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BACKGROUND AND PURPOSE: Wernicke encephalopathy is a severe neurologic disorder that results from a dietary vitamin B1 deficiency. It is characterized by changes in consciousness, ocular abnormalities, and ataxia. This study was undertaken to analyze and compare findings on MR imaging and neurologic symptoms at clinical presentations of patients with Wernicke encephalopathy with and without a history of alcohol abuse. MATERIALS AND METHODS: A multicenter study group retrospectively reviewed MR brain imaging findings, clinical histories, and presentations of 26 patients (14 female, 12 male) diagnosed between 1999 and 2006 with Wernicke encephalopathy. The age range was 6-81 years (mean age, 46 .6+/-19 years). RESULTS: Fifty percent of the patients had a history of alcohol abuse, and 50% had no history of alcohol abuse. Eighty percent showed changes in consciousness, 77% had ocular symptoms, and 54% had ataxia. Only 38% of the patients showed the classic triad of the disease at clinical presentation. At MR examination, 85% of the patients showed symmetric lesions in the medial thalami and the periventricular region of the third ventricle, 65% in the periaqueductal area, 58% in the mamillary bodies, 38% in the tectal plate, and 8% in the dorsal medulla. Contrast enhancement of the mamillary bodies was statistically positively correlated with the alcohol abuse group. CONCLUSIONS: Our study confirms the usefulness of MR in reaching a prompt diagnosis of Wernicke encephalopathy to avoid irreversible damage to brain tissue. Contrast enhancement in the mamillary bodies is a typical finding of the disease in the alcoholic population.

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The clinical manifestations of localised or early systemic forms of Wegener's granulomatosis (WG) do not require immediate treatment to save the patient's life and/or the function of a vital organ. The organs mainly involved are the ear, nose, throat (ENT) and lung, and the results of antineutrophil cytoplasmic antibody (ANCA) assays are frequently negative. We here describe three cases of the ANCA-negative early systemic form of WG with prevalent ENT involvement complicated by severe central nervous system (CNS) disease; in two cases, the only symptom was a mild headache. We conclude that, although apparently mild, the localised and early systemic forms of WG can hide CNS involvement and may require immediate treatment. This complication should be suspected and investigated in the case of patients with localised or early systemic disease especially in the presence of ENT involvement and negative ANCA assays.

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We report a case of widespread immune activation with moderate cytopenia during acute infection with human parvovirus B19 in an adult female patient, in whom five criteria for the diagnosis of systemic lupus erythematosus were present at disease onset. Our case is unusual due to the presence of a cutaneous rash mimicking leukocytoclastic vasculitis at presentation, moderate leukopenia with thrombocytopenia and the presence of a broad array of autoantibodies. Diagnosis was established on the grounds of serological tests confirming recent infection with human parvovirus B19; spontaneous regression of clinical and laboratory abnormalities was observed within 16 weeks, ruling out classic systemic lupus erythematosus. We conclude by proposing that human parvovirus B19 infection should be included in the differential diagnosis of lupus-like syndromes in adult patients.

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Heart rate variability (HRV) gives information about sympathetic parasympathetic autonomic balance. Our purpose was to determine whether HRV is abnormal in patients with Sjögren's syndrome. In 16 patients with Sjögren's syndrome and 30 matched controls, a short time analysis of HRV was performed for both the frequency and the time domain. In the time domain, patients tended to display a slower heart rate, greater R-R variability and higher standard deviation of the mean (SDNN) than did healthy subjects, but the differences were not statistically significant. In the frequency domain the spectral measures of HRV showed a slight reduction of LF and an increase of HF; as a result, the ratio between high and low frequencies, representative of sympathovagal modulation, was significantly reduced. Our data suggest an increase in the parasympathetic control of heart rate in patients with Sjögren's syndrome. This predominance in vagal tone could exert a protective and antiarrhythmic role in patients with primary Sjögren's syndrome, and may be relevant with reference to the lower incidence of sudden death in this disorder compared to other major autoimmune diseases.

