Neuroimaging insights into poor prognosis paraneoplastic encephalomyelitis: A case report on a challenging diagnosis revealed by MR imaging in a patient with Hodgkin's lymphoma.

Paraneoplastic encephalomyelitis (PEM) is a rare complication associated with malignancies, often presenting before the cancer diagnosis. A 42-year-old male with a history of chronic smoking presented with acute urinary retention and neurological deficits, all evolving in a febrile context with general deterioration. Laboratory tests were conducted, followed by a cerebral MRI which revealed multiple T2 and FLAIR hyperintense lesions in the periventricular and periaqueductal regions, medial temporal lobes, and bilateral postero-medial thalamus. Enhanced CT scans of the chest and abdomen identified multiple cervical, axillary, and inguinal lymphadenopathies. Subsequently, an ultrasound-guided biopsy of a cervical node was performed. His condition deteriorated rapidly, requiring intubation and sedation. A subsequent MRI revealed worsening cerebral and spinal cord lesions with new contrast enhancement in the brainstem. The differential diagnosis included toxic/metabolic and paraneoplastic causes. Biopsy results confirmed Hodgkin's lymphoma, leading to a diagnosis of progressive paraneoplastic encephalomyelitis (PEM). Despite adequate treatment, the patient's condition worsened, leading to death from pneumonitis and metabolic complications. This case underscores the importance of considering PEM in patients with neurological deficits and malignancy, with MRI playing a crucial role in diagnosis. Early detection and treatment are essential to improving outcomes.

Catastrophic Case of West Nile Virus Rhombencephalitis in AIDS.

West Nile Virus (WNV) belongs to the Flaviviridae family of viruses. It was first isolated and identified in 1937. Patients typically present with flu-like symptoms or are asymptomatic; however, neuroinvasive West Nile can lead to significant neurological impairment. Herein presented is a catastrophic case of WNV rhombencephalitis in a male patient newly diagnosed with AIDS. This report sheds light on the potential for severe neurological complications in co-infected patients and emphasizes the importance of early recognition.

The rapidly progressing and fatal outcome of rhombencephalitis by listeriosis in a 61-year-old male.

Listeria monocytogenes is a Gram-positive facultative anaerobic bacterium that is ubiquitous in the environment and can cause severe infections in immunocompromised individuals, pregnant women, and newborns. Listeriosis can manifest as meningitis, encephalitis, or sepsis, and its diagnosis requires a high index of suspicion. The case is reported of a rare presentation of rhombencephalitis by listeriosis in a 61-year-old male who initially suffered from subacute gastric disturbances and fever. Neurological consultation showed abnormal functions of cranial nerves and meningeal signs were observed. MRI revealed a poorly demarcated focus of approximately 45 × 16 × 15mm, indicating possible inflammatory processes, necessitating a lumbar puncture. Assessment of the CSF indicated infection with the bacterium- Listeria Monocytogenes, with the final diagnosis of Listeriosis encephalitis. Despite antibiotic therapy of Ceftazidine and Ampicillin, the patient's condition deteriorated, followed by death.

A case of Adult-onset Acute Flaccid Myelitis Accompanied by Rhombencephalitis which First Presented with Prominent Psychiatric Symptoms and Dysautonomia Mimicking Anti-N-methyl-d-aspartate Receptor Encephalitis.

A 44-year-old woman with a subacute onset of an altered mental status, urinary retention, and fluctuating blood pressure was initially diagnosed with anti-N-methyl-d-aspartate receptor (NMDAR) encephalitis, meeting the criteria of Graus et al. Cardiac arrest occurred, which required pacemaker placement. She subsequently showed profound flaccid limb paralysis, with magnetic resonance imaging demonstrating focal necrotic lesions localized in the anterior horn of the longitudinal segments of the spinal cord and in the pontine tegmentum. Enteroviruses or autoimmune encephalitis-associated autoantibodies were not detected. We herein report a case of acute flaccid myelitis with profound psychiatric symptoms and dysautonomia, resembling NMDAR encephalitis.

