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J Figols

Publications and source records attributed to J Figols.

At least 19 recordsLinked to original sources
PubMed2002

[Cauda equina meningioma in a girl: presentation of a case and review of the literature].

Meningiomas are unusual in childhood, and the intraspinal location is very uncommon. Those arising from lumbar dura matter are the most rare among spinal neoplasms of meningeal origin. We present the case of a 12-years-old girl with a cauda equina meningioma. As in previously reported cases, the initial complain was back pain with radicular irradiation. The girl had few neurological findings, with pyramidal signs of both lower limbs as single neurological impairment. She underwent surgical treatment through an open door laminoplasty and the tumour was completely removed without neurological deficit. After 2-year of follow-up she remains asymptomatic.

Back Pain↗
PubMed2001

Primary brain lymphoma presenting as Parkinson's disease.

Neoplasm is an uncommon cause of a parkinsonian syndrome. We report a woman with primary brain B-cell lymphoma presenting as Parkinson's disease. After 1 year of the illness, CT and MRI showed lesions without mass effect in the basal ganglia and corpus callosum. The patient did not respond to levodopa and right cerebellar and brain-stem signs appeared, which prompted further neuroimaging, showing an increase in size of the lesions and a right cerebellar and pontine mass. Stereotactic biopsy of the basal ganglia showed high-grade B-cell lymphoma. Despite the basal ganglia frequently being involved in lymphoma of the brain, presentation with typical or atypical parkinsonism is exceptional.

Brain↗
PubMed2001

Cardiac epithelioid angiosarcoma presenting as cutaneous metastases.

BACKGROUND: Cardiac angiosarcoma is a rare tumor that has a predilection for middle-aged males and a marked predominance in the right atrium. The tumor may present abruptly with a fulminant clinical course. Initial presentation with metastatic disease is rare. Only one case has been reported of a patient who presented with cutaneous metastases. METHODS AND RESULTS: We here report the case of a 51-year-old man who initially presented with cutaneous metastases in the absence of cardiac symptoms. The skin biopsy was diagnosed as metastatic undifferentiated sarcoma. The patient died 26 days later with widely disseminated disease. At autopsy a tumor arising in the wall of the left atrium and in the interatrial septum was found. After an immunohistochemical study including CD31 and CD34 stains the diagnosis of cardiac pure epithelioid angiosarcoma was made. To the best of our knowledge this tumor variant has not been documented in the heart until now. CONCLUSION: Although cardiac angiosarcoma is a rare neoplasm, its presence should be suspected in patients with cutaneous metastatic angiosarcoma without an evident source of the tumor, even in absence of cardiac symptoms.

Antigens, CD34↗
PubMed2000

Perineurium contributes to axonal damage in acute inflammatory demyelinating polyneuropathy.

OBJECTIVE: To assess if axonal damage in severe acute inflammatory demyelinating polyneuropathy (AIDP) correlates with the appearance of epiperineurium in nerve trunks. BACKGROUND: Increase of endoneurial fluid pressure in nerve trunks possessing epiperineurium may be an important mechanism of axonal damage in AIDP. METHODS: A 79-year-old man had a 2-day history of acroparesthesias and ascending paralysis culminating in quadriplegia, bilateral facial palsy, and mechanical ventilation. Five intravenous immunoglobulin cycles were given without response. He died on day 60. Electrophysiologic studies (days 4, 17, and 50) initially showed normal nerve conduction velocities with further slowing, progressive attenuation of compound muscle action potentials, and profuse denervation. The authors studied the preforaminal anterior and posterior L3 and L5 spinal roots, third and fifth lumbar nerves and their branches, and femoral and sural nerves. RESULTS: Density of myelinated fibers was preserved in L5 ventral and dorsal roots and reduced in sural nerve. Mild de-remyelination was observed in lumbar roots. In both lumbar nerves and their branches, there were extensive de-remyelination and centrofascicular or wedge-shaped areas with marked loss of large myelinated fibers. Axonal degeneration was the predominant lesion in sural nerve. CONCLUSION: The presence of epiperineurium correlates with a drastic change of pathology with superimposed ischemic lesions and distally accentuated axonal loss, suggesting that endoneurial fluid pressure increase could cause axonal damage in AIDP.

