Significant Clinical Improvement Research Articles

Case Report: Acquired hemophilia A following COVID-19 vaccine

Background: In December 2019, severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) was identified and has subsequently been responsible for immense morbidity and mortality. This resulted in the development of vaccines in record time, potentially posing safety issues. Several cases of acquired hemophilia A (AHA) following coronavirus disease 2019 (COVID-19) vaccines have been described in the literature. AHA is a rare autoimmune disease due to the production of IgG autoantibodies to coagulation FVIII that burdens high morbidity and mortality. It is presumed mainly on identifying an increased activated partial thromboplastin time (aPTT), even without bleeding and is confirmed by determining a FVIII inhibitor. To the best of our knowledge, this is the first report of AHA after inactivated virus COVID-19 treated with low dose rituximab (RTX). Lower RTX doses may lead to shorter infusion duration, lower risk of adverse events and lower costs. Case presentation: We report a case of AHA occurring in a 69-year-old man 60 days after an inactivated Sinovac-coronavac COVID-19 vaccine. Diagnosis of AHA was based on clinical presentation and confirmed with prolonged activated partial thromboplastin time, Factor VIII deficiency and the presence of an FVIII inhibitor. Follow-up showed a significant clinical and biological improvement, aPTT was normalized as of 18 days of treatment with corticosteroids along with low dose rituximab. Conclusions: In the literature, the reported cases of AHA following COVID-19 vaccination occurred after Adenoviral Vector DNA- and SARS-CoV-2 mRNA-based COVID-19 vaccines. Here, AHA was presented following inactivated virus COVID-19 vaccine. AHA treated with low dose rituximab can limit occurrence of side effects and the cost of treatment.

Changes in cerebral cortex activity during a simple motor task after MRgFUS treatment in patients affected by essential tremor and Parkinson's disease: a pilot study using functional NIRS

Objective. Magnetic resonance imaging-guided focused ultrasound surgery (MRgFUS) is a non-invasive thermal ablation method that involves high-intensity focused ultrasound surgery (FUS) and Magnetic Resonance Imaging for anatomical imaging and real-time thermal mapping. This technique is widely employed for the treatment of patients affected by essential tremor (ET) and Parkinson’s disease (PD). In the current study, functional near-infrared spectroscopy (fNIRS) was used to highlight hemodynamics changes in cerebral cortex activity, during a simple hand motor task, i.e. unimanual left and right finger-tapping, in ET and PD patients. Approach. All patients were evaluated before, one week and one month after MRgFUS treatment. Main results. fNIRS revealed cerebral hemodynamic changes one week and one month after MRgFUS treatment, especially in the ET group, that showed a significant clinical improvement in tremor clinical scores. Significance. To our knowledge, our study is the first that showed the use of fNIRS system to measure the cortical activity changes following unilateral ventral intermediate nucleus thalamotomy after MRgFUS treatment. Our findings showed that therapeutic MRgFUS promoted the remodeling of neuronal networks and changes in cortical activity in association with symptomatic improvements.

A Novel 3D Tunneling Surgical Technique (3DT) for the Treatment of Gingival Recessions with Reconstruction of the Deficient Interdental Papilla and Interproximal Attachment Regeneration: Case Series.

Current concepts in periodontology emphasize an important distinguishing aspect of the interproximal attachment, assigning its major influential significance as a key diagnostic and prognostic factor. A complete regeneration of the interproximal clinical attachment has become a primary determinant of the periodontal success. Since mid - 20th century have been published numerous articles on root coverage procedures but mostly for non-proximal gingival recessions. When it comes to the interdental area, literature does not inspire the same level of confidence. This study is aimed to introduce an innovative 3D - Tunneling Surgical Technique for the gingival papilla reconstruction and supra-alveolar interproximal attachment regeneration. The technique is described step by step and shown on three selected clinical cases of multiple bilateral adjacent gingival recessions type 2 and 3 in the lower anterior area with follow - up results of up to 6.5 years. A total of 18 proximal, mid-buccal and mid-lingual recessions were treated simultaneously. Mean root coverage up to 88.9% has been achieved in the proximal area. Within its limits, this study demonstrates a possibility of treating gingival recessions with deficient papilla in the mandibular anterior region, achieving a significant clinical improvement with the long-term stability.

