PubMed Health⌕ Search

Biomedical subjects

A Hietaharju

Publications and source records attributed to A Hietaharju.

At least 19 recordsLinked to original sources
PubMed2001

The prevalence of neuropsychiatric syndromes in systemic lupus erythematosus.

OBJECTIVE: To describe the prevalence of neuropsychiatric (NP) syndromes in a Finnish population of patients with systemic lupus erythematosus (SLE) and to classify them according to the recently developed American College of Rheumatology (ACR) nomenclature and case definitions for NPSLE. METHODS: Cross-sectional, population-based study covering an area with 440,000 people. A total of 58 patients with a definite diagnosis of SLE and aged 16 to 65 years were found in the computerized database of the area hospitals. Of these, 46 (79%) agreed to participate. The diagnosis of various NP syndromes was based on clinical impression (H.A.) following history, examination, review of medical records, and neuropsychologic testing. RESULTS: At least one NP syndrome was identified in 42 patients (91%). The most frequent manifestation was cognitive dysfunction (n = 37; 81%), followed by headache (n = 25; 54%) and mood disorder (n = 20; 43%). When mild NP syndromes (mild cognitive deficit, headache, mild depression, anxiety, electroneuromyography-negative polyneuropathy) were excluded, the prevalence of NPSLE dropped to 46%. CONCLUSIONS: According to the ACR nomenclature, there is a high prevalence of NP manifestations in a population-based sample of patients with SLE. Most NP syndromes were classified as minor; if they were excluded, the 46% prevalence of NPSLE would be slightly less than estimated in previous studies.

Adult↗
PubMed2001

Validity of the new American College of Rheumatology criteria for neuropsychiatric lupus syndromes: a population-based evaluation.

OBJECTIVE: To assess the validity of the recently developed American College of Rheumatology (ACR) nomenclature for neuropsychiatric systemic lupus erythematosus (NPSLE). METHODS: We conducted a cross-sectional, population-based study covering an area with 440,000 people. A total of 46 patients aged 16 to 65 years fulfilled the criteria for a definite diagnosis of SLE. One control for each patient matched by age, sex, education, and place of residence was randomly identified from the population register. All patients and controls underwent a clinical neurologic examination and neuropsychological testing. The data were analyzed using conditional logistic regression methods. RESULTS: Forty-two patients (91%) and 25 controls (56%) fulfilled at least one of the ACR NPSLE criteria, which gave an odds ratio (OR) of 9.5 (95% confidence interval [CI] 2.2-40.8) but low specificity (0.46). Cognitive dysfunction was the most common syndrome detected in 37 patients (80%). A revised set of 16 criteria excluding the syndromes without evidence for neuronal damage resulted in improved specificity (OR 7.0, 95% CI 2.1-23.5, specificity 0.93). CONCLUSION: The proposed 19 ACR criteria did not differentiate SLE patients from controls, nor NPSLE patients from other SLE patients. The revised NPSLE criteria proposed by us performed well in our population but should be evaluated in a larger patient population.

Adolescent↗
PubMed2001

The coexistence of systemic lupus erythematosus and multiple sclerosis in a mother and daughter.

The occurrence of both multiple sclerosis (MS) and systemic lupus erythematosus (SLE) has previously been described in several members within the same family and in twins of successive generations, but the finding of both diseases in one patient is a great rarity. We here report on a rare coexistence of MS and SLE both in mother and daughter. Both patients fulfill the diagnostic criteria of primary-progressive subtype of MS as well as SLE. The finding constitutes supporting evidence of a common genetic background for these two autoimmune disorders.

Adult↗
PubMed2000

Chronic neuropathic pain in treated leprosy.

The existence of chronic neuropathic pain in treated leprosy has received scant attention. We describe the clinical findings of 16 patients with multibacillary leprosy who had chronic stimulus-independent pain despite finishing their treatment. With confirmation, our results could be of importance in the establishment of "care after cure" programmes for patients with leprosy.

Adult↗
PubMed2000

Reversible posterior leukoencephalopathy after combination chemotherapy.

We describe a young woman with Burkitt's lymphoma, treated with intravenous adriamycine and cyclophosphamide and intrathecal cytarabine. She developed a reversible posterior leukoencephalopathy syndrome (RPLS) with typical MRI findings. Diffusion-weighted images during the first days after the onset of symptoms predicted a small irreversible lesion in the frontal lobe, verified on T2-weighted images 1 month later. The patient showed full recovery after high-dose steroid treatment.

