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1.
Diagn Microbiol Infect Dis ; 110(1): 116418, 2024 Jun 28.
Article in English | MEDLINE | ID: mdl-38954862

ABSTRACT

Mycoplasma pneumoniae is a common cause of community-acquired pneumonia in children and young adults. It is responsible of a broad array of extrapulmonary manifestations, the most severe affecting the central nervous system. We report a challenging diagnosis of macrolide-resistant M. pneumoniae-induced Bickerstaff encephalitis in a 16-year-old man.

2.
CNS Neurosci Ther ; 30(7): e14821, 2024 Jul.
Article in English | MEDLINE | ID: mdl-38948940

ABSTRACT

AIMS: To investigate the diagnostic and predictive role of 18F-FDG PET/CT in patients with autoimmune encephalitis (AE) as a whole group. METHODS: Thrty-five patients (20 females and 15 males) with AE were recruited. A voxel-to-voxel semi-quantitative analysis based on SPM12 was used to analyze 18F-FDG PET/CT imaging data compared to healthy controls. Further comparison was made in different prognostic groups categorized by modified Rankin Scale (mRS). RESULTS: In total, 24 patients (68.6%) were tested positive neuronal antibodies in serum and/or CSF. Psychiatric symptoms and seizure attacks were major clinical symptoms. In the acute stage, 13 patients (37.1%) demonstrated abnormal brain MRI results, while 33 (94.3%) presented abnormal metabolism patterns. 18F-FDG PET/CT was more sensitive than MRI (p < 0.05). Patients with AE mainly presented mixed metabolism patterns compared to the matched controls, demonstrating hypermetabolism mainly in the cerebellum, BG, MTL, brainstem, insula, middle frontal gyrus, and relatively hypometabolism in the frontal cortex, occipital cortex, temporal gyrus, right parietal gyrus, left cingulate gyrus (p < 0.05, FWE corrected). After a median follow-up of 26 months, the multivariable analysis identified a decreased level of consciousness as an independent risk factor associated with poor outcome of AE (HR = 3.591, p = 0.016). Meanwhile, decreased metabolism of right superior frontal gyrus along with increased metabolism of the middle and upper brainstem was more evident in patients with poor outcome (p < 0.001, uncorrected). CONCLUSION: 18F-FDG PET/CT was more sensitive than MRI to detect neuroimaging abnormalities of AE. A mixed metabolic pattern, characterized by large areas of cortical hypometabolism with focal hypermetabolism was a general metabolic pattern. Decreased metabolism of right superior frontal gyrus with increased metabolism of the middle and upper brainstem may predict poor long-term prognosis of AE.


Subject(s)
Encephalitis , Fluorodeoxyglucose F18 , Positron Emission Tomography Computed Tomography , Humans , Female , Male , Positron Emission Tomography Computed Tomography/methods , Adult , Middle Aged , Encephalitis/diagnostic imaging , Encephalitis/metabolism , Young Adult , Cohort Studies , Predictive Value of Tests , Hashimoto Disease/diagnostic imaging , Hashimoto Disease/metabolism , Brain/diagnostic imaging , Brain/metabolism , Adolescent , China , Radiopharmaceuticals , Aged , Magnetic Resonance Imaging , East Asian People
3.
Heliyon ; 10(11): e32248, 2024 Jun 15.
Article in English | MEDLINE | ID: mdl-38947434

ABSTRACT

A middle-aged gentleman, presented to our outpatient department with painful skin lesions suggestive of disseminated herpes zoster. Further examination revealed bilateral cerebellar signs. He had a history of receiving a third dose of AZD1222 vaccine fourteen days prior to the onset of skin lesions but had no other significant medical history. The patient was also evaluated for retroviral infection and other immunodeficient states, workup for which were negative. The patient was initially treated with intravenous acyclovir 7.5 mg/kg/q8H; however, the patient developed varicella encephalitis on treatment, which was followed by pneumonia and haemorrhagic cystitis. Subsequently, treatment was started with acyclovir 10 mg/kg/q8H for 14 days, followed by valacyclovir for eight days, following which there was near-complete resolution of symptoms with the persistence of minimal rigidity. Although there have been several reports of herpes zoster following SARS-CoV-2 vaccination, we found few reports of varicella zoster with systemic manifestations following ChAdOx1 nCoV-19 (AZD1222) vaccination. This case highlights the importance of considering varicella zoster reactivation in a patient presenting with encephalitis or pneumonia post SARS-CoV-2 vaccination.

