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Biomedical subjects

F Gray

Publications and source records attributed to F Gray.

At least 163 records · Page 9Linked to original sources
PubMed1990

MRI pattern of progressive multifocal leukoencephalopathy (PML) in AIDS. Pathological correlations.

Progressive multifocal leukoencephalopathy (PML) is a demyelinating disease which occurs in immunodepressed subjects and is particularly frequent in AIDS. Some authors having drawn attention to the protean aspect of the disease and claimed that AIDS may lose its basic characteristics and affect the grey matter as well as the white matter, we reviewed a series of 8 patients who had been biopsied and/or autopsied and had been examined at least once by MRI. In this series, contrary to what is regularly observed in toxoplasmic abscesses we did not find any lesion of the grey matter or any mass effect. On the other hand, we confirmed that PLM is not multifocal in all cases and that it course may be interrupted by prolonged remissions. The MRI criteria for PML therefore are reliable, provided multiple T2-weighted slices in coronal plane are performed, clearly showing the anatomy of the white fibres affected. However, it must be borne in mind that HIV-infected patients often have other associated brain pathologies, especially when the immune deficiency increases.

Acquired Immunodeficiency Syndrome↗
PubMed1990

[Lesions of the spinal cord and spinal roots in human immunodeficiency virus infection].

Spinal cord lesions, although far less frequent than brain lesions, are not uncommon in AIDS. Almost all diffuse or multifocal pathological processes involving the central nervous system and/or leptomeninges may also affect the spinal cord. However, some of them involve it predominantly causing specific, clinically overt, myelopathic diseases. The most characteristic of these is vacuolar myelopathy which usually manifests as progressive spastic ataxia. Its incidence varies in different pathological series and its pathogenesis is still controversial. Acute meningomyeloradiculitis in AIDS patients presents a remarkably uniform and distinct clinical and cerebrospinal fluid profile although they might be due to various causes including cytomegalovirus infection, syphilis, mycobacterial infection or leptomeningeal malignancy. Tumoral focal syndromes have exceptionally been recorded due to epidural lymphomas, intraspinal gliomas or toxoplasma abscesses. Spinal cord ischemic lesions have been documented in two cases: they were related to inflammatory lesions of the blood vessels in one case and to diffuse intravascular coagulation in the other. An ascending myelitis syndrome, secondary to spinal cord infection by both herpes simplex virus type 2 and cytomegalovirus, without significant vasculitis has also been reported.

HIV Infections↗
PubMed1990

[Cytomegalovirus encephalo-myelo-radiculitis in acquired immunodeficiency syndrome].

A 30 year-old male, with the acquired immune deficiency syndrome (AIDS) presented with rapidly progressive flaccid paraplegia and sphincter incontinence. Cerebrospinal fluid examination showed elevated protein and pleocytosis. Death occurred 2 months after the onset of neurological signs. Post-mortem examination showed inflammatory necrotic lesions, relatively sparing the axons and predominantly involving the roots of the cord. Numerous cytomegalovirus (CMV) inclusion bodies were found in the necrotic lesions, in the subarachnoid spaces and in Schwann cells. CMV encephalitis and involvement of the 3rd cranial nerves were also observed. Only 8 well-documented clinico-pathological cases of acute CMV myeloradiculitis, which all presented as progressive cauda equina syndrome, have been reported until now in AIDS patients.

Acquired Immunodeficiency Syndrome↗
PubMed1990

[Cytomegalovirus (CMV) and human immunodeficiency virus (HIV) co-infection, of multinucleated giant cells in acquired immunodeficiency syndrome (AIDS) encephalopathy].

A 31-year-old HIV-1-seropositive Haitian male presented with HIV-nephropathy and typical features of subcortical dementia. He died 4 months after the onset of neurological signs. Neuropathological examination revealed HIV encephalitis with diffuse progressive leukoencephalopathy, diffuse poliodystrophy and massive calcifications of white matter, basal ganglia and dentate nuclei probably partly related to renal failure. It was associated with focal, subependymal CMV encephalitis. In the periventricular regions, morphologically characteristic multinucleated giant cells, positive for CD68, and negative for GFAP, contained early CMV antigens (E13) in their nuclei and HIV antigens (gp41 and p24) in their cytoplasm. The co-infection of a single cell by both viruses was confirmed by electron microscopy. The finding that HIV and CMV can co-infect in vivo the same cell raises the question of a direct cooperation of both viruses at the single cell level, and suggests the possibility of a role for CMV as co-factor in the pathogenesis of HIV encephalopathy.