Action Potentials↗
PubMed2000

Loss of dopamine uptake sites and dopamine D2 receptors in striatonigral degeneration.

To explore the mechanisms underlying L-dopa response, we studied, by postmortem autoradiography, selective makers of dopamine presynaptic terminals, [3H]WIN 35428, and dopamine D2 receptors, [3H]nemonapride, in the putamen of four Parkinson's disease (PD) and one striatonigral degeneration (SND) neuropathologically confirmed brains as compared with six matched control brains. Dopamine uptake transporter was dramatically decreased (> 90%) both in PD and SND striatum. Dopamine D2 receptors were preserved in PD, but clearly reduced (> 76%) in the SND putamen. These data confirm that L-dopa response is closely associated with the preservation of striatal dopamine D2 receptors.

Aged↗
PubMed2000

Biologic parameters that correlate with the prognosis of human gliomas.

Much clinical and biologic data have been processed in the search for useful objective parameters to predict brain tumor behavior. Seventy cases of astrocytic glioma collected by a single clinical team were studied using a full complement of clinical procedures: follow up (7 years), histologic analysis, DNA content estimation, and cell kinetics by flow cytometry. Proliferating cell nuclear antigen (PCNA) was determined by immunocytochemical-coupling flow cytometry (PFC) and also by counting under light microscopy (PIHC). A statistical evaluation was carried out to establish the usefulness of several parameters for glioma prognosis. The cases were histologically classified as 14 low-grade astrocytomas, 20 anaplastic astrocytomas, and 36 glioblastomas multiforme. The survival curve showed significant differences between histologic groups. Diploid populations were more frequent in low-grade astrocytomas, and aneuploid tumors often had increased S-phase and proliferative fractions. The PCNA-labeled index (PCNA-LI) increased with malignancy and correlated with histologic grading (P = 0.01). The PCNA-LI and age segregated low- from high-grade astrocytomas (including anaplastic astrocytoma and glioblastoma multiforme), but none of the variables considered differentiated anaplastic astrocytoma from glioblastoma multiforme. The Cox regression test displayed significant values for age, histologic diagnosis, and PCNA determinations when considered in tandem. Discriminant analysis obtained a function integrating age and specifically PIHC-LI to help in the prognosis of doubtful cases. The results emphasize the importance of parameters integrating different variables in an attempt to provide an accurate prognosis, the most significant being age, histopathologic diagnosis, and the proliferative fraction determined by PCNA.

Adult↗
PubMed2000

Fatal familial insomnia: clinical, neuropathological, and genetic description of a Spanish family.

The clinical presentation and evolution, neuropathological findings, and genotyping of three members of a Spanish family affected with fatal familial insomnia are reported. The mother and two of her offspring developed a rapidly evolving disease with insomnia and behavioural disorders as the initial symptoms and died between 5 and 10 months after the onset of the illness. Frontal brain biopsy in the mother disclosed only non-significant spongiosis, and full neuropathological examination of her offspring showed thalamic and olivary degeneration with isolated focal cortical spongiosis. Genetic examination could only be performed in the contemporary patients and both harboured the prion protein (PrP) 178Asn mutation and homozygous 129 Met/Met genotype.

Biopsy↗
PubMed1998

5-HT1B receptor binding in degenerative movement disorders.

Using [3H]sumatriptan as a radioligand, 5-hydroxytryptamine (5-HT)1B receptors were examined in posterior striatum and midbrain post-mortem tissue sections of 12 patients who had died from representative degenerative movement disorders as compared to nine controls. In the control human basal ganglia, the highest densities of [3H]sumatriptan binding were observed in the globus pallidus and substantia nigra. No significant change in the density of [3H]sumatriptan binding sites was found in the striatum and substantia nigra of the six Parkinson's disease brains. In the two brains from patients with progressive supranuclear palsy an increase was found in the densities of [3H]sumatriptan binding sites, most marked in the substantia nigra. In contrast, [3H]sumatriptan labelling was almost absent in the striatonigral degeneration brain and was markedly reduced in the three Huntington's disease brains. This study indicates that the status of 5-HT1B receptors is different in each degenerative movement disorder and suggests that human 5-HT1B receptors are located somatodendritically on GABAergic and peptidergic caudate-putamen neurons which project to the substantia nigra and globus pallidus, where these receptors are presynaptic.

