[Meningitis and myelitis by hypervirulent Klebsiella pneumoniae]. / Meningitis y mielitis por Klebsiella pneumoniae hipervirulenta.

Invasive liver abscess syndrome caused by hypervirulent Klebsiella. pneumoniae is a rare disease. This type of K. pneumoniae is aggressive and invasive, despite its sensitivity profile. We report the case of a 62-year-old man with diabetes mellitus, who was admitted to our hospital with meningeal syndrome. Within 24 hours of admission, Gram negative bacilli were isolated blood and cerebrospinal fluid cultures, which were later identified as K. pneumoniae. Liver abscess was detected by computed tomography. Despite early antibiotic treatment, the patient developed back pain that prevented him from moving and right hemiparesis. Increased signal from the central region of the spinal medulla compatible with myelitis was identified by magnetic resonance, for which he received methylprednisolone 1 g/day for 5 days. The patient evolved favorably. Infections caused by hypermucoviscous K. pneumoniae are aggressive and invasive, and more common in men with a history of diabetes mellitus, as in this case. These infections require early antibiotic treatment and the search of metastatic infections.

El síndrome de absceso hepático invasivo causado por cepas hipermucoviscosas de Klebsiella pneumoniae es una enfermedad poco frecuente. Esta serovariedad de Klebsiella se caracteriza por ser agresiva e invasiva pese a su perfil de sensibilidad. Se presenta el caso de un varón de 62 años con antecedentes de diabetes mellitus, que ingresó a nuestro centro con síndrome meníngeo. A las 24 horas del ingreso se aislaron en hemocultivos y en líquido cefalorraquídeo (LCR) bacilos Gram negativos que luego fueron tipificados como Klebsiella pneumoniae. Se identificó la presencia de un absceso hepático mediante tomografía computarizada. Pese al tratamiento antibiótico instaurado de manera temprana, el paciente evolucionó con dolor dorsal que le impedía movilizarse y hemiparesia derecha. En la resonancia magnética nuclear de columna se identificó aumento de la señal de la región central de la médula espinal compatible con mielitis por lo cual recibió tratamiento con metilprednisolona 1g/día por 5 días consecutivos. El paciente evolucionó de manera favorable. Las infecciones por K. pneumoniae hipermucoviscosas son agresivas e invasoras y más frecuentes en varones con antecedentes de diabetes mellitus, como en este caso. Su control requiere de un tratamiento antibiótico temprano y búsqueda de focos a distancia.

A tropical myelitis.

Spinal cord inflammation is a rare presentation of schistosomiasis infection. The present report describes the case of a young patient presenting subacute medullary symptoms revealing extensive longitudinal myelitis related to schistosomiasis, also known as bilharzia. The diagnosis was based on detection of parasite eggs in stool. The patient was treated with Praziquantel, corticosteroids and plasma exchanges, leading to a favorable clinical course.

Clinical characteristics of overlapping syndrome in patients with GFAP-IgG and MOG-IgG: a case series of 8 patients and literature review.

OBJECTIVE: The overlapping syndrome of anti-GFAP and anti-MOG antibodies is extremely rare. This retrospective study reports 8 adult cases of the GFAP-MOG overlapping syndrome. METHODS: We reviewed the clinical characteristics of 8 adult patients with the GFAP-MOG overlapping syndrome from Jan 2019 and Sep 2023 at the Third Affiliated Hospital, Sun Yat-sen University. Moreover, we searched the literature and included all case reports with this overlapping syndrome since 2018 on PubMed. RESULTS: The predominant clinical syndrome was meningoencephalomyelitis (5/8), followed by meningoencephalitis (2/8), and myelitis (1/8). Five patients had a flu-like prodromal symptom or diarrhea. No neoplasms were found in these patients. Regarding brain MRI, T2-weighted/fluid-attenuated inversion recovery hyperintensities were in 7 patients and leptomeningeal enhancement was in 4 patients. However, only one patient had periventricular radial linear enhancement. Besides, two patients had large space-occupying lesions. For spinal MRI, T2-hyperintensities were observed in 4 patients, in which 3 patients had longitudinally extensive lesions. All patients were treated with immunotherapy, the median follow-up period was 18 months (range, 3-36 months). Three patients presented relapses during the follow-up, but all cases recovered to mRS scores ≤ 2 at last follow-up. In addition, we also reviewed 14 cases (including 7 adults and 7 children) with this overlapping syndrome by literature review. CONCLUSION: Our findings provide data to understand the clinical features and prognosis of the GFAP-MOG overlapping syndrome. Recognizing this overlapping syndrome will expand our knowledge, allowing for better management of these patients.

[Clinical characteristics analysis of 4 cases with acute flaccid myelitis in children].

Objective: To summarize the clinical manifestations, diagnosis, treatment and prognosis of acute flaccid myelitis (AFM) in children. Methods: Clinical characteristics of 4 AFM cases from Department of Neurology, Children's Hospital Affiliated to Capital Institute of Pediatrics, from September 2018 to November 2022, were analyzed retrospectively. Results: The age of 4 children with AFM was 7 years, 4 years and 3 months, 7 years and 1 month, 6 years and 5 months, respectively. There were 2 boys and 2 girls. Prodromal infection status showed 3 children of respiratory tract infection and 1 child of digestive tract infection. The main manifestation was asymmetrical limb weakness after infection, and the affected limb range was from monoplegia to quadriplegia. Cranial nerve injury was involved in 1 child, no encephalopathy. Magnetic resonance imaging in the spinal cord of all 4