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Biomedical subjects

V J Ojeda

Publications and source records attributed to V J Ojeda.

At least 37 records · Page 2Linked to original sources

Scleromyxedema and inflammatory myopathy: a clinicopathologic study of three patients.

Scleromyxedema (lichen myxedematosus) is a rare cutaneous manifestation in patients with idiopathic inflammatory myopathy. The clinical and histological findings in three patients with this association are presented. Two patients had a severe inflammatory polymyopathy which responded incompletely to corticosteroid therapy, while in the third, who developed esophageal carcinoma, the myopathy was relatively mild and the skin changes were the dominant feature. The occurrence of scleromyxedema in patients with inflammatory myopathy appears to carry a poor prognosis.

Adult↗

Primary cerebral neuroblastomas. A clinicopathological study of one adolescent and five adult patients.

Neuronal differentiation was demonstrated by light microscopy, immunohistochemistry and electron microscopy in the cerebral neoplasms of one adolescent and five adult patients. The tumours exhibited a wide spectrum of cellular differentiation from low to high grade malignancy which seems to correlate well with their biological behaviour. The natural history of these 6 cerebral neuroblastomas is rather long compared to that of other malignant primary cerebral neoplasms of adulthood; however, 2 patients died, one survived about 5 yr after initial symptoms whilst an untreated patient survived more than 12 yr. It is suggested that morphological grading along the lines of Kernohan's system may be useful in determining the prognosis and postoperative management of patients with cerebral neuroblastomas.

Adolescent↗

The neuropathology of progressive autonomic failure of central origin (the Shy-Drager syndrome).

The neuropathological features of the "Shy-Drager syndrome" have, in the past, been unsettled. The position was recently clarified by Oppenheimer who reviewed the 51 reported patients with progressive autonomic failure in whom neuropathological findings were given. He divided these cases into two groups. Group I included those with lesions of the pigmented nuclei of the brain stem which contained Lewy bodies. These he further subdivided into subgroups IA or IB according to whether or not the patient showed clinical evidence of Parkinson's disease in addition to the common denominator of orthostatic hypotension. In group II he placed those patients in whom multiple system atrophy was found at necropsy. These were the majority. Loss of neurons from the intermediolateral columns of the thoracic spinal cord, thought to be the cause of the postural hypotension, was present in all cases. To illustrate the new classification the necropsy findings in 3 patients taken from the files of the Royal Perth Hospital are described. One of these showed the lesions of Parkinson's disease with Lewy bodies, and thus conformed to Oppenheimer's type IA. The two other patients showed multiple system atrophy and thus belonged to Oppenheimer's type II. All three showed loss of neurons in the intermediolateral columns of the thoracic spinal cord. We believe that Oppenheimer's classification of progressive orthostatic hypotension improves the clinicopathological understanding of the syndrome and is thus useful as well as informative to the practising neurologist.

Aged↗

Medulloblastoma: a clinicopathological study of 42 cases.

Clinicopathological data have been collected for 42 patients with cerebellar medulloblastoma diagnosed and treated in Western Australia between the years 1961 and 1984. Thirty-one patients were male and 12 were over 14 years of age. In 31 patients the neoplasms were localized to the midline, while in 10 they were placed laterally in the cerebellar hemispheres. The remaining patient had a diffusely spreading neoplasm in the meninges of the posterior fossa. Thirty-nine underwent surgical treatment followed by postoperative radiotherapy. Three patients died in the early postoperative period. Twenty-four patients survived for one year or longer, 15 for two or more years, 10 for five years, and five survived longer than 10 years. The survival proportions estimated by the life-table method were 66% at one year, 48% at two years and 32% at 5 and 10 years. In one patient who survived for 23 years a meningioma developed, possibly due to radiotherapy.

Adolescent↗

Non-bacterial thrombotic endocarditis associated with malignant disease: a clinicopathological study of 16 cases.