Evaluating the clinical efficacy of pulsed dye laser with sirolimus for treatment of capillary malformations: A systematic review.

Port-wine stains (PWS) are capillary vascular anomalies that are often treated with pulsed-dye laser (PDL). Revascularization limits persistent clearance; however, the anti-angiogenic effects of sirolimus (SIRO) may inhibit revascularization. This review aims to determine differences in PWS outcomes when treated with PDL monotherapy or in combination with SIRO. A systematic review was conducted using PubMed, Cochrane, and Embase databases. The following search terms were used: 'port wine stain PDL SIRO', 'port wine stain PDL', and 'port wine stain PDL and topical treatment' with (MeSH) and (Title/Abstract) limits. The search was limited to the English language and human-subject studies conducted between 1 January 2000 and 1 June 2023. Inclusion criteria included studies evaluating SIRO as an adjunct to PDL in patients with PWS. Data extraction and quality assessment were performed by two independent reviewers. A total of nine studies met the inclusion criteria, which included randomized controlled trials (3), case series (2), case reports (3), and a prospective intrapatient study (1), which represented a total of 58 patients. Five studies showed improvement of a measured post-treatment PDL parameter including shortening treatment time and less frequent dosing. A subset of studies (4/9) which did not demonstrate significant clinical improvements exhibited significant photographic evidence of improvement. Heterogeneity among the studies highlights the need for further research and standardization. While adjunctive SIRO shows promise, larger studies and comprehensive evaluation methods are required to establish conclusive safety and efficacy guidelines to shape clinical decision-making.

Is Ultrasound-Guided Percutaneous Nephrostomy Efficacious and Safe in Pregnant Patients?: A Single Center Experience

Aim: To evaluate the safety and efficacy of ultrasound-guided percutaneous nephrostomy (PCN) placement in pregnancy. Method: Medical records of 26 pregnant patients who were inserted with a total of 27 PCN catheters between June 2020 to June 2023 were retrospectively analyzed to assess imaging findings, procedural parameters, technical success, delivery outcomes, and procedural complications. Results: At the time of placement of the PCN, the mean gestational age was 29.04±7.01 weeks (range 7 - 36 weeks). The indications for insertion of the PCN catheter included: 1) obstructing calculi (46.2%), 2) pyelonephritis (34.6%), and 3) symptomatic hydronephrosis without calculi (19.2%). Successful catheter placement was achieved in 25/27 procedures, yielding a success rate of 92.59%, and no major complications. Minor instances of transient hematuria were encountered in three cases. The mean duration of catheter retention was 72.58 days. All patients exhibited significant clinical and symptomatic improvement. Among these patients, 22 patients (84.6%) delivered their babies at full-term, 3 patients (11.5%) delivered prematurely, and 1 patient (3.8%) experienced a miscarriage. Conclusion: Ultrasound-guided PCN placement is a reliable method with high success and efficacy that can be applied during pregnancy. This procedure avoids the use of ionizing radiation, ensuring safety for both the fetus and the mother.

Pelvic AVM Embolization: A Tricky Affair-Multicenter Retrospective Experience.

Pelvic arteriovenous malformations (pAVMs) are congenital or acquired vascular anomalies, presenting with hematuria, menometrorrhagia, pelvic pain, and varices; they can be life-threatening in case of rupture. Surgical therapies have been proposed but endovascular embolization has been recognized as the primary modality. The aim of this article was to report a retrospective multicenter experience concerning embolization of pelvic AVMs and provide literature overview. We describe 18 patients (14 female and 4 male) diagnosed with pAVM and treated with minimally invasive methods. The pre-procedural imaging evaluation was based on transpelvic and/or transvaginal color Doppler ultrasound, contrast-enhanced computed tomography, and/or magnetic resonance. In 3 cases, the malformation was congenital and in other 15, acquired. Most common symptoms were menometrorrhagia, hematuria, pelvic pain and pressure, and heaviness in the lower abdominal region. In 10 cases (56%), only 1 procedure was required. Eight patients underwent multistage treatment. Complete occlusion of the lesion in post-procedural angiography was observed in 12 patients (67%). No major periprocedural complications were observed. In 14 cases (78%), both satisfactory embolization and significant clinical improvement was achieved in long-term follow-up. Sixteen patients (88%) were at least satisfied with the clinical outcome. One patient reported subsequent successful pregnancy 5 years after the treatment. Hemodynamics of pAVM are variable and thorough understanding of the vessel anatomy is crucial in planning and choosing proper treatment. Both transarterial and percutaneous direct puncture embolization strategies appear safe, technically feasible, and clinically effective. In this manuscript, we discuss the role of interventional radiology methods in the treatment of pelvic arteriovenous malformations along with its advantages, limitations and possible complications. In addition to this, we review the current literature and confront our findings with those made by other authors. We believe that modern endovascular methods offer safe and reliable alternative for traditional surgical therapy and should be therefore considered during multidisciplinary treatment of these patients.