4.
Cureus ; 16(5): e61419, 2024 May.
Article in English | MEDLINE | ID: mdl-38947631

ABSTRACT

Varicella zoster virus (VZV) vasculopathy is a rare yet potentially severe neurological manifestation resulting from VZV reactivation, primarily affecting immunocompromised individuals. We present a case report of a 61-year-old male with VZV vasculopathy who initially presented with herpes zoster ophthalmicus, subsequently complicated by meningoencephalitis and an acute infarct in the territory of the left middle cerebral artery (MCA). Imaging revealed acute and chronic infarcts in the capsuloganglionic regions, accompanied by thickening and enhancement of the left MCA wall. Treatment involved a 14-day course of intravenous acyclovir, supplemented with oral prednisolone, resulting in modest clinical improvement. VZV vasculopathy represents an infrequently acknowledged neurological syndrome, particularly prevalent among immunocompromised individuals. Early recognition and appropriate intervention offer promise in ameliorating outcomes for affected patients. This case emphasizes the importance of including VZV vasculopathy in the differential diagnosis of neurological deficits, especially within high-risk populations.

5.
J Med Virol ; 96(7): e29763, 2024 Jul.
Article in English | MEDLINE | ID: mdl-38949193

ABSTRACT

Tick-borne encephalitis (TBE) is one of the main diseases transmitted by ticks, the incidence of which is increasing. Moreover, its diagnosis and therapy are often long and difficult according to nonspecific symptoms and complex etiology. This study aimed to observe changes in the proteome of cerebrospinal fluid from TBE patients. Cerebrospinal fluid (CSF) of TBE patients (n = 20) and healthy individuals (n = 10) was analyzed using a proteomic approach (QExactiveHF-Orbitrap mass spectrometer) and zymography. Obtained results show that in CSF of TBE patients, the top-upregulated proteins are involved in pro-inflammatory reaction (interleukins), as well as antioxidant/protective response (peroxiredoxins, heat shock proteins). Moreover, changes in the proteome of CSF are not only the result of this disease development, but they can also be an indicator of its course. This mainly applies to proteins involved in proteolysis including serpins and metalloproteinases, whose activity is proportional to the length of patients' convalescence. The obtained proteomic data strongly direct attention to the changes caused by the development of TBE to antioxidant, pro-inflammatory, and proteolytic proteins, knowledge about which can significantly contribute to faster and more accurate diagnosis of various clinical forms of TBE.


Subject(s)
Encephalitis, Tick-Borne , Proteome , Humans , Encephalitis, Tick-Borne/cerebrospinal fluid , Encephalitis, Tick-Borne/diagnosis , Proteome/analysis , Male , Female , Adult , Middle Aged , Proteomics/methods , Young Adult , Aged
6.
Neurol Sci ; 2024 Jun 29.
Article in English | MEDLINE | ID: mdl-38949742

ABSTRACT

Here we described an 18-year-old woman who were initially misdiagnosed as psychiatric disorders in a psychiatric institution. She was transferred to our neurological ward because of impaired consciousness. Neuronal antibody testing confirmed the diagnosis of anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis. Cerebral magnetic resonance imaging (MRI) revealed a concomitant disorder named reversible splenial lesion syndrome (RESLES). After administration of combined immunotherapy, the patient recovered completely 3 months after discharge. To our knowledge, co-occurrence of RESLES and anti-NMDAR encephalitis was only described in two patients with teratoma and we provide another case without teratoma. We highlight that anti-NMDAR antibodies can be added to the multiple causes of RESLES. It is therefore imperative for clinicians to detect anti-neuronal antibodies in patients with RESLES to avoid missed diagnosis.