Aged↗
PubMed1997

[McArdle's disease in adults: clinical and genetic study].

McArdle's disease is a rare metabolic myopathy resulting from an absence of functional muscle glycogen phosphorylase that is inherited as an autosomal recessive condition. Recent molecular genetic studies have identified more than ten different mutations in patients with McArdle's disease, although a nonsense point mutation at codon 49 in exon 1 (R49X) accounts for approximately 85% of mutant alleles in American and British patients. We describe clinical, biochemical and genetic characteristics of five adults patients with McArdle's disease studied at our hospital during the last 10 years.

Adolescent↗
PubMed1997

Fulminant Guillain-Barré syndrome with universal inexcitability of peripheral nerves: a clinicopathological study.

The pathological basis of nerve inexcitability in Guillain-Barré syndrome has not been established with certainty. We report the clinicopathological findings in a 67-year-old patient with fulminant Guillain-Barré syndrome who died 18 days after onset. Three serial electrophysiological studies revealed nerve inexcitability. Antibodies to Campylobacter jejuni were present but there was no antiganglioside reactivity. Spinal root sections revealed extensive and almost pure macrophage-associated demyelination with occasional presence of T lymphocytes and neutrophil leukocytes. Conversely, in femoral, median, and sural nerves the outstanding lesion was axonal degeneration, with some denuded axons remaining. Unmyelinated fibers, posterior root ganglia, and dorsal columns were preserved. Endoneurial postcapillary venules showed plump endothelial cells with loss of their tight junctions. We conclude that both primary demyelination and axonal degeneration secondary to inflammation account for nerve inexcitability. Our findings lend support to the hypothesis of increased endoneurial pressure as the cause of wallerian degeneration in nerve trunks.

Action Potentials↗
PubMed1997

Lectin histochemistry of ethylnitrosourea-induced oligodendrogliomas in the rat.

Oligodendrogliomas (n = 26) induced by ethylnitrosourea (ENU) in wistar rats were examined to assess the lectin specificity to oligodendroglial membranes. Two different types of oligodendrogliomas were found in our material: an isomorphous type (n = 12), and a polymorphous type (n = 14). The first one, with two variants according to its size, macro- (n = 9) and microtumors (n = 3), had predominantly a honey-comb pattern with 'clear halos' around the nuclei without anaplasia. The second type, composed mostly by macrotumours, was anaplastic, with high cellular density, necrosis and intratumoral hemorrhages. Peanut agglutinin (PNA) labelled plasma membranes of well-differentiated cellular components of the first group. The tumoral oligodendrocytes lost the property to bind PNA in the second group of tumours, while Concanavalin A (Con A) showed affinity to intracytoplasmic structures of these tumours. PNA is a reliable marker of oligodendroglial plasma membrane of well-differentiated ENU-induced oligodendrogliomas. This experimental model, using PNA and Con A, may have important clinical applications regarding the biological behaviour of this type of neoplasm.

Animals↗
PubMed1997

Prognostic analysis of astrocytic gliomas correlating histological parameters with the proliferating cell nuclear antigen labelling index (PCNA-LI).

Eighty out of 250 cases of astrocytic glioma collected from a practice served by a single clinical team over a 15-year period were studied using a full complement of clinical, follow up, histopathological analysis and proliferating cell nuclear antigen (PCNA) immunostaining for the obtention of the PCNA-labelling index (LI). A statistical evaluation and discriminant analysis were carried out with the aim of clarifying the importance of various parameters as predictors of tumor behaviour. Data are correlated with survival (with a 10-year follow up). A significant correlation with survival was found when histological grouping and the PCNA-LI were studied with the Cox test. Most significant features were histological as detected using classical techniques including histological grading. The utilization of objective values (mitosis, cellular density and necrosis) appears to be useful in grading astrocytic tumors. Our results emphasize the importance of cytological, histological and PCNA-LI parameters as predictors of tumor behaviour.