Collinsella aerofaciens as a predictive marker of response to probiotic treatment in non-constipated irritable bowel syndrome

ABSTRACT Probiotics are exploited for adjuvant treatment in IBS, but reliable guidance for selecting the appropriate probiotic to adopt for different forms of IBS is lacking. We aimed to identify markers for recognizing non-constipated (NC) IBS patients that may show significant clinical improvements upon treatment with the probiotic strain Lacticaseibacillus paracasei DG (LDG). To this purpose, we performed a post-hoc analysis of samples collected during a multicenter, double-blind, parallel-group, placebo-controlled trial in which NC-IBS patients were randomized to receive at least 24 billion CFU LDG or placebo capsules b.i.d. for 12 weeks. The primary clinical endpoint was the composite response based on improved abdominal pain and fecal type. The fecal microbiome and serum markers of intestinal (PV1 and zonulin), liver, and kidney functions were investigated. We found that responders (R) in the probiotic arm (25%) differed from non-responders (NR) based on the abundance of 18 bacterial taxa, including the families Coriobacteriaceae, Dorea spp. and Collinsella aerofaciens, which were overrepresented in R patients. These taxa also distinguished R (but not NR) patients from healthy controls. Probiotic intervention significantly reduced the abundance of these bacteria in R, but not in NR. Analogous results emerged for C. aerofaciens from the analysis of data from a previous trial on IBS with the same probiotic. Finally, C. aerofaciens was positively correlated with the plasmalemmal vesicle associated protein-1 (PV-1) and the markers of liver function. In conclusion, LDG is effective on NC-IBS patients with NC-IBS with a greater abundance of potential pathobionts. Among these, C. aerofaciens has emerged as a potential predictor of probiotic efficacy.

Fusion with and without lever reduction in degenerative lumbar spondylolisthesis: a retrospective study.

The reduction of slipped vertebra is often performed during surgery for degenerative lumbar spondylolisthesis (DLS). This approach, while potentially improving clinical and radiological outcomes, also carries a risk of increased complications due to the reduction process. To address this, we introduced an innovative lever reduction technique for DLS treatment. This study aims to investigate the clinical efficacy, radiological outcomes, and complications of fusion with or without lever reduction. We conducted a retrospective review of prospectively collected data from a registry of patients who underwent lumbar fusion surgery for DLS, with a follow-up of at least 24months. Self-reported measures included visual analog scale (VAS) for back or leg pain, Oswestry Disability Index (ODI), and the achievement of minimal clinically important difference (MCID). Radiological assessments encompassed spondylolisthesis percentage (SP), focal lordosis (FL), and lumbar lordosis (LL). Complications were categorized using the modified Clavien-Dindo classification (MCDC) scheme. Patients were assigned to the reduction group (RG) and non-reduction group (NRG) based on the application of the lever reduction technique. Clinical and radiological outcomes at baseline, immediately after surgery, and at the last follow-up were compared. A total of 281 patients were analyzed (123 NRG, 158 RG). Baseline patient demographics, comorbidities, and surgical characteristics were similarly distributed between groups except for operating time (NRG 129.25min, RG 138.04min, P = .009). Both groups exhibited significant clinical improvement after surgery (all, P = .000), with no substantial difference between groups (VAS, ODI, or the ability to reach MCID). Patients in RG showed statistically lower SP and higher FL during follow-up (all, P = .000). LL was comparable at different time points within each group or at the same time point between the two groups (all, P > .050). The overall complication rate (NRG 38.2%, RG 27.2%, P = .050) or specific complication rates per MCDC were similar between groups (all, P > .050). Patients in RG were predisposed to a lower risk of adjacent segment degeneration (ASDeg) (NRG 9.8%, RG 6.3%, P = .035). There were no significant differences in postoperative measures such as VAS scores for back and leg pain, ODI, the ability to reach MCID, overall complication rate, or specific complication rates per MCDC between surgical approaches. However, fusion with lever reduction demonstrated a notable advantage in restoring segmental spinal sagittal alignment and reducing the occurrence of ASDeg compared to in situ fusion.