7.
Trop Med Health ; 52(1): 45, 2024 Jul 08.
Article in English | MEDLINE | ID: mdl-38978129

ABSTRACT

BACKGROUND: Rapid and accurate diagnosis of toxoplasmosis is critical, particularly for immunocompromised patients. Several molecular methods could have value for toxoplasmosis diagnosis, but often require sophisticated and expensive equipment, and as such are impractical for use in resource-limited countries. Our study aimed to develop a new rapid diagnostic test for toxoplasmosis that can be used in developed countries as well as low- or middle-income countries. METHODS: Common primers for conventional loop-mediated isothermal amplification (LAMP) and the new LAMP DNA chromatography method were designed based on a 529-bp repeat present in Toxoplasma gondii genomic DNA. A total of 91 clinical samples from 44 patients suspected of having toxoplasmosis who were treated at several hospitals across Japan were tested using the new LAMP DNA chromatography method, conventional LAMP, and nested PCR and the sensitivity and specificity of the methods was compared. RESULTS: The LAMP DNA chromatography method showed better sensitivity and specificity (68.2% and 100%, respectively) compared with the nested PCR (45.4% and 100%, respectively) and conventional LAMP (63.6% and 100%, respectively) methods for diagnosis of toxoplasmosis in immunocompromised patients. LAMP DNA chromatography also has better sensitivity and specificity (75% and 100%, respectively) than nested PCR (50.0% and 93.5%, respectively) and conventional LAMP (62.5% and 100%, respectively) to diagnose toxoplasma encephalitis using CSF samples. CONCLUSION: We developed a LAMP DNA chromatography method to detect T. gondii DNA in clinical samples. This method also successfully detected T. gondii DNA in CSF from patients with toxoplasma encephalitis. This newly developed method can be a valuable rapid diagnostic test for toxoplasmosis in a range of settings, including resource-limited areas like those in low- or middle-income countries.

8.
J Neurol ; 2024 Jul 09.
Article in English | MEDLINE | ID: mdl-38981871

ABSTRACT

BACKGROUND: Anti leucine-rich, glioma inactivated 1 (LGI1) antibody-associated autoimmune encephalitis (AE) is the second most common AE, where the trafficking and recycling of the pathogenic immunoglobulin (IgG) can be controlled by the neonatal crystallizable fragment receptor (FcRn), making the latter as a candidate therapeutic target. Efgartigimod is an antagonist of FcRn, its ability to increase the degradation of IgGs and improve the health and quality of life of patients. ADAPT trail indicated its rapid efficacy and safety on myasthenia gravis. However, there is currently no case reported using efgartigimod for the treatment of anti-LGI1-associated AE. CASE DESCRIPTION: The patient presented with five episodes of generalized tonic-clonic seizures in the past 2 weeks. The patient had no abnormal signs on magnetic resonance imaging. Electroencephalogram examinations showed an increase in bilateral symmetric or asymmetric slow activity, without any clear epileptic waves. The cerebrospinal fluid (CSF) examination results indicated a slight increase in protein (47 mg/dL). The anti-LGI1 antibody titer in serum was 1:100 and that in CSF was 1:3.2. The treatment with intravenous methylprednisolone 1000 mg once a day combined with levetiracetam tablets failed to completely control the patient's seizures. Thus, 10 mg/kg efgartigimod was administered intravenously once a week for 2 weeks. After 2 weeks of treatment, serum levels of anti-LGI1 antibody and IgG decreased and the patient's epilepsy did not recur in the next 3 months. CONCLUSIONS: This is the first case report of using efgartigimod to treat anti-LGI1-associated AE. The combination of efgartigimod and methylprednisolone resulted in favorable outcomes, indicating that this is an optional treatment plan.

