Clinical Reasoning: Longitudinally Extensive Spinal Cord Lesions in a Middle-aged Man

An immunocompetent 47-year-old man presented with a five-month history of progressive lower limb weakness, back pain, sphincter dysfunction, and intermittent fever, suggesting myelopathy in a chronic deteriorating course. A comprehensive analysis comprising of blood tests, neuroimaging, CSF profiling, molecular analysis, and histopathology was performed. Notably, enhanced spinal cord MRI revealed longitudinally extensive intradural-extramedullary lesions involving the cervical, thoracic, and lumbosacral spinal cord, with homogeneous enhancement and spinal cord compression. Serum TPHA and RPR tests were positive. CSF profiling showed pleocytosis, significant protein elevations, hypoglycorrhachia, and positive TPHA test. 18F-FDG-PET/CT indicated slightly increased intraspinal FDG uptake. Spinal cord biopsy further showed small round blue cells in poorly differentiated tissues. Immunostaining was positive for NKX2.2, CD56, CD99, Synaptophysin, and Ki67 (50%). Molecular analysis detected a novel MALAT-CYSLTR1 fusion protein and variants in oncogenic genes including PTCH1, TERT, CREBBP, SPEN, and STK11. The diagnosis of intraspinal extraosseous Ewing’s sarcoma (ES) was confirmed. Briefly, our case details the diagnosis of a patient with intradural-extramedullary ES and highlights the value of spinal cord biopsy in progressive myelopathy of unknown causes.

Case Report on Guillain-Barre Syndrome (GBS) in Adult

Introduction: Guillain-Barre-syndrome is when the immune system attacks the peripheral nervous system were disease progresses to trembling and muscle weakness in both hands and legs, which progress to upper body and arms. Clinical Findings: High grade intermittent fever, low back pain, B/L LL weak, urinary incontinence which is intermittent in nature. Diagnostic Evaluation: Neurological examination- revealed B/L UL and LL weakness, acute onset of quadriparesis. X-Ray – revealed normal sinus rhythm. CSF examination – revealed No RBC; No pus cell; No Organism seen. Lab investigation – Hb% 10%, total RBC count 4.45, total WBC 10400, total platelet 2.33, SGOT 226 SGPT 83, Peripheral Smear RBCs - Normocytic Normochromic platelets adequate smear no Haemoparasite seen. Blood Culture: revealed Growth of Acinetobacter species. Therapeutic Intervention: Inj. Optineurone 1gm, Inj.Pantop 40mg, Tab.PCM,Inj. Tramadol 500mg, Immunoglobin (Ig) 100ml , physiotherapy and supportive therapy. Outcome: The symptoms and clinical state of the patient improved over time. The patient's weakness began to improve after 5 days of IV-Ig therapy. Conclusion: The patient was hospitalised to the neurosurgery ICU AVBRH on 05/06/20 with the known case of guillain-barre syndrome-(GBS). After receiving therapy, she showed significant progress.

Acute Disseminated Encephalomyelitis in a 2-Year-Old Patient Following COVID-19

This report presents the case of acute disseminated encephalomyelitis in a 2-year-old patient following a positive severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) test. She presented with ataxic gait, truncal ataxia, and reduced coordination following 10 days of intermittent fever and lethargy. She did not have any respiratory symptoms. Magnetic resonance imaging of the brain and spine showed widespread T2 high signal within the gray and white matters and within the spinal cord. She was treated with intravenous methylprednisolone followed by tapering oral prednisolone; this led to resolution of her neurological symptoms. This case highlights that neurological complications can occur secondary to SARS-CoV-2 infection.

Macrophage Activation Syndrome secondary to Systemic Juvenile Idiopathic Arthritis: A Case Report

Macrophage activation syndrome is a rare but a life threatening condition commonly associated with Systemic Juvenile Idiopathic Arthritis. Its clinical presentation includes fever, hepatosplenomegaly, hypertriglyceridemia, hypofibrinogenemia, hyperferritinemia and impaired liver enzymes. The symptoms are alarming yet non-specific and often lead to a delayed diagnosis. A 12 year male presented with a history of intermittent fever and was started on antibiotics but failed to respond after several days of hospital stay. After a series of investigations to rule out multiple diagnoses he was diagnosed as a case of Macrophage Activation Syndrome secondary to Systemic onset Juvenile Arthritis and was treated with steroids.