Primary mediastinal seminoma presenting with paraneoplastic anti-Hu encephalitis: a case report and literature review.

Primary mediastinal seminomas are exceedingly rare tumors, often localized to the anterior mediastinum. They may present with numerous complications, including superior vena cava syndrome, chylothorax, and pericardial effusions. Less commonly, they may present with paraneoplastic encephalitis. In this report we describe a case of a 19-year-old male with no significant past medical history who presented with bilateral hearing loss, progressive neuropathy, and ataxia. Subsequently the patient was found to have mediastinal mass with a high-titer anti-Hu antibody. To our knowledge, only one other case of mediastinal seminoma presenting with anti-Hu antibodies has been described in the literature. In this report, we describe a rare case of mediastinal seminoma, describe treatment options, and discuss additional known cases presenting with paraneoplastic encephalitis.

Rhombencephalitis and longitudinal extensive myelitis associated with dinutuximab use in high-risk neuroblastoma.

BACKGROUND: Dinutuximab is a monoclonal antibody that targets the GD2 antigen used in the treatment of high-risk neuroblastoma. Dinutuximab-associated rhombencephalitis and myelitis is a rare, steroid-responsive, serious, but reversible pathology. To date, three transverse myelitis cases and one rhombencephalitis case due to dinutuximab have already been reported. Moreover, a recently published article identified five inflammatory CNS demyelination cases (four myelitis and one rhombencephalitis). We present a 5-year-old patient with rhombencephalitis and myelitis following dinutuximab-beta treatment. CASE: A 5-year-old patient with a left-sided retroperitoneal mass infiltrating the left kidney and multiple lytic bone lesions was diagnosed with neuroblastoma with a percutaneous biopsy from the abdominal mass. Surgery was performed after a prominent treatment response was detected on the abdominal CT. Radiotherapy was applied to the abdomen. While she was still undergoing maintenance treatment with 13-cis retinoic acid, a metaiodobenzylguanidine (MIBG) scan detected new bone lesions, and brain MRG identified pachymeningeal involvement. A new chemotherapy regimen was started and decreased MIBG uptake was seen in all previous bone lesions. However, newly developed eighth rib metastasis was seen in the following MIBG scan. Autologous stem cell transplantation was done. Soon after, dinutuximab-beta, together with temozolomide and irinotecan, was initiated. Following the third cycle hypotension, somnolence, paraparesis, and unilateral fixed dilated pupil were developed. Afterward, hemiballismus-like irregular limb movements were observed. Work-up studies were unremarkable, except for hypodensity in the brain stem on the brain CT. MRI revealed T2 hyperintensity of the brainstem and spinal cord extending from the cervicomedullary junction to the T7 level. Moreover, incomplete contrast enhancement and facilitated diffusion were observed. Imaging findings suggested demyelination. Steroids and intravenous immune globulin (IVIG) treatment were initiated. Both imaging abnormalities and clinical symptoms resolved partially at one month and disappeared at six months. CONCLUSIONS: Awareness of the radiological findings of dinutuximab toxicity will lead to prompt diagnosis and treatment.

Secondary dengue serotype 1 infection causing dengue shock syndrome with rhombencephalitis and bleeding associated with refractory thrombocytopenia: A case report.

