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Ureaplasma spp. are a rare cause of invasive disease outside of the neonatal period. Recent studies have shown that immunosuppression with B-cell depleting therapies, such as rituximab or obinutuzumab, can increase the risk of invasive infection. We present a case in which a patient receiving B-cell depleting therapy for anti-neutrophil cytoplasmic antibody-associated vasculitis and pauci-immune nephritis was diagnosed with disseminated, invasive Ureaplasma infection. A 17-year-old female with documented B-cell depletion presented to the emergency department from the pediatric infectious disease clinic with persistent shoulder pain. Within the first month of admission, the patient developed multifocal abscesses with joint involvement and an echodensity resembling a possible vegetation, all of which were unresponsive to broad-spectrum antibiotics. A broad-spectrum polymerase chain reaction test detected Ureaplasma urealyticum in aspirated joint fluid from multiple anatomic sites. Clinical improvement occurred after initiation of combination therapy with intravenous doxycycline and levofloxacin. We describe a patient with documented iatrogenic B cell depletion presenting with a disseminated, multifocal invasive Ureaplasma infection including osteoarthritis, cellulitis, and soft tissue abscess. Following a 6-week course of levofloxacin, the patient's C-reactive protein, fever, and pain resolved without further evidence of disease. This case report highlights the importance of considering disseminated, invasive Ureaplasma spp. when evaluating B-cell depleted patients with challenging multifocal symptom complexes. In addition, this case report highlights the critical role of molecular diagnosis in identifying this fastidious pathogen.
The Enterobacter cloacae complex (ECC) includes opportunistic pathogens that can be carbapenem resistant, thus complicating treatment regimens. Here, we characterized a carbapenem- and colistin-resistant ECC O89H7 isolate expressing KPC carbapenemase. ECC O89H7 was isolated in January 2021 from an axillary swab performed during routine screening for multidrug-resistant (MDR) bacteria from a patient after 39 days of hospitalization in the intensive care unit (ICU) for severe COVID-19 pneumonia at Nini hospital (Tripoli, Lebanon). The ECC O89H7 was identified as E. roggenkampii (Er) belonging to sequence type (ST)422 and contained eight different plasmids as revealed by whole-genome sequencing (WGS). Er O89H7 was predicted to be a human pathogen (96.3%), harboring 51 virulence factors as well as genes conferring resistance to heavy metals and quaternary ammonium compounds. Er O89H7 was highly drug resistant, including resistance to carbapenems and colistin. The resistome revealed six β-lactamase genes: the chromosome-encoded bla MIR-3 cephalosporinase, bla LAP-2 and bla SHV-12 encoded on a 115-kb IncM-1 plasmid, and bla OXA-10 , bla TEM-40, and bla KPC-2 on a mobilizable IncP-6 plasmid of 51 kb, as revealed by mating-out assays. Here, we have characterized a human pathogenic Er ST422 harboring bla KPC-2 carbapenemase on an IncP-6 plasmid from Lebanon. Er O89H7 represents a major health threat due to limited therapeutic options, especially because novel β-lactam/inhibitor combinations are not available in Lebanon. Our results highlight an urgent need for improved carbapenemase screening and detection capacity in clinical laboratories and for enhanced genomic surveillance of MDR bacteria to implement intervention strategies to control their spread in Lebanon and beyond.
Hypervirulent Klebsiella pneumoniae (hvKp) is a globally emerging pathotype known for causing severe and disseminated infections across multiple organ systems. Diabetes is a risk factor for hvKp infection; however, otherwise healthy individuals from the community are also at risk. Two patients from Nebraska presented with complex abscesses of unknown origin. Both cases required interventional procedures and prolonged hospitalization. PCR-based testing, animal studies, and whole-genome sequencing analysis confirmed that hvKp was the offending pathotype. This report describes the first confirmed cases of hvKp infection in Nebraska, USA. These cases demonstrated the utility of using molecular methods to identify hvKp. The microbiology lab can be operationalized to provide valuable insights into the genetic makeup of pathogens, inform the institutional clinical decision-making processes, and epidemiological surveillance. This study highlights the collaborative relationships between the Infectious Diseases staff and the Clinical Microbiology Laboratory to work up complex cases.
