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Biomedical subjects

J Berciano

Publications and source records attributed to J Berciano.

At least 91 records · Page 5Linked to original sources

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↗

[Bimanual mirror letter writing: a post-traumatic case].

Mirror writing is inverse writing that can only be read with the help of a mirror. It is generally associated with dominant hemisphere lesions and affects only one hand. We describe the case of a patient who, after cranial injury, developed bimanual mirror writing for letters but no other focal neurological symptom. The only abnormal complementary finding was a zone of left parieto-occipital hypoperfusion visibly by SPECT. No signs of psychological disorder were present to which the dysgraphia could be attributed. Symptoms disappeared after three months. We conclude that isolated bimanual mirror letter writing may be an expression of reversible parieto-occipital dysfunction of the dominant hemisphere.

Adolescent↗

[Headaches due to cough, exertion and sexual intercourse].

The aim of this study was to review the advances in cough, exertional and sexual headaches. We perform a critical analysis of the literature and of our experience with 72 patients. About one-half of patients having cough headache will have benign cough headache. The remaining half suffer from Chiari type I malformation. Benign cough headache begins typically in men above age 50, while symptomatic cough headache begins under 50 and appears with the same frequency in both sexes. Indomethacin is the treatment of choice of benign cough headache, but has no effect in symptomatic cases, which respond to suboccipital craniectomy. Although also showing male predominance, exertional and sexual headaches begin under age 50. Subarachnoid bleeding due to aneurism is the most frequent etiology for exertional and sexual headaches. Cough headache and exertional headache are separate clinical entities, whereas exertional headache and sexual headache seem to be different expressions of the same clinical entity. These data allow, in addition, the clinical differentiation between benign and symptomatic cough headache.

Adult↗

The insulin-like growth factor I system in cerebellar degeneration.

Brain insulin-like growth factor I (IGF-I) and its related molecules may be involved in neurodegenerative processes in which IGF-I-containing pathways are compromised. Since IGF-I is present in the olivocerebellar circuitry, two types of late-onset cerebellar ataxias (olivopontocerebellar and idiopathic cerebellar cortical atrophy) were chosen to test this hypothesis. The following significant changes in the peripheral IGF-I system of these patients were found: low IGF-I levels, and high IGF-binding protein 1 (BP-1), and BP-3 affinity for IGF-1. Sixty percent of the patients also had significantly low insulin levels. Patients suffering from other neurological diseases with cerebellar dysfunction and ataxia not involving the olivocerebellar pathway also had low IGF-I levels, while IGFBPs and insulin levels were normal. Our data indicate that degeneration of an IGF-I-containing neuronal pathway produces significant changes in the peripheral IGF system. This suggests strongly that the endocrine (bloodborne) and the paracrine/autocrine (brain) IGF systems are linked functionally. We propose that alterations in the blood IGF-I system may constitute a marker of some cerebellar diseases.

Adult↗

Intramedullary tuberculoma of the spinal cord with syringomyelia.

We describe a patient with a presumed intramedullary tuberculoma treated only with antituberculous therapy. MRI showed a ring-enhancing annular lesion in the thoracic spinal cord with a distant syringomyelic cavity, which improved after therapy. MRI in this case allowed us not only to achieve a presumptive diagnosis but also to assess the therapeutic response, making surgical exploration unnecessary.

Adult↗

MRI in radiation-induced myelopathy and pharyngocutaneous fistula.

A patient developed a cervical myelopathy 20 months after radiotherapy for a carcinoma of the larynx. MRI showed an intramedullary lesion at C7. Although radiation myelopathy was suspected, tumour recurrence could not be excluded. A radiation-induced pharyngocutaneous fistula, confirmed histologically, appeared a month later. The fistula lay just anterior to the level of the spinal cord lesion, a finding useful in supporting a diagnosis of simultaneous radiation myelopathy.

Aged↗