Primary Sjogren’s Syndrome Presenting as Autoimmune Cytopenia

Sjogren’s syndrome (SS) is a chronic systemic autoimmune disease, characterized by lymphocytic infiltration of lacrimal and salivary glands. Although extra glandular manifestations are uncommon, they can occur with the musculoskeletal, renal, pulmonary and hematological disease. We report the case of a 35-year-old woman presented to us with persistent unexplained bicytopenia (anemia and thrombocytopenia). Antinuclear antibody and direct Coombs test were positive. Anti-Ro/SSA and anti-La/SSB antibodies were also positive in high titer. The final diagnosis of primary SS with autoimmune cytopenia was made. Cytopenias in SS are rarely reported. Our case illustrates that clinically significant cytopenias may present as an extraglandular manifestation of SS

Acute Brucellosis Presenting as an Autoimmune Hemolytic Anemia

Brucellosis is one of the most widespread zoonosis in the world. Hematological complications in brucellosis usually present as mild anemia, leukopenia, or pancytopenia. Autoimmune hemolytic anemia in brucellosis is rarely reported. Here, we report an 18-year-old female presented to us with progressive fatigue, jaundice, and fever. Hematological investigations revealed hemolytic anemia. Direct Coombs test was positive. Further evaluation showed positive serology and culture for Brucella. The patient was diagnosed with brucellosis with autoimmune hemolytic anemia. She was put on rifampicin and doxycycline along with corticosteroids. After 6 weeks, the patient was symptomatically improved with complete remission of hemolytic anemia. The possibility of brucellosis should be considered as a differential diagnosis of autoimmune hemolytic anemia, especially those living in the endemic areas

Hyperhomocysteinemia in a Patient with Moyamoya Disease

Moyamoya disease is a chronic progressive cerebrovascular disease characterized by bilateral occlusion or stenosis of arteries around circle of Willis. We report a case of 18-year-old female presented with recurrent episodes of headache and vertigo. On cerebral angiography, the patient was diagnosed to have moyamoya disease. On further evaluation, thrombophilia profile showed increased homocysteine level. The patient was treated conservatively with cobalamin and aspirin and advised for revascularization. According to the literature, there are few case reports of moyamoya disease with thrombotic disorders. Hence, we are reporting this interesting and rare case

Epidemiology, clinical presentation, and predictors of outcome in nontuberculous mycobacterial central nervous system infection: a systematic review

Abstract Background CNS manifestations represent an emerging facet of NTM infection with significant mortality. Due to protean presentation and low index of suspicion, many cases are often treated erroneously as tubercular meningitis or fungal infections. Objectives Literature on NTM CNS disease is scarce, with most available data on pulmonary disease. This systematic review aimed to evaluate the epidemiology, clinical presentation, diagnostic modalities, and predictors of outcome in CNS NTM infection. Methods The literature search was performed in major electronic databases (PubMed, Google Scholar, and Scopus) using keywords “CNS,” “Central nervous system,” “brain abscess,” “meningitis,” “spinal,” “Nontuberculous mycobacteria,” “NTM”. All cases of CNS NTM infection reported between January 1980 and December 2022 were included. Results A total of 77 studies (112 cases) were included in the final analysis. The mean age of all patients was 38 years, with most patients male (62.5%). Mycobacterium avium complex (MAC) was the most common aetiology, followed by M. fortuitum and M. abscessus (34.8%, 21.4% and 15.2%, respectively). The disseminated disease was found in 33% of cases. HIV (33.9%) and neurosurgical hardware (22.3%) were the common risk factors. Intracranial abscess (36.6%) and leptomeningeal enhancement (28%) were the most prevalent findings in neuroimaging. The overall case fatality rate was 37.5%. On multivariate analysis, male gender (adjusted OR 2.4, 95% CI 1.2–7.9) and HIV (adjusted OR 3.7, 95% CI 1.8–6.1) were the independent predictors of mortality). M. fortuitum infection was significantly associated with increased survival (adjusted OR 0.18, 95% CI (0.08–0.45), p value 0.012). Conclusions Current evidence shows the emerging role of rapid-grower NTM in CNS disease. Male gender and HIV positivity were associated with significant mortality, while M fortuitum carries favourable outcomes

