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Biomedical subjects

R Gherardi

Publications and source records attributed to R Gherardi.

At least 109 records · Page 6Linked to original sources
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

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

[Dysglobulinemic neuropathies].

Monoclonal gammopathies are frequently associated with peripheral neuropathies of which clinical, electrophysiological, pathological and possibly pathogenetical aspects are heterogeneous. Nevertheless some clinico-biological entities, which account for the majority of cases, have been recently recognized: 1) The IgM neuropathy is a chronic demyelinating sensori-motor polyneuropathy with tremor and ataxia as prominent features. It can be either associated with MGUS or Waldenstrom macroglobulinemia. The light chain of the gammopathy is kappa in a majority of cases. Numerous reports have demonstrated specific antibody activities supported by the M-protein and directed against various peripheral nerve antigens, usually myelin components such as the myelin associated glycoprotein (MAG). The ultrastructural evidence of widely spaced myelin is suggestive of the diagnosis but is not consistent. Treatment directed towards the gammopathy is occasionally associated with improvement of the symptoms. 2) The neuropathy of the osteosclerotic myelomas and solitary plasmacytomas present as a chronic sensori-motor polyradiculoneuropathy with conspicuous demyelination and may be associated with one or more of the systemic clinical features of the Crow-Fukase or POEMS syndrome (Polyneuropathy, Organomegaly, Endocrinopathy, M-protein, Skin changes). The POEMS syndrome may also be associated with "benign" monoclonal or even polyclonal dysproteinemias. The M-proteins are almost all IgG or IgA with lambda light chains. There are some relations between POEMS syndrome and Castleman's disease. The pathogenesis of both disorders remains obscure. Treatment is most favorable in case of solitary plasmacytomas, which may be completely removed. 3) The neuropathy observed in patients with primary AL amyloidosis or amylosis associated with malignant plasma-cell dyscrasias is rare. Sensory deficit and autonomic dysfunction are related to a prominent involvement of small myelinated and unmyelinated fibers. A clinical and/or electro-physiological carpal tunnel syndrome is frequent. In a majority of cases the light chain of the M-protein is lambda. Amyloid deposits are observed on nerve biopsy. Treatment is inefficient. 4) The neuropathy associated with cryoglobulinemias may be asymmetric, painful, cryosensitive and associated with cutaneous purpura and neuromuscular vasculitis. In fact, in a majority of cases the symptoms are less suggestive raising the problem of an incidental laboratory finding. 5) A motoneuron disease-like syndrome may develop in patients with various types of monoclonal gammopathies.(ABSTRACT TRUNCATED AT 400 WORDS)

Amyloidosis↗
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

[Neuropathologic study of 15 cases of multinucleated giant cell encephalitis in acquired immunodeficiency syndrome (AIDS)].

The central nervous system (CNS) of 40 patients who died of acquired immune deficiency syndrome (AIDS) between August 1982 and August 1987 was examined. In 15 cases, multinucleated giant cells (MGC) characteristic of Human Immunodeficiency Virus (HIV) infection were observed. In 3 cases ultrastructural examination disclosed HIV-like viral particles in the cytoplasm of some MGC. All cases with MGC showed, in addition, predominant white matter lesion: diffuse myelin pallor, multiple small, usually subcortical, necrotic foci, vacuolar myelopathy, proliferation of rod-shade microglia and microglial nodules, reactive astrocytosis, mineralization of the vessel walls. These changes, typical of HIV encephalitis, were isolated in 3 cases. In the other cases, they were associated with other AIDS-related CNS lesions, i.e., cerebral toxoplasmosis (9 cases), cytomegalovirus infection (5 cases), progressive multifocal leukoencephalitis (1 case), cryptococcosis (1 case) and cerebral lymphoma (1 case). The involvement of MGC with these lesions was remarkable.

Acquired Immunodeficiency Syndrome↗
PubMed1987

Extensive form of progressive multifocal leucoencephalopathy associated with laryngeal carcinoma.