Background: Dengue has a wide spectrum of manifestations, from an asymptomatic condition to dengue shock syndrome. Extensive plasma leakage, severe bleeding, or both, could lead to dengue shock syndrome, a common cause of death in dengue-infected patients. Thrombocytopenia is a common laboratory finding in dengue, which correlates with the disease severity and rapidly resolves during the recovery phase. Therefore, refractory thrombocytopenia is rare in patients with dengue. Rhombencephalitis is an inflammatory disease affecting the hindbrain, rarely associated with dengue. We report the second case of dengue-associated rhombencephalitis, wherein the patient developed dengue shock syndrome and severe bleeding associated with refractory thrombocytopenia. Case report: A 47-year-old Thai female with secondary dengue serotype 1 infection developed dengue shock syndrome with rhombencephalitis, manifested as altered sensorium and status epilepticus in the critical phase. Cerebrospinal fluid analysis showed pleocytosis with predominantly mononuclear cells and high protein levels. Magnetic resonance imaging of the brain showed multifocal brain signal abnormalities involving the medulla oblongata, pons, midbrain, bilateral hippocampi, thalami, posterior limb of internal capsules, external capsules, and deep hemispheric white matter. The patient had partial neurological recovery following rhombencephalitis for one month. During the recovery phase, severe bleeding with refractory thrombocytopenia and acute kidney injury were observed. Methylprednisolone with eltrombopag was administered, which resulted in an increased the platelet count, cessation of bleeding and recovery of kidney function within 4 days. Conclusions: Dengue is a potential cause of rhombencephalitis. Dengue-associated rhombencephalitis develops during the critical phase, with only partial neurological recovery. However, severe bleeding and refractory thrombocytopenia were also observed during the recovery phase. Methylprednisolone with a thrombopoietin receptor agonist could be an effective treatment for increasing platelet count and stopping bleeding in dengue.

Epidemiología, clínica y resultados de imagen de rombencefalitis causada por L. monocytogenes. Un estudio observacional / Epidemiology, clinical and imaging features of rhombencephalitis caused by L. monocytogenes. A retrospective observational study

Introducción: Hasta la fecha, pocos estudios han explorado los factores de riesgo específicos de los pacientes con listeriosis que desarrollan rombencefalitis, y no hay suficiente información sobre los hallazgos de imagen y los síntomas clínicos en pacientes con esta enfermedad. El objetivo de este trabajo fue analizar los hallazgos de imagen asociados a la rombencefalitis por L. monocytogenes en una cohorte de pacientes con listeriosis. Materiales y métodos: Se realizó un estudio observacional retrospectivo de todos los casos declarados de listeriosis en un hospital terciario de Granada, España, desde 2008 hasta 2021. Se recogieron los factores de riesgo, las comorbilidades y los resultados clínicos de todos los pacientes. Además, se incluyeron los síntomas clínicos y los hallazgos de resonancia magnética (RM) de los pacientes que desarrollaron rombencefalitis. Se realizaron análisis descriptivos y bivariados utilizando el software estadístico SPSS (IBM SPSS, versión 21). Resultados: Nuestra cohorte incluyó a 120 pacientes con listeriosis (41,7%, mujeres; edad media: 58,6 ± 23,8 años), de los cuales 10 (8,3%) tenían rombencefalitis. Los hallazgos más frecuentes en la RM de los pacientes con rombencefalitis confirmada fueron hiperintensidad en T2-FLAIR (100%), hipointensidad en T1 (80%), realce parenquimatoso disperso (80%) y realce de los nervios craneales (70%), mientras que la afectación anatómica más frecuente fue en la protuberancia, la médula oblongada y el cerebelo. Se produjeron complicaciones en seis pacientes (absceso en cuatro, hemorragia en dos e hidrocefalia en uno). Conclusiones: La rombencefalitis se asocia a un aumento de la mortalidad intrahospitalaria en pacientes con listeriosis. La distribución anatómica y las características de imagen de la neurolisteriosis podrían ser útiles para sugerir el diagnóstico. Futuros estudios con mayor tamaño muestral deberían explorar la asociación entre la...