AIDS Dementia Complex↗
PubMed1990

[Obsessive-compulsive behavior and progressive supranuclear palsy].

A case of progressive supranuclear palsy characterized by a loss of self-activation and a compulsive behaviour of the obsessive type is reported. The pathological examination was remarkable for the intensity of pallidal lesions and their diffusion to both the external and internal segments. While the loss of self-activation seemed to result from a damaged cortico-subcortical circuit forming a limbic loop, the compulsive behaviour of the obsessive type may have resulted from the interruption of a frontal-caudal-pallidal-luysian circuit.

Globus Pallidus↗
PubMed1989

Diffuse "encephalitic" cerebral toxoplasmosis in AIDS. Report of four cases.

Four patients with AIDS presented with a rapidly fatal global neurological illness. CT did not show any focal lesion and gross post mortem examination of the brain was normal in three of the four cases. Microscopic examination revealed numerous widespread microglial nodules in the brain parenchyma, most containing central toxoplama cysts or free tachyzoites. Such diffuse, non-necrotic, "encephalitic" forms of cerebral toxoplasmosis appear unique to AIDS and, to our knowledge, have not been documented previously. They represent a treatable, often misdiagnosed cause of diffuse neurological involvement in AIDS patients.

Acquired Immunodeficiency Syndrome↗
PubMed1989

Acute myeloradiculitis due to cytomegalovirus as the initial manifestation of AIDS.

A 26 year old male intravenous drug abuser presented with rapidly progressive paraplegia and total incontinence. CSF examination showed elevated protein level and pleocytosis. HIV testing was positive. Anti CMV titres were mildly elevated in serum and CSF. Death occurred 26 days after the onset of neurological signs. Necrotic and inflammatory lesions with numerous inclusion bodies characteristic of CMV were found in the roots of the cauda equina, conus terminalis and lumbar segments of the spinal cord. CMV subependymal encephalitis and HIV encephalitis were also present.

Acquired Immunodeficiency Syndrome↗
PubMed1989

[Vertebral hemangioma. Definition, limitations, anatomopathologic aspects].

Vertebral hemangiomas are benign, malformative vascular tumours composed of newly formed blood vessels with normal (capillary, venous, or veno-capillary) structure and without arterio-venous shunt; they involve firstly the vertebra and may secondary extend to the epidural space. These features allow to separate vertebral hemangiomas from bone lymphangiomas, from true neoplastic vascular tumours such as hemangioendotheliomas, hemangiopericytomas, angiosarcomas, from hemangioblastomas and from arteriovenous malformations with shunt. The latter are usually associated with other vascular malformation of the skin and spinal cord in the same metameric segment(s) (metameric angiomatosis or Cobb syndrome). There are two microscopic types of vertebral hemangiomas which frequently coexist. Cavernous angiomas are composed of large dilated blood vessels closely clustered together, so that they are not separated by normal bone tissue. Capillary angiomas are formed of thin-walled capillary vessels of various sizes separated by normal bone tissue. The vascular malformation is not separated from the bone tissue by a capsule; the surrounding osseous lamellae usually show secondary osteolysis and osteocondensation, and the bone marrow undergo fibrous and/or adipose involution.

Arteriovenous Malformations↗
PubMed1988

Quantitative study of lateral horn cells in 15 cases of multiple system atrophy.

Nerve cell counts of the lateral horns of the spinal cord were performed in 15 cases of multiple system atrophy (MSA) and three age- and sex-matched controls. The patients with MSA were comprised of eight with severe postural hypotension, three with orthostatic hypotension and little or no disability and four without any signs of autonomic failure. All cases of MSA had lost more than half their lateral horn cells but nerve cell loss could not be correlated with the degree of dysautonomia. These results confirm the involvement of the intermediolateral columns of the spinal cord in MSA. However, the lack of correlation between cell loss and severity of autonomic failure in our cases, suggests that lesions of other sites, such as sympathetic ganglia or the dorsal vagal nuclei, are also implicated in dysautonomia.