9.
Thorac Cancer ; 2024 Jul 10.
Article in English | MEDLINE | ID: mdl-38984426

ABSTRACT

Anti-gamma-aminobutyric acid receptor type A (GABAA) encephalitis is a relatively rare autoimmune encephalitis, and often associated with thymoma. Here, a 44-year-old female was diagnosed as having a thymoma with autoimmune encephalitis. At 4-month follow-up she was without recurrence of symptoms after treatment with methylprednisolone pulse therapy and immunotherapy. This case report provides a reference for the identification of this type of paraneoplastic encephalitis and for a therapeutic schedule. It also highlights that conservative treatment may be effective for patients with a tumor and GABAA encephalitis.

10.
Int J Biol Macromol ; 275(Pt 2): 133720, 2024 Jul 08.
Article in English | MEDLINE | ID: mdl-38987000

ABSTRACT

Tick-borne encephalitis virus (TBEV), a zoonotic pathogen, can cause severe neurological complications and fatal outcomes in humans. Early diagnosis of TBEV infection is crucial for clinical practice. Although serological assays are frequently employed for detection, the lack of antibodies in the early stages of infection and the cross-reactivity of antibodies limit their efficacy. Conventional molecular diagnostic methods such as RT-qPCR can achieve early and accurate identification but require specialized instrumentation and professionals, hindering their application in resource-limited areas. Our study developed a rapid and visual TBEV molecular detection method by combining RT-recombinase-aided amplification, the CRISPR/Cas13a system, and lateral flow dipsticks. The diagnostic sensitivity of this method is 50 CFU/ml, with no cross-reactivity with a variety of viruses. The detection can be carried out within 1 h at a temperature between 37 and 42 °C, and the results can be visually determined without the need for complex instruments and professionals. Subsequently, this assay was used to analyze clinical samples from 15 patients suspected of TBEV infection and 10 healthy volunteers, and its sensitivity and specificity reached 100 %, which was consistent with the results of RT-qPCR. These results indicate that this new method can be a promising point-of-care test for the diagnosis of tick-borne encephalitis.

11.
Cureus ; 16(6): e62056, 2024 Jun.
Article in English | MEDLINE | ID: mdl-38989350

ABSTRACT

COVID-19 might present with a wide range of clinical manifestations, from mild respiratory distress to severe multi-organ dysfunction. We present a unique case of complex COVID-19 presentation in a 45-year-old female who initially developed general symptoms such as fever, cough, headache, and weakness, which escalated to coma, requiring intubation and ICU admission. A brain MRI revealed lesions compatible with encephalitis, the cause of which remained unexplained after an in-depth clinical, laboratory, and imaging investigation. While in the ICU, the patient also developed cardiac tamponade, requiring pericardiocentesis, and atypical electrocardiographic changes. After treatment with steroids, her condition improved, and the patient was extubated and transferred to the ward. Upon checkup, cardiac MRI revealed fibrous tissue in the inferior cardiac wall and the adjacent intraventricular septum. In the absence of an alternative diagnosis, it might be important to consider the central nervous system and cardiac involvement in patients with COVID-19.

12.
Clin Neurol Neurosurg ; 244: 108422, 2024 Jul 03.
Article in English | MEDLINE | ID: mdl-38991392

ABSTRACT

We presented a case of a 34-year-old male with postoperative brainstem cavernous malformations complicated with LGI1 encephalitis and secondary hypertrophic olivary degeneration (HOD). Due to recurrent dizziness and headache, the patient was diagnosed as brainstem cavernous malformations with recurrent hemorrhage and underwent resection. He subsequently developed unexplained abnormal mental behavior 1 month after the surgery, and diagnosed with LGI1 encephalitis. Six months later, cranial MRI showed HOD. This condition is rare in clinical practice,and a complex mechanism underlies the occurrence.