A case is reported of the rare association between progressive multifocal leucoencephalopathy and carcinoma. A 66-year-old man underwent a laryngectomy for carcinoma. Four years later he developed a local recurrence. Simultaneously there were behavioural disturbances and a left motor neglect followed by dense hemiplegia and coma. The patient died a further 5 months later. Anergy was demonstrated by skin tests. CT scan showed asymmetrical non-enhancing low-density areas in the hemispheric white matter, brain-stem and cerebellum and neuropathological examination confirmed extensive myelin loss with typical papovavirus inclusions in oligodendrocytes identified by electron microscopy.

Aged↗
PubMed1987

Almitrine neuropathy. A nerve biopsy study of 8 cases.

We report the quantitative and qualitative, light and electron microscopic studies, including teased fiber preparations of nerve biopsies obtained from eight patients, treated with almitrine, presenting with the characteristic association of a sensory peripheral neuropathy with a recent body weight loss. The data were consistent with an axonal damage affecting myelinated fibers, predominantly large ones and to a lesser degree unmyelinated fibers, some degree of segmental demyelination. Marked axonal regeneration was observed when the nerve biopsy was delayed after withdrawal of the drug, Micro-angiopathy secondary to the formation of concentric lamellae from the basement membrane was observed in five patients suffering from chronic hypoxemia. Almitrine is an agonist of the chemoreceptors. The pathogenesis of the toxic neuropathy induced by this compound remains obscure. Clinical features do not fit with a purely hypoxic mechanism at the origin of the neuropathy.

Almitrine↗
PubMed1987

Leucoencephalopathy with multinucleated giant cells containing human immune deficiency virus-like particles and multiple opportunistic cerebral infections in one patient with AIDS.

A 29-year-old homosexual male with AIDS presented with progressive encephalopathy and cytomegalovirus (CMV) pneumonia. Neuropathological examination revealed toxoplasma abscesses in corpus callosum, basal ganglia and cerebellar white matter; demyelinating foci in the parietal white matter, with microscopic changes typical of progressive multifocal leucoencephalopathy and intranuclear papovavirus inclusions in oligodendrocytes; and lesions of subacute encephalitis in the periventricular regions with large cells positive by immunostaining for CMV. Diffuse myelin loss was observed in the cerebral white matter. Multinucleated giant cells were numerous in the demyelinated areas, they were also observed in close relationship with papova, CMV and Toxoplasma lesions. Immunostaining of these cells was positive for histiocyte markers and negative with the leucocyte common antigen monoclonal antibody. Some of them contained virus-like particles measuring around 100 nm similar to human immune deficiency virus (HIV) as observed in human brain.

Acquired Immunodeficiency Syndrome↗
PubMed1987

Vertebral hemangiomas presenting with neurologic symptoms.

Six cases of spinal hemangiomas with neurologic symptoms are reported. In three of them the diagnosis was obtained before surgery using plain x-rays and spinal computed tomography scan or biopsy. In the other cases the diagnosis was not done before surgery. In two cases preoperative embolization had minimized blood loss during surgery. Laminectomy was performed in three cases. Large removal and/or vertebrectomy was carried out in the other cases. Our results and a review of the literature about the investigations and treatment of vertebral hemangiomas led us to propose a stereotypical management of spinal hemangiomas with neurologic symptoms.

Adolescent↗
PubMed1987

Symptomatic xanthogranuloma of the choroid plexus of the third ventricle. A new case with ultrastructural study.

The clinicopathological findings in a new case of a surgically removed, symptomatic xanthogranuloma of the third ventricle are reported. Ultrastructural study showed that the lining epithelium of the tumor was composed of typical leptomeningeal cells, and that transitional forms from leptomeningeal cells into xanthomatous cells were present in the underlying fibrous capsule. These findings support the hypothesis of a leptomeningeal origin of the foamy cells in the present case. The ten cases reported previously of symptomatic xanthogranuloma of the third ventricle are reviewed. We suggest that xanthogranulomas of the choroid plexus might represent a heterogenous entity arising from xanthomatous changes involving various types of cells.