Aged↗
PubMed1988

Pathological findings in three non-Japanese patients with the POEMS syndrome.

The pathological features of three European patients with plasma cell dyscrasia, osteosclerosis and a multisystem disorder, most frequent in Japan, that includes polyneuropathy, organomegaly, endocrinopathy, M-protein and skin changes (POEMS syndrome), are reported. The material was obtained from biopsies (peroneal nerve, lymph node) and general autopsy, including hypophysis, in one case. The most salient findings were: peripheral nerve lesions, including both segmental demyelination and axonal degeneration, with so-called uncompacted myelin lamellae (UML); angiofollicular lymph node hyperplasia (AFLNH); and non inflammatory vascular changes. Though not specific, it appears that UML and AFLNH may be contributive findings in atypical cases of POEMS syndrome (incomplete forms, lack of underlying malignant plasma cell dyscrasia or circulating monoclonal immunoglobulin). Among the various autopsy findings we emphasize the skin thickening which was secondary to a hyaline sclerosis of the papillary dermis, and the presence in adenohypophysis of numerous cells showing positive reactions with the anti-alpha MSH antibody. Though immunological, vascular and hormonal disturbances have been implicated at the origin of several manifestations of the disorder, the pathogenesis of the POEMS syndrome remains obscure.

Adult↗
PubMed1988

Tumor-simulating giant serpentine aneurysm of the posterior cerebral artery.

A case of a giant aneurysm of the proximal segment of the posterior cerebral artery is reported. Complete neuroradiologic (computed tomography scan, angiography, magnetic resonance imaging) and pathological studies were performed. This type of aneurysm is extremely rare and may be difficult to differentiate from a cerebral tumor, both clinically and on computed tomography scan. Vertebral angiography is usually necessary to make, or confirm, the diagnosis.

Aged↗
PubMed1988

The neuropathology of the acquired immune deficiency syndrome (AIDS). A review.

The nervous system has been involved in the majority (at least 75%), of cases of acquired immune deficiency syndrome (AIDS) examined postmortem, but the pathogenetic mechanisms involved are not well understood. The predominant pathological process is opportunistic infection secondary to the decrease of T-helper (T4) cells and includes toxoplasmosis, encephalitis due to cytomegalovirus and progressive multifocal leucoencephalopathy. On the other hand, mycoses (mainly cryptococcosis) are relatively uncommon. Primary lymphomas are three times more common than secondary lymphoma spreading from other sites. Cerebral involvement by Kaposi sarcoma is metastatic, probably from primary foci in the lungs. Lesions due to the direct involvement of the nervous system by the human immune deficiency virus (HIV) include subacute encephalitis and vacuolar myelopathy. The former is reported with increasing frequency and is localized predominantly to the white matter in which multinucleated giant cells can be found. These are considered typical of AIDS and have been shown to contain HIV particles in their cytoplasm. AIDS lesions due to infectious agents do not always conform to the typical pattern of the uncomplicated disease and not uncommonly there is evidence of more than one infectious agent in the same area. Peripheral nervous system lesions in HIV infections, responsible for a variety of clinical symptoms, usually appear, in biopsy material, as nonspecific inflammatory in type. CMV inclusions and lymphomatous infiltrations of peripheral nerve have been reported in autopsy cases.

Acquired Immunodeficiency Syndrome↗
PubMed1988

Adult polyglucosan body disease (APBD).

Three patients aged 63, 63 and 74 years had various combinations of progressive lower and upper motor neuron dysfunction, sensory loss, urinary incontinence and dementia. Postmortem examinations in two cases showed moderate cerebral and spinal atrophy, ill-defined areas of incomplete myelin loss in white matter and small necrotic foci in the white matter of gyri, around the basal ganglia and near the dentate nuclei. The main microscopic abnormality was a massive accumulation of PAS-positive polyglucosan bodies (PB) of various sizes and shapes in the cerebral hemispheres, brainstem, cerebellum, spinal cord, nerve roots and nerves. These PB were found in the processes of nerve cells and astrocytes, but not in their perikarya. Similar PB were present in peripheral nerves and in the lungs, heart, liver and kidneys. In the third case, a nerve biopsy revealed several, unusually large, PB in the axons of myelinated fibers. These clinicopathologic features are consistent with adult polyglucosan body disease (APBD) and are distinctive from other conditions in which PB may accumulate. Twelve similar cases have been reported previously. The diagnosis can be made by nerve biopsy. The pathogenesis of APBD is not known, but it may be a polysaccharide storage disease.