13.
Cureus ; 16(6): e61899, 2024 Jun.
Article in English | MEDLINE | ID: mdl-38975432

ABSTRACT

Mild encephalitis/encephalopathy with reversible splenial lesion (MERS) is characterized by mild neurological manifestations associated with spontaneously reversible lesions of the splenium of the corpus callosum. While various conditions and diseases can trigger MERS, infectious causes predominate, with mumps being notably linked to MERS in the pediatric population. Although rare in adults, there are sporadic case reports associating mumps with MERS. Here we report a 23-year-old male patient with a typical presentation of mumps who presented with meningeal syndrome, dizziness, seizures, and right orchitis. Brain MRI showed classic findings of MERS syndrome while cerebrospinal fluid analysis demonstrated lymphocytic pleocytosis. Our patient had a confirmed diagnosis of mumps disease with multiple complications, including MERS, meningitis, and orchitis, and was managed with symptomatic medications and antiviral therapy. Subsequently, there was a gradual resolution of these manifestations and the outcome was favorable, with no residual sequelae.

14.
J Neurol ; 2024 Jul 08.
Article in English | MEDLINE | ID: mdl-38977462

ABSTRACT

BACKGROUND: Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is characterized by distinct structural and functional brain alterations, predominantly affecting the medial temporal lobes and the hippocampus. Structural connectome analysis with graph-based investigations of network properties allows for an in-depth characterization of global and local network changes and their relationship with clinical deficits in NMDAR encephalitis. METHODS: Structural networks from 61 NMDAR encephalitis patients in the post-acute stage (median time from acute hospital discharge: 18 months) and 61 age- and sex-matched healthy controls (HC) were analyzed using diffusion-weighted imaging (DWI)-based probabilistic anatomically constrained tractography and volumetry of a selection of subcortical and white matter brain volumes was performed. We calculated global, modular, and nodal graph measures with special focus on default-mode network, medial temporal lobe, and hippocampus. Pathologically altered metrics were investigated regarding their potential association with clinical course, disease severity, and cognitive outcome. RESULTS: Patients with NMDAR encephalitis showed regular global graph metrics, but bilateral reductions of hippocampal node strength (left: p = 0.049; right: p = 0.013) and increased node strength of right precuneus (p = 0.013) compared to HC. Betweenness centrality was decreased for left-sided entorhinal cortex (p = 0.042) and left caudal middle frontal gyrus (p = 0.037). Correlation analyses showed a significant association between reduced left hippocampal node strength and verbal long-term memory impairment (p = 0.021). We found decreased left (p = 0.013) and right (p = 0.001) hippocampal volumes that were associated with hippocampal node strength (left p = 0.009; right p < 0.001). CONCLUSIONS: Focal network property changes of the medial temporal lobes indicate hippocampal hub failure that is associated with memory impairment in NMDAR encephalitis at the post-acute stage, while global structural network properties remain unaltered. Graph theory analysis provides new pathophysiological insight into structural network changes and their association with persistent cognitive deficits in NMDAR encephalitis.

15.
J Neuroimmunol ; 393: 578398, 2024 Jun 20.
Article in English | MEDLINE | ID: mdl-39002186

ABSTRACT

The classification of autoimmune encephalitis (AE) is based on the presence of different types of antibodies. Currently, the clinical manifestations and treatment regimens of patients with all types of AE exhibit similarities. However, the presence of immunological distinctions among different types of AE remains uncertain. In this study, we prospectively collected clinical data, as well as blood and cerebrospinal fluid (CSF) samples from patients diagnosed with MOG antibody-associated disease (MOGAD) or GFAP astrocytopathy (GFAP-A), in order to assess changes in inflammatory biomarkers such as immunoglobulin oligoclonal bands, cytokines in serum and CSF, as well as peripheral blood lymphocyte subtypes within different subsets. To further distinguish the immune response in patients with MOGAD and GFAP-A from that of healthy individuals, we prospectively recruited 20 hospitalized patients diagnosed with AE. Among them, 15 (75%) tested positive for MOG antibodies, 4 (20%) tested positive for GFAP antibodies, and 1 (5%) tested positive for both MOG and GFAP antibodies. These patients were then followed up for a period of 18 months. Compared to healthy controls (HC), AE patients exhibited elevated levels of MIP-1beta, SDF-1alpha, IL-12p70, IL-5, IL-1RA, IL-8 and decreased levels of IL-23, IL-31, IFN-alpha, IL-7, TNF-beta and TNF-alpha in serum. The CSF of AE patients showed increased levels of IL-1RA, IL-6 and IL-2 while decreased levels of RANTES, IL-18,IL-7,TNF-beta,TNF-alpha,RANTES,Eotaxin,and IL-9. The level of MCP-1 in the CSF of GFAP-A patients was found to be lower compared to that of MOGAD patients, while RANTES levels were higher. And the levels of IL-17A, Eotaxin, GRO-alpha, IL-8, IL-1beta, MIP-1beta were higher in the CSF of patients with epilepsy. The presence of intrathecal immune responses is also observed in patients with spinal muscular atrophy (SMA). However, no biomarker was found to be associated with disease severity in patients with AE. Among the 17 patients, recovery was observed, while 2 patients experienced persistent symptoms after an 18-month follow-up period. Additionally, within one year of onset, 8 patients had a single recurrence. Therefore, the immunological profiles of MOGAD and GFAP-A patients differ from those of normal individuals, and the alterations in cytokine levels may also exhibit a causal association with the clinical presentations, such as seizure.

