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Biomedical subjects

B Schott

Publications and source records attributed to B Schott.

At least 73 records · Page 4Linked to original sources

[Amnesic syndrome of posterior cerebral ischemia].

30 patients with acute onset of memory disturbances and visual impairment (cortical blindness or hemianopsia) are reported. For all of them, there was evidence of posterior cerebral artery ischemia. This clinical syndrome is compared with Dide and Botcazo's case report. The amnesia never recovered in 17 patients and was transient in 13 patients: in 4 of them it occurred during vertebral angiography and in 4 during general anaesthesia with anoxia. The main clinical features of the syndrome and the related bibliography are reviewed.

Adult↗

[Radioimmunofixation: a new technic for characterizing immunoglobulins in unconcentrated cerebrospinal fluid. Preliminary results].

The restriction of heterogenicity of immunoglobulins G (IgG), or oligoclonal distribution, in the cerebrospinal fluid (CSF) can be observed after electrophoresis or electrofocalization, the IgG nature of each oligoclonal band being confirmed by subsequent immuno-electrophoresis. Using immunofixation, the oligoclonal bands can be visualized and characterized simultaneously, but requires previous concentration of the CSF, which is a source of error. The technique of radioimmunofixation, here described, allows the study of IgG with 20 microliters of non-concentrated CSF. It demonstrates that the oligoclonal characteristic is present in normal CSF and is a more quantitative than qualitative feature. Preliminary results show that the method can be applied to the study of all CSF proteins. By using a viral antigen labelled with I-125, for example, it should be possible to reveal and visualize the antibody activity of each oligoclonal band and to determine whether the quantitative increase in an oligoclonal band corresponds to a definite antigenic stimulation.

Humans↗

[Chronic polyradiculitis with cutaneous and endocrine signs suggesting a plasmocytic dyscrasia to IgA (author's transl)].

A case of chronic disabling sensory-motor polyradiculitis occurring in a female patient and followed up for 18 months is reported. Clinical findings were papilledema, hypertrichosis, abnormal pigmentation of the skin, generalized edema, and spontaneous cutaneous necrosis. The diagnosis of a plasmocytic dyscrasia to IgA Lambda was made at a late stage of the disease. There were no signs of a solitary plasmocytoma or of diffuse myeloma. At autopsy, there were mild lesions in the peripheral nervous system, but no plasmocytic infiltration or amylosis was seen on optical or electron microscopy. This case is similar to other clinical cases reported mainly by Japanese authors.

Chronic Disease↗

[Three case histories of cortical and sub-cortical auditory lesions. Audiological and tomodensimetrical confrontations (author's transl)].

The authors describe three case histories of auditory lesions involving the cortex or subcortex of the temporal lobes. In two of the cases there are minor lesions of the hemisphere producing an amusia without verbal deafness or agnosia of non-verbal sounds. In one of the two patients, the total transitory deafness makes the use of the term cortical deafness debatable. In the third case, a bilateral vascular lesion of the temporal lobes had produced a pure verbal deafness without loss of tone discrimination or loss of the capacity to recognise changes in rhythm or sound frequency. In these three patients in whom there was no anatomical verification, the data given by the scanner and the study of evoked cortical potentials recorded over the two cerebral hemispheres allowed a fairly good study of the correlations between the topography of the lesions and their semiotic consequences.

Audiometry↗

[Granulomatous myositis during collagenosis (one case) and suprasellar dysgerminoma (one case) (author's transl)].

Two cases of mainly interstitial myositis with epitheloid and giant cells are reported. In the first case, the late amyotrophic-type paralysis of the two girdles, of the clinical myositis type, was associated with severe myocardial damage, the atrioventricular conduction disorder requiring a pace-maker. This observation would appear to be a true polymyositis of the collagen diseases, in view of the associated skin affection, death occurring within a year, a large increase in serum muscle enzymes and urine creatine, the existence of a rheumatoid factor and striated muscle antibodies, and a large increase in M and G immunoglobulins. In the 2nd case, the amyotrophic affection of the pelvic girdle was associated with progressive blindness, diabetes insipidus, and anterior pituitary insufficiency. Death occurred after two years and autopsy showed a suprasellar dysgerminoma (ectopic pinealoma) without any visceral localization of sarcoidosis. The authors discuss the concept of granulomatous polymyositis, autonomous with respect to the sarcoidosis, and sometimes symptomatic of an inflammatory connective tissue condition or a malignant tumor.