Adolescent↗
PubMed1997

Value of endothelin in cellular rejection after liver transplantation.

Endothelin-1 is a vasoconstrictor peptide released by the vascular endothelium by various chemical and mechanical factors. Cellular rejection is one of the most common complications following orthotopic liver transplantation, endotheliitis being its most specific and consistent histological feature. To assess the role of endothelin in cellular hepatic rejection, we studied 21 cirrhotic patients undergoing elective liver transplantation. These patients were divided in two groups: Group I comprised 10 patients without cellular rejection, and Group II comprised 11 patients with cellular rejection. Endothelin was measured on day 7 after transplantation, on the day of liver biopsy, and after treatment for rejection. We found a significant increase in the plasma endothelin levels in the early postoperative period in the patients with moderate or severe cellular rejection compared with the non-rejection group. It remained significantly elevated until the clinical diagnosis of rejection was made. This value returned to baseline with successful treatment. So, the monitorization of this peptide may be of help in the diagnosis of rejection, its severity, and the evaluation of its resolution.

Adult↗
PubMed1997

[Osterosclerotic myeloma and polyneuropathy].

INTRODUCTION: The peripheral neuropathy is a common complication in patients with osteosclerotic myeloma. The association with hepatosplenomegaly, lymphadenopathy and variable endocrine disturbances and skin changes constitute the POEMS syndrome. The pathogenesis of this syndrome is unclear, but the presence of M protein and elevated levels of interleukin-6 in the serum of patients, suggests an immunologic mechanism. CASE: A 68 year old woman presented with a 2 year history of progressive weakness and paresthesias in both legs. Neurological examination revealed a sensorimotor polyneuropathy, involving predominantly the lower extremities. In addition to neurological findings, the patient had lymphadenopathy and skin hyperpigmentation. Serum protein immunoelectrophoresis revealed a peak of monoclonal IgA lambda paraprotein. Radiographic examination disclosed multiple osteosclerotic lesions. Electrophysiological evaluation showed a severe axonal and demyelinating sensorimotor polyneuropathy. Biopsy of sural nerve revealed demyelination and axonal degeneration. Axillary lymph node biopsy were consistent with giant lymph node hyperplasia. Interleukin-6 serum levels were normal. CONCLUSIONS: The POEMS syndrome or Crow-Fusake syndrome is a rare multisystem disorder often associated with osteosclerotic myeloma. A severe demyelinating sensorimotor polyneuropathy frequently constitutes the chief complaint. These patients have a polyneu-ropathy which resembles idiopathic chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) and the electrophysi-ological studies are essential in the diagnosis. The pathogenesis has not been explained. Presumably, the plasma cells secrete an immunoglobulin or another substance that is toxic to peripheral nerves. Interleukin-6 is implicated in the pathogenesis of this disease.

Aged↗
PubMed1997

Intracranial hemangiopericytomas. Histological and immunohistochemical study.

The authors report on 8 cases of intracranial supratentorial tumors which clinically corresponded to meningeal neoplasms. Only one patient revealed the recurrence. The age of patients was 39-74, prevailed women (5). Histological features of tumors, except some variations in two cases agreed with common morphologic pattern of hemangiopericytomas. Immunohistochemical study with following antibodies was performed: vimentin, epithelial membrane antigen, cytokeratin, S-100 protein, fibronectin, desmin, collagen IV, factor RAg, and glial fibrillary acidic protein. Two atypical cases which presented scarce pseudocalcifications and tendency to concentric cellular structures did not differ from other hemangiopericytomas by their intrinsic immunohistochemical properties such as negative reaction to epithelial membrane antigen and cytokeratin. Atypical positive immunostaining with S-100 protein and with glial fibrillary acidic protein was found solely in the group or perivascular cells in one of those atypical cases reflecting probably local astrocytes embedded in tumor tissue. Immunohistochemical investigations did not present univocal data to explain controversial origin of hemangiopericytomas either from meningeal cup cells or from vascular pericytes.

Adult↗