PubMed Health⌕ Search

Biomedical subjects

M M Ruchoux

Publications and source records attributed to M M Ruchoux.

81 records · Page 5Linked to original sources

[Erythromelalgia, arterial hypertension and increased excretion of urinary catecholamines].

A 7 year-old girl was hospitalized with acute, severe and drug resistant erythromelalgia. During her stay in the hospital, she presented with an epileptic seizure associated with hypertension (220-120 mmHg). Catecholamine urinary excretion was markedly increased. Diagnoses of pheochromocytoma and acrodynia were excluded. Erythromelalgia and hypertension both disappeared in a few days without any relapse after a 2 year-follow-up. Catecholamine urinary excretion returned to normal levels in a few weeks. A skin biopsy which was performed in an affected site, showed a slight and questionable reduction of the density of autonomic adrenergic nerve terminals in the periarterial and glandular plexuses. The relevance of transitory excess catecholamine excretion and its link with erythromelalgia and hypertension are discussed. The hypothesis that the disorder could be explained by an abnormality of distal autonomic axons is likely.

Catecholamines↗

[Histopathology of the vein of Galen].

Histology of cerebral arteries and veins are reviewed to show variances with extracerebral blood vessels. Muscles fibers even in large cerebral veins are easily overlooked. The thick wall of the vein of Galen grossly and microscopically resembled dura mater fed by microscopic blood vessels and capillaries. The authors reported 4 cases of arteriovenous aneurysms of the great vein of Galen. When compared to the rest of the histological reports on this subject published till now, it is obvious that this new cases bring new facts. First is the presence of an irregular muscle coat in the aneurysmal wall suggesting early hemodynamic troubles. The second and most important data is the association of multiple malformations of the pineal gland, of the vascular elements of the tela choroidea of the third ventricle between which the aneurysm of the great cerebral vein is found. These unusual data suggested complex congenital malformation in the early stages of development.

Cerebral Veins↗

Orthostatic hypotension from a cerebellar gangliocytoma (Lhermitte-Duclos disease). Case report.

A 49-year-old woman presented with orthostatic hypotension. Vertebral angiography and ventriculography revealed a tumor of the left cerebellar hemisphere. Ventriculoperitoneal shunting was followed by complete disappearance of the orthostatic hypotension. The tumor was subsequently removed and microscopic study showed Lhermitte-Duclos disease. Orthostatic hypotension has been rarely reported in association with tumors of the posterior fossa except for those tumors destroying the medullary centers and interrupting the baroreceptor reflex arc. This case is of interest because the tumor was restricted to the cerebellum. The authors have found no previous case in which orthostatic hypotension was a presenting symptom of Lhermitte-Duclos disease.

Cerebellar Neoplasms↗

Intracerebral medulloepithelioma with a long survival.

This report concerns a 3-month-old boy where neuroimaging examination showed a large, well-circumscribed, mildly heterogeneous tumor arising in the left ventricle. Pathological findings were compatible with a medulloepithelioma. A survey of published cases of medulloepitheliomas showed this tumor to be highly malignant, possibly displaying the entire range of differentiation from embryonal primitive neuroepithelium to mature cells and usually involving the cerebral hemispheres with a very poor prognosis in this location. On the other hand, medulloepitheliomas occurring in the eye or the orbit generally benefit from a gross-total resection and may present a good prognosis. Curiously, the patient reported here is doing well 7 years after the resection without any postoperative treatment. The exclusive intraventricular location of the tumor and its gross-total resection clearly seems to have contributed to this unusual recovery.

Brain Neoplasms↗

Large-cell medulloblastoma with arrestin-like protein expression.

We report a case of a cerebellar large-cell medulloblastoma in a 12-year-old patient. Despite a gross-total resection followed by a radiation therapy and then a chemotherapy, the death occurred 6 months later. The cyogenetic analysis showed an isochromosome 17q. Immunoreactivity for synaptophysin, neurofilaments, chromogranin and arrestin-like proteins was detected, whereas rhodopsin, vimentin, EMA and PAX-6 were negative. In this study, we demonstrate that large-cell medulloblastoma with translocation in chromosome 17q is a neuronal differentiated medulloblastoma with non-photoreceptor characterization. By reverse transcription and polymerase chain reaction (RT-PCR) method, using primers for beta1, beta2 and visual arrestin, we demonstrate corresponding mRNA for beta1, beta2 arrestin but not for visual arrestin. These results suggest that arrestin immunoreactivity in this tumor corresponds to non-visual arrestin. This case corresponds to a new entity of large-cell medulloblastoma. The potential role of a new marker linked to a beta2 adrenergic receptor needs further molecular characterization to be useful.

Arrestin↗

Solitary brain metastasis as only recurrence of a carcinoma of the bladder.

A case of solitary brain metastasis from a transitional carcinoma of the bladder occurring 20 months after resection is presented. The patient underwent gross total removal followed by external irradiation and intravenous chemotherapy. The tumor in this quite unusual occurrence had a very characteristic aspect, both operatively and at pathologic examination. We believe that these patients should be managed aggressively whenever possible.

Adult↗