Brain Diseases↗
PubMed1987

Aspergillosis of ethmoid sinus and optic nerve, with arteritis and rupture of the internal carotid artery.

A 54-year-old woman had been treated 26 years previously for Hodgkin's disease. Eight months before presentation, the disease had recurred and the patient had received cytotoxic drugs and steroids. She presented with loss of vision of the right eye and died 5 months later from extensive subarachnoid hemorrhage. Postmortem examination revealed an aspergillus abscess of the ethmoid sinus, extending to the right frontal lobe and optic nerve, and to the wall of the right internal carotid artery (ICA). Death was due to rupture of the ICA. Such a presentation of aspergillosis is unusual. Three cases of aspergillus arteritis causing rupture of the ICA have been reported previously.

Arteritis↗
PubMed1987

Pigmentary type of orthochromatic leukodystrophy (OLD): a new case with ultrastructural and biochemical study.

A 34-year-old woman with no family history of orthochromatic leukodystrophy (OLD) developed progressive intellectual deterioration, a frontal syndrome and spastic tetraparesis. She died four years after the onset of the clinical illness. Neuropathological studies included light and electron microscopy of cerebral and nerve biopsies, and a complete postmortem examination. Light microscopy demonstrated OLD with pigmented macrophages and glial cells. 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 oligodendrocytes in other forms of OLD. Biochemical study did not show evidence of demyelination. Galactolipids were normal. Polyunsaturated fatty acids were decreased. The most striking feature was an increase in plasmalogens.

Adult↗
PubMed1987

[Neuropathy caused by cisplatin. 7 cases including one with an autopsy study].

The clinical, electrophysiological and histopathological features in seven cases of cisplatinum peripheral neuropathy are reported and compared with the literature data. The neuropathy appears for an average intake of 500 mg/m2 of DDP. The symptoms are those of a symmetric, distal, predominantly sensitive neuropathy of an axonal type with major involvement of proprioception. Neurological improvement is poor after withdrawal of the drug. A post mortem study performed in one case showed a degeneration of the posterior column in the cord and residual nodules of Nageotte in a lumbar spinal ganglion. The systematic study of the tendon reflexes and distal pallesthesia in subjects treated with the drug, may reveal the neuropathy before the onset of the most disabling symptoms (paresthesia, ataxia, pain, Lhermitte's sign).

Adult↗
PubMed1986

T-cell lymphoma revealed by a peripheral neuropathy. A report of two cases with an immunohistologic study on lymph node and nerve biopsies.

In two patients a peripheral neuropathy was the presenting symptom of a noncutaneous peripheral T-cell lymphoma. In the first patient, the neuropathy had a relapsing and remitting course, the symptoms improved under corticosteroid therapy. The second patient suffered from a relentless neuropathy. In both cases the lymphoma infiltrated the peroneal nerve with an angiocentric and perivascular pattern resembling that observed in central nervous system lymphomas. The characterization of T-cell subsets in the lymph node showed cells with the helper/inducer and suppressor/cytotoxic phenotype in the first case and a predominance of cells with the helper/inducer phenotype in the second case. In the nerve, lymphocytes beard the helper/inducer phenotype antigen. A typical paraneoplastic vasculitis of nerve showed clearly different immunologic features.

Adult↗
PubMed1986

Diffuse subependymal periventricular metastases. Report of three cases.

Three clinicopathologic cases with a remarkable pattern of extensive diffuse subependymal periventricular spread of cerebral metastases from solid systemic cancer are reported. Two patients had a small cell carcinoma of the lung. In the third case, the histologic features of the brain metastases were consistent with a neuron-specific enolase-positive, small cell anaplastic carcinoma. Involvement of the choroid plexus and leptomeninges was moderate or absent. Intraparenchymatous nodular metastases were not found except in one case in which rare nodular superficial cortical metastases were present. The clinical data were nonspecific except for orthostatic hypotension, in one patient, which was probably due to the infiltration of the floor of the third and fourth ventricles. Results of the cerebrospinal fluid examination, available in two cases, were normal. The only diagnostic investigation was contrast-enhanced computed tomography scanning.

Carcinoma↗