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Riedel's thyroiditis is a rare condition in which the thyroid gland is replaced by fibrous tissue. Fibrosis in various distant sites is a possible concomitant event. We report a case of Riedel's thyroiditis complicated by mediastinal fibrosis, a tumefactive fibro-inflammatory lesion of the neck and primary hypothyroidism. A review of the literature in which only 8 previous cases of hypoparathyroidism secondary to Riedel's thyroiditis have been recounted concludes the report.

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Kikuchi's disease or histiocytic necrotizing lymphadenitis is a recognized cause of benign lymphadenopathy, although the current clinical literature also supports a link with systemic lupus erythematosus (SLE). Eight patients represent all the cases of Kikuchi's disease admitted to our hospital from 1990 to 1997. They were all young women of median age of 31 years (range 22-43 years). All patients showed cervical lymphadenopathy, and in two cases splenomegaly was present. In one patient, the diagnosis of lupus preceded the onset of Kikuchi's disease by 5 months. In 4 other patients, antinuclear antibodies (ANA) were positive at titers ranging between 1:80 and 1:2560 and were associated with leukopenia ranging from 1190 to 2800 white blood cells/mm3 when the diagnosis of Kikuchi's disease was made. Among these, 3 patients developed SLE after periods ranging from 6 to 25 months. The other ANA positive patient and the 2 remaining ANA negative patients did not develop any autoimmune disease after 2-A years of follow-up.The differential diagnosis between Kikuchi's disease and lupus lymphadenitis cannot always be clarified histologically. We recommend that patients with necrotizing lymphadenitis, leukopenia, and ANA be closely followed. This should ensure an early diagnosis of development of an autoimmune disorder that will require treatment, rather than always considering these patients to be affected by a benign self-limited disorder like Kikuchi's disease.

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Platelet-derived growth factor (PDGF) could play a role in both vascular hypertrophy and atherosclerotic disease associated with hypertension. To assess whether plasma PDGF level is increased in mild essential hypertension, we measured plasma PDGF concentration in 25 never-treated patients with uncomplicated mild essential hypertension and in 22 normotensive healthy subjects. To evaluate the contribution of platelets to plasma PDGF in the two groups, we also measured plasma beta-thromboglobulin (BTG). Measurement of PDGF was carried out through an enzyme-linked immunoadsorbent assay, which detects two PDGF dimers, namely PDGF-BB and PDGF-AB. Both plasma PDGF and BTG were higher in the hypertensive than in the normotensive subjects. The ratio of PDGF to BTG was similar in the two groups. Plasma PDGF was weakly correlated with plasma BTG in the normotensive subjects, whereas this relationship was lost in the hypertensive patients. Our results suggest that the increase in plasma PDGF (PDGF-AB + PDGF-BB) in never-treated essential hypertension is mainly due to platelet activation. The increased circulating level of PDGF could play a role in the vascular structural changes associated with hypertension.

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It is well known that sex hormones can regulate the course of systemic lupus erythematosus (SLE). We describe a young woman who had a fatal exacerbation of SLE after controlled ovarian hyperstimulation for induction of ovulation. We recommend careful attention to the immunological status of the infertile women before starting any ovulation inducing treatment for assisted reproduction.

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BACKGROUND: Ear involvement is not unusual in autoimmune diseases, but few data on this problem in the Sjögren syndrome are available. OBJECTIVES: To determine whether the incidence of hearing loss is increased in patients with the Sjögren syndrome and to determine what factors might be involved in the pathogenesis of Sjögren syndrome-related hearing loss. DESIGN: Cross-sectional study. SETTING: Secondary referral center in Italy. PATIENTS: 30 women with the Sjögren syndrome were evaluated for evidence of audiovestibular disorder. Their results were compared with those of 40 age-matched healthy women. MEASUREMENTS: Evaluation techniques included pure tone audiometry thresholds at octave frequencies of 125 Hz to 8000 Hz, tympanometry, and the stapedial reflex test in both ears. The presence of vasculitis, the Raynaud phenomenon, nervous system involvement, and serum anticardiolipin antibodies was recorded. RESULTS: 14 patients with the Sjögren syndrome (46% [95% CI, 28% to 66%]) had sensorineural hearing loss. Only one control (2.5% [CI, 0.06% to 13%]) had a similar hearing impairment (P < 0.001). Nine of the 14 patients who had the Sjögren syndrome and sensorineural hearing loss (64% [CI, 36% to 87%]) had anticardiolipin antibodies compared with only 3 controls (18% [CI, 4% to 45%]) (P = 0.02). CONCLUSION: The high prevalence of hearing loss in the Sjögren syndrome supports the value of doing audiometric studies before excluding cranial nerve involvement in this disease. The correlation of sensorineural hearing loss with anticardiolipin antibodies must be investigated further.

