A Diagnostic Dilemma: A Case of Neurosarcoidosis Without Systemic Sarcoidosis.

Sarcoidosis is a multisystem granulomatous disorder of unknown etiology characterized by non-caseating granulomas in involved organs. Approximately 10% of patients with sarcoidosis exhibit central nervous system involvement. However, the occurrence of isolated neurosarcoidosis without concurrent systemic signs is very rare, affecting less than 1% of patients. We report a case of isolated neurosarcoidosis in a previously healthy patient who initially presented with a single episode of seizure and loss of consciousness. Brain MRI showed T2/fluid-attenuated inversion recovery (FLAIR) hyperintense extra-axial soft tissue mass over the left cerebral convexity measuring approximately 14 mm in maximum depth. Excisional biopsy of the brain mass showed chronic non-caseating granulomatous inflammation with epitheloid cells that was consistent with sarcoidosis. Treatment with high dose-steroids led to significant clinical improvement. At a two-year follow-up, there were no signs of systemic disease or recurrence of the meningeal mass. This case emphasizes the rarity of such presentation, diagnostic difficulties, and the importance of high suspicion and timely management to prevent debilitating neurologic complications.

One in a Million: A Case Report of Stiff Person Syndrome.

Stiff person syndrome (SPS) is a rare autoimmune disease caused by lack of inhibition to excitatory neurotransmitters in the central nervous system (CNS) leading to inappropriate motor unit firing. The pathophysiology is incompletely understood; however, high titers of antiglutamic acid decarboxylase antibody (anti-GAD Ab) are strongly associated with this disease. We present a 50-year-old woman with a history of ongoing gait and balance issues for 5 years with multiple negative workups. She recently had an acute exacerbation which left her bedbound, unable to move her legs or turn from side to side. After a negative workup at an outside hospital, the patient was discharged to a subacute rehabilitation facility. She then presented to our institution due to worsening of her condition and was ultimately diagnosed with SPS which was successfully treated. We review the case presentation and treatment options in the context of a severe disabling disease presentation.

Disseminated nocardiosis in an immunosuppressed patient with systemic lupus erythematosus and neuromyelitis optica spectrum disorder.

Opportunistic infections are an ongoing concern in patients with autoimmune disease who are being treated with immunosuppressive agents. Nocardiosis is an uncommon opportunistic infection which has been reported in association with immunosuppressed patients and autoimmune disease. It is challenging to diagnose and can have multisystem manifestations. Failure to diagnose and appropriately treat can result in significant mortality. We present a 49 year old woman with systemic lupus erythematosus and neuromyelitis optica spectrum disorder who was treated with mycophenolate mofetil, prednisone and recent plasmapheresis. She developed acute onset of shortness of breath and fevers and was ultimately diagnosed with disseminated nocardiosis with lung, brain and muscle abscesses.

Complementary Medicine Use, Symptom Burden and Non-Adherence in Kidney Transplant Recipients.

BACKGROUND: Complementary Medicine (CAM) is often used by patients with chronic illness and may not be disclosed to health care providers without prompting. In other populations, patients who use CAM were more likely to alter medications without discussing it with their provider. This study examined the relationship between self-reported use of CAM, attitudes toward care, and adherence to medical therapy in a population of inner-City kidney transplant recipients. METHODS: Cross-sectional observational analysis in a random convenience sample from the outpatient transplant clinic. Data were obtained via face-to-face structured closed-ended interview using validated survey instruments. RESULTS: 45% of patients reported using CAM. Of the study participants who used CAM, 39.1% reported non-adherence to immunosuppressant medications within the past three months, while among the non-CAM users, 17.9% reported non-adherence (p value=0.084). Adherence to hypoglycemic medication was significantly lower CAM users,(p=0.029). Patients who reported having somatic symptoms were more likely to use CAM. Symptom sum was significantly associated with CAM use, p=0.030, with 47.8% CAM users reporting skin problems vs. 10.7% non-CAM users,p=0.003 and 17.4% CAM users noting loss of appetite, compared to 3.5% of non-CAM users,p=0.002 In a random subgroup of 26 patients, 15% who did not use CAM reported medication side effects, while 53% of CAM users reported them, p =0.039. CONCLUSIONS: Use of CAM was common in our kidney transplant population. Patients who use CAM reported more somatic symptoms, more medication side effects and were more likely to be non-adherent to non-immunosuppressant medications. Positive response to questions about CAM use may be a surrogate marker for high symptom burden and risk of non-adherence to non-immunosuppresion medications in kidney transplant recipients.

Granulomatosis with Polyangiitis Presenting as Pyrexia of Unknown Origin, Leukocytosis, and Microangiopathic Haemolytic Anemia.

A 66-year-old woman presented to the Emergency Department with a florid sepsis-like picture, a two-week history of fever, relative hypotension with end organ ischemia (unexplained liver enzyme and troponin elevations), and nonspecific constitutional symptoms. She was initially found to have a urinary tract infection but, despite appropriate treatment, her fever persisted and her white blood cell count continued to rise. During her hospitalization the patient manifested leukocytosis to 47,000 WBC/µL, ESR 67 mm/hr (normal range 0-42 mm/hr), CRP 17.5 mg/dL (normal range 0.02-1.20 mg/dL), and microangiopathic haemolytic anemia, with declining haemoglobin and haematocrit. An infectious aetiology was not found despite extensive bacteriologic studies and radiographic imaging. The patient progressed to acute kidney injury with "active" urinary sediment and proteinuria. Kidney biopsy results and serological titres of myeloperoxidase positive perinuclear-antineutrophil cytoplasmic antibodies (MPO+ p-ANCA) led to a diagnosis of granulomatosis with polyangiitis. Immunosuppressive treatment with high dose methylprednisolone and rituximab led to resolution of the leukocytosis and return of the haemoglobin and haematocrit values toward normal without further signs of hemolysis.