Niemann-Pick disease type B (NPD-B) is a rare lysosomal storage disorder characterized by residual activity of acid sphingomyelinase (ASM). While functional inhibitors of ASM (FIASMAs) are widely prescribed as psychotropic medications, they may pose a particular risk to patients with NPD-B by further reducing the already impaired enzymatic function. Here, we report the case of a 20-year-old male with genetically confirmed NPD-B who experienced rapid clinical deterioration following the administration of zuclopenthixol, a drug not previously associated with FIASMA activity. Within 48 hours of treatment initiation, the patient developed profound lethargy and markedly elevated creatine kinase (CK) levels of up to 22,000 U/L, consistent with rhabdomyolysis. Symptoms resolved quickly after discontinuation of zuclopenthixol. In vitro experiments using a radioactive [1 4 C]-sphingomyelin assay in Jurkat cells demonstrated that zuclopenthixol dose-dependently inhibited ASM activity by up to 71.5%. Zuclopenthixol had not previously been recognized as a FIASMA and might therefore have been considered a rational choice for treating patients with NPD-B. Our findings challenge this assumption by identifying zuclopenthixol as a potent inhibitor of ASM activity. This novel insight is of high clinical relevance, given the frequent use of antipsychotics in the management of neuropsychiatric symptoms in lysosomal storage disorders. We propose that zuclopenthixol and other potential FIASMAs be carefully re-evaluated for use in this vulnerable patient population.
Phytobacter spp., a recently recognized human pathogen, presents significant diagnostic challenges. Conventional biochemical and automated identification methods often fail to distinguish Phytobacter from other Enterobacterales, which hinders accurate diagnosis and may potentially delay appropriate treatment. Since 2018, our state public health laboratory (LACEN-PR) has adopted a polyphasic identification strategy, integrating matrix-assisted laser desorption/ionization time-of-flight mass spectrometry, API 20E, targeted quantitative PCR, and whole-genome sequencing to investigate closely related Enterobacterales isolates that were initially misidentified and subsequently confirmed as Phytobacter spp. This study reports three cases of Phytobacter-associated sepsis, including a putative novel Phytobacter species and the first documented case of bla NDM-positive Phytobacter ursingii. Case 1: A 31-year-old man living with HIV developed sepsis following respiratory failure. Initial testing misidentified the isolate as Leclercia adecarboxylata, but the polyphasic approach revealed the potential for a novel Phytobacter species. The patient showed clinical improvement following targeted antimicrobial therapy. Case 2: A 50-year-old trauma patient developed sepsis caused by a Phytobacter spp. The isolate was closely related to that of Case 1. Despite treatment, he succumbed to the infection. Case 3: A 68-year-old woman with multiple comorbidities who developed septic shock. Blood cultures identified bla NDM-positive Phytobacter ursingii. The patient did not survive. These cases underscore the clinical significance of Phytobacter spp. as an emerging pathogen and highlight the challenges posed by their misidentification. The discovery of a novel species and multidrug-resistant P. ursingii highlights the need for improved diagnostic accuracy and effective treatment to enhance surveillance and improve patient outcomes.
Cerebral cavernous malformations (CCMs) are vascular lesions that can cause focal epilepsy due to local hemosiderin deposition, gliosis, and cortical irritation. Epileptic seizures attributable to CCMs contribute significantly to morbidity and often require long-term management with antiseizure medications (ASMs), but the psychiatric side effect profiles of ASMs remain underreported in the literature. Herein, we present a case report of a patient with homicidal ideation following zonisamide use and uptitration following an Epilepsy Monitoring Unit (EMU) stay. Detailed is his hospitalization for this side effect and transition to another ASM so that he could return to a normal life outside the hospital. To contextualize this case, a literature review was conducted using PubMed from 1990 to 2026 using combinations of the terms "zonisamide," "antiepileptic drugs," "homicidal ideation," and "psychiatric adverse effects." Relevant articles were manually reviewed and no prior cases explicitly describing homicidal ideation associated with zonisamide were identified. Homicidal ideation should be contextualized within a spectrum of ASM-associated psychiatric adverse effects (PAEs). Zonisamide may have contributed to symptom emergence in a susceptible individual within a multifactorial neuropsychiatric context. To our knowledge, this is the first case report explicitly documenting homicidal ideation as an adverse event of zonisamide.
