Biomedical subjects

J-P Guichard

Publications and source records attributed to J-P Guichard.

4 recordsLinked to original sources

PubMed2006

Dilation of Virchow-Robin spaces in CADASIL.

To precise the severity of dilated Virchow-Robin spaces (VRS) in CADASIL patients and to determine their correlation with clinical presentation and other abnormalities on cerebral Magnetic Resonance Imaging (MRI). Dilated VRS were previously associated with aging, hypertension, dementia, epilepsy or migraine. We already reported increased frequency of enlarged VRS in CADASIL patients when compared with family members without the affected haplotype. We analysed clinical and MRI data from 50 CADASIL patients collected prospectively in our center. The presence of dilated VRS was assessed in the subcortical white matter of temporal lobes, the centrum semi-ovale and the basal ganglia. Their severity in each region was evaluated according to the scale proposed by Heier. We compared the clinical data, the severity of white matter abnormalities and the presence of microbleeds in patients with and without dilated VRS. Seventy-eight percent of patients in our series had dilated VRS, mostly located in the lentiform nuclei (94%) and subcortical white matter of the temporal lobes (66%). The severity of these lesions was variable but not correlated neither to the extent of white matter abnormalities nor to the clinical presentation in our patients. Only the age was found to be related to the extent of dilated VRS. Dilated VRS are frequent in CADASIL and mostly located in the temporal white matter and basal ganglia. The dilation of perivascular spaces does not seem to be directly related to the occurrence of ischemic or hemorrhagic lesions in CADASIL. In contrast, the relation with age suggests that either aging, progression of vascular wall alterations during the course of the disease, or both of these processes can favour the extension of VRS in CADASIL.

Adult↗

PubMed2004

[Cadasil].

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CADASIL↗

PubMed2003

Hereditary infantile hemiparesis, retinal arteriolar tortuosity, and leukoencephalopathy.

BACKGROUND: The main hereditary vascular conditions involving both retinal and cerebral vessels include cerebroretinal vasculopathy, HERNS (hereditary endotheliopathy with retinopathy, nephropathy, and stroke), and hereditary vascular retinopathy; all are linked to the same locus on chromosome 3p21. Hereditary retinal arteriolar tortuosity is a distinct, autosomal dominant condition characterized by retinal arteriolar tortuosity and recurrent retinal hemorrhages. This condition is known to affect only retinal vessels. METHODS: Clinical and brain MRI investigations of eight members of a three-generation family and extensive biological and systemic vascular investigations within one affected family member were conducted. RESULTS: Six of eight family members were clinically symptomatic; disorders included infantile hemiparesis (2), migraine with aura (3), and retinal hemorrhage (1). Five individuals had retinal arteriolar tortuosities. A diffuse leukoencephalopathy in association with dilated perivascular spaces was observed in six individuals. Two family members had silent, deep cerebral infarcts as demonstrated on MRI. Genetic linkage analysis strongly suggests that this disorder is not linked to the 3p21 hereditary vascular retinopathy/cerebroretinal vasculopathy/HERNS locus. CONCLUSIONS: The authors describe a novel hereditary autosomal dominant condition affecting both retinal and cerebral vessels and characterized by infantile hemiparesis, migraine with aura, retinal hemorrhage, retinal arterial tortuosity, and leukoencephalopathy with dilatation of perivascular spaces and microbleeds on brain MRI. Investigation of additional families should help to map the gene and to better categorize the spectrum of hereditary cerebroretinal small vessel diseases.

Adolescent↗

PubMed2002

[Scintigraphy by l'Octreoscan in the management of head and neck paragangliomas].

OBJECTIVE: To assess the usefulness of somatostatin receptor scintigraphy [Octreoscan] in a series of 18 patients referred for a suspicion of paraganglioma of the head and neck between July 2001 and February 2002. PATIENTS AND METHODS: Sixteen patients had one or several paragangliomas of the head and neck diagnosed on conclusive conventional imaging including CT and MR scan. In two patients, radiological data were not conclusive. Planar images were obtained 4 and 24 hr after the iv injection of 148-185 MBq [Octreoscan]. RESULTS: Twenty-two hot spot lesions were detected. Twenty of these lesions corresponded to the twenty known paragangliomas. The volume of the smallest tumor was 0.2 cm(3). In one patient, intense thyroid nodule uptake led to the surgical diagnosis of oncocytoma. In two lesions, where conventional imaging was not conclusive, arteriography showed a typical aspect of meningioma; one patient was operated on and histology confirmed this diagnosis. No evidence of abnormal uptake was seen in site previously operated on (3 patients). CONCLUSION: Octreotide scintigraphy is a very sensitive method for detection of paraganglioma of head and neck. It provides information on potential tumor sites in the whole body after one single injection. It could be used as a screening test in patients at risk (familial or known paraganglioma) in order to detect paraganglioma at an early stage and, thus to reduce the surgical morbidity, as well as in the follow-up after surgery to detect recurrences.

Adult↗