Celiac disease with Budd–Chiari syndrome: A rare association

Budd–Chiari syndrome is characterized by hepatic venous outflow tract obstruction. We describe an 18-year-old female of known celiac disease presented with progressive abdomen distention and shortness of breath for the last 1 month. Computed tomography of abdomen revealed hepatic vein obstruction. The patient was diagnosed with Budd–Chiari syndrome. Coagulation profile showed an increased homocysteine level. Serum folate level was also decreased. The patient was put on oral anticoagulant with a gluten-free diet. After 4 weeks, the patient showed significant improvement with decreased ascites. The association of Budd–Chiari syndrome with Celiac disease has not yet been fully understood. There have been few reports that described this rare association. Budd–Chiari syndrome should be considered as an important differential in a patient with unexplained ascites and celiac disease

Hypercalcemic Encephalopathy as an Initial Presentation of Multiple Myeloma

We report the case of an 84-year-old female presented to us with acute onset altered sensorium. On investigation, neurological and infectious causes were ruled out. On further evaluation, her serum calcium was found elevated (15.07 gm/dl). The diagnosis of hypercalcemic encephalopathy was made with the possibility of multiple myeloma due to raised total protein and globulin levels. Serum electrophoresis, immunofixation, and bone marrow examination confirmed the diagnosis of multiple myeloma. The patient was treated with bortezomib, dexamethasone, and lenalidomide. After 1 week, she improved with normalization of serum calcium. Herein, we highlight hypercalcemia as an important cause of encephalopathy. As our report suggests, metabolic encephalopathy can be the first presentation in multiple myeloma

Additional file 1 of Epidemiology, clinical presentation, and predictors of outcome in nontuberculous mycobacterial central nervous system infection: a systematic review

Additional file 1. Supplemental files

The epidemiology, clinical presentation and treatment outcomes in CNS actinomycosis: a systematic review of reported cases

Abstract Background CNS actinomycosis is a rare chronic suppurative infection with non-specific clinical features. Diagnosis is difficult due to its similarity to malignancy, nocardiosis and other granulomatous diseases. This systematic review aimed to evaluate the epidemiology, clinical characteristics, diagnostic modalities and treatment outcomes in CNS actinomycosis. Methods The major electronic databases (PubMed, Google Scholar, and Scopus) were searched for the literature review by using distinct keywords: "CNS" or "intracranial" or "brain abscess" or "meningitis" OR "spinal" OR "epidural abscess" and "actinomycosis." All cases with CNS actinomycosis reported between January 1988 to March 2022 were included. Results A total of 118 cases of CNS disease were included in the final analysis. The mean age of patients was 44 years, and a significant proportion was male (57%). Actinomycosis israelii was the most prevalent species (41.5%), followed by Actinomyces meyeri (22.6%). Disseminated disease was found in 19.5% of cases. Most commonly involved extra-CNS organs are lung (10.2%) and abdomen (5.1%). Brain abscess (55%) followed by leptomeningeal enhancement (22%) were the most common neuroimaging findings. Culture positivity was found in nearly half of the cases (53.4%). The overall case-fatality rate was 11%. Neurological sequelae were present in 22% of the patients. On multivariate analysis, patients who underwent surgery with antimicrobials had better survival (adjusted OR 0.14, 95% CI 0.04–0.28, p value 0.039) compared to those treated with antimicrobials alone. Conclusion CNS actinomycosis carries significant morbidity and mortality despite its indolent nature. Early aggressive surgery, along with prolonged antimicrobial treatment is vital to improve outcomes

Pulmonary Nocardiosis with Aspergillosis in a patient with COPD: A rare co-infection

Pulmonary Nocardiosis and invasive Aspergillosis are well documented in immunocompromised patients. The coexistence of both infections is a diagnostic rarity, especially in patients with underlying structural lung diseases. We describe this rare association in a 46-year-old female with a history of pulmonary tuberculosis and COPD. The diagnosis of pulmonary Nocardiosis is challenging due to non-specific clinical features, inherent ability to mimic malignancy, tuberculosis and difficulty in the cultivation of the organism. The treating physicians should aware of the rare occurrence of such co-infections in order to prevent misdiagnosis and prompt treatment