Therapeutic effects of extracorporeal shock wave therapy on patients with spastic cerebral palsy and Rett syndrome: clinical and ultrasonographic findings

BackgroundExtracorporeal shock wave therapy (ESWT) is reportedly effective for improving spasticity and motor function in children with cerebral palsy (CP). Because late-stage Rett syndrome has a similar presentation, this study aimed to investigate the effects of ESWT on these two diseases.Material and MethodsPatients diagnosed with spastic CP and Rett syndrome received 1500 impulses of ESWT at 4 Hz and 0.1 mJ/mm2, on their spastic legsonce weekly for a total of 12 weeks. Outcomes were assessed before and 4 and 12 weeks after ESWT. Clinical assessments included the Modified Ashworth Scale (MAS), passive range of motion (PROM), and Gross Motor Function Measure 88 (GMFM-88). Ultrasonographic assessments included muscle thickness, acoustic radiation force impulse (ARFI), and strain elastography.ResultsFifteen patients with CP and six with Rett syndrome were enrolled in this study. After ESWT, patients with CP showed significant clinical improvement in the MAS (P = 0.011), ankle PROM (P = 0.002), walking/running/jumping function (P = 0.003), and total function (P < 0.001) of the GMFM-88. The patients with Rett syndrome showed improved MAS scores (P = 0.061) and significantly improved total gross motor function (P = 0.030). Under ARFI, patients with CP demonstrated decreased shear wave speed in the gastrocnemius medial head (P = 0.038). Conversely, patients with Rett syndrome show increased shear-wave speeds after ESWT.ConclusionOur study provides evidence that a weekly course of low-dose ESWT for 12 weeks is beneficial for children with both CP and Rett syndrome, with the clinical effects of reducing spasticity and improving the gross motor function of the lower limbs. The ARFI sonoelastography reveals improvement of muscle stiffness in patients with CP after ESWT, but deteriorated in patients with Rett syndrome. The diverse therapeutic response to ESWT may be caused by the MECP2 mutation in Rett syndrome, having a continuous impact and driving the pathophysiology differently as compared to CP, which is secondary to a static insult.Trial registration IRB 201700462A3. Registered 22March 2017, https://cghhrpms.cgmh.org.tw/HRPMS/Default.aspx.

Doença da arranhadura do Gato - relato de caso

INTRODUCTION: Cat Scratch Disease (CSD) presents most commonly with fever and lymphadenopathy, but up to 25% of cases develop systemic manifestations. OBJECTIVE: to report a pediatric case of hepatosplenic form of CSD. METODOLOGY: this is a case report of a nine-year-old child hospitalized from October to November 2019. The report was written based on medical records and literature review, through Pubmed platform. Informed consent was given by the legal sponsor and the assent by the child. RESULT: a nine-year-old girl presents with swollen, tender and erythematous nodes in the right popliteal fossa and fever. Initial suspicion was of cellulitis, complicated with a popliteal abscess. Treatment was initiated with oxacillin for 3 days and later on with clindamycin and ceftriaxone for 11 days. On the eleventh day of hospital stay, the patient manifested hepatosplenic abscesses and antibiotic treatment was upscaled to vancomycin and piperacillin-tazobactam until she was diagnosed with a hepatosplenic form of CSD, which happened seventeen days after admission. Therefore, treatment was provided with Rifampicin and Clarithromycin for 14 days, with significant clinical improvement. CONCLUSION: this report aims to raise awareness regarding the wide clinical spectrum of this infection and how to better manage it.

