Cases reported "Sjogren's Syndrome"

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1/126. Autonomic failure and proximal skeletal myopathy in a patient with primary Sjogren syndrome.

    Autonomic failure and proximal skeletal myopathy are rare features of the Sjogren syndrome (SS). We describe a 51-year-old woman with primary SS who had development of esophageal dysmotility, urinary retention, severe orthostatism, and skeletal myopathy during a 3-month period after the diagnosis of SS. Her symptoms and signs responded well to corticosteroid therapy. Although dysfunction of the peripheral nervous system has a prevalence rate of 20% in patients with SS, most commonly the nerve dysfunction is a sensory deficit, and autonomic neuropathy is less frequent. Autonomic neuropathy due to SS may be underreported. The cause of our patient's myopathy remains undetermined. We speculate that the myopathy was due to either a form of polymyositis or an immune-mediated neuropathy with muscle involvement.
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ranking = 1
keywords = peripheral nervous system, neuropathy, peripheral, nervous system, nerve
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2/126. Primary sjogren's syndrome in children and adolescents: proposal for diagnostic criteria.

    OBJECTIVE: Primary sjogren's syndrome (pSS) in childhood is a rare disease. Diagnostic criteria are available for adult patients only. In order to establish diagnostic criteria for juvenile pSS an analysis of 7 girls and one boy suffering from pSS with early onset is reported. Due to the rarity of the disease, data on patients with pSS reported in the literature are included in the proposal for modified diagnostic criteria. methods: The diagnosis of pSS was established according to the criteria for adulthood pSS, duly modified, which include clinical symptoms and laboratory immunological evaluation. RESULTS: The average age of our patients at clinical onset was 13.5 years (range: 10-17 yrs.). Clinical signs included systemic (fever, fatigue) as well as local (parotitis, vulvovaginitis, conjunctivitis) symptoms. paralysis due to hypokalemia linked to renal tubular acidosis and central nervous system (CNS) involvement was seen in one patient. Asymptomatic renal tubular acidosis was diagnosed in another 2 patients. Autoimmune hepatitis was present in 2 patients. All patients had laboratory abnormalities: hyperimmunoglobulinemia IgG, high titers of antinuclear antibodies (anti-SS-A and/or anti-SS-B) and elevated serum amylases. Sicca syndrome was never seen during childhood, although it developed later in 3 patients, after 7 to 10 years of follow-up. CONCLUSIONS: It has been stressed that the classical diagnostic criteria for adult sjogren's syndrome, especially sicca syndrome, are not applicable to a pediatric onset of the disease. On the other hand, the presence of typical laboratory abnormalities can allow the diagnosis of these patients in the early stages. Both laboratory and clinical symptoms typical for childhood are included in our proposal for diagnostic criteria applicable to juvenile pSS. life-threatening conditions such as hypokalemic paralysis, CNS involvement and hepatitis may also occur in children. Sicca syndrome tends to develop much later in pediatric patients.
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ranking = 0.019679096142489
keywords = nervous system
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3/126. Primary sjogren's syndrome with severe central nervous system disease.

    OBJECTIVE: central nervous system (CNS) involvement in primary sjogren's syndrome (pSS) is controversial with regard to frequency, significance, and etiology. methods: We describe a young woman with pSS and severe CNS disease and review the literature on the pathophysiology, clinical significance, symptoms, diagnostic examinations, and treatment of CNS disease with concomitant pSS (CNS-SS). RESULTS: Our patient with pSS had a 5-month history of benign lymphadenopathy and myositis, after which she developed severe CNS disease, vasculitic lesions on her hands, and a neurogenic bladder attributable to spinal cord involvement. The diagnosis was based on the clinical picture and the results of a brain magnetic resonance imaging (MRI) scan, electroencephalography (EEG), and cerebrospinal fluid (CSF) analysis. The disease did not respond to corticosteroids, but the administration of cyclophosphamide resulted in recovery. In the literature, the incidence of CNS-SS varies widely, from rare to incidence rates of 20% to 25%. The clinical picture is diverse, ranging from mild cognitive symptoms to fatal cerebrovascular accidents. The pathophysiology of CNS-SS is unclear, specific diagnostic methods are not available, and diagnosis is based on the clinical picture and a combination of examinations. MRI is the most sensitive test and cerebral angiography the most specific. CSF reflects involvement of the leptomeninges, and EEG is nonspecific. There are no controlled studies of the treatment of CNS-SS. Regimens for vasculitis are commonly used. CONCLUSIONS: CNS-SS is uncommonly recognized and difficult to diagnose. Increasingly accurate and available diagnostic examinations will yield more information about the association of CNS disease with pSS.
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ranking = 44.634352235262
keywords = nervous system disease, system disease, nervous system
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4/126. A combination of autoimmune hepatitis, sensory-dominant peripheral neuropathy, and primary sjogren's syndrome in the same patient: a rare association.