Cladophialophora bantiana is a globally distributed dematiaceous mold which acts as an uncommon source of human infection. With a predilection for the central nervous system (CNS), this organism accounts for nearly half of all cerebral phaeohyphomycosis, with a mortality rate over 60%. Due to the rarity of this disease, treatment regimens are primarily based on retrospective reviews of case reports. The best clinical response occurred with a dual approach of surgical resection and antifungal therapy, though mortality rates remain high. A 68-year-old immunocompromised woman presented with imaging findings concerning for a neoplastic process, such as a glial neoplasm or CNS lymphoma. With microbiology, flow cytometry, and cytology of the cerebrospinal fluid negative and frozen section of the lesion favoring glial neoplasm, the scarce pigmented fungal hyphae on permanent sections were unexpected. Broad-range fungal DNA testing identified the organism as C. bantiana. Without further surgical resection, dual antifungal therapy was utilized to achieve clinical stability and return to pre-infection baselines. This is a rare case of a C. bantiana fungal brain abscess in an immunocompromised patient. Complete surgical resection was not possible; however, this case provides additional data to show the benefits of a dual antifungal treatment approach without additional surgical intervention, and the dosages used to achieve stability at 18 months post-presentation. This case highlights the importance of prompt efforts in determining causative organisms in unexpected fungal brain abscesses, which allows for targeted and improved success in the treatment of this highly fatal disease.
Antiseizure medications (ASMs) are the cornerstone of epilepsy treatment, but their potential reproductive toxicity may impact male fertility. Globally, approximately 15% of couples are affected by infertility, with male factors accounting for 50%. This study aimed to analyze the association between ASMs and male infertility using the FDA Adverse Event Reporting System (FAERS). Data from January 1, 2004, to September 30, 2024, were extracted from FAERS. A hybrid signal detection framework combining non-Bayesian (Reporting Odds Ratio, ROR) and Bayesian (Bayesian Confidence Propagation Neural Network, BCPNN) methods was employed to evaluate the reporting frequency and risk of male infertility adverse events for different ASMs. Among 81,618 deduplicated case reports involving specified ASMs, 60 were related to male infertility. Disproportionality analysis revealed that carbamazepine (ROR = 8.73; IC = 3.10) and valproic acid (ROR = 6.82; IC = 2.74) posed the highest risks. Oxcarbazepine, lamotrigine, and levetiracetam also showed positive signals. Phenytoin sodium, topiramate, and clonazepam showed no significant risk. Regarding overall ASM-related reports, the majority originated from the United States and involved patients aged 18-65. Despite the limitations of the FAERS database, these findings emphasize the importance of monitoring reproductive health in male patients, particularly those of childbearing age, and highlight the need to balance ASM efficacy with potential reproductive toxicity in clinical practice. Further research is needed to validate these findings and explore underlying mechanisms.