16.
Neurol Sci ; 2024 Jul 11.
Article in English | MEDLINE | ID: mdl-38987510

ABSTRACT

INTRODUCTION: Since the initial identification of Miller Fisher syndrome (MFS) and Bickerstaff brainstem encephalitis (BBE),significant milestones have been achieved in understanding these diseases.Discoveries of common serum antibodies (IgG anti-GQ1b), antecedent infections, neurophysiological data, andneuroimaging suggested a shared autoimmune pathogenetic mechanism rather than distinct pathogenesis, leadingto the hypothesis that both diseases are part of a unified syndrome, termed "Fisher-Bickerstaff syndrome". The subsequent identification of atypical anti-GQ1b-positive forms expanded the classification to a broader condition known as "Anti-GQ1b-Antibody syndrome". METHODS: An exhaustive literature review was conducted, analyzing a substantial body of research spanning from the initialdescriptions of the syndrome's components to recent developments in diagnostic classification and researchperspectives. RESULTS: Anti-GQ1b syndrome encompasses a continuous spectrum of conditions defined by a common serological profilewith varying degrees of peripheral (PNS) and central nervous system (CNS) involvement. MFS and BBE represent theopposite ends of this spectrum, with MFS primarily affecting the PNS and BBE predominantly involving the CNS.Recently identified atypical forms, such as acute ophthalmoparesis, acute ataxic neuropathy withoutophthalmoparesis, Guillain-Barré syndrome (GBS) with ophthalmoparesis, MFS-GBS and BBE-GBS overlap syndromes,have broadened this spectrum. CONCLUSION: This work aims to provide an extensive, detailed, and updated overview of all aspects of the anti-GQ1b syndromewith the intention of serving as a stepping stone for further shaping thereof. Special attention was given to therecently identified atypical forms, underscoring their significance in redefining the boundaries of the syndrome.

17.
Mol Ther ; 2024 Jul 02.
Article in English | MEDLINE | ID: mdl-38956870

ABSTRACT

Several viruses hijack various forms of endocytosis in order to infect host cells. Here, we report the discovery of a molecule with antiviral properties that we named virapinib, which limits viral entry by macropinocytosis. The identification of virapinib derives from a chemical screen using high-throughput microscopy, where we identified chemical entities capable of preventing infection with a pseudotype virus expressing the spike (S) protein from SARS-CoV-2. Subsequent experiments confirmed the capacity of virapinib to inhibit infection by SARS-CoV-2, as well as by additional viruses, such as mpox virus and TBEV. Mechanistic analyses revealed that the compound inhibited macropinocytosis, limiting this entry route for the viruses. Importantly, virapinib has no significant toxicity to host cells. In summary, we present the discovery of a molecule that inhibits macropinocytosis, thereby limiting the infectivity of viruses that use this entry route such as SARS-CoV2.

