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J Figols

Publications and source records attributed to J Figols.

At least 55 records · Page 3Linked to original sources

Lectins as differentiation markers of human gliomas.

The lectins Concanavalin A (Con A), Ricinus communis agglutinin (RCA-I), Peanut agglutinin (PNA) and Wheat germ agglutinin (WGA) as well as the immunomarkers for glial fibrillary acidic protein (GFAP) and myelin basic protein (MBP) were used in a series of 21 glial tumors (4 pylocytic astrocytomas, 5 grade II astrocytomas, 3 anaplastic astrocytomas, 4 glioblastomas and 5 oligodendrogliomas). ConA binds to all tumoral astrocytes in low grade astrocytomas, as well as to well differentiated tumoral astrocytes in anaplastic astrocytomas and glioblastomas. RCA-I has a similar behaviour. PNA, and to a lesser degree WGA, binds selectively to the oligodendroglial plasma membrane in well differentiated oligodendrogliomas. The results suggest that these lectins are markers of differentiation in gliomas rather than of malignancy.

Astrocytoma↗

Creutzfeldt-Jakob disease with severe involvement of cerebral white matter and cerebellum.

We describe a patient with Creutzfeldt-Jakob disease (CJD) of the ataxic and panencephalopathic type. Postmortem examination revealed the characteristic lesions of CJD in the grey matter and profound white matter involvement was seen with immunocytochemical techniques. Ultrastructural white matter lesions were identical to those described in experimentally transmitted CJD. There was marked loss of cerebellar granule cells with virtual disappearance of parallel fibres, but Purkinje cells were only slightly reduced. Electron microscopic studies revealed extensive degenerative changes including cytoplasmic vacuoles in both cell types. Silver methods disclosed massive impregnation of white matter and striking abnormalities of Purkinje cells consisting of hypertrophy and flattening of thick dendritic branches, reduction in the number of terminal branchlets, segmentary loss of spines and polymorphic spines. These findings show the extensive involvement of all three cerebellar cortical layers and the reactive plasticity of Purkinje cells to deafferentiation. They favour the hypothesis that demyelination represents a primary lesion of the white matter.

Brain↗

Choroid plexus papillomas: an immunohistological study of 16 cases.

Eleven benign and five malignant choroid plexus papillomas in children and adults were studied immunohistologically with a panel of antibodies against glial fibrillary acidic protein, S-100 protein, vimentin, desmin, epithelial membrane antigen and two different cytokeratins (LP34 and CAM 5.2). Glial fibrillary acidic protein was focally present in epithelial tumour cells, in cells within solid areas and in clusters of cells within the stroma. S-100 protein was diffusely present in tumour cells with focal accentuation. Vimentin was present in all cases, the epithelial tumour cells demonstrating strong and diffuse positivity with perinuclear accentuation; malignant tumours, however, showed stronger positivity than benign ones. Desmin was negative in all tumours. Epithelial membrane antigen and cytokeratin (LP34) were demonstrated in four of five malignant tumours but were absent in the benign ones; CAM 5.2 reacted with four of five malignant tumours and also reacted with eight of the 11 benign ones. The significance of these findings is discussed in respect of the ontogeny of these tumours.

Adolescent↗

Histologic characterization of 41 ependymomas with the help of a personal computer.

Fourty-one ependymomas were histologically analyzed in relation to patient age and sex and tumor location. A discriminant analysis model using Bayes' formula and a personal computer were employed. Ependymomas situated in the posterior fossa had a higher incidence in children. Ependymoblastomas were all situated above the tentorium and occurred only in young children. We identified three tumor groups on the basis of common histologic characteristics: benign ependymomas, anaplastic ependymomas, and ependymoblastomas. The main features useful for the grouping were the degree of differentiation and anaplasia. Ependymomas from the cauda equina showed histologic characteristics that allowed them to be differentiated from other benign ependymomas. In each group the particular histologic characteristics, age, and location were significant in terms of diagnosis and prognosis. This method of analysis may help to more precisely define ependymomas and may provide pathologists and clinicians with a quantifiable diagnostic tool that may be of help in establishing the appropriate treatment.

Adolescent↗

[Idiopathic form of late hemorrhagic disease caused by a deficiency of vitamin K. A presentation of 3 cases].

Three one month old infants with idiopathic late haemorrhagic disease due to vitamin K deficiency are reported. Patients had been exclusively breast fed and had not received vitamin K at birth. Initial symptomatology was typical in the three cases. Two of them had an intracranial haemorrhage with exitus in one of them. Coagulation study showed non measurable levels of PT and PTT and a significant reduction in vitamin K dependent factors. Coagulopathy was corrected with parenteral administration of vitamin K and fresh plasma. Pathogenic factors of this entity are reviewed and present day recommendations to prevent occurrence of this problem are discussed.

Female↗

Hereditary motor and sensory neuropathy type II. Clinicopathological study of a family.

A family with hereditary motor and sensory neuropathy (HMSN) type II is described in which 10 affected and 17 unaffected members in three generations were examined. The peak age of onset was in the second decade. In the youngest generation, the proportion of affected to unaffected individuals at risk significantly differed from the expected 50%. There was slight slowing of conduction velocities in 36% of nerves; however, only 3 out of 10 affected members had entirely normal conduction studies. The amplitude of the sensory potentials of median and peroneal nerves was almost uniformly reduced. In all affected patients electromyography of anterior tibial muscles showed signs of neurogenic involvement. Histological study of two sural nerves and a sciatic nerve and its branches revealed loss of myelinated fibres with a proximal-to-distal gradient in this fibre loss, clusters of small regenerating fibres, and atrophic axons. Postmortem study of the proband showed loss of anterior horn and dorsal root ganglion neurons in the lumbar and sacral segments and degeneration of the fasciculus gracilis. Morphometric evaluation of L5 ventral and dorsal roots revealed a normal number of myelinated fibres, diameter histograms being shifted to the left because of a significant loss of large myelinated fibres and regeneration. These anatomical findings are consistent with the hypothesis that HMSN type II represents a primary neuronopathy affecting motor and sensory neurons.