Aged↗
PubMed1988

[The neuropathology of Parkinson syndrome].

Parkinsonism is due to an alteration of the dopaminergic nigro-striatal pathway the main morphological basis of which is a lesion of the substantia nigra (SN). Parkinson's disease, the etiology of which is unknown, is responsible for 90 p. cent of the cases of parkinsonism. It is characterized by a neuronal loss involving mainly the pigmented brainstem nuclei (substantia nigra, locus coeruleus and dorsal vagal nuclei) and a specific neuronal inclusion: the Lewy inclusion body. Involvement of other structures such as the nucleus basalis of Meynert, hypothalamus, reticular formations of the brainstem, spinal lateral horns, sympathetic ganglia, may be responsible for dementia and/or autonomic failure which are often associated with the extrapyramidal signs. Various other pathological processes, infectious, toxic, vascular, tumoral or traumatic, involving usually the SN, are occasionally responsible for parkinsonism. A number of degenerative diseases involving the SN may develop parkinsonism as major presenting sign. Most of them are diseases of the basal ganglia such as multiple system atrophy, primitive pallidal atrophies, Steele-Richardson-Olszewski disease, Hallervorden-Spatz disease. However, changes in the SN at the origin of parkinsonism are occasionally found in cortical degenerative diseases such as senile dementia of Alzheimer's type, Pick disease or the complex of Guam, in cerebellar atrophies or in motor neuron disease.

Aged↗
PubMed1988

[Pigmentary orthochromatic leukodystrophy. Van Bogaert and Nyssen disease].

A 43 year old woman with a strong likelihood of familial history, developed progressively a spastic tetraparesis associated with intellectual deterioration and terminal epileptic fits. She died 11 years after onset of the clinical disorders. Neuropathological study revealed an orthochromatic leukodystrophy. Macrophages and glial cells of the white matter contained a brown-yellow, autofluorescent pigment which stained positively with PAS, Perls stain for iron and Masson-Fontana. Electron microscopy showed electron dense, membrane bound intracytoplasmic lamellar inclusions with curved or straight parallel arrangement or fingerprint pattern, in white matter macrophages, astrocytes and oligodendrocytes. Cortical cells contained lipofuscin which was normal in type and amount. This suggests that the material in white matter glial cells and macrophages is ceroid pigment; however, the distribution is not that seen in ceroid-lipofuscinosis. Similar inclusions have been found in oligodentrocytes in other forms of orthochromatic leucodystrophy. Ten similar cases of pigmentary type of orthochromatic leucodystrophy have been reported previously; only one had had an ultrastructural study.

Adult↗
PubMed1988

[Dolichoectatic intracranial arteries. Association with aneurysms of the abdominal aorta].

Dolichoectatic intracranial arteries (DIA) have been given several names, e.g. fusiform aneurysms, dolichomega-arteries. It is not an uncommon condition and it raises interesting points: 1) symptoms and signs result from compression (cranial nerve palsies; hydrocephalus) from ischemic or hemorrhagic episodes; 2) the diagnosis is made easier with CT since the arterial wall is often calcified; 3) the pathophysiology has long been assumed to be atherosclerotic in nature, but controversial assumptions are discussed; 4) associated arterial abnormalities are common: one of our cases and approximately half of the post mortem reported DIA were associated with aneurysm of the abdominal aorta. Two cases are reported here: 1) A 56 y/o male presented with progressive gait disorder, deafness and ischemic episodes. CT showed DIA and hydrocephalus. DIA involved the basilar artery, both intracranial carotid arteries, the abdominal aorta and probably one renal artery. A ventriculo-atrial shunt was performed unsuccessfully. Post mortem findings are reported; 2) A 52 y/o male had suffered from trigeminal neuralgia for about one year and from trigeminal permanent pain for two weeks. He had developed left hemiparesis and dysarthria. DIA involved the basilar artery and the left intracranial internal carotid artery. The right internal carotid artery and the right vertebral artery were hypoplastic. Clinical, radiographical and pathophysiological features are reviewed.

Aorta, Abdominal↗