Niemann-Pick disease (NPD) is a rare autosomal recessive lysosomal storage disorder caused by acid sphingomyelinase (ASM) deficiency, resulting in progressive lipid accumulation in multiple cell types and organs. To describe a rare case of suspected NPD type B with secondary sea-blue histiocytosis and to explore its diagnostic implications. Comprehensive clinical and laboratory evaluations were conducted to assess the patient's condition. We report a rare case of Niemann-Pick disease type B accompanied with secondary sea-blue histiocytosis in a 32-year-old woman who had previously undergone splenectomy for congenital splenomegaly. She presented with abdominal distension, poor appetite and abdominal pain. Clinical evaluations revealed decompensated cirrhosis with no neurologic abnormalities. Transjugular liver biopsy demonstrated foamy cells infiltration, while bone marrow examination identified sea-blue histiocytes (approximately 4.5% of nucleated cells) and Niemann-Pick cells (approximately 2.5%). Although ASM activity testing and SMPD1 sequencing were recommended, the patient declined further evaluation for financial reasons. This case highlights the significance of considering ASM deficiency in patients with cryptogenic cirrhosis and underscores the diagnostic value of histopathology when definitive gene or enzyme testing is unavailable. It also raises the possibility that prior splenectomy may influence the clinical presentation of the disease.
Pluralibacter gergoviae is a rare, opportunistic, urease-producing gram-negative organism infrequently implicated in invasive infections. Reports in organ transplant or extracorporeal membrane oxygenation (ECMO) settings are exceedingly uncommon. This case is distinctive for describing P. gergoviae bacteremia potentially associated with severe hyperammonemia and encephalopathy in a lung transplant recipient on prolonged ECMO support, expanding the clinical spectrum of infection-related hyperammonemia beyond the traditionally recognized Ureaplasma and Mycoplasma species. A 32-year-old woman with cystic fibrosis underwent bilateral lung transplantation while on ECMO for refractory hypoxemic respiratory failure. Her post-operative course was complicated by multiorgan failure, Candida parapsilosis fungemia, and later, surgical site infection. During the fourth month of hospitalization, P. gergoviae was isolated from surgical wound and blood cultures. Around the time of bacteremia, she developed acute encephalopathy with markedly elevated plasma ammonia (262 µmol/L) in the absence of hepatic dysfunction. The P. gergoviae isolate was confirmed to be urease positive. Management included targeted antimicrobial therapy with ceftriaxone and meropenem, along with lactulose, rifaximin, and zinc for hyperammonemia. Ammonia levels and mental status gradually normalized following treatment, and blood cultures cleared. This case illustrates P. gergoviae as an emerging opportunistic pathogen capable of causing invasive infection and possibly metabolic complications in profoundly immunocompromised hosts.
Pathogenic variants in HCN1 are associated with a spectrum of epilepsies, including drug-resistant developmental and epileptic encephalopathies. However, evidence to guide antiseizure medication (ASM) selection in HCN1-related epilepsies remains limited. In this intentionally selected, retrospective case series, we identified seven patients with confirmed pathogenic or likely pathogenic HCN1 variants associated with gain-of-function effects. In all cases, treatment with sodium-channel-blocking ASMs including phenytoin, lamotrigine, oxcarbazepine, and lacosamide was associated with seizure worsening, with clinical improvement after drug discontinuation. These findings identify a clinically actionable gene-drug interaction in HCN1-related epilepsies and support a mechanism-based approach to ASM selection. Our data provide translational evidence to inform precision treatment strategies and help avoid potentially harmful therapies in affected children.
Intravesical instillation of Mycobacterium bovis bacillus Calmette-Guérin (BCG) was first used to treat bladder cancer in 1976. Since then, it has become a common therapy for non-muscle invasive bladder cancer. A live, attenuated mycobacterium, M. bovis BCG causes infectious complications in approximately 1% of patients. These infections can involve various anatomic sites and have a range of presentations. Diagnosis may require several testing modalities, and treatment typically involves multiple antimicrobials for a long duration. We report three cases of M. bovis BCG infection following intravesical BCG instillation for the treatment of bladder cancer. The patients are men ranging in age from 71 to 83 years old. One had a ruptured mycotic aneurysm of the aorta, another had a presumed anaphylactic reaction, and the final had a bone marrow infection. Isolation of BCG was achieved through mycobacterial blood culture in two cases and also through mycobacterial culture of bone marrow and hematoma aspirate. Nucleic acid amplification testing for Mycobacterium tuberculosis complex (of which BCG is a member) was utilized in two cases, and histologic examination identified granulomas in one case. Two of the patients received mycobacterial treatment, including rifampin, isoniazid, ethambutol, and linezolid. One patient remains living over 3 years after infection, one died despite treatment, and one died from unrelated causes. These cases highlight the diversity of BCG infection symptoms and sites and the various laboratory testing modalities that can inform diagnosis and treatment.
