Nonvasculitic autoimmune inflammatory meningoencephalitis (NAIM): a reversible form of encephalopathy.

Five patients, age 54 to 80 years, presented between 3 weeks and 18 months after symptomatic onset of progressive cognitive decline, psychosis, and unsteady gait that proved to be due to a steroid-responsive nonvasculitic autoimmune inflammatory meningoencephalitic syndrome. CSF examination showed elevated immunoglobulin (Ig)G index and IgG synthesis rate in all three patients in whom it was checked, and brain biopsy revealed perivascular lymphocytic infiltrates without vessel wall invasion.

A study of the cutaneous manifestations of Behçet's disease in patients from the United States.

BACKGROUND: The type and frequency of different manifestations of Behçet's disease (BD) vary in different geographic areas. This variability could affect the ability to diagnose the disease in certain areas by using standardized criteria. The frequency of cutaneous lesions in patients from the United States, where the disease is less prevalent, is not known. OBJECTIVE: We sought to determine the frequency and type of skin lesions in a series of patients with BD from the United States and to identify methods of confirmation of these lesions as part of the disease process. RESULTS: Cutaneous manifestations were present in 64% of patients with BD. Clinicians most often relied on their clinical diagnosis to identify lesions as part of the spectrum of BD. Skin biopsy specimens were generally nonspecific. CONCLUSION: Cutaneous manifestations were common in patients with BD from the United States and usually were necessary to fulfill the diagnostic criteria of the disease in most cases.

Whipple's arthritis: direct detection of Tropheryma whippelii in synovial fluid and tissue.

We describe 2 patients presenting with polyarthritis in whom the synovial fluid (1 patient) or synovial tissue (1 patient) was positive for Tropheryma whippelii, the Whipple's disease-associated bacillus, when examined by polymerase chain reaction (PCR) and DNA sequencing. Histopathologic findings were consistent with articular Whipple's disease in the synovial fluid of 1 patient and the synovial tissue of the other. In both patients, bowel mucosal specimens were negative for Whipple's disease features by histologic and PCR methods. One patient was positive for T whippelii in the peripheral blood. Control synovial fluid specimens from 40 patients with other arthritides, including Lyme arthritis, were negative. Sequencing of a 284-basepair region of the 16S ribosomal RNA gene confirmed that the sequence is closely related to the known T whippelii sequence. Both patients responded to treatment with antibiotics.

Improvement of myelopathy in Sjögren's syndrome with chlorambucil and prednisone therapy.

The authors present a patient with Sjögren's syndrome with a fluctuating and then progressive myelopathic syndrome, and optic nerve involvement. Treatment with chlorambucil and prednisone improved the patient's function from being wheelchair bound to walking unaided. Spinal MRI showed multiple, extensive intraparenchymal areas of abnormal T2-weighted signal intensity, gadolinium enhancement, and cord swelling, which also improved during the period of treatment.

Interferon-alpha treatment of Behçet's disease.

OBJECTIVE: To determine if interferon-alpha (IFN-alpha) is effective in Behçet's disease (BD). METHODS: Ten patients having active BD were entered into a 6 month open label trial of IFN-alpha therapy given by self-administered dose of 3 million units subcutaneously daily. Provision was made for downward or upward dosing depending on predetermined responses. Four study centers followed the same protocol. Patients having ocular or neurologic inflammation at onset of the study were excluded. RESULTS: Seven patients completed the trial, while 3 were removed at 3 and 4 months for side effects or lack of efficacy. There was a substantial reduction in the number of oral and genital ulcers, cutaneous lesions, and articular signs and symptoms by the end of the study. Pain scores, global assessments, and prednisone dose also declined. The IFN-alpha dose at end of study averaged 1.5 million units. Significant side effects, besides the anticipated flu-like syndrome experienced by all the patients, included single instances of seizure, psychosis, psoriasis, and hyperthyroidism. CONCLUSION: The results suggest that IFN-alpha is effective therapy for some manifestations of Behçet's disease.

Polyarticular blastomycotic arthritis.

Blastomycotic arthritis usually presents as acute monoarticular septic arthritis resembling a bacterial process. Only 2 cases in the literature have subsequently developed arthritis in a second joint. We describe the case of an elderly woman with polyarticular blastomycotic arthritis associated with lung, skin, and bone involvement. The organism was identified in the synovial fluid and skin biopsies. Our patient underwent open drainage and aspiration of the involved joints and received antimycotic therapy.

A prospective study of the safety of joint and soft tissue aspirations and injections in patients taking warfarin sodium.

OBJECTIVE: To determine the safety of joint or soft tissue aspirations and injections in patients taking warfarin sodium. METHODS: The outcome of 32 joint or soft tissue aspirations or injections in patients receiving stable doses of warfarin sodium was assessed through a standardized interview 4 weeks after the procedure. The primary outcome measure was significant joint or soft tissue hemorrhage, ascertained by patient-reported increases in swelling or warmth at the procedure site. RESULTS: None of 32 procedures was complicated by joint or soft tissue hemorrhage reported by the patients, yielding, by the "rule of threes," a risk of significant hemorrhage of < 10% (with 95% certainty). Diagnostic information was obtained for 53% of aspirated sites (8 of 15) and therapeutic benefit was noted in 74% of corticosteroid-injected sites (17 of 23). CONCLUSION: Joint or soft tissue injections and aspirations in selected patients taking warfarin sodium are associated with a low risk of hemorrhage and are often of diagnostic or therapeutic value.