Introduction: To date, few studies have explored the specific risk factors of patients with listeriosis who develop rhombencephalitis, and there is insufficient information regarding imaging findings and clinical symptoms in patients with this disease. This work aimed to analyze the imaging findings associated with L. monocytogenes rhombencephalitis in a cohort of patients with listeriosis. Materials and methods: We conducted a retrospective observational study of all declared cases of listeriosis in a tertiary hospital from Granada, Spain, from 2008 to 2021. Risk factors, comorbidities, and clinical outcomes were collected for all patients. In addition, clinical symptoms and magnetic resonance imaging (MRI) findings were included for those patients who developed rhombencephalitis. Descriptive and bivariate analyses were performed using SPSS statistical software (IBM SPSS, version 21). Results: Our cohort comprised 120 patients with listeriosis (41.7% women, mean age: 58.6 ± 23.8 years), of which 10 (8.3%) had rhombencephalitis. The most frequent MRI findings in patients with confirmed rhombencephalitis were T2-FLAIR hyperintensity (100%), T1 hypointensity (80%), scattered parenchymal enhancement (80%), and cranial nerve enhancement (70%), while the most frequent anatomical involvement were pons, medulla oblongata, and cerebellum. Complications occurred in 6 patients (abscess in 4, hemorrhage in 2, hydrocephalus in 1). Conclusions: Rhombencephalitis is associated with an increased in-hospital mortality in patients with listeriosis. The anatomical distribution and imaging characteristics of neurolisteriosis could be useful to suggest the diagnosis. Future studies with greater sample size should explore the association between anatomical location, imaging patterns, and associated complications (e.g., hydrocephalus, hemorrhage), and clinical outcomes.(AU)

Listeria rhombencephalitis mimicking stroke in a patient with giant cell arteritis.

Listeria monocytogenes sometimes causes central nervous system infections. However, rhombencephalitis is a rare form of L. monocytogenes infection. Its clinical symptoms and magnetic resonance imaging (MRI) findings are often similar to those of vertebrobasilar stroke. We present the case of a 79-year-old woman with Listeria rhombencephalitis presenting with rhinorrhea and productive cough. She had giant cell arteritis (GCA) treated with prednisolone and methotrexate. She was admitted for loss of appetite, rhinorrhea, and productive cough. These symptoms were alleviated without specific treatment; however, she suddenly developed multiple cranial nerve palsies, and MRI showed hyperintense signals on diffusion-weighted imaging and hypointense signals on apparent diffusion coefficient in the brainstem. Ischemic stroke due to exacerbation of GCA was suspected, and treatment with intravenous methylprednisolone was initiated; however, seizures occurred, and a lumbar puncture was performed. Cerebrospinal fluid and blood cultures revealed L. monocytogenes, and she was diagnosed with Listeria rhombencephalitis. Although antibiotic treatment was continued, the patient died. Thus, when patients with rhinorrhea or productive cough develop sudden cranial nerve palsy, Listeria rhombencephalitis should be considered as a differential diagnosis, and lumbar puncture should be performed.

Next-Generation Sequencing of Cerebrospinal Fluid for the Diagnosis of VZV-Associated Rhombencephalitis.

BACKGROUND: Rhombencephalitis (RE) is a general term for a group of inflammatory diseases of the rhombencephalon caused by different etiologies. Patients of RE caused by the varicella-zoster virus (VZV) are sporadic in medical practice. The VZV-RE is easily misdiagnosed and causes a poor prognosis for patients. METHODS: In this study, we analyzed the clinical symptoms and imaging features of five patients with VZV-RE diagnosed by the next-generation sequencing (NGS) technique of cerebrospinal fluid. Magnetic resonance imaging (MRI) examination was used to characterize the imaging of the patients. The McNemar test was used to analyze the cerebrospinal fluid testing (CSF) values and MRI test of the 5 patients. RESULTS: We finally used NGS technology to confirm the diagnosis in 5 patients with VZV-RE. MRI revealed T2/FLAIR high signal lesions in the patients' medulla oblongata, pons, and cerebellum. All patients had early signs of cranial nerve palsy; some had herpes or pain in the corresponding cranial nerve distribution areas. The patients develop headaches, fever, nausea, vomiting, and other signs and symptoms of brainstem cerebellar involvement. McNemar's test showed no statistical difference between multi-mode MRI and CSF values for diagnosing VZV-RE (p = 0.513). CONCLUSIONS: This study showed that patients with herpes in the skin and mucous membranes at the distribution area of the cranial nerves and with the underlying disease were prone to RE. We suggest that the NGS analysis should be considered and selected based on the level of parameters, such as MRI lesion characteristics.

High fever in myelin oligodendrocyte glycoprotein-associated disorder (MOGAD): A diagnostic challenge.