Adolescent↗
PubMed1998

A reversible neuronal antibody (anti-Tr) associated paraneoplastic cerebellar degeneration in Hodgkin's disease.

Paraneoplastic cerebellar degeneration (PCD) has been associated with a variety of neoplasms, most commonly with gynecologic tumors, breast cancer, small cell lung cancer, and Hodgkin's disease (HD). In some patients PCD is associated with circulating antineuronal antibodies like anti-Hu, anti-Yo or anti-Ri. Previously, only 5 patients with a new antineuronal antibody called anti-Tr, proposed to be specific for HD, have been reported. We describe 1 further patient with HD and reversible PCD with a decline in anti-Tr antibody titers in cerebrospinal fluid and serum corresponding to the improvement of clinical symptoms. At the present time the immunoreactive pattern observed in rat cerebellum is the only way to identify anti-Tr antibodies and differentiate them from other antibodies that immunoreact with the Purkinje cells.

Adult↗
PubMed1994

Association of HLA antigens with anti-Scl-70-antibodies and clinical manifestations of systemic sclerosis (scleroderma)

Thirty patients with systemic sclerosis (scleroderma) and 188 healthy controls were studied for class I and class II MHC antigens. All patients with scleroderma fulfilled the ARA preliminary criteria for systemic sclerosis. The frequencies of HLA antigens B8, DR3 and DR52 were higher in patients with scleroderma as compared with controls. Ten patients had anti-Scl-70 antibodies, and four of them (40%) had HLA-DR5 antigen compared to 19 of 188 controls (10%), P = 0.013. The relative risk for DR5 in anti-Scl-70-positive patients was 3.6 compared to 1.6 of DQ3 suggesting the primary significance of DR5 compared to DQ related factors. Patients with neurological manifestations had higher frequencies of B8 and DR3 than those with no neurological manifestations of scleroderma. Our results support the view that immunogenetic background is associated with different clinical subsets of systemic sclerosis.

Adult↗
PubMed1994

Association of anti-U1RNP- and anti-Scl-70-antibodies with neurological manifestations in systemic sclerosis (scleroderma).

Thirty-one patients with SSc were studied. Eleven patients had anti-Scl-70-, six had anti-U1RNP-, three had anticentromere antibodies, and one both anti-Scl-70- and anticentromere antibodies. Eleven patients (35%) had neurological findings (trigeminal neuropathy, polyneuropathy, in some with myopathy). Eight of these patients (73%) had either anti-U1RNP- or anti-Scl-70-antibodies in their serum. These findings suggest that neurological manifestations are not as uncommon in SSc as previously reported. There are probably subgroups of patients who are more prone to neurological manifestations of scleroderma (mostly patients with anti-U1RNP and possibly those with anti-Scl-70 antibodies).

Adult↗
PubMed1993

Peripheral neuromuscular manifestations in systemic sclerosis (scleroderma).

Systemic sclerosis (scleroderma) is thought to be the least likely of the collagen vascular disorders to cause nervous system damage. We evaluated the peripheral neuromuscular manifestations in 32 patients with scleroderma. A clinically defined peripheral nervous system (PNS) lesion was manifest in 5 of 32 patients (16%), including 2 patients with trigeminal neuropathy and single cases of polyneuropathy, brachial plexopathy, and lumbosacral radiculopathy. Neurophysiological studies suggested subclinical PNS involvement in 6 additional patients (3 with distal axonal polyneuropathy, 1 with probable myopathy and superimposed polyneuropathy, 1 with trigeminal neuropathy, and 1 with focal ulnar neuropathy). Even though subjective muscular complaints were numerous (16 patients, 50%), a defined primary muscular disease could be demonstrated only in 5 patients (16%). Our results indicate that peripheral neuropathy in scleroderma is not as uncommon as previously estimated.

Action Potentials↗
PubMed1993

Central nervous system involvement and psychiatric manifestations in systemic sclerosis (scleroderma): clinical and neurophysiological evaluation.

