Chronic recurrent multifocal osteomyelitis is an idiopathic nonpyogenic autoinflammatory bone disorder involving multiple sites, with clinical progression persisting for more than 6 months and which may have episodes of remission and exacerbation in the long term. It represents up to 2-5% of the cases of osteomyelitis, with an approximate incidence of up to 4/1,000,000 individuals, and average age of disease onset estimated between 8-11 years, predominantly in females. The legs are the most affected, with a predilection for metaphyseal regions along the growth plate. We describe the case of a female patient, aged 2 years and 5 months, with involvement of the left ulna, right jaw and left tibia, showing a predominance of periosteal reaction as main finding.Keywords: osteomyelitis, chronic, multifocal, recurrent, periosteal.case report A female patient aged 2 years and 5 months, being investigated due to pain and swelling in the left forearm for one month. Her parents denied episodes of fever, trauma, and other comorbidities. On physical examination, the child presented normal weight, normal skin color, she was well-hydrated, acyanotic, breathing normally and in good general conditions. Blood counts analyzed in the previous month revealed mild leukocytosis and normocytic normochromic anemia. Levels of C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR) were slightly elevated.Computed tomography (CT) of the left forearm without intravenous contrast revealed a periosteal reaction and a small area of loss of cortical compaction, especially in the ulna ( Figure 1A).A biopsy of the left ulna was performed with results describing signs suggestive of chronic osteomyelitis. Culture of the biopsy material did not show growth of microorganisms.Empirical antibiotic therapy was used starting one month after diagnosis, but there was no change in clinical status. A new biopsy was performed in the third month, showing the same findings associated with negative blood culture. Radiographs of the left forearm from the second to the fifth month showed progression of the multilamellar periosteal reaction to a solid type ( Figure 1B).Chest radiography, echocardiography, levels of TSH, T4, IgG, IgA, IgM and complement components were all normal. A hypothesis of chronic recurrent multifocal osteomyelitis (CRMO) was suggested and thus treated with ibuprofen combined with methotrexate, later replaced with sulfasalazine.Between the eighth and ninth month, pain and swelling appeared in the right mandibular region and in the left leg. A facial CT scan showed mainly periosteal reaction at the right mandibular angle with mild bone sclerosis (Figure 2). CT scan of the left leg also characterized a periosteal reaction in the tibial diaphysis with a small area of cortical alteration (Figure 3), similar to that found in the ulna.After 6 months of therapy, there was clinical and general laboratory improvement, with left forearm radiography in the 11 th month showing a regression of the periosteal reaction, despite persistence of corti...
Multiple sclerosis (MS) is a recurrent chronic inflammatory demyelinating disease, restricted to the central nervous system (CNS), whose histological feature is the occurrence of perivenular inflammatory infiltrate, leading to demyelination with relative axonal preservation. The classic form of the disease is characterized by the recurrence of attacks (outbreaks), followed by remission of symptoms (RRMS), with the presence of multiple focal lesions dispersed by the CNS (spatial dissociation) with exuberant inflammatory process in the acute phase, coexisting with chronic lesions (Temporal dissociation) without inflammatory activity evidenced by the breakdown of blood-brain barrier and contrast-enhanced contrast-enhanced magnetic resonance imaging (MRI). Some patients have a benign course, remaining free of significant sequelae for more than 20 years of the disease. Other patients have an aggressive form of the disease, being restricted to the wheelchair in 8 to 10 years of evolution. One challenge is to modify the course in this aggressive way, which can be done with the use of immunomodulators, chemotherapeutics and, eventually, autologous hematopoietic stem cell transplantation (aHSCT). The goal of using aHSCT is to restore immune activity free of myelin attacks ("autoimmune reset"). One of the ways to monitor treatment efficacy is to identify the occurrence of new lesions and visible reinforcing lesions on serial MRI scans. Objective: To test the hypothesis that the treatment of patients with MS using AHSCT was effective in avoiding the recurrence of inflammation and the appearance of new visible lesions in SB at the long-term examination of MRI. Results: At our institution, approximately 66 patients with MS were treated with aHSCT from 2004 to 2011, followed by the hematology, neurology / neuroimmunology and MRI sections of the CCIFM-HCRP. Methods: Brain and MRI scans acquired in a prospective and protocolized way from 76 patients who underwent aHSCT were followed up with MRI for more than five years. The MR images were archived on the CCIFM servers, retrieved, anonymised and reviewed by at least two experienced radiologists, independently and by confrontation. The identification of new lesions and / or lesions with inflammatory reactivation was considered therapeutic failure. Results: Ten patients were excluded. Twelve patients (18.18%) presented new lesions or recurrence of the inflammatory process, with reinforcement. Conclusion: In our sample, aHSCT was able to control the recurrence of lesions and the inflammatory activity detected in MRI in more than 87% of the cases, characterizing an important second line therapeutic option in the cases of greater aggressiveness of the disease.
A previously-healthy 16-year-old male presented with fever, headache, lethargy and seizures for a day. A hemogram showed mild lymphocytosis. Spinal fluid analysis revealed 75 leukocytes/mm³ (62% neutrophils, 35% lymphocytes, 3% monocytes) and no growth of micro-organisms in culture. Cerebral imaging studies showed an abnormal bilateral symmetrical appearance of the basal ganglia, which, together with positive IgG and IgM serology for the Epstein-Barr virus, suggested acute encephalitis by this agent (Figure). Some of the differential diagnoses were: hypoglycemia, hypoxia, intoxication (methanol, carbon monoxide, cyanide), herpes simplex infection, infantile bilateral striatal necrosis and central extrapontine myelinolysis 1,2,3,4,5 .
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