Among 2627 necropsies performed in the Sir Charles Gairdner Hospital, Perth, over a period of 11 years, 16 cases of non-bacterial thrombotic endocarditis (NBTE) were found in patients with cancer (13 adenocarcinomas). The final stay in hospital of seven of these patients was complicated by a major embolic cerebral (six patients), or spinal cord (one patient), stroke. In all cases, the diagnosis of NBTE was made at necropsy. The aortic valve was affected in 10 patients, the mitral valve in five, and both the mitral and tricuspid valves in one. The diagnosis of NBTE should be considered in any patient with a known, or suspected, malignant neoplasm who suffers a stroke or other unexplained embolic events.

Adenocarcinoma↗

Inflammatory myopathies: Part 1.

The inflammatory myopathies have diverse clinical and pathological features and multiple etiologies. Some are confined to a single muscle or group of muscles (e.g., orbital myositis and localized nodular myositis) while others are diffuse. Infective forms may be due to viral, bacterial, fungal, protozoal, or parasitic organisms. Viruses may cause acute self-limited forms of myositis and have been isolated from muscle in some cases of acute rhabdomyolysis and inclusion body myositis. They have also been implicated in some cases of congenital myopathy and in polymyositis and dermatomyositis, but there is no evidence of viral invasion of muscle in these conditions. In polymyositis and dermatomyositis there are derangements in humoral and cellular immune function, and recent evidence suggests an underlying disturbance of immunoregulation. The roles of genetic factors, drugs, and Toxoplasma infection have been under scrutiny. There is increasing recognition of immunological and pathological differences in polymyositis and juvenile and adult dermatomyositis, and in cases with associated connective tissue diseases, suggesting different underlying pathogenetic mechanisms. Inclusion body myositis, eosinophilic myositis, and granulomatous myositis can be separated from the other idiopathic inflammatory myopathies because of distinctive clinical and pathological features and this may also reflect different mechanisms of muscle injury. Recent developments in the treatment of the idiopathic inflammatory myopathies include the use of plasmapheresis and total-body irradiation in cases that are resistant to corticosteroids and immunosuppressive drugs.

Bacterial Infections↗

Premalignant epithelial lesions of the gall bladder: a prospective study of 120 cholecystectomy specimens.

One hundred and twenty gall-bladders obtained at cholecystectomy for gall bladder diseases formed the basis of a prospective study of premalignant epithelial lesions. Six cases displayed abnormal mucosa (5%); 4 had atypical hyperplasia (dysplasia) and 2 gall-bladders had carcinoma-in-situ; all were associated with chronic cholecystitis and lithiasis. These changes are considered premalignant and are probably precursors of gall-bladder carcinoma. It is impossible to predict which patient with chronic gall-bladder disease is likely to harbour premalignant epithelial changes. In any event, thorough histological examination of all gall bladders removed surgically is more than justified.

Adult↗

Familial motor neurone disease associated with non-specific organic dementia. A clinico-pathological study of a family.

Two sisters developed motor neurone disease (MND), which was associated with progressive intellectual impairment in one; they survived for 18 and 24 months, respectively. At necropsy, the features of MND were confirmed, and the demented patient had severe non-specific cerebral atrophy. No evidence of Alzheimer's disease was found. Organic dementia of non-specific type can be associated with up to 15% of the familial cases of MND and with about 2% of the sporadic cases of MND in the Western world.

Aged↗

Multifocal progressive neurological disorder caused by neoplastic angioendotheliosis.

The case of a 46-year-old woman who suffered a multifocal neurological illness, which rapidly progressed to dementia, is reported; the clinical signs were suggestive of multiple sclerosis. At necropsy, polyfocal cerebral white-matter lesions, associated with intravascular plugs of neoplastic cells which appeared to arise from the vascular endothelium, were found. The findings were typical of a rare form of malignant disease of the vascular endothelium known as neoplastic angioendotheliosis, which shows a striking predilection for the central nervous system.

Brain↗

Necrotizing myelopathy associated with malignancy. A clinicopathologic study of two cases and literature review.

