1/46. 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.- - - - - - - - - - ranking = 1keywords = nervous system (Clic here for more details about this article) |
2/46. 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.- - - - - - - - - - ranking = 3.2812279402903keywords = central nervous system, nervous system (Clic here for more details about this article) |
3/46. 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.- - - - - - - - - - ranking = 14.156146225853keywords = central nervous system, nervous system, brain (Clic here for more details about this article) |
4/46. 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.- - - - - - - - - - ranking = 0.031234464691699keywords = brain (Clic here for more details about this article) |
5/46. 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.- - - - - - - - - - ranking = 1keywords = nervous system (Clic here for more details about this article) |
6/46. 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.- - - - - - - - - - ranking = 1keywords = nervous system (Clic here for more details about this article) |
7/46. Treatment of myelopathy in Sjogren syndrome with a combination of prednisone and cyclophosphamide.BACKGROUND: Peripheral neuropathy is a common complication of primary Sjogren syndrome, but central nervous system involvement also occurs and may be the only extraglandular manifestation. Sicca symptoms may also be minimal. Combinations of lesions along with relapses and remissions can suggest multiple sclerosis in the proper clinical setting, making the correct diagnosis elusive. OBJECTIVES: To report a case of progressive transverse myelopathy with previous optic neuropathy in primary central nervous system Sjogren syndrome (CNS-SS), and to review 17 previously reported cases and the patient's responses to various therapies. DESIGN: Case report and literature review. SETTING: University hospital. PATIENT: A 63-year-old Hispanic woman with a 10-month history of progressive spastic paraparesis associated with optic neuropathy and a T10 sensory level. magnetic resonance imaging demonstrated multifocal, contrast-enhancing lesions in the spinal cord. The patient was diagnosed as having CNS-SS because of the presence of sicca symptoms, abnormal serological test results, and salivary gland biopsy results, which fulfilled San Diego criteria for "definite" Sjogren syndrome. She responded to treatment with a combination of prednisone and cyclophosphamide. CONCLUSIONS: diagnosis of primary CNS-SS requires a high index of suspicion and specialized clinical testing. Treatment with pulse doses of corticosteroids alone may be suboptimal, but results of treatment with a combination of corticosteroids and either cyclophosphamide or chlorambucil have been encouraging.- - - - - - - - - - ranking = 6.5624558805806keywords = central nervous system, nervous system (Clic here for more details about this article) |
8/46. central nervous system sjogren's syndrome in a child: case report and review of the literature.We describe a case of pediatric sjogren's syndrome with progressive neurologic involvement. At age 4 years, she had been diagnosed with melkersson-rosenthal syndrome. After being stable with facial diplegia and swelling for 5 years, she acutely presented with diplopia, vertigo, and ataxia. Cranial magnetic resonance imaging (MRI) showed a left dorsal midbrain lesion. Serologic and histopathologic findings confirmed primary sjogren's syndrome. She responded well to intravenous methylprednisolone, with subsequent clinical improvement and MRI resolution. This report reviews the pediatric literature and underscores the importance of considering sjogren's syndrome in a child with unexplained facial weakness and in the differential diagnosis of pediatric stroke.- - - - - - - - - - ranking = 4.0312344646917keywords = nervous system, brain (Clic here for more details about this article) |
9/46. Neuropsychological, neuroimage and psychiatric aspects of primary sjogren's syndrome.We report a case of a 49-year-old woman diagnosed with primary Sjogrens Syndrome (pSS) who was submitted to extensive neuropsychobiological assessment. Examination revealed a Wechsler adult intelligence Scale-Revised (WAIS-R) Full Scale IQ of 97 with no Verbal/Performance IQ discrepancy and performance below estimated premorbid levels on arithmetic skills, visual tracking, naming and delayed paired associate learning/memory. CT scans of the brain were normal. However, there were subcortical hyperintensities on MRI and left parieto-temporal hypoperfusion on SPECT. Neuropsychological impairment is consistent with the pattern of neuroimage findings. We hypothesize that the pathophysiological mechanisms of pSS involve direct immune attack on neurons in addition to indirect effects through small-vessel angiopathy and thereby induce natural fracture lines in behavior according to location in the central nervous system.- - - - - - - - - - ranking = 3.312462404982keywords = central nervous system, nervous system, brain (Clic here for more details about this article) |
10/46. Angiocentric T cell lymphoma of the central nervous system in a patient with sjogren's syndrome.patients with sjogren's syndrome (SS) have an increased risk of developing lymphoproliferative disorders, mainly non-Hodgkin's lymphoma and pseudolymphoma. We describe a young Caucasian woman with a 7 year history of SS, who presented with meningeal signs and symptoms and was found to have non-Hodgkin's lymphoma of the central nervous system.- - - - - - - - - - ranking = 16.406139701451keywords = central nervous system, nervous system (Clic here for more details about this article) |
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