TMR for Peripheral Sensory Nerve Neuroma around the Wrist Utilizing the Distal Anterior Interosseous Nerve.

Injury to the peripheral sensory nerves of the hand and wrist is common and can lead to debilitating neuromas and significantly impair patients' quality of life. Target-muscle reinnervation (TMR) is a novel method for treating neuromas that can result in significant clinical improvement. However, TMR for the peripheral sensory nerves in the hand and wrist is restricted by the limited options for motor branches. The adaptability of the anterior interosseous nerve (AIN) as a target for TMR treating peripheral sensory neuroma has not been thoroughly investigated or implemented therapeutically. This study aimed to evaluate the use of AIN as a viable recipient of TMR for treating peripheral sensory neuromas around the wrist. In this retrospective study, eight patients were included over 18 months from June 2021 to January 2023 at Hamad Medical Corporation. The average follow-up time after TMR was 13 months. The peripheral sensory nerves involved were the radial sensory nerve in five cases, the palmar cutaneous branch of the median nerve in one case, and the median nerve in one case. The preoperative average VAS pain score was 7 of 10 compared with the postoperative pain score of 2 of 10. In conclusion, the AIN can be used as a first-choice motor target for all peripheral sensory neuromas around the wrist for the following reasons: first, it can be reached by the peripheral sensory nerves around the wrist; second, the pronator quadratus muscle is expandable; and third, the AIN can be taken with a long proximal tail for flexible coaptation with the peripheral sensory nerves.

Neuroblastoma estágio 4S e hepatomegalia maciça: relato de caso

Objective: To report a case of stage 4S neuroblastoma and massive hepatomegaly that worsened the disease prognosis. Case Description: A 3-month-old male infant presented with 7 days of worsening abdominal distension, significant sweating, incessant crying, and irritability. His abdomen was globular and exhibited massive hepatomegaly. Imaging examinations detected multiple nodules in the liver parenchyma and a hypoechoic nodule in the right adrenal gland. The lesions were biopsied, with findings revealing infiltrative neuroblastoma in the liver tissue. The patient was diagnosed with International Neuroblastoma Staging System stage 4S, and treatment was started according to the International Society of Paediatric Oncology - Paediatric Oncology in Developing Countries protocol. During the first chemotherapy cycle, his condition required prolonged mechanical ventilation and hemodialysis. It was then decided to switch to the POG 9341-Modified regimen in an emergency mode. A significant clinical improvement response was observed after the third chemotherapy cycle. Subsequent cycles were administered based on the initial proposed protocol. Complete remission was achieved after the eighth cycle. Comment: Stage 4S neuroblastoma usually has a good prognosis; however, when associated with massive hepatomegaly, the prognosis becomes unfavorable. Complications during the first chemotherapy cycle required a change in management to the POG 9341-Modified regimen, which resulted in a reduction in the number and size of hepatic and adrenal nodules. Owing to significant clinical improvement, the remaining cycles were performed using the initial protocol. Despite the uncommon association and the lack of consensus for a specific treatment, complete remission was achieved by combining therapeutic regimens.

Bee Sting-induced Nephrotic Syndrome in a Child: A Case Report with Review of Literature

Abstract Background: Nephrotic syndrome (NS) is common in children, and it is usually idiopathic. Bee sting-induced NS in children is rarely reported. Clinical Description: A 9-year-old female child presented with a complaint of generalized edema for 2 days which started to develop 4 days after being stung on the dorsum of her right foot by a bee. She had pedal edema, facial puffiness, and abdominal distension. Management and Outcome: Laboratory investigations showed proteinuria, low serum protein, elevated serum cholesterol, serum triglycerides, and immunoglobulin E levels. A diagnosis of NS secondary to bee sting was made. The patient was treated with supportive measures for edema. There was a significant clinical and biochemical improvement by 10 days. Conclusion: This case reiterates the association between allergen exposure and NS, which is hardly reported in children. Such a phenomenon may be transient with requirement of only supportive therapy for symptom relief.