18.
World J Pediatr ; 2024 Jul 12.
Article in English | MEDLINE | ID: mdl-38997604

ABSTRACT

BACKGROUND: Following the standardized nomenclature proposed by the American Clinical Neurophysiology Society (ACNS), rhythmic high-amplitude delta activity with superimposed spikes (RHADS) can be reported as an extreme delta brush (EDB). The clinical implications of similar electrographic patterns being reported as RHADS versus EDB are important to highlight. We aim to review the electrographic characteristics of RHADS, evaluate whether RHADS is seen in other neurological disorders, and identify the similar and unique characteristics between RHADS and EDB to ultimately determine the most accurate way to differentiate and report these patterns. We believe that the differentiation of RHADS and EDB is important as there is a vast difference in the diagnostic approach and the medical management of associated underlying etiologies. DATA SOURCE: We conducted an extensive search on MEDLINE and Pubmed utilizing various combinations of keywords. Searching for "gamma polymerase and EEG", or "RHADS" or "Alpers syndrome and EEG" or "EEG" AND "Alpers-Huttenlocher syndrome". RESULTS: Three articles were found to be focused on the description of "RHADS" pattern in Alpers Syndrome. No publication to date were found when searching for the terms "EDB" AND "children", AND "infant" AND "adolescent" excluding "encephalitis" and "neonate". Although RHADS and EDB appear as similar EEG patterns, meticulous analysis can differentiate them. RHADS is not exclusive to patients with Alpers-Huttenlocher syndrome and may manifest in regions beyond the posterior head region. Reactivity to eye-opening and response to anesthesia can be two other elements that help in the differentiation of these patterns. CONCLUSION: RHADS is not exclusive to patients with AHS and may manifest in regions beyond the posterior head region. Reactivity to eye-opening and response to anesthesia are features that help in the differentiation of these patterns.

19.
Cancers (Basel) ; 16(13)2024 Jul 04.
Article in English | MEDLINE | ID: mdl-39001514

ABSTRACT

Autoantibodies targeting the neuronal antigen metabotropic glutamate receptor 5 (mGluR5) have been identified in patients with Ophelia syndrome, which describes a co-occurrence of paraneoplastic limbic encephalitis and Hodgkin lymphoma (HL). Little data exist regarding frequency and function of mGluR5 in HL and its potential role in causing seropositive paraneoplastic disease. We studied a representative cohort of pediatric HL and NHL patients (n = 57) using immunohistochemistry and fluorescence staining to investigate mGluR5 expression. All lymphoma tissues displayed positive mGluR5 staining, with focus on Hodgkin-Reed-Sternberg (H-RS) cells. We did not detect any mGluR5 staining in tumor-free lymph nodes, which is consistent with the absence of GRM5 transcripts in RNA-sequencing data from non-malignant B and T cells. The frequent presence in pediatric lymphoma falls in line with reports of mGluR5 expression and associated tumor progression in other malignancies. We tested for correlation with clinical features, focusing on disease progression and neurological symptoms. Low mGluR5 expression in H-RS cells correlated with young patient age (<15 years) and positive histology for EBV infection. Paraneoplastic or neurological symptoms were found exclusively in HL patients. While an impact of mGluR5 on HL severity remains possible, a prognostic value of mGluR5 expression levels requires further investigation.

20.
IDCases ; 37: e02027, 2024.
Article in English | MEDLINE | ID: mdl-39045035

ABSTRACT

HSV-1 encephalitis (HSE) is the most common cause of fatal sporadic encephalitis in the United States. HSE in adults is most commonly due to the reactivation of HSV in the central nervous system (CNS) which results in CNS necrosis leading to neurological compromise. The most common symptoms include altered mentation, fever, seizures, and focal neurological deficits. HSE most commonly involves damage to the temporal lobes however can rarely involve other CNS structures such as the brainstem and cerebellum. Immunocompromised status may increase the risk of atypical HSE. HSE involvement of the brainstem, particularly the pons, most commonly cause neuro-ocular and neuro-bulbar deficits. Rarely can HSV brainstem encephalitis cause quadriplegia or locked-in syndrome. We present a case of HSV-1 rhombencephalitis complicated by locked-in syndrome in a patient with CLL.

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