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A 55-year-old woman with a six-year history of Sjögren's syndrome (SS) and the positivity of IgG and IgM antiphospholipid antibodies (aPL) developed a sudden onset of sensorineural hearing loss associated with vertigo. This suggested the presence of an atypical Cogan's syndrome (CS), which might be a focal, neurological complication of aPL.

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The pharmacological treatment of systemic sclerosis (SSc) has been discouraging. In view of this, therapeutic trials with cyclosporine A (CyA) are outstanding particularly in patients with a rapid cutaneous involvement. We report the case of a patient whose SSc cutaneous manifestations dramatically improved with CyA therapy. Its subsequent withdrawal led to a quick onset of a fatal sclerodermal renal crisis. We believe that in our patient there could have been a cause and effect relationship between the suspension of CyA and kidney sclerodermal complication.

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Kikuchi's necrotizing lymphadenitis is a histologically alarming but self-limiting lesion typically affecting the cervical lymph nodes of young women. The authors report 2 cases of systemic lupus erythematosus with the finding of Kikuchi's disease on lymph node biopsy. Considering the common infectious and/or immunologic etiologies described in both disorders, coexistence of both conditions suggests the possibility that they share a common etiology.

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OBJECTIVE: To investigate the efficacy of high-dose intravenous immunoglobulin (IVIg) in the treatment of refractory rheumatoid arthritis (RA). METHODS: Ten patients with active, severe RA that was unresponsive to first- and second-line agents were administered IVIg monthly, for 6 months. RESULTS: Following IVIg treatment, there was significant improvement in both subjective and objective parameters of disease activity in all 9 patients who completed the protocol. This improvement was noted to occur as early as after the second infusion of IVIg. After discontinuation of the treatment, all patients had a relapse of the disease within a few weeks. CONCLUSION: Since the reduction in clinical activity paralleled a decrease in the CD4+CDw29+:CD4+CD45RA+ cell ratio, some of the therapeutic benefits associated with IVIg may be due to a direct influence on the CD4+CD45RA+ subset. Although the possibility of carrying out further controlled studies on a larger scale is limited by the high cost of the treatment, IVIg appears to be an effective therapy for refractory RA.

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We describe a 68-year-old woman suffering from rheumatoid arthritis treated with low doses of prednisone who developed Kaposi's sarcoma (KS). This patient was not affected with AIDS, and two years previously, her sister had also complained of KS. In the literature only 8 rheumatoid arthritis patients who developed KS during steroid therapy have been reported. We present a review of the literature and question the responsibility of corticosteroids and autoimmune diseases as the cause of KS.

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A patient with rheumatoid damage of the cervical spine with cervical cord compression, complaining of a typical syringomyelic syndrome is described. Magnetic resonance imaging revealed cord compression due to an upward displacement of the odontoid peg with a mass of pannus behind the dens, and a syrinx cavity extending from C1 to T1. We postulate that this patient's syringomyelia probably represents a complication of cervical rheumatoid arthritis that has not been described.

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The authors describe necrotizing histiocytic lymphadenitis (Kikuchi's disease) in association with systemic lupus erythematosus (SLE). To our knowledge this is the first case report where SLE preceded Kikuchi's disease. Whether Kikuchi's disease is an independent event or directly connected with SLE is discussed.

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