The phenotypic spectrum of myelin oligodendrocyte glycoprotein (MOG)-IgG associated disorders (MOGAD) has broadened in the past few years, and atypical phenotypes are increasingly recognized. Febrile meningoencephalitis has rarely been reported as a feature of MOGAD and represents a diagnostic challenge. We report the case of 24-year-old women with high-grade fever, meningoencephalomyelitis, and persistently positive MOG-IgG, for whom an extensive infectious work-up was negative and who responded to high-dose intravenous methylprednisolone. The full clinical spectrum of MOGAD is yet to be completely elucidated. In patients presenting with febrile meningoencephalitis, MOG-IgG testing should be considered particularly if infectious work-up is negative.

Listeria Rhombencephalitis and Infratentorial Brain Abcesses in a Young Adult with Stroke Like Presentation.

Rhombencephalitis refers to the inflammation of rhombencephalon, and Listeria monocytogenes is one of the causes of infectious rhombencephalitis. Listeria rhombencephalitis is a rare and severe infection with high mortality and morbidity. As the disease can present with a variety of neurological symptoms and nonspecific laboratory tests, it can easily be misdiagnosed. Sudden onset of neurological signs without fever can resemble stroke. Magnetic resonance imaging can be useful in patients for confirmation of the diagnosis and during the follow-up. Early diagnosis and treatment are especially important for improvement of the outcomes. Here we report a case with stroke-like presentation that was diagnosed as Listeria rhombencephalitis in follow-up and present the serial brain magnetic resonance imaging features.

Rhombencephalitis Possibly Caused By Mycoplasma Pneumoniae.

We report a rare case of encephalitis that is not often described in clinical settings in neurology. Our case was 11-year-old female patient who had presented with features of meningoencephalitis, but not responded to the conventional treatment. Her magnetic resonance imaging revealed lesions in thalami, cerebellum and brainstem. The differentials in this age were infective and inflammatory causes of meningoencephalitis and acute disseminated encephalomyelitis (ADEM). Paraneoplastic was another differential. Mycoplasma serology came out positive. As a result, diagnosis of mycoplasma pneumoiae associated Rhombencephalitis was made based on diagnosis of exclusion.

Rhombencephalitis in Pregnancy-A Challenging Case of Probable Listeria Infection.

Rhombencephalitis refers to inflammation of the brainstem and cerebellum, and can be caused by infections, autoimmune disorders or paraneoplastic syndromes. The most common infective cause is the bacterium Listeria monocytogenes. Listeria monocytogenes is the predominant species to cause human listeriosis, and is commonly due to the ingestion of contaminated foods. Symptoms include a mild gastroenteritis, fever (often with extreme temperature variations), headache, and myalgia. In more severe cases, invasive disease may lead to bacteraemia and neurolisteriosis. Pregnant women are more susceptible to listeriosis, which is believed to be due to pregnancy-related immune modulation. Maternal-neonatal infection with adverse pregnancy outcomes include neonatal listeriosis, spontaneous miscarriage and intrauterine fetal demise. Diagnosis may be challenging due to initial nonspecific symptoms and low sensitivity and specificity of confirmatory diagnostic laboratory tests. Here, we describe a case of rhombencephalitis in pregnancy, attributed to Listeria, and review the clinical features, diagnosis and multidisciplinary management. Lastly, we describe the immunological response to Listeria monocytogenes and show in vitro pro-inflammatory effects of Listeria monocytogenes on peripheral blood mononuclear cells and placental explants.

Kelch-like protein 11 antibody-associated paraneoplastic neurological syndrome: A state-of-the-art review.

