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C Massot

Publications and source records attributed to C Massot.

At least 19 recordsLinked to original sources

[Tuberous sclerosis without mental impairment, diagnosed in adulthood].

INTRODUCTION: Tuberous sclerosis complex (TSC) is an autosomal dominant inherited phakomatosis, usually diagnosed in childhood and characterized by cutaneous and neurological tumors, the latter often leading to epilepsy and mental retardation. EXEGESIS: We report a case of TSC diagnosed in a 33-year-old man, without any known family history of phakomatosis, presenting with facial angiofibromas, hypomelanotic macules, a giant-cell astrocytoma and retinal phakomas without any mental impairment or epilepsy. CONCLUSION: TSC may occur in patients who do not have any family history of phakomatosis because de novo mutations are frequent. TSC may be diagnosed in adulthood since a high phenotypic variability is observed. Facial angiofibromas are highly suggestive of tuberous sclerosis complex. They should lead to brain imaging in search for astrocytoma, subependymal nodules and cortical tubers which number is directly correlated with the risk of seizures and the degree of mental impairment.

Adult↗

[Non-histaminic angiodema management: diagnostic and therapeutic interest of tranexamic acid].

INTRODUCTION: The diagnosis of chronic angioedema (localised and reversible oedema) is sometimes very difficult when there is no efficiency of anti-histaminics and corticosteroids. In these cases, bradykinin angioedema must be mentioned. Tranexamic acid is efficient and must be proposed. STUDY: We reported seven patients who have a non-histaminergic angioedema since an average of 8 years. Corticosteroids and histamine-1 blockers were not efficient. Daily tranexamic acid treatment (1 g x 3 per day) controlled the disease. Three patients had no more attack. The C1Inh antigen and function levels were normal. No secondary effect was described and all patients are still being treated (average of 20 months). CONCLUSION: When a non-histaminergic angioedema is suspected, tranexamic acid must be proposed as attack's treatment and as prophylactic treatment.

Adolescent↗

[Chronic meningitis: aetiologies, diagnosis and treatment].

PURPOSE: Chronic meningitis are very uncommon and account for less than 10% of all meningitis cases. Their symptoms are uncunth and there outcome is insidious. Therefore, they remain often unknown. There are only a few published reports on this disease, so diagnosis and therapeutic approachs are difficult. CURRENT KNOWLEDGE AND KEY POINTS: Positive chronic meningitis diagnosis is easy. However, determining the cause of chronic meningitis remains dilemma, as many infectious and noninfectious processes (including inflammatory, neoplastic or autoimmune aetiologies or as a result of a chemical exposure) can result in the chronic meningitis syndrome. In order to institute a pertinent treatment, sometimes urgently needed, diagnostic approach must be extremely rigourous and accutely orientated. Nevertheless, although extensive investigations, 30% of the aetiologies remain undetermined. Only two choices are left for the medical physician: an aggressive attitude based on complementary investigations or a contemplated therapy with a close clinical and biological control. On the other hand, when the patient's condition is quickly deterioring without a clear and proved aetiology, it is sometimes necessary to institute an empirical treatment, not always properly determined and sometimes contreversial. Besides, few reports on prognosis and outcome od idiopathic chronic meningitis have been published. FUTURE PROSPECTS AND PROJECTS: After a review of aetiologies and diagnostic investigations chronic meningitis, we propose a practical experience attitude about management and treatment of chronic meningitis. Thus, large-scale studies about the follow up chronic meningitis in long term, in particular those without aetiology, treated or no, should improve the outcome of this chronic syndrome.

Cerebrospinal Fluid↗

[Systemic lupus erythematosus with disease onset after age 65].

OBJECTIVES: Systemic lupus erythematosus with disease-onset in the elderly has rarely been studied (only one report about 21 patients with disease onset at 65 and older). Is the management of this pathology modified in this population? METHODS: Seventeen hospitalised cases of lupus patients with disease onset at 65 or older are retrospectively reported between 1988 and 2000. The results are compared with those of younger subjects. RESULTS: The female to male ratio is 1.83. Mean age at disease onset is 71.9 +/- 3.5 years. Mean duration of follow-up is 3.5 +/- 2.4 years. Main initial symptoms are: deterioration of general status (41%), arthritis (35%), cutaneous manifestations (35%), thrombo-embolism (24%) and pleuritis (18%). Malar rash is uncommon (12%). Nephropathy is never a revealing symptom and is rarely serious during the disease's evolution. Like in neurologic manifestations, the etiology has to be discussed in relation to associated co-morbidities. Concerning haematologic features, lymphopenia is found in 82% of the cases with a questionable specificity. Antinuclear antibodies are constant, anti-dsDNA antibodies are found in 82% of the cases, antibodies to extractable nuclear antigens in 50%, and anticoagulant circulating activity in 59%. Prognosis is difficult to assess in such a limited series but 5-years survival probability is 83%. Glucocorticoid lead to 50% of major complications. CONCLUSIONS: This study focuses on the particular initial manifestations of systemic lupus erythematosus in the elderly (deterioration of general status, thrombosis, unusual cutaneous symptoms), and on the specificity of differential diagnosis and treatment.

Age Distribution↗

Angioedema and oral contraception.

