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We report the case of a 36-year-old-man who first presented two relapses of chronic inflammatory demyelinating polyneuropathy (CIDP) before a diagnosis of sarcoidosis was made. He subsequently presented two combined relapses of CIDP and sarcoidosis. Each time, the outcome was favorable after treatment with intravenous immunglobulins and steroids. The possible relationship between CIDP and sarcoidosis is discussed.

Whipple disease is an uncommon chronic bacterial infection due to Tropheryma whipplei. Clinical manifestations are protean (joint pain, fever, weight loss, abdominal pain, lymphadenopathies), and the diagnosis is often delayed. Although previously considered a late manifestation of Whipple disease, neurological involvement is now frequently the initial clinical manifestation and represents the greatest risk for long-term disability. All patients should be treated and monitored as if they had central nervous system disease even if they are asymptomatic. Neurological manifestations include dementia (56 percent), abnormalities of eye movements (33p. cent), involuntary movements (28 percent), seizures, hypothalamic dysfunction, myelopathy, ataxia and psychiatric manifestations. Uveitis, retinitis, optic neuritis and papilloedema may be found. 80 percent of the reported cases of neuro-Whipple had associated systemic symptoms or signs but many patients are presenting without concurrent intestinal manifestation. Thus, the disease may remain undiagnosed or misdiagnosed, as rheumatoid arthritis or sarcoidosis. Traditionally, the diagnostic procedure of choice is biopsy of the duodenal mucosa by demonstrating PAS-positive foamy macrophages. However, not all cases have small bowel infiltration and tissue obtained from sites clinically affected may be helpful. CT and MR images of the central nervous system are normal or not specific: atrophic changes, mass lesions, focal abnormalities and hydrocephalus. The application of a PCR assay against Tropheryma whipplei has transformed the diagnosis. Positive results have been obtained from several tissues and from CSF and PCR is more sensitive than other techniques. All patients must be treated with antibiotics which cross the blood-brain barrier. Most agree that initial treatment with a combination of parenteral penicillin and streptomycin for at least 14 days is appropriate, thereafter cotrimoxazole orally 3 times a day for at least one and probably for two years. Third generation cephalosporins, rifampicin and chloramphenicol have been used successfully. PCR is recognized to be a useful tool for monitoring progress but it is sometimes difficult to reverse established neurological defects.

A physician's activity is not limited to, but does start at diagnosis. An accurate diagnosis requires collection of pertinent clinical information through history taking and physical examination and selection and organization of the observed symptoms to develop a diagnostic hypothesis to be confirmed or refuted. The prerequisites are adequate knowledge, a good patient-physician relationship and technical qualification. Learning to make a diagnosis is an individual endeavor. Teaching requires a good analysis of the diagnostic process and possible errors to design anatomoclinical conferences or diagnostic exercises. Experience can be transmitted directly; the 19th century clinical lessons are an excellent example. Progress in medical diagnosis is largely linked to advances in fundamental sciences and investigation techniques, but for the most difficult cases, clinical analysis remains the cornerstone of diagnosis.

Fibromyalgia is a chronic pain syndrome, more common in women. Its prevalence is estimated around 2% in the general population, and up to 20% among rheumatology outpatients. Besides musculoskeletal pain, symptoms as fatigue and sleep disturbance are considered characteristic. Research criteria have been set up, but their seemingly preciseness is unable to distinguish clearly between fibromyalgia and other functional somatic syndromes (chronic fatigue syndrome, irritable bowel syndrome) and psychiatric disorders (depression, anxiety), with which a striking comorbidity is documented. The diagnosis of fibromyalgia does not theoretically require the exclusion of muscle, joint, or metabolic diseases, but in clinical practice this problem proves to be of crucial importance. There are numbers of pathophysiological hypothesis for fibromyalgia, but none of them is fully satisfying: muscle is probably innocent; sleep disturbance, although sometimes considered a landmark of the syndrome, is unspecific; stress response studies show subtle anomaly; psychiatric disorders may represent factors of vulnerability and perpetuation rather than causes. We propose to include some of these etiological contributors in vicious circles leading to a "final common pathway" characterized by generalized hyperalgesia. Treatments of fibromyalgia, whether pharmacological (antidepressants) or psychological (cognitive-behavioral therapies) are of little efficacy, and the global prognosis of fibromyalgia is poor. However, the outcome might prove better outside the specialized clinics in which studies of chronic sufferers with severe abnormal illness behaviors are done. The social consequences of the popularization of the diagnosis of fibromyalgia should not be neglected.