Paenibacillus species are rod-shaped, gram-variable, endospore-forming, environmental bacteria. Once classified within Bacillus spp., it was reclassified based on 16S rRNA sequencing. Paenibacillus spp. has rarely been found to be pathogenic in humans; however, recent reports have been described in immunocompromised patients. A 60-year-old male with past medical history of hypertension, hyperlipidemia, atrial fibrillation, and bicuspid aortic valve presented to an outside hospital for 3 days of chest pain without associated symptoms. He was transferred to our institution after computed tomography (CT) demonstrated fluid around the aortic root concerning for aortic pseudoaneurysm. Admission and follow-up blood cultures were negative. He was started on ceftriaxone and vancomycin and underwent aortic root repair. Intraoperative cultures grew gram-indeterminate bacilli identified as Paenibacillus pueri by 16S broad-range PCR at an outside institution. Initial pathogen identification was complicated without discernible morphological differences between Paenibacillus spp. and Bacillus spp. Final identification often relies on molecular methods. Previous cases utilized amoxicillin-clavulanate or trimethoprim-sulfamethoxazole, with resistance demonstrated to penicillins and clindamycin. In our case, doxycycline was chosen based on limited susceptibility profiles and considering antibiotic side effect profiles. This is the third documented human case of Paenibacillus pueri infection. Paenibacillus spp. infections have varied clinical presentations, typically described in immunocompromised patients. This case demonstrates the difficulties in identifying Paenibacillus pueri. While rare, Paenibacillus spp. has been occasionally implicated in human disease. Even in immunocompetent patients, suspicion for true pathogens should remain high with repeated isolation on separate specimens.
Posterior ankle pain is a nonspecific symptom that can result from various pathologies. Rare accessory muscle variants, including the accessory soleus muscle, may cause overlapping clinical conditions due to mass effect, dynamic impingement, chronic compartment syndrome, or tibial nerve compression. Limited familiarity with this anatomical variant often leads to diagnostic delay, misdiagnosis, or inappropriate treatment. The authors report three cases of chronic posterior ankle pain in athletes caused by the accessory soleus muscle with the aim to highlight the importance of considering the accessory soleus muscle (ASM) as a possible cause of this symptomatology. The diagnostic and therapeutic management, and clinical-functional outcomes are analysed. All patients reported posterior ankle pain for more than one year, exacerbated by sports activity and forced plantar flexion, with relief at rest. Magnetic Resonance Imaging (MRI) or Computed Tomography (CT) demonstrated the presence of ectopic muscle tissue compatible with an accessory soleus muscle. Clinical data, imaging findings, treatment modalities, and clinical-functional outcomes were retrospectively collected for each case. Preoperative, one year postoperative, and final follow-up assessments included the Visual Analog Scale (VAS) for pain evaluation, 17-Item Italian Foot Function Index (17-IFFI), and the American Orthopaedic Foot and Ankle Society (AOFAS) ankle-hindfoot score validated in Italian. All patients underwent surgical excision of the accessory soleus muscle through a posteromedial approach. Surgical excision was the definitive treatment for each patient, resulting in pain relief, clinical and functional improvement, and return to sports activity. No complications or recurrences were observed. The accessory soleus muscle is a potential cause of chronic posterior ankle pain and must be considered in the differential diagnosis to avoid diagnostic delays or misdiagnosis. Surgical excision has represented the effective and definitive treatment of the three reported cases.