Blindness↗

[Adrenomyeloneuropathy: an adult form of adrenoleukodystrophy spastic paraparesis, and chronic adrenal insufficiency (concerning 3 cases) (author's transl)].

Three cases of adult males with spastic paraparesis and adrenal insufficiency are reported. The adrenal insufficiency is primary and in one case associated with Leydig cell insufficiency. Ultrastructural examination of peripheral nerve revealed abnormal cytoplasmic inclusions in Schwann cells. A decrease in the percentage of linoleate to total fatty acids was found in the sera. A child with adrenoleucodystrophy and an adult with adreno myeloneuropathy were observed in the same family. It allows adrenomyeloneuropathy to be considered as an adult form of adrenoleucodystrophy.

Addison Disease↗

[Subacute sclerosing panencephalitis. A case with a prolonged course in an adult. Isolation and characteristics of a "defective" measles virus (author's transl)].

A 33-year-old man had a 6-year history of clinical signs suggesting multiple sclerosis : visual lesion at 27 years of age, cerebellar and visual disturbances at 31, which partly regressed, lymphocytosis and increased-gammaglobulin levels in the cerebrospinal fluid. Biological and anatomical (optical and ultrastructural) examinations gave results typical of a subacute sclerosing panencephalitis. A cytopathogenic measles virus was isolated from a cerebral biopsy specimen. The agent was transmissable to vero cells by co-culture but infectivity was always related to the cells and was therefore an incomplete viral infection. Virus-like particles were found in the nucleus and cytoplasm after electron microscopy examination of the co-cultures. Biochemical tests demonstrated that the viral proteins were all synthesized except hemagglutinin, which is a characterist abnormality of a "defective" measles virus.

Adult↗

[The neuropathies of monoclonal gammapathies. Immunofluorescence and immunolabelling in the electron microscopy of immunoglobulins with amyloid structure (author's transl)].

Five cases of peripheral neuropathies occurring with monoclonal gammapathies are studied by means of nerve biopsies. Immunofluorescence and HRP-immunolabelling with the electron microscope are performed. The direct pathogenic role of a subperineurial immunoglobulin deposit is pointed out. The relationship of the heavy chain monoclonal immunoglobulin with amyloid fibrils is discussed.

Aged↗

[Neurological manifestations in monoclonal gammapathies. Pure neurological manifestations. Immunofluorescence study].

Analysis of 105 peripheral and central nervous system complications in 1062 monoclonal gammapathies draws attention to two types of phenomena. The possibility of pure neurological manifestations of IgM monoclonal gammapathies with macroglobulinorachia leads to discussion of their nosological position in relation to Waldenström's disease, Burkitt's lymphoma and Marek's disease. It is suggested that these cases should be reclassified under the heading "secreting neurolymphomatosis". Immunofluorescence and electron microscopy of 10 biopsies of the peripheral nerve showed deposits of monoclonal immunoglobulin whose function in determining peripheral neuropathies is discussed. The simultaneous presence of lymphoid infiltration, amyloid deposits and the monoclonal immunoglobulin (M component) suggests that this immunoglobulin could be the link between the cellular infiltrate secreting it and amyloid infiltration which would be the visible manifestation of it.

Amyotrophic Lateral Sclerosis↗

[Striato-nigral degeneration. A propos of 2 anatomo-clinical cases].

Two sporadic cases of striato-nigral degeneration were characterized by the onset at the age of 61 of an akinetic-hypertonic syndrome and of a minimal or absent rest tremor, with a pyramidal syndrome, sphincter disorders, dysphonia, difficulty in swallowing and an unfavorable development in the space of two or three years not affected by L-Dopa. In one case, total insomnia was demonstrated by means of 3 polygraphic recordings during the night, two of them consecutive, and aprobenecide test showed a definite fall in H.V.A. and 5 H.I.A.A. in the lumbar cerebrospinal fluid. Anatomical verification showed, in one case, isolated putamino-nigral degeneration, and in the other associated with lesions of the olivo-cerebellopontine system. Much of the excess pigmentation found only in the putamen was melanotic in character and resulted perhaps from the striato-nigral degeneration, making it possible to classify this more accurately among the other multisystem degenerations, especially olivo-ponto-cerebellar atrophy and Shy-Drager syndrome

Brain Diseases↗