Manifestations of dementia occurred in two young patients with disseminated lupus erythematosus who had no signs of focalized neurological deficit. In case 1 followed for 8 years, a probably autonomous and familial psychiatric syndrome was complicated during a lupus flare-up by a prolonged dementia syndrome which regressed to a large extent. In the second patient with moderate dementia and familial hearing loss, severe mental deterioration suddenly occurred with long-term degradation of the cognitive capacity. In both of these cases with neurolupus, the frontal clinical signs, the neuropsychological deficits evidenced at testing, and the lesions demonstrated at CT scan and magnetic resonance imaging favored a frontal and/or frontobasal type dementia. Treatment with cyclophosphamide was effective in case 1 after failure of corticosteroid therapy.

The aim of this study was to determine, in a population of medical inpatients, the sensitivity, specificity, positive and negative predictive values of two self-rating depression scales, and of the physicians' judgment, compared to a structured interview derived from the Composite Interview Diagnostic Interview (CIDI) designed to assess the diagnosis of major depressive episodes. The setting of the study was a general internal medicine inpatient ward of a French university hospital. Patients between 15 and 75 were asked to fill, within the first week of their hospitalization, two self-rating depression scales: the Beck Depression Inventory - short form (BDI-short form), a 13-item scale with established reliability and validity; and the Center for Epidemiologic Studies-Depression Scale (CES-D), a 20-item scale designed for epidemiological use in the general population, recently translated in French. Patients were then interviewed by a psychologist, blind to the results of the self-rating scales, using a slightly simplified version of the depression section of the CIDI. The ward physicians' recognition of depression was assessed six months later by a careful chart review, conducted by an investigator blind to the results of depression scales and structured interview. One hundred consecutive patients were studied: 63% were women, mean age was 53.6 +/- 16.5 years. According to the results of the CIDI, the prevalence of current major depression was 29.7%, and the lifetime prevalence of major depression was 59.8%. The correlation between BDI-short form and CES-D was +0.81. BDI-short form proved to have a slightly better acceptability than the CES-D.(ABSTRACT TRUNCATED AT 250 WORDS)

A case of facial hemiatrophy and homolateral cervical band of scleroderma, complicated by hypothyroidism is reported. This case raises two problems: one is the problem of distinction between Romberg's disease and facial hemiatrophy due to a genuine localized scleroderma; the other concerns the relationship between localized scleroderma and dysthyroidism. The generalized scleroderma-dysthyroidism association has now been recognized, but the coexistence of thyroid disease and localized scleroderma has not yet been reported. Several pathogenetic hypotheses on this association are discussed.

Two cases of mainly interstitial myositis with epitheloid and giant cells are reported. In the first case, the late amyotrophic-type paralysis of the two girdles, of the clinical myositis type, was associated with severe myocardial damage, the atrioventricular conduction disorder requiring a pace-maker. This observation would appear to be a true polymyositis of the collagen diseases, in view of the associated skin affection, death occurring within a year, a large increase in serum muscle enzymes and urine creatine, the existence of a rheumatoid factor and striated muscle antibodies, and a large increase in M and G immunoglobulins. In the 2nd case, the amyotrophic affection of the pelvic girdle was associated with progressive blindness, diabetes insipidus, and anterior pituitary insufficiency. Death occurred after two years and autopsy showed a suprasellar dysgerminoma (ectopic pinealoma) without any visceral localization of sarcoidosis. The authors discuss the concept of granulomatous polymyositis, autonomous with respect to the sarcoidosis, and sometimes symptomatic of an inflammatory connective tissue condition or a malignant tumor.

Authors describe three cases of agranulocytosis in patients with giant cell (temporal) arterities treated with Dapsone and corticosteroids. The culture in vitro of CFU.GM. cells of two patients, four healthy subject and two patients treated with Dapsone with out granulopenia, demonstrates no any toxic effect of Dapsone. A review of the literature finds 59 cases of agranulocytosis in patients treated with Dapsone alone or in combination with other drugs. Doses of Dapsone ranged from 25 mg to 300 mg a day. Agranulocytosis occurred between the 4th and the 12th week, more often near the 8th week. Agranulocytosis occurred suddenly without previous granulocytopenia. These facts argue for immunoallergic mechanism. This Dapsone side effect requires to restrict its use and to reserve it to serious diseases or failures of other therapeutic drugs. It needs too to keep a close eye on the patients between the 6th and 12th week.

In this paper, we have compared the results obtained for hemoglobin A1c and C-peptide concentrations, was not convenient for the diagnosis of anomalies in the regulation of carbohydrate metabolism in obesity or in latent diabetes. Nevertheless, hemoglobin A1c allowed us to check carbohydrate metabolism and to discriminate diabetes treated by oral therapy from insulin dependent diabetes; in these latter cases, hemoglobin A1c concentration varied inversely as C-peptide concentration as it was shown by the method of factor analysis in particular "principal components analysis". In the control of insulin dependent diabetes in a remission, hemoglobin A1c allowed assessment of the regulation of carbohydrate metabolism after suppression of insulin therapy. Thus, hemoglobin A1c is an index of the adaptation of insulin secretion in anomalies of carbohydrate metabolism.