Trichophyton indotineae is an emerging Trichophyton species associated with extensive dermatophytosis frequently demonstrating terbinafine resistance. While early cases were reported in South Asia, T. indotineae has since spread globally, with several cases reported in the United States since 2023. To our knowledge, we describe the first cases reported in Virginia. Patients A and B each presented to the dermatology clinic with a diffuse, pruritic rash unresponsive to previous courses of topical and antifungal therapy. Both presentations raised concern for T. indotineae infection, which was confirmed with internal transcribed spacer (ITS) genome sequencing of isolates from the lesions. Both patients were initially prescribed an 8-week course of itraconazole 200 mg daily. Patient A required an additional month of treatment, but both patients had improved at follow-up. The present cases highlight several key points regarding the epidemiology, diagnosis, and management of T. indotineae infections, including continued expansion within the US, the need for ITS sequencing to distinguish T. indotineae from Trichophyton mentagrophytes and Trichophyton interdigitale, and the growing role of oral itraconazole as first-line therapy.
Granulicatella adiacens is a fastidious Gram-positive coccus and is a rare but recognized cause of infective endocarditis. Infective endocarditis during pregnancy is uncommon but carries substantial maternal and fetal risk. Plasma metagenomic analysis of microbial cell-free DNA has emerged as a complementary diagnostic tool in culture-negative infections. We describe a 35-year-old pregnant woman with known mitral valve prolapse who presented at 21 weeks of gestation with an acute ischemic stroke. Initial etiological work-up, including transesophageal echocardiography, was unremarkable. Ten days later, she re-presented with left-arm pain and neurologic symptoms. Repeat echocardiography revealed multiple mitral vegetations compatible with infective endocarditis. Despite multiple sets of prolonged-incubation blood cultures and extensive serological testing, all microbiological investigations remained negative. Empirical intravenous ceftriaxone was initiated based on the working diagnosis of HACEK endocarditis. A plasma metagenomic cell-free DNA test ultimately identified G. adiacens, which was suspected to have entered the body through dental treatment received a few weeks earlier. Ceftriaxone was continued given the favorable clinical response, with vegetation resolution, troponin decline, and uncomplicated term delivery of a healthy infant. This case illustrates the diagnostic challenges of culture-negative infective endocarditis in pregnancy and underscores the value of plasma microbial cell-free DNA sequencing as a complementary tool when conventional methods fail. It also emphasizes the need to repeat echocardiography when clinical suspicion remains high and raises the question of antibiotic prophylaxis for high-risk dental procedures in pregnant women with underlying valvular heart disease.
Epilepsia partialis continua (EPC) is a form of focal motor status epilepticus (SE), which is commonly drug-resistant requiring treatment with multiple antiseizure medications (ASM). There are no established guidelines for pharmacological management. Perampanel (PER), an AMPA receptor antagonist, has gained attention in treating SE based on limited case reports. This study evaluates the efficacy of PER in treating EPC. We retrospectively analyzed the treatment response and adverse effects of PER in patients with EPC at our local centre between January 2024 and March 2025. Eight patients with EPC were included. The loading dose of PER ranged from 6 mg to 20 mg. A clinical response was defined as seizure freedom within 72 h of initiating PER, with PER being the last ASM added. PER resulted in seizure termination in eight out of eight patients. Time to response ranged from less than 24 h to 72 h. Oral PER, up to a dose of 16 mg, was well-tolerated in conscious patients. Common side effects included sedation, delirium, agitation, and nausea. Our real-world data suggest that PER is an effective and well-tolerated early adjunct therapy in EPC, particularly at higher oral loading doses in conscious patients.