Modified triple pelvic osteotomy for residual acetabular dysplasia through double incisions: Technical note and review of short-term results.

To assess validity, safety, and efficacy of the modified triple pelvic osteotomy (TPO) approach for correction of residual acetabular dysplasia. This is a retrospective case series conducted on 15 hips in 15 patients from 2019 to 2023 with residual acetabular dysplasia treated by modified TPO as described by Tonnis with two modifications. The first modification is using a single medial incision for pubic and ischial cuts (the Vladimirov modification). The second modification is having the ischial cut closer to the acetabulum (Li modification) allowing free movement of the acetabular fragment for better femoral head coverage. The mean age at the time of surgery was 11.85years, (range 8-23). Cases presenting were 10 males (66.7%) and 5 females (33.3%). The mean follow-up period was 36.533 months (24-60 months). Our study revealed significant clinical and radiological improvement. The CE angle improved from a mean value of 10° (range 2-17) pre-operatively to 32.785° (range 18°-40°) post-operatively. The AI improved from a mean value of 32° pre-operatively to a mean value of 13.89° post-operatively. HHS increased from a preoperative mean value of 74.80° to a post-operative mean value of 90.67°. Also, there was a significant improvement in ROM (abduction and internal rotation). LLD improved from a mean value of 2.60cm preoperatively to a mean value of 0.37cm postoperatively. Delayed union was found in 3 cases. No cases of osteonecrosis or neurovascular complication were encountered in our study. The modified TPO technique using dual incisions can be considered safe and effective, providing adequate coverage of the femoral head in acetabular dysplasia with less surgical time, satisfactory functional outcomes, and minimal complications. IV.

A Young Farmer with Multi-Organ Failure: A Case Report on Weil’s Disease in Bangladesh

Weil’s disease is a zoonotic infection caused by organism of Leptospira icterohaemorrhagiae, having a very high mortality. This article reports a case of a young male farmer who presented to a tertiary-level hospital in north-eastern Bangladesh with severe fatigue, fever, abdominal pain and yellow discoloration of the whole body. Meticulous history taking revealed that he worked bare-footed in an animal farm and had a cut injury in his right foot 15 days back. On the basis of this piece of information, serological tests for leptospirosis were done that showed positive results. There was significant clinical and biochemical improvement after treatment. This case report revealed that a patient with fever, hepatitis and, pancreatitis with a background history of suggestive exposure, leptospirosis should be considered.

Change in Neuropsychological Test Performance Seen in a Longitudinal Study of Patients With Post-acute Sequelae of COVID-19: A 6-Month Follow-up Study

BackgroundPost-acute sequelae of COVID-19 (PASC) may include physical, psychiatric, and neurocognitive symptoms. Few studies of cognitive symptoms have been longitudinal, with many following participants briefly after infection, relying on subjective complaints, screening instruments, or computerized testing. This group previously reported diminished neuropsychological (NP) test performance in over half of 60 individuals tested in-person 7 months post-COVID-19, particularly those seeking care for cognitive complaints. The current study describes the intial and 6-month follow-up results of an expanded cohort of 75 participants. MethodsParticipants underwent NP, psychiatric, and medical assessments approximately 7 months after acute COVID-19 infection. Sixty-three (84%) returned approximately 6 months later for repeat evaluation. ResultsAt the initial visit, 29 (38.7%) met criteria for low NP performance and 16 (21.3%) met criteria for extremely low NP performance. At 6-month follow-up, several NP domains which were significantly below normative values at the initial visit were no longer abnormal, with the exception of language. Only measures of delayed memory and fatigue showed significant improvements between the two time points. Discussion/ConclusionA substantial proportion of individuals recovered from acute COVID-19 infection have persistent neuropsychiatric symptoms over 1 year after infection. While the overall sample in this study showed some improvement in NP test performance relative to norms, only fatigue and delayed memory improved significantly between times one and two. No individual declined in NP test performance, though relatively few individuals made significant clinical improvement, indicating the need for serial neuropsychiatric assessment and treatment supports. Longitudinal follow-up of this cohort is in progress.