Adult↗

Encephalopathy with astrocytic residual bodies. Report of a case and review of the literature.

Biopsy and autopsy findings in a girl who died at 7 1/2 months after having suffered from progressive axial hypotonia, myoclonus, EEG changes and retarded psychomotor development. Inclusions consisting of lamellar profiles, situated in membrane-bound cytosomes were found mainly in astrocytes, but also in neurones and in axons of peripheral nerves. Lipofuscin bodies were also increased in number. The patient belongs in the same category as cases studied by Towfighi et al. (1975) and Martin et al. (1977). Etiology and pathogenesis of this syndrome remain unknown. It is suggested, however, that the pathological changes observed might have been caused by the administration soon after birth of anti-epileptic drugs (diphenylhydantoin, clonazepam and nitrazepam).

Anticonvulsants↗

Dominantly inherited motor and sensory neuropathy type I. Genetic, clinical, electrophysiological and pathological features in four families.

This report describes the genetic, clinical, electrophysiological and sural nerve biopsy features in 26 affected members and 21 unaffected relatives from 4 families with autosomal dominant inherited motor and sensory neuropathy (HMSN), Type I. In all age categories, the proportion of affected to unaffected individuals at risk did not significantly differ from the expected 50%. The peak age of onset was in the first decade. There was a complete concordance between nerve conduction velocity in the propositi and that in their affected relatives within each family. Marked slowing of conduction velocities was present as early as the age of 2.5 years, while precocious clinical signs and symptoms were quite subtle. Determination of conduction velocity is a valuable aid aid to the early diagnosis of the disease.

Biopsy↗

[Guillain-Barré syndrome associated to a type B acute hepatitis (author's transl)].

A 42-year-old male patient suffers an acute hepatitis with positive HBs Ag and approximately 2 months after its onset, an acute polyneuritis with lessening of conduction velocity and albumino-cytologic dissociation appeared. Both conditions recuperated synchronously in a few months. This association has been only slightly referred to previously, and the majority of cases lacked facts important to the establishment of a diagnosis. The polyneuritis is possibly secondary to the viral alteration, either directly or due to an ensuing immunological alteration. Besides, there is the possibility that a clinical or sub-clinical demyelinizing neuropathy that does not fill the criteria of a Guillain-Barré syndrome may complicate a hepatitis, or that an acute polyneuritis may associate itself to an autoimmune hepatitis.

Acute Disease↗

Immuno-lectin histochemistry and ultrastructure in two cases of globoid cell leukodystrophy (Krabbe's disease).

Two cases of globoid cell leukodystrophy (Krabbe's disease) have been studied by means of conventional histological, immunohistochemical and ultrastructural methods. Lectins specific for several different carbohydrates, Ricinus communis (RCA-1), peanut agglutinin (PNA), wheat germ agglutinin (WGA) and Concanavalin A (Con A) have been utilized both at optical and ultrastructural level. In both cases a strong positive labeling of the globoid cells could be observed light-microscopically. Ultrastructurally immunogold deposits only of RCA-I and WGA in the typical Krabbe's inclusions have been clearly demonstrated.

Acetylgalactosamine↗

[Buttock mass and malignant sciatic nerve tumor].

Malignant peripheral nerve sheath tumors (MPNST) are rare. We report a case of a MPNST of the proximal sciatic nerve in the thigh, unassociated with pre-existing type 1 neurofibromatosis or history of radiation therapy. A 71-year-old man had a 6 month history of constant, severe, burning pain affecting the sole of the left foot. One month after the onset, the pain radiated to the left calf, posterior aspect of the thigh and buttock, and distal leg weakness followed. Three months prior to admission, the patient developed a large and painful mass in the buttock, that occupied the entire left gluteal region on examination. There was severe weakness of ankle and toe dorsiflexion and plantarflexion, decreased sensation on the lateral and posterior aspect of the left leg as well as on the dorsal and plantar surfaces of the foot, and absent ankle jerk. EMG showed denervation and motor unit loss in the short head of biceps femoris and muscles supplied by tibial and peroneal nerves on the left side. Magnetic resonance imaging revealed a 10-cm enhancing mass of the left sciatic nerve from the upper thigh to the greater sciatic notch. In surgery, a large MPNST with a high Ki67 labeling index (> 60 %) was subtotally removed from the sciatic nerve, and adjuvant radiation therapy was administered. In the ensuing months the tumor invaded the entire pelvic region. A high sciatic malignant tumor can present with a rapidly growing buttock mass and unilateral, neuropathic foot pain imitating the tarsal tunnel syndrome.

Aged↗

Myelin basic protein (MBP) in human gliomas: a study of twenty-five cases.

Twenty five gliomas (5 oligodendrogliomas, 8 oligoastrocytomas, 5 astrocytomas grades I-II, and 7 malignant astrocytomas-glioblastomas) were studied with the immunohistochemical technique avidin-biotin complex (ABC) for myelin basic protein (MBP). We found four patterns of positivity with variable degrees and frequencies in the different types of tumors. We believe this method can help in the diagnosis of non-characteristic gliomas, particularly oligoastrocytomas.

Astrocytoma↗