less. Prematurity, first‐time delivery and multiple gestation all significantly increased the odds of perinatal death. Approximately 70% of END occurred during the first 3 postnatal days, and the most common causes of death were birth asphyxia and injury, infections and prematurity. Conclusion Stillbirths and END remain a significant problem in Navrongo. The main causes of END occur during the first 3 days and may be modifiable with simple targeted perinatal policies.