On 91 patients with temporal arteritis (TA) and/or polymyalgia rheumatica (PMR), we observed 7 females aged 62 to 73 years with upper extremities ischemia. Arm claudication and/or Raynaud's phenomenon were the initial manifestations of the disease in 2 cases, or appeared simultaneously with other symptoms in 2 cases, or complicated decreasing corticosteroid therapy in 3 cases. A temporal artery biopsy was performed on 6 patients with, in all of them, typical giant cell granulomatous arteritis pathology findings. Angiograms showed, in all cases, multiple bilateral smooth stenosis and/or obliterations of post vertebral subclavian arteries and/or axillary arteries. Symptoms always improved on corticosteroid treatment and no patient needed reconstructive surgery. In conclusion, large arteries involvement, which can occur in TA and/or PMR, affect in our experience most commonly the subclavian and axillary arteries, with female predominance as found in Takayasu's arteries. These disorders should be considered in cases of occlusive disease of the arms in elderly women and the response to steroids is usually adequate to eliminate the need for early surgical intervention. Early recognition of asymptomatic large artery involvement by Doppler evaluation, in all TA and/or PMR patients, and transient anticoagulant therapy might prevent vessels occlusions.

Twelve patients suffering from mental anorexia were examined on clinical and biological grounds, based on the hypothesis of the functional depression of the noradrenergic track. The initial values of MHPG and of catecholamines were below normal. The quantitative results for depression and retardation were lessened significantly under beta-stimulant treatment. Only glucuro-conjugate MHPG excretion increased significantly, but the MHPG values were much lower than normal at the end of the treatment. The correlations between biochemical and behavioural parameters were worth noticing as far as the retardation scale was concerned. The present study shows the advantage of the dexamethasone suppression test and of the response of TSH under TRH.

About a case of Von Hippel-Lindau syndrome with association of retinal angioma, bilateral pheochromocytomas, bilateral renal cysts, the authors had many difficulties to confirm associated unilateral renal cell carcinoma. Three solid left renal tumors were not seen neither with abdominal ultrasound, nor with CT scan, but only with hypervascularity on renal angiography. Even intra-operative histologic examination fail to confirm the malignity. After the complete histologic examination of the tumorectomy piece, the unilateral renal cell carcinoma was secondary managed by radical nephrectomy, although the risk of bilateral carcinoma is known in this disease.

The authors report a prospective study of the thyroid function of 18 consecutive patients with systemic scleroderma and 7 patients with morphea. A history was taken and patients were examined for thyroid disease. The patients were tested for free thyroxin, TSH, cholesterolemia, circulating TeBG, anti-microsomial and anti-thyroglobulin antibodies and had a TRH test. The incidence of thyroid diseases was correlated with an age and sex-matched control population consisting of 2,534 subjects of the Loire department. None of the patients with morphea had a history of thyroid pathology and the thyroid screenings were within normal limits. A familial history of thyroid disease was found in 7 out of 18 patients with systemic scleroderma, and 8 out of 18 patients had a thyreopathy (one Hashimoto's disease, one Graves' disease, one toxic adenoma, one hypothyroidism, two euthyroid goiters, two nodular thyroids). The TRH test only revealed a toxic adenoma. A thyroid disease preceded systemic scleroderma in 5 out of 18 cases with delays ranging from 1 to 38 years. In 3 cases, the thyroid disease occurred within two years after the onset of systemic scleroderma. The prevalence of thyroid disease was significantly higher than in a comparable population sample of subjects from the same geographic region (p less than 0.01). The authors review the literature and discuss the physiopathologic relationships between the two clinical entities. The authors suggest to perform a thyroid screening (ultrasensitive TSH assay) on all patients with systemic sclerosis and especially on those whose clinical follow-up is atypical and on those with a familial and/or personal history of thyroid disease.

A 44-year old female farmer and pigeon-breeder presented with paroxysmal attacks of dyspnoea caused by recurrent subglottic laryngeal oedema. The symptoms disappeared under corticosteroid therapy but returned after discharge from hospital. The avian origin of the disease, suggested by a strongly positive search for avian precipitins, was confirmed when the attacks ceased after antigen exclusion. A realistic inhalation challenge test performed on two occasions with pigeon's feathers and droppings as antigens resulted, within 15 minutes, in a 4-hour long attack of paroxysmal laryngeal dyspnoea. The dyspnoea was caused by subglottic laryngeal oedema visualized, 15 minutes after the inhalation, by pharyngeal and bronchial fibroscopy. Nasal manifestations have been reported in patients with hypersensitivity of the lung, but it seems that no case of subglottic oedema in pigeon-breeder's disease has yet been published.