Adult Onset Acute Disseminated Encephalomyelitis (ADEM): A Comprehensive Case Repot

INTRODUCTION: Acute disseminated encephalomyelitis (ADEM) is a rare immune-mediated condition affecting the central nervous system, characterized by acute encephalopathy and multifocal neurological deficits. This disorder predominantly impacts children but here, we document a rare adult case, emphasizing the necessity for considering organic causes in psychiatric symptomatology [1 ; 2]. AIMS / OBJECTIVES: A detailed review of literature was conducted through PubMed focusing on the atypical presentations of ADEM and the incidence of organic diseases manifesting psychiatric complaints. This approach informed our diagnostic strategy and management of an unusual adult case of ADEM, underscoring the importance of maintaining a wide differential diagnosis in emergency medicine [3 ; 4] CASE DESCRIPTION: A 22-year-old female presented with generalized weakness, confusion, apathy, and reduced appetite, following an upper respiratory tract infection and a distressing dental visit. Her neurological examination revealed dysarthria, dysmetria, and ataxia, with a significant reduction in motor strength and generalized hyperreflexia. Laboratory tests were largely unremarkable except for elevated ESR and CRP. MRI findings were consistent with a demyelinating process, See Figure 1A and 1B, fulfilling the criteria for ADEM diagnosis [5 ; 6]. RESULTS: Results: Initiation of a steroid regimen led to significant clinical improvement, and by day 11 of hospitalization, there was a marked improvement in the patient&amp;apos;s condition, enabling discharge with outpatient neurology and ophthalmology follow-ups. A six-week steroid taper was prescribed. Follow-up MRI confirmed resolution of the demyelinating lesions, confirming the effectiveness of the treatment protocol [7 ; 8]. DISCUSSION: This case underscores the complexity of diagnosing ADEM in adults, particularly when presenting with psychiatric symptoms. It highlights the critical importance of comprehensive evaluation in emergency settings to identify rare organic causes of psychiatric presentations. This report reinforces the need for awareness among clinicians to consider ADEM in differential diagnoses, ensuring timely and appropriate management for affected patients [9; 10; 11]. REFERENCES: 1. [Pohl D, Alper G, Van Haren K, Kornberg AJ, Lucchinetti CF, Tenembaum S, Belman AL. Acute disseminated encephalomyelitis: Updates on an inflammatory CNS syndrome. Neurology. 2016 Aug 30;87(9 Suppl 2):S38-45. doi 10.1212/WNL.0000000000002825. PMID: 27572859.] 2. [De Fino C, Nociti V, Modoni A, Bizzarro A, Mirabella M. An atypical case of acute disseminated encephalomyelitis associated with cytomegalovirus infection. Mult Scler Relat Disord. 2016 Jan;5:70-2. doi: 10.1016/j.msard.2015.11.003. Epub 2015 Nov 10. PMID: 26856946.] 3. [Kamel MG, Nam NT, Han NHB, El-Shabouny AE, Makram AM, Abd-Elhay FA, Dang TN, Hieu NLT, Huong VTQ, Tung TH, Hirayama K, Huy NT. Post-dengue acute disseminated encephalomyelitis: A case report and meta-analysis. PLoS Negl Trop Dis. 2017 Jun 30;11(6):e0005715. doi: 10.1371/journal.pntd.0005715. PMID: 28665957; PMCID: PMC5509372.] 4. [Obermeier PE, Karsch K, Hoppe C, Seeber L, Schneider J, Muhlhans S, Chen X, Tief F, Kaindl AM, Weschke B, Bottcher S, Diedrich S, Rath B. Acute Disseminated Encephalomyelitis After Human Parechovirus Infection. Pediatr Infect Dis J. 2016 Jan;35(1):35-8. doi: 10.1097/INF.0000000000000928. PMID: 26741581.] 5. [Neeki MM, Au C, Richard A, Peace C, Jaques S, Johansson J. Acute Disseminated Encephalomyelitis in an Incarcerated Adolescent Presents as Acute Psychosis: Case Report and Literature Review. Pediatr Emerg Care. 2019 Feb;35(2):e22-e25. doi: 10.1097/PEC.0000000000000919. PMID: 27749808.] 6. [Chaudhry LA, Babur W, Chaudhry GA, Al-Atawi FE, Robert AA. Acute disseminated encephalomyelitis: a call to the clinicians for keeping this rare condition on clinical radar. Pan Afr Med J. 2018 M