Cytomegalovirus Cholangiopathy in an Immunocompetent Patient: A Case Report and Literature Review

A 37-year-old man presented with jaundice, upper right quadrant pain, and intermittent fever with chills. Laboratory assessment showed biliary stasis, with total bilirubin of 203 µmol/L (2–20), conjugated bilirubin of 105 µmol/L, and alkaline phosphatase of 556 U/L (30–120). Markers for hepatitis A–E viruses were negative. Serology assessment for rubeola, herpes simplex virus, Epstein-Barr virus, and Toxoplasma gondii showed negative IgM antibodies. HIV serology status was negative. For cytomegalovirus, both types of antibodies (IgM and IgG) were positive, with an IgM level >300 U/mL. pp65 antigen was also detected as well as CMV DNA. Diagnostic imaging of the abdomen except the dilated common bile duct showed a normal appearance of the gallbladder, liver, pancreas, spleen, and both kidneys. To our knowledge, cytomegalovirus cholangiopathy in the absence of any other underlying disease has not been reported. Therefore, the presence of cholangiopathy in our patient is interesting from an imaging, laboratory, and clinical point of view.

263. Antibody Spill-Over vs. True Coccidioidal Meningitis in a Patient with HIV/AIDS and Disseminated Coccidioidomycosis

Background The diagnosis of coccidioidal meningitis merits life-long antifungal therapy given high rates of disease recurrence. Accurate diagnosis is important. Antibody spill-over into cerebrospinal fluid (CSF) can happen when serum titers are high. We present a case of antibody spill-over vs. true fluconazole-resistant coccidioidal meningitis. Methods A 49-year-old man presented with 6 months of intermittent fever, myalgias, decreased appetite, vomiting, diarrhea, unsteadiness and 60-pound weight loss. He was recently diagnosed with HIV and a prior lymph node biopsy had grown Coccidioides immitis (C. i) for which he was given fluconazole 100 mg twice daily. Figure 1. Timeline of Coccidioides immitis lab results in relation to treatment regimen. CF: Complement fixation, EIA: Enzyme immunoassay for Coccidioides antigen, CSF: Cerebrospinal fluid, c/mL: copies/mL, R: resistant, S: susceptible. Results Vitals revealed a temperature of 102°F. He was cachectic and a 0.5 cm right supraclavicular lymph node was palpable. No meningeal signs were appreciated. CD4 count was 50/µL (18%), HIV-1 viral load 2,969,945 copies/mL. Computed tomography (CT) of the abdomen/pelvis suggested lung and spleen involvement. Serum C. i enzyme immunoassay (EIA) was 1.38 ng/mL, immunodiffusion (ID) was positive and complement fixation (CF) titer was 1:256. C. i was isolated from expectorated sputum. CSF cell count was normal, but ID was positive and CF titer was 1:2 however, lab reported concern for spill-over due to high serum IgG titers. He left against medical advice with fluconazole 400 mg daily. He was hospitalized a month later for failure to thrive. MRI head revealed enlarged lateral and third ventricles with increased periventricular hyperintensity concerning for coccidioidal meningitis. Repeat serum studies were stable. CSF revealed CF 1:4 and C. i antigen by EIA 1.31ng/mL, distinguishing between spill-over and meningitis. Susceptibility results showed resistance to fluconazole and amphotericin B with minimum inhibitory concentrations (MICs) of 50 and 4 respectively, posaconazole susceptibility (MIC < 1) and itraconazole borderline (MIC 3.7). Despite amphotericin B resistance, it was used for bridge to posaconazole. ART was initiated after concern for immune reconstitution had resolved. Conclusion This case highlights the difficulty in making an accurate diagnosis of coccidioidal meningitis. It also describes a fluconazole-resistant C. i isolate in the setting of prolonged low-dose fluconazole therapy. Disclosures All Authors: No reported disclosures

Clinical characteristics of Kawasaki disease in adolescents

Objective Studies focusing on Kawasaki disease (KD) in adolescents are lacking in Southwest China. We systematically summarized the clinical characteristics of KD in adolescents to improve pediatricians’ recognition of this condition. Methods The clinical data of patients with adolescent-onset KD in our center were retrospectively analyzed. The patients were divided into Group A (n = 7), whose first hospitalization was at our hospital, and Group B (n = 10), who were transferred from their local hospital or community health center. Results Seventeen patients with adolescent-onset KD were identified (constituent ratio of 0.8%). Seven patients had an intermittent fever for >10 days. The incidence of incomplete KD was 52.9%. These patients had a high incidence of other atypical clinical manifestations. Fifteen patients were initially misdiagnosed with other infectious diseases. Although the incidence of typical KD was higher in Group B, the overall misdiagnosis rate at the initial stages was higher and the average fever duration on arrival and before IVIG administration were much longer in Group B than A. Conclusions KD in adolescents was frequently misdiagnosed, which might be associated with its atypical, diverse clinical features and pediatricians’ poor recognition. Pediatricians must be aware of the possibility of KD in adolescents.