Evaluation of glucocorticosteroid injection for the treatment of trochanteric bursitis.

OBJECTIVE: We conducted an open observational study to assess the short and longterm effect of single local glucocorticosteroid injection for trochanteric bursitis. METHODS: 75 patient diagnosed with trochanteric bursitis based on clinical criteria were injected; 20, 32, and 22 patients each received 6, 12, and 24 mg betamethasone, respectively, mixed with 4 cm3 of 1% lidocaine. A standardized baseline questionnaire was administered to assess the severity and functional limitation due to trochanteric pain, including the visual analog scale for pain. Patients were followed at Weeks 1, 6, and 26 to determine their response to treatment. RESULTS: 77.1, 68.8, 61.3% of responding patients reported improvement in pain at Week 1, 6, and 26, respectively. Patients receiving higher doses of betamethasone were more likely to experience pain relief (p < 0.0123). CONCLUSION: Corticosteroid and lidocaine injection for trochanteric bursitis is an effective therapy with prolonged benefit.

Common rheumatologic diseases in elderly patients.

OBJECTIVE: To review common rheumatologic disorders that affect elderly patients and emphasize the unique diagnostic and therapeutic challenges inherent in the management of rheumatologic diseases in this age-group. DESIGN: We summarize our approach to treatment and management of specific rheumatologic problems in geriatric patients and discuss pertinent studies from the literature. RESULTS: Among the spectrum of rheumatologic disorders frequently encountered in the elderly population are polymyalgia rheumatica, fibromyalgia, giant cell arteritis, crystalline arthropathies (gout and pseudogout), and degenerative joint disease. The initial manifestations of these rheumatologic diseases in elderly patients may differ from the typical findings in younger patients. Geriatric patients may have nonspecific complaints, a decline in physical function, or even confusion. Because of physiologic changes associated with aging and a decrease in functional reserves, elderly patients are susceptible to adverse effects of pharmacologic therapy (including nonsteroidal anti-inflammatory medications, corticosteroids, narcotic analgesics, allopurinol, and colchicine). Clinicians should be alert for such problems as hepatotoxicity and occult gastrointestinal blood loss. Comorbid conditions such as cardiovascular disease and cognitive impairment may complicate management strategies and may limit the goals of both surgical intervention and rehabilitation programs in elderly patients. CONCLUSION: Rheumatologic disorders in geriatric patients pose special challenges to primary-care physicians. In the selection of optimal pharmacologic and nonpharmacologic therapeutic modalities, clinicians should focus on maintaining or improving the patient's quality of life and level of independent function.

Survival of low birthweight infants in central Queensland.

The survival of low birthweight infants < or = 2000 g born in the central Queensland area during the years 1979 to 1991 was examined. Five hundred and sixty such infants were either delivered at one of the two Rockhampton obstetric units (Rockhampton Base Hospital and the Mater Misericordiae Hospital) or retrieved from outlying central Queensland areas. Both hospitals had intensive care nurseries capable of ventilation and infants were transferred to tertiary centres only for cardiac or surgical treatment. The study involved all liveborn infants, including those with lethal malformations and all deaths up to the time of discharge. Survival rates were as follows: < or = 500 g 0%, 501 < or = 750 g 30%, 751 < or = 1000 g 51%, 1001 < or = 1500 g 79%, 1501 < or = 2000 g 93%. The survival of Aboriginal infants and outborn infants were found to be essentially the same as the overall group.

Behçet's disease.

The International Study Group's diagnostic criteria for Behçet's disease are gaining acceptance and may be further refined before the decade is over. Previously neglected clinical features of the disease, especially arterial aneurysms and phlebitis, are increasingly reported. Each poses a dilemma in the choice of treatment, which ranges from anticoagulant to immunosuppressant regimens, old and new. T-lymphocyte abnormalities, both in subpopulation and function, suggest ongoing presentation of an unknown antigen. Research on the role of HLA-B51, a split of B5, is moving centromeric to it in pursuit of a better disease marker. The choice of immunosuppressive therapy in severe disease such as uveitis and arteritis is between alkylating agents and agents that inhibit interleukin-2 production.

Chronic inflammatory meningoencephalitis should not be mistaken for Alzheimer's disease.

We describe two patients with a chronic encephalopathy that clinically resembled dementia but that resolved after oral administration of high-dose corticosteroid therapy. Both patients had serologically documented Sjögren's syndrome, a diagnosis that was further supported by biopsy of a salivary gland in one. Neither patient had radiologic evidence of vasculitis of the central nervous system. In one patient, meningeal and brain biopsy specimens showed perivascular inflammatory lymphocytic infiltrates. Chronic inflammatory meningoencephalitis is a treatable cause of chronic encephalopathy that should be clinically distinguished from dementia associated with Alzheimer's disease.