    Although autoimmune hepatitis and sensory-dominant neuropathy have been known to independently accompany primary sjogren's syndrome, the combination of all these conditions in the same patient has not been described. We report the case of a woman who initially suffered from autoimmune hepatitis and later was diagnosed with primary sjogren's syndrome upon the development of sensory-dominant neuropathy. In this patient, autoimmune hepatitis preceded neuropathy by one year. All of the diagnoses were confirmed by histological examinations of the liver, sural nerve, and minor salivary gland. Her autoimmune hepatitis was relieved with conservative treatment, and her sensory-dominant neuropathy was alleviated by prednisolone therapy. Our case indicated that the multiple organ involvement, especially that in the liver and peripheral nerves, should be taken into account in the course of primary sjogren's syndrome.
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ranking = 1.854550045956
keywords = peripheral neuropathy, neuropathy, peripheral, peripheral nerve, nerve
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5/126. Systemic mononuclear inflammatory vasculopathy associated with sjogren's syndrome in a patient with primary biliary cirrhosis.

    We report a 46-year-old woman with primary biliary cirrhosis (PBC) presenting with sjogren's syndrome and systemic mononuclear inflammatory vasculopathy. biopsy specimens of sural nerve showed findings consistent with vasculitic neuropathy. Perivascular inflammatory mononuclear cell infiltration was observed on muscle biopsy specimen. The findings of abdominal computed tomography and brain magnetic resonance imaging were suggestive of vasculitis. Clinical manifestations and radiologic findings were improved after high dose prednisolone therapy.
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ranking = 0.089905322114738
keywords = neuropathy, nerve
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6/126. Primary sjogren's syndrome manifested as multiple sclerosis and cutaneous erythematous lesions: a case report.

    sjogren's syndrome is a chronic autoimmune disorder characterized by lymphocytic infiltration of the lacrimal and salivary glands, leading to dryness of eyes (kerato-conjunctivitis sicca) and mouth (xerostomia). The skin lesions in sjogren's syndrome are usually manifested as xeroderma, but sometimes appear as annular erythema or vasculitis. central nervous system symptoms may be presented as one of extraglandular manifestations, though rare in incidence, and need differential diagnosis from multiple sclerosis. We report a case of a 45-year-old woman diagnosed as multiple sclerosis at first but later as neurologic manifestation of primary sjogren's syndrome, showing signs of multiple sclerosis and cutaneous erythematous lesions.
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ranking = 0.019679096142489
keywords = nervous system
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7/126. Acute optic neuropathy and transverse myelopathy in patients with antiphospholipid antibody syndrome: favorable outcome after treatment with anticoagulants and glucocorticoids.

    We describe two patients with established antiphospholipid syndrome, who during periods of subtherapeutic anticoagulation, developed acute optic neuropathy and transverse myelopathy. Treatment with optimal anticoagulation and high dose glucocorticoids was followed by resolution of the neurologic deficits.
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ranking = 0.44332954762266
keywords = neuropathy
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8/126. Cowden's syndrome (multiple hamartoma and neoplasia syndrome): diagnostic dilemmas in three cases.

    Cowden's syndrome is a multisystem disease inherited as an autosomal dominant trait with incomplete penetrance and variable expression. The disease has typical oral manifestations which often precede more systemic involvement, and the dental professional is therefore well placed to institute a regime of regular checks to ensure early treatment of any neoplasms which may occur. However, since not all of the classical signs are present in all patients, diagnosis may be difficult. The case report of a patient with most of the features of Cowden's syndrome is presented and features compared with two other possible cases.
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ranking = 2.0010734871545
keywords = system disease
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9/126. A case of sensory neuropathy associated with childhood Sjogren syndrome.

    Complications observed in adulthood Sjogren syndrome also occur in the childhood disease and suggest that Sjogren syndrome should be considered as a cause of neuropathy in children. Treatment with corticosteroid is a choice for such cases.
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ranking = 0.44332954762266
keywords = neuropathy
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10/126. trochlear nerve palsy in sjogren's syndrome.

    sjogren's syndrome (SS) is a systemic lymphoproliferative, autoimmune disease, which is characterized by dryness of the eyes, mouth, and other mucous membranes. The nervous system may be affected in up to 20% of the cases of primary or secondary SS. We present a case of a 54-year-old woman with trochlear nerve palsy complicating sjogren's syndrome secondary to rheumatoid arthritis. We suggest that all patients with multiple cranial neuropathies, especially when associated with rheumatoid arthritis, should be carefully examined for the possible presence of secondary SS.
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ranking = 0.025876159093517
keywords = nervous system, nerve
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