Two cancer patients had extensive spinal cord necrosis at necropsy. One had carcinoma of the breast and the other, carcinoma of the lung. Case 1 was treated by mastectomy, systemic chemotherapy and cranial irradiation, where as Case 2 received local irradiation to the right upper chest and right supraclavicular zone. In both cases, the spinal cord necrosis affected grey and white matter along most of its extent. Neither local nor systemic causes to explain the spinal cord lesion were disclosed at necropsy. It is suggested that these are two new examples of paraneoplastic necrotizing myelopathy. There are 22 such cases in the English language medical literature since 1903.

Adult↗

Giant cell granulomatous angiitis of the central nervous system in a patient with leukemia and cutaneous herpes zoster.

Pontine infarcts associated with granulomatous (giant cell) arteritis were the terminal events in a 71-year-old man treated for chronic myeloid leukemia with intermittent Busulphan therapy during the previous ten years. The final admission was precipitated by a severe episode of herpes-zoster infection of the scalp. Since 1950 there have been 25 papers in the English language medical literature describing 36 cases of granulomatous angiitis of the central nervous system (CNS). Seven cases, including this report, were associated with lymphoid malignancies, and five had cutaneous herpes-zoster as well. Virus-like particles were detected in the affected vessels in three patients and in the nearby glial cells of a further case.

Aged↗

Familial myopathy associated with Marfanoid features and multicores.

The cases of a mother and son with a slowly progressive generalised myopathy and Marfanoid features are described. Muscle biopsies showed atrophy, type 1 fibre preponderance, excessive variation in fibre size and shape, and multiple foci of myofibrillar disorganisation with loss of oxidative enzyme activity of the type found in multicore disease.

Adult↗

Cerebral gliosarcoma, pulmonary adenoid-cystic carcinoma, and pulmonary metastatic gliosarcoma: report of an untreated case.

An elderly woman had a cerebral gliosarcoma with a single pulmonary metastasis, together with a separate pulmonary adenoid-cystic carcinoma. Spontaneous extraneural metastases of primary intracranial neoplasms are exceedingly unusual; so far, only 8 proven cases are recorded in the English language medical literature. The association of cerebral gliosarcoma and a pulmonary adenoid-cystic carcinoma is presumably coincidental.

Aged↗

Non-specific senile dementia mimicking Huntington's disease in two siblings.

Two elderly brothers developed a progressive organic dementia associated with choreiform movements which was clinically suggestive of Huntington's disease. Necropsy studies disclosed non-specific cerebral atrophy in both patients. A precise diagnosis is of paramount importance in cases such as this because of the social and genetic implications of the diagnosis of Huntington's disease.

Aged↗

Primary malignant lymphomas of the brain. A clinicopathological study of five cases.

We report here the cases of five patients with primary malignant lymphoma of the brain; all died and a necropsy was performed in each case. Although these lymphomas remain localized, their prognosis is worse than that of lymphomas arising in any other site. Our five cases confirmed the reported natural history of this lesion as a highly malignant neoplasm. There was a discrepancy between the macroscopic appearance of the new growth and its microscopic extent; and the extensive infiltration of the surrounding tissue was not always detected by CT scan. Although primary malignant lymphomas of the brain are radiosensitive and radiotherapy can prolong life, careful clinical judgment is necessary to assess the desirability of the poor quality of life that may follow. Patients with severe intellectual impairment may receive no benefit from aggressive radiotherapy.

Adult↗

Necropsy study of the olfactory portal of entry in herpes simplex encephalitis.

Two fatal cases of sporadic herpes simplex virus (HSV) encephalitis were seen and diagnosed at the Sir Charles Gairdner Hospital, Perth, between 1979 and 1981. Necropsy studies were directed to the possible portal of entry of HSV into the central nervous system. Viruses were present in the left olfactory nerve in one patient. No viruses were found in the Gasserian ganglia of either patient. The finding of HSV within an olfactory nerve provides further evidence of an olfactory portal of entry to the central nervous system in sporadic human HSV encephalitis.

Aged↗