Epilepsy surgery remains underutilized despite strong evidence supporting its efficacy for appropriately selected patients with drug-resistant epilepsy (DRE). We describe the case of a 23-year-old woman with a 4-year history of refractory focal impaired awareness seizures, experiencing up to four episodes per week despite multiple trialed antiseizure medications at tolerated doses. Initial investigations including magnetic resonance imaging (MRI), EEG, and paraneoplastic screening were unremarkable, and she was managed as having non-lesional left temporal lobe epilepsy. As part of an artificial intelligence driven research initiative, a natural language processing based random forest algorithm reviewing neurology clinic documentation identified her as a potential surgical candidate. Complementary large language model analysis supported extraction of relevant seizure and imaging information. This prompted re-evaluation of the case, with PET demonstrating left temporal hypometabolism and repeat MRI revealing a previously unrecognized encephalocele. She subsequently underwent left temporal lobe polectomy with encephalocele disconnection. The procedure was successful, and she has remained seizure-free for 1 year postoperatively, allowing initiation of ASM weaning and the possibility of long-term cure. This case illustrates the transformative potential of epilepsy surgery for individuals with DRE and the promising role of artificial intelligence augmented triage systems in reducing delays to surgical referral. Given the morbidity, mortality, and economic burden associated with DRE, timely surgical evaluation should be considered the standard of care for eligible patients. Integrating artificial intelligence tools into clinical workflows may help overcome longstanding barriers to access and ensure that life-changing interventions are offered earlier in the disease course.
The expanding use of B-cell-depleting anti-CD20 monoclonal antibodies has introduced diagnostic challenges for infections that are traditionally confirmed through serologic testing. Seronegative secondary syphilis is exceptionally rare and has historically been described only in profoundly immunocompromised patients with advanced HIV/AIDS. This case represents a novel clinical scenario in the era of targeted immunologic therapy. We report the case of a 44-year-old man with marginal zone lymphoma receiving maintenance obinutuzumab, who presented with a symmetric, pruritic, palmoplantar predominant papulosquamous eruption accompanied by fatigue and elevated inflammatory markers. Extensive serologic testing for syphilis, including repeated treponemal (TP-PA, treponemal IgG) and nontreponemal (RPR) assays, remained completely nonreactive on multiple occasions. However, a skin punch biopsy demonstrated epidermal hyperplasia with a dense plasma cell-rich lymphohistiocytic infiltrate. Immunohistochemical staining for Treponema pallidum revealed numerous spirochetes in the epidermis and superficial dermis, confirming the diagnosis of secondary syphilis. The patient received three weekly intramuscular doses of benzathine penicillin G (2.4 million units per dose) with complete clinical resolution and no adverse effects. Serologic tests remained nonreactive on follow-up evaluation. This case is the first documented report of persistent seronegative secondary syphilis in a patient receiving obinutuzumab therapy. It underscores the critical importance of maintaining a high index of clinical suspicion and pursuing tissue-based diagnostics when serologic tests fail to explain compelling clinical findings in patients with profound B-cell depletion. Clinicians managing patients on anti-CD20 therapies must recognize the limitations of serologic diagnosis for infections that typically depend on antibody detection.
Neuronal intranuclear inclusion disease (NIID) is a rare neurodegenerative disease. We investigated a role of nonconvulsive status epilepticus (NCSE) in cognitive dysfunction in pathologically confirmed NIID. We also analyzed distinctive factors to differentiate NIID from Alzheimer's disease (AD). A 63-year-old man presented with transient consciousness disturbance (Day 1). For previous 6 years he had been suffering from similar episodes and gradually progressive cognitive decline. Clinical characteristics and response to antiseizure medications (ASM) were analyzed with a narrative literature review. Neurological examination showed disorientation, memory disturbance, aphasia, agraphia, and impaired visuospatial ability. On Day 27, his MMSE scored 10. Diffusion-weighted MRI showed high intensity signal in the corticomedullary junction of the frontal lobe, which could not explain his neurological manifestations. EEG showed seizure patterns arising from the bilateral occipital areas. ASM improved MMSE score to 23. Skin biopsy confirmed his diagnosis as dementia-dominant sporadic NIID. He died on Day 77. Cognitive dysfunction in visuospatial execution, manifested by impaired pentagon drawing and agraphia of both kanji (Japanese morphograms) and kana (Japanese syllabograms), its fluctuating course, and reactivity to ASM were clear distinction from AD. NCSE can accelerate cognitive decline and ASM can improve cognitive function in NIID. Cognitive evaluation using pentagon drawing and handwriting of both morphograms and syllabograms can be useful to differentiate NIID from AD.