Vemurafenib combined with chemotherapy achieved sustained remission in pediatric LCH: a multi-center observational study

BackgroundLangerhans cell histiocytosis (LCH) is a myeloid neoplasia with potentially fatal consequences, and about 2/3 of cases involve the BRAFV600E kinase-activated mutation. Vemurafenib, a BRAF inhibitor, has demonstrated significant clinical improvements in LCH. However, the high relapse rate of LCH following cessation of vemurafenib therapy remains a major challenge, and alternative treatment strategies require further investigation.MethodsIn this retrospective multi-center study, we evaluated the efficacy and safety of vemurafenib combined with conventional chemotherapy in patients with severe or refractory LCH.ResultsSeventeen patients were enrolled in the study, with eleven classified as risk organ involvement (RO +). Six received the combination therapy as the primary treatment, and eleven after being refractory to prior chemotherapy. The overall response rate was 94.1%. Progression-free survival among all 17 patients was 70.6% (12/17) at a median follow-up of 32 months, and relapse-free survival among the 15 patients with discontinuation after a response was 73.3%(11/15) at a median follow-up of 34 months. Five of six patients (83.3%) with myeloid BRAFV600E mutations demonstrated molecular remission. The overall survival rate was 100%. Adverse events were mostly classified as grades 1 or 2.ConclusionOur data suggest that the combination of vemurafenib and chemotherapy can achieve sustained clinical and molecular level relief in children with LCH, and side effects are tolerable.

Extensive Deep Vein Thrombosis: A Rare Manifestation of Disseminated Tuberculosis

A 20-Year-Old Male With Disseminated Tuberculosis (Pulmonary, Pleural, And Lymph Node TB) Presented With Unusual Manifestation In The Form Of Extensive Deep Vein Thrombosis (DVT) Involving Bilateral Popliteal Veins, Femoral Veins, Iliac Veins, And Infra-Hepatic Inferior Vena Cava (IVC). Upon Follow-Up With The Patient After Two Months Of Therapy, There Was A Significant Clinical Improvement, And Edema And Tenderness Of The Lower Limb Had Decreased.

Primary central nervous system lymphoma with initial presentation of cerebral salt-wasting syndrome shifting to syndrome of inappropriate antidiuretic hormone secretion: A case report

Background. Primary central nervous system lymphoma (PCNSL) is a rare form of non-Hodgkin lymphoma which may affect brain parenchyma. Hyponatremia can be present as one of the complications, either due to neuronal injury arising from in-situ tumor and/or surgical resection (resulting in cerebral salt wasting syndrome/CSWS) or indolent, slowly progressing and often asymptomatic one as a consequence of ongoing chronic illness (so-called syndrome of inappropriate antidiuretic hormone/SIADH). Differentiating between the two is critical, since each diagnosis has a starkly different treatment approach which may exacerbate one another. Case presentation. Herein, we reported a case of a 48-year-old immunocompetent male who presented with diplopia, hallucination, imbalance, and dizziness, later to be diagnosed with PCNSL on multiple brain regions. He suffered from symptomatic hyponatremia post-surgically as a result of CSWS which in turn responding well to intensive dose of desmopressin acetate and fluid replenishment. However, as the disease progressed, hyponatremia also relapsed but was refractory to the same treatment approach. After subsequent investigations, patient was known to have SIADH. Treatments tailored to SIADH including fluid restriction and administration of hypertonic saline as well as tolvaptan lead to stabilization of sodium levels and significant clinical improvements. Conclusion. Post-operative hyponatremia in the setting of a PCNSL continues to be a complex issue. It underscores the importance of careful clinical assessment, monitoring, and the need for flexibility in diagnosis and management strategies. Furthermore, it highlights the challenges of distinguishing between CSWS and SIADH, two conditions with similar laboratory findings but divergent management approaches. Recognizing the subtleties in clinical presentation and responding appropriately can greatly influence patient outcomes. The rising incidence of PCNSL in immunocompetent individuals also needs a heightened awareness and understanding of its presentations and potential post-operative complications among clinicians.