Case Report: Pyogenic Arthritis, Pyoderma Gangrenosum, and Acne: A Single-Center Experience and Literature Review

ObjectivesThis study aims to describe the characteristics of patients diagnosed with pyogenic arthritis, pyoderma gangrenosum, and acne (PAPA) syndrome at a single center in China and provide an up-to-date literature review.MethodsThe clinical data and genotype of three Chinese Han patients were carefully documented and studied. We also conducted a systematic literature review on PAPA syndrome.ResultsA total of three patients were diagnosed with PAPA syndrome at our center from 2018 to 2020. Arthritis was observed in all three patients, while pyoderma gangrenosum (PG) was found in two patients and acne in one patient. Other manifestations included pathergy reaction, intermittent fever, oral ulcer, keratitis, proteinuria, and hematuria. The PSTPIP1 A230T mutation was identified in two patients, and a novel Y119C variation was revealed in a sporadic patient. A total of 76 patients with PAPA syndrome reported in 29 articles were included in our literature review. The classical triad of arthritis, PG, and acne was visible in only 16 (25.4%) patients, while 24 (38.1%) exhibited only one major symptom. Skin lesions were more commonly seen in patients with adult-onset disease than those with childhood-onset disease (100 vs. 83%), whereas arthritis was less common (50 vs. 98.1%). Steroid and/or biological agents were effective in most patients.ConclusionsThe rarity and phenotypic heterogeneity associated with PAPA syndrome make the diagnosis a huge challenge to physicians, especially in adult patients. A significant portion of patients did not exhibit the full spectrum of the classical triad. Accordingly, gene testing is critically helpful for diagnosis.

PHYTOCHEMICAL STUDIES OF STAR FRUIT AVERRHOA CARAMBOLA L.

The paper deals with a phytochemical investigation on the fruit of Averrhoa carambola L. belonging to the family Oxalidaceae. Commonly known as “star fruit”. Fruits and leaves are used widely in Ayurveda preparations to pacify impaired Kapha, pitta, skin diseases, pruritis, worm infestations, diarrhea, vomiting, hemorrhoids, intermittent fever, over-perspiration, and general debility. In the present study fruits of Averrhoa carambola L. were screened for their phytochemical constituents following hot continuous and successive extraction by Soxhlet apparatus. A qualitative assay was done using a range of solvents. The extraction process was carried using different solvents successively in the order of increasing polarity. Qualitative analysis of the extracts using standard procedures revealed the presence of alkaloids, avonoids, tannins, saponins, glycosides, steroids&terpenoids, phenols, coumarins, and phytosterols. Preliminary screening of phytochemicals is a valuable step, in the detection of the bioactive principles present in medicinal plants and subsequently, may lead to drug discovery and development.

Retained foreign needle in the thoracic spinal canal in a child: Case report

Background: The presence of retained foreign bodies in the spinal canal has been reported in the literature. They are attributed to retained pieces of medical equipment after surgery, or, following trauma, to residual bullets, glass fragments, or knife blades. Although some retained materials do not cause any neurological deficits in the short run, others may become symptomatic months later. Case Description: A 2-year-old male presented with a history of intermittent fever and mild lower extremity weakness. Notably, the original infectious workup was negative. However, a noncontrast CT scan later documented a needle-shaped foreign body in the spinal canal at the T10 level. During the T10 laminectomy, a needle (i.e. from a medical syringe) was removed, the patient remained neurologically intact. The foreign body turned out to be a medical syringe needle tip. Conclusion: A 2-year-old male presented with fevers and mild lower extremity weakness attributed to an intraspinal needle tip found utilizing CT at the T10 level. T10 laminectomy allowed for removal of a small needle tip. This shows the importance of removing retained spinal foreign bodies to avoid further/future neurological injury, and/or the potential risks/complications of foreign body migration/sequestration.