BACKGROUND: Oral contraceptives can precipitate attacks of hereditary angioedema (ANE) or induce acquired forms. OBJECTIVE: We studied 5 patients who had an ANE which had begun under oral contraception and disappeared after stopping the pill. METHODS: We explored the clinical and biological characteristics of these patients. RESULTS: The symptoms developed during the first year or later after starting contraception; the patients reported relapsing swelling of the lips, hands, larynx and abdomen. All women had normal serum C4 and C1 inhibitor (C1Inh) antigen levels, but a lowered C1Inh activity, with a marked protein cleavage on the immunoblot. The suppression of the pill was associated with the regression of the edema and normalization of C1Inh function. CONCLUSION: The mechanism of these ANE is unknown. The could be due to a modulation of C1Inh expression upon androgens or an imbalance between coagulation proteins favoring C1Inh cleavage by its target proteases.

Adult↗

[Non-allergic angioedema: update].

PURPOSE: Nonallergic isolated angioedema is an uncommon clinical syndrome raising difficult diagnosis and therapeutic problems. Occurrences linked to a C1Inh are the predominant ones and have to be examined as a priority, taking into account the specificity of the associated follow-up. CURRENT KNOWLEDGE AND KEY POINTS: Diseases with a clinical profile close to hereditary angioneurotic edema, but without C1Inh anomaly, have been described recently. It is in fact family cases, concerning only women, where estrogens seem to play a dominant role. Angioedema's secondary aspects are gathering various pathologies (vasculitis, Gleich's syndrome, angioedema initiated by physical agents). The role played by some drugs must not be forgotten, mainly angiotensin converting enzyme inhibitors, which are at the origin of angiodema in nearly 0.5% of users. FUTURE PROSPECT AND PROJECTS: Uncontrolled activation of the contact system seems to play a major role in the main part of these angiodemas. The efficiency of the tranexaminic acid (which modulates its activation) is to be taken as evident. The key to the future seems to be the development of plasmin and bradykinin inhibitors.

Angioedema↗

CT findings in a case of laryngeal sarcoidosis.

Laryngeal sarcoidosis is a rare manifestation of systemic sarcoidosis. It affects mainly the supraglottic larynx. Involvement of the glottic and subglottic levels are exceptional. We present the case of a 56-year-old man with a 2-year history of systemic sarcoidosis, involving the mucosa of paranasal sinuses, a joint, and mediastinal lymph nodes, who developed laryngeal sarcoidosis. We emphasize the CT appearance of laryngeal sarcoidosis.

Humans↗

[Acquired angioneurotic edema. Clinical and biological characteristics in 9 patients].

OBJECTIVE: Angioneurotic edema (AE) is a rare but severe disease. Hereditary AE is the more well-known form. The acquired form is exceptional: the symptoms are the same but there are some biologic and treatment differences. We investigated the clinical and biochemical features in nine patients with acquired angioneurotic edema (AAE). PATIENTS AND METHODS: Four of the patients with type I AAE presented an accelerated metabolism of C1Inh, associated with a hematology disease. Their C4, C1q and C1Inh plasma levels were low. Four patients had type II AAE associated with an autoantibody to C1Inh. Their C1Inh plasma levels were normal or low but the functional levels were low in all four. One patient had AAE induced by oral contraceptives. The C1Inh plasma level was normal but the functional level was very low; there were no autoantibodies. Symptoms resolved when oral contraceptives were withdrawn and the C1Inh level returned to normal. DISCUSSION: Treatment of AAE is a difficult matter. For type I AAE, it consists in treating the associated disease. For type II AAE, the treatment goal is to lower the autoantibody level. Management of these diseases requires close collaboration between clinicians and biologists.

Adult↗

[Renal inflammatory pseudotumor manifesting as a prolonged fever].

INTRODUCTION: Inflammatory pseudo-tumors (IPT) are benign neoplasms associated with local or general manifestations. Renal localization seems exceptional, and no case of fever of unknown origin caused by renal inflammatory pseudo-tumors have been reported. EXEGESIS: The authors report the case of a man presenting fever of unknown origin and inflammatory syndrome. Computerized tomography showed a renal mass. Nephrectomy was performed, and renal inflammatory pseudo-tumor was diagnosed. After surgical exeresis, the clinical and biological abnormalities vanished. CONCLUSION: This rare diagnosis must be considered in these situations. The outcome is excellent.

Biopsy, Needle↗

[Diagnostic strategy in uveitis: a prospective study in 125 cases].

BACKGROUND: The diagnosis of uveitis is a difficult one and subject to much debate between ophthalmologists and internists. In 1992, we carried out a prospective study to assess the importance of the internist's consultation, the contribution of an extensive laboratory evaluation and to propose a well considered diagnostic strategy. MATERIAL: and methods: From 1992 to 1995, all patients seen at consultation at the Grenoble University Hospital had a complete ophthalmologic evaluation, an internist's consultation, an extensive laboratory evaluation and a follow-up for at least 6 months. RESULTS: One hundred twenty five patients were included. The rate of diagnosis was 72%. The internist's help was useful in at least 68% of the diagnoses. Laboratory and radiography tests provided pertinent information in 77% of the cases. Follow-up allowed finding 11% of the diagnoses. The analysis of the clinical and laboratory tests enabled us to establish a diagnostic strategy chart where the key pint is clinical and ophthalmologic evaluation. A minimal laboratory evaluation systematically performed for all cases of uveitis is proposed and, according to some characteristics of uveitis, a well-targeted laboratory evaluation is recommended. CONCLUSION: This diagnostic strategy enabled establishment of the diagnosis in 92% of the cases and limited laboratory evaluation for 68 patients. We are performing a new prospective study to better validate this diagnostic chart.

Adult↗