We evaluated central nervous system and psychiatric involvement in a clinical sample of 32 patients with systemic sclerosis (SSc) (scleroderma). All patients underwent clinical neurological examination. Electroencephalography (EEG) and visual evoked potentials (VEPs) were also recorded. Prominent central nervous system (CNS) or psychiatric symptoms were present in 5 patients (16%), including encephalopathy, psychosis, anxiety disorder, grand mal seizures and transient ischemic attack. In addition, abnormal VEPs were recorded from 5/32 patients (16%), suggesting optic neuropathy. EEGs were mainly normal or showed only slight, nonspecific changes. Primary CNS involvement in scleroderma, however, could not be shown in any of the 5 cases with neuropsychiatric symptoms. Our results suggest that neuropsychiatric symptoms in SSc are, if not coincidental, indirectly caused by internal organ involvement of SSc or by possible overlapping connective tissue diseases. On the other hand, optic neuropathy might be a primary complication of SSc.

Adult↗
PubMed1993

Nervous system involvement in systemic lupus erythematosus, Sjögren syndrome and scleroderma.

INTRODUCTION: The purpose of this study was to determine, whether there are any differences in the occurrence of nervous system involvement in different systemic rheumatic diseases. The further aim of the present study was to identify and distinguish primary involvement of the nervous system by these diseases and involvement that may be secondary to confounding factors. MATERIAL AND METHODS: The patient population consisted of 122 patients with a connective tissue disease (42 with systemic lupus erythematosus (SLE), 48 with Sjögren's syndrome and 32 with scleroderma). The methods included neurological examination and standard electrophysiological tests. RESULTS: At least one neurological defect was diagnosed in 69% of SLE patients, in 71% of Sjögren's syndrome patients and in 66% of scleroderma patients. Secondary factors might have contributed to the pathogenesis of neurological symptoms and signs in up to 25-34% of events. CONCLUSION: No significant differences were noted in the occurrence of neurological events in patients with SLE, Sjögren's syndrome and scleroderma. The necessity to differentiate between neurological phenomena directly attributed to the systemic rheumatic disease and those which are totally unrelated or secondary events resulting indirectly from involvement of other organ systems is emphasized.

Adult↗
PubMed1992

Nervous system disease, immunological features, and HLA phenotype in Sjögren's syndrome.

Twenty seven patients with primary or possible Sjögren's syndrome with neurological manifestations were compared immunologically with 21 patients with Sjögren's syndrome with an intact nervous system. Patients with Sjögren's syndrome were divided into seropositive and seronegative subgroups with respect to the occurrence of one or more autoantibodies (antinuclear antibodies, rheumatoid factor, antibodies to SS-B) in their serum samples. This study of 48 patients indicates that the spectrum of nervous system disease in seronegative and seropositive subgroups is almost indistinguishable. No significant differences were found in the occurrence of circulating immune complexes, the levels of serum complement C3 and C4, or serum IgA, IgM, and beta 2 microglobulin with respect to the neurological manifestations. The serum IgG level, however, was significantly higher in the patients with Sjögren's syndrome with intact nervous systems than in those with neurological manifestations. No significant association was found between the HLA phenotype and nervous system disease. The occurrence of HLA-B8 and DR3 antigens was, however, significantly higher in those patients with antibodies to SS-B than in those without. This finding supports the suggestion that HLA-B8/DR3 may modulate autoantibody responses rather than disease expression in Sjögren's syndrome.

Adult↗
PubMed1990

Nervous system manifestations in Sjögren's syndrome.

Central and peripheral manifestations of the nervous system were evaluated in 48 Sjögren's syndrome patients. Fifty-six percent of the patients had neurological disturbances. The most common manifestations were entrapment neuropathies (19%) and polyneuropathy (15%). Electrophysiological tests gave further evidence of subclinical nervous system involvement in Sjögren's syndrome: electroencephalography (EEG) was abnormal in 48%, and visual evoked potentials (VEP) in 12% of patients tested. To find possible neuropsychiatric abnormalities, the Minnesota Multiphasic Personality Inventory was applied, and 33/43 patients were found to have psychiatric symptoms. The most frequent were depressive symptoms. In 44% of the patients there was additional evidence of extraglandular involvement or autoimmune disorders. No correlation could be found between the groups of patients with or without neurological disturbances in relation to simultaneous occurrence of associated disorders. It is suggested that nervous system involvement in Sjögren's syndrome reflects the pathogenetic consequences of Sjögren's syndrome alone, and not those of associated autoimmune diseases of extraglandular disorders.

Adult↗