The Kelch-like protein 11 antibody-associated paraneoplastic neurological syndrome (KLHL 11-PNS) was first identified in 2019. This novel antibody, targeting the intracellular KLHL 11 antigen, can be detected in serum and cerebrospinal fluid using tissue-based and cell-based assays. It is thought to be a biomarker for a T-cell autoimmunity response. The most likely immunopathogenesis of KLHL 11-PNS appears to be linked to cytotoxic T-cell-mediated neuronal injury and loss. Patients have adult-male predilection, rhombencephalitis (brainstem and / or cerebellar involvement), and a robust oncological correlation with testicular germ cell tumors (predominately seminoma). Brain magnetic resonance imaging demonstrated T2 / fluid-attenuated inversion recovery hyperintensities and atrophy of the temporal lobe, cerebellum, and brainstem. Most patients responded poorly to immunotherapy and oncotherapy and thus had a poor long-term prognosis. We review the literature and provide an update of current knowledge regarding KLHL 11-PNS, including epidemiology, underlying mechanism, clinical presentations, paraclinical and oncological findings, diagnostic workup, and treatment approaches.

Enteroviral Rhombencephalitis with Abducens Nerve Palsy and Cardio-Pulmonary Failure in a 2-Year-Old Boy.

Enterovirus infection is endemic in many areas, especially in Southeast Asia. Enterovirus infection with severe complications (EVSC) is life-threatening, and timely diagnosis and management are crucial for successful management. Here, we report on a 2-year-old boy with hand, foot, and mouth disease. Myoclonic jerks developed and left abducens nerve palsy followed. Brain magnetic resonance imaging (MRI) showed rhombencephalitis. Pulmonary edema and cardiopulmonary failure developed, and intravenous immunoglobulin and extracorporeal membrane oxygenation were administered. He had a tracheostomy with home ventilator use after 64 days of hospitalization. At a 5-year follow-up, his neurodevelopment was normal with complete recovery from the abducens nerve palsy. The progress of EVSC may be rapid and fulminant, and timely diagnosis is critical for patient prognosis and outcomes. The presence of abducens nerve palsy is an indicator of enteroviral rhombencephalitis, and immediate and appropriate management is suggested.

Neuroborreliosis with involvement of rhombencephalon: A case report.

We describe a case of a 52 year-old woman who was hospitalized with rhombencephalitis caused by Borrelia burgdorferi sensu lato. The patient presented with intermittent fever, dry cough, fatigue, global headache, night sweats, unintentional weight loss, and neurological symptoms like diplopia, tremor, paresthesia and ataxia. Examination of serum and cerebrospinal fluid (CSF) revealed positive Borrelia burgdorferi-specific antibody index and presence of CSF oligoclonal IgG bands, indicating intrathecal synthesis of Borrelia-specific antibodies. The clinical and biochemical picture thus suggested neuroborreliosis. Unexpectedly a magnetic resonance imaging (MRI) scan demonstrated inflammation in rhombencephalon that are extremely rare in patients with neuroborreliosis. The patient was treated with intravenous ceftriaxone with rapid improvement of her symptoms. The MRI findings were in regress six weeks after onset of antibiotic treatment, and normalized after about seven months.

Neuro-Behcet Disease and Ocular Inflammation: A Case Report and Literature Review.

Purpose: To describe a case of panuveitis with occlusive vasculitis leading to the diagnosis of neuro-Behcet disease (NBD) and discuss the relationship between uveitis and NBD.Methods: Case report with a literature review of ocular inflammation in NBD.Results: A 26-year-old woman with a seven-month history of recurrent cerebral venous sinus thromboses (CVST) and right-sided hemiparalysis secondary to rhombencephalitis presented with bilateral panuveitis and occlusive retinal vasculitis. Systemic evaluations were negative for hypercoagulability and infection. Although HLA-51 negative, the diagnosis was consistent with NBD.Conclusion: NBD is a rare subset of BD with a limited number of studies and patients. However, uveitis is more common in adults with parenchymal disease; may predate the development of neurological symptoms. The most common locations of ocular inflammation were posterior and panuveitis. MRI/V of the brain can identify enhancing lesions in the rhombencephalon or CVST in patients with uveitis with neurological findings.

Community-acquired Klebsiella pneumoniae systemic infection complicated with rhombencephalitis.

Rhombencephalitis is a rapidly progressing disease that should be taken into consideration in a patient with abrupt onset of cerebellar ataxia with rapid neurologic deterioration (tetraparesis, coma) after vascular etiology has been ruled out.