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Hugues Rousset

Publications and source records attributed to Hugues Rousset.

10 recordsLinked to original sources
PubMed2006

Neurological manifestations and neuroradiological presentation of Erdheim-Chester disease: report of 6 cases and systematic review of the literature.

Erdheim-Chester disease (ECD) is a rare, non-Langerhans form of histiocytosis of unknown etiology that affects multiple organs. We report 6 cases of ECD with neurological involvement and neuroradiological abnormalities on brain MRI. A literature review revealed 60 other cases of ECD with neurological involvement. We therefore analyzed 66 ECD patients with neurological involvement. Cerebellar and pyramidal syndromes were the most frequent clinical manifestations (41% and 45% of cases), but seizures, headaches, neuropsychiatric or cognitive troubles, sensory disturbances, cranial nerve paralysis or asymptomatic lesions were also reported. Neurological manifestations were always associated with other organ involvement, especially of bones (at least 86%) and diabetes insipidus (47%). Neurological involvement was responsible for severe functional handicaps in almost all patients and was responsible for the death of 6 of the 66 patients (9%). Neuroradiological findings could be separated into three patterns: the infiltrative pattern (44%), with widespread lesions, nodules or intracerebral masses, the meningeal pattern (37%), with either thickening of the dura mater or meningioma-like tumors, and the composite pattern (19%), with both infiltrative and meningeal lesions.

Adult↗
PubMed2005

[Weight loss].

Explore the source record for details and available documents.

Humans↗
PubMed2005

Systemic mastocytosis: predictable factors of poor prognosis present at the onset of the disease.

Systemic mastocytosis (SM) refers to a group of heterogeneous diseases that can be divided into indolent SM, for which prognosis is favorable, and malignant SM, which has a poor prognosis. While the diagnosis of SM is often a challenge since clinical and biological abnormalities are not specific, prognosis is even more difficult to predict. Thus, we aimed to highlight predictable factors in a cohort of 28 cases of SM. Among the 13 women and 15 men studied were 7 patients who had an aggressive form of SM that ultimately led to death in 3 of them. We found common characteristics among these seven patients. First, they were older when the first symptoms appeared and when the diagnosis was confirmed. Second, ascitis, lymphadenopathy, anemia, and thrombocytopenia were significantly more frequent, while cutaneous lesions and flush were less frequent. Moreover, general symptoms, gastrointestinal disorders, neutropenia, and coagulation abnormalities also seemed to characterize this group of patients. Understanding the factors that predict SM is essential in order to provide patients with the malignant form of the disease with specific treatments.

Journal Article↗
PubMed2005

Lupus-like presentation of parvovirus B19 infection.

OBJECTIVES: To describe 2 cases of parvovirus B19 (B19) infection mimicking systemic lupus erythematosus (SLE) and to identify all cases of SLE imitated by and/or associated with B19 in the medical literature. METHODS: A computer-assisted (PubMed) search of the medical literature from 1975 to 2003 was performed using the following key words: parvovirus, B19, SLE, lupus, antibodies, auto-immunity. RESULTS: Thirty-eight patients were identified: 35 women, 3 men; mean age = 28.8 years. Clinical manifestations were as follows: fever (24 patients); articular involvement (36 patients); cutaneous lesions (28 patients); lymphadenopathy (9 patients); hepato- and/or splenomegaly (6 patients); serositis (6 patients); renal involvement (4 patients); cerebral impairment (10 patients). Cytopenia was observed in 23 cases. Antinuclear antibodies were detected in 34 patients, anti-double-stranded DNA antibodies in 20 patients, anti-Sm antibodies in 4 patients, antinuclear ribonucleoprotein antibodies in 5 patients, anti-Ro-SSA antibodies in 4 patients, anti-La-SSB antibodies in 4 patients, and anticardiolipin and/or anti-beta2-glycoprotein I antibodies in 8 patients. Hypocomplementemia was found in 15 of 26 patients. In 19 cases, the B19 infection had a self-limiting course. In 6 cases, B19 infection occurred in a context of previously established SLE, simulating SLE exacerbation. In 6 observations, symptoms persisted several months after the viral infection. In 7 cases, the exact relationship between SLE and B19 could not be determined. CONCLUSIONS: B19 infection may present a clinical and serological tableau making it difficult to distinguish between a viral infection and the first episode of SLE. Although B19 may modulate the clinical and biological features of rheumatic disease, studies in large series do not support a causative role for B19 in the pathogenesis of SLE.

Adult↗
PubMed2003

[Acquired angioneurotic edema induced by hormonal contraception].

OBJECTIVE: Although frequently pointed out in the aetiology of chronic angioedema or chronic abdominal pain, food allergy is frequently a diagnosis for lack of anything better, as exemplified by disappointing results of eviction regimens. This has resulted in the search for other aetiologies, such as an iatrogenic effect of oral contraceptives. METHODS: Detailed medical history was obtained on 38 young women, aged a mean of 26 years, experiencing chronic angioedema initially ascribed to food hypersensitivity, but in whom a deficit in C1 inhibitor was demonstrated. We investigated the effects of oral contraception on their level of C1-INH, functional C1-INH assay and their clinical symptoms. RESULTS: An interruption of oral contraception induced an increase of the C1-INH level from 0.201 to 0.224 g/L (p < 0.002) and of the C1-INH functional assay fom 0.396 to 0.702 U (p < 0.0001), associated with a recovery or a marked improvement of the clinical symptoms formerly ascribed to food allergy. The replacement of the initial contraception containing ethinyloestradiol by a progestogen maintained or even improved their clinical and biological state. CONCLUSION: Exogenous estrogen such as those contained in most oral contraceptive may play an iatrogenic role in the aetiology of chronic angioedema.

Adolescent↗
PubMed2003

Exogenous oestrogen as an alternative to food allergy in the aetiology of angioneurotic oedema.

Although frequently reported as an aetiology for chronic angioneurotic oedema or urticaria, food allergy is often a diagnosis proposed in the absence of more convincing evidence, as illustrated by the disappointing results of eviction regimens. We report a series of women with an initial diagnosis of food allergy, but in whom the role of oral contraceptives was subsequently demonstrated. Detailed medical history was obtained from 26 young women presenting with chronic angioneurotic oedema or urticaria initially attributed to food allergy, but in whom C1-esterase inhibitor (C1 INH) deficiency was demonstrated. We investigated the effects of oral contraception on C1 INH levels, C1 INH activity and clinical symptoms of these patients. Discontinuation of oral contraception induced an increase in C1 INH levels and C1 INH activity, associated with recovery or marked improvement of the clinical symptoms formerly attributed to food allergy. The relatively high frequency of women taking cyproterone acetate in this population appeared to be a remarkable finding. Replacement of the initial contraception containing ethinylestradiol by a progestogen maintained or even accentuated these good therapeutic results. Exogenous oestrogens, such as those contained in most oral contraceptives, may play an iatrogenic role in the aetiology of chronic angioneurotic oedema or urticaria.

Adolescent↗
PubMed2003

Prevalence and pattern of antinuclear autoantibodies in 347 patients with non-Hodgkin's lymphoma.

The presence of antinuclear autoantibodies (ANA) was investigated in a large cohort of patients with non-Hodgkin's lymphoma (NHL) in order to assess their frequency, specificity and prognostic relevance. ANA were analysed in 347 patients with different histological subgroups of NHL and in 213 controls using an indirect immunofluorescence technique on HEp2 cells. As the appearance of autoantibodies may be found after treatment of NHL, samples were collected at the time of diagnosis of NHL before any therapy. Sixty-six (19%) NHL patients and 12 (5.6%) patients from the control group displayed ANA. The prevalence between the two groups was found to be significantly higher in NHL patients (P < 0.0001) with a marked increased prevalence in follicular and mantle cell lymphoma subgroups. Autoantibodies directed against mitotic proteins or mitotic-associated proteins were found in 6.9% of NHL patients versus 0.5% in the control group (P < 0.001), with a significantly increased incidence in follicular and mantle cell lymphoma subgroups (P < 0.0001). Some 28% of the patients with positive ANA displayed clinical symptoms that could correspond to classical autoimmune manifestations, this frequency appearing to be higher in the marginal zone/mucosa-associated lymphoid tissue lymphoma subgroup. These data demonstrate a significant incidence of ANA before any treatment in NHL occurrence, which seems to be higher in some histological subgroups with particular ANA, such as ANA directed against mitotic proteins or mitotic-associated proteins.

Adolescent↗
PubMed2002

[Immunological diagnosis of scleroderma].

Systemic sclerosis is characterized by a fibrosis and a microvascular injury. These vascular lesions can affect internal organs causing severe visceral damages. The pathogenesis of this disease is complex but some immunological disorders with a production of antinuclear auto-antibodies can be useful to the clinicians, especially for an early diagnosis and to distinguish the scleroderma/myositis overlap syndromes. This review presents the target antigens and the methods of detection of the different antinuclear auto-antibodies. Sensitivity, specificity and clinical association between the antinuclear auto-antibodies and the different subclasses of scleroderma are also presented in detail.

Autoantibodies↗
PubMed2002

Neurologic presentation of Whipple disease: report of 12 cases and review of the literature.

We report 12 cases of Whipple disease in patients with prominent neurologic symptoms, along with 122 cases of Whipple disease with nervous system involvement reported in the literature. We analyzed the clinical signs and results of additional examinations in 2 groups: the first group included patients with predominantly but not exclusively neurologic signs, and the second included patients with clinically isolated neurologic presentation of the disease. Whipple disease is a multisystemic infectious disease due to Tropheryma whippelii that may present with prominent or isolated symptoms of either the central or the peripheral nervous system. Recent reports stress the importance of polymerase chain reaction (PCR) analysis of cerebrospinal fluid, magnetic resonance imaging (MRI) during follow-up, and prolonged antibiotic therapy with drugs able to cross the blood-brain barrier. Cerebrospinal fluid should be analyzed repeatedly during follow-up, and treatment should be discontinued only when the results of PCR assay performed on cerebrospinal fluid are negative. Other examinations to be done include searching for gastrointestinal tract involvement with multiple duodenal biopsies and searching for systemic involvement with lymph node biopsies, which should be analyzed with light microscopy, electron microscopy, and PCR. When all examinations are negative, if Whipple disease is suspected and a lesion is found on brain MRI, a stereotactic cerebral biopsy should be performed. Treating Whipple disease with long-term trimethoprim-sulfamethoxazole is usually effective, but the use of third-generation cephalosporins in case of incomplete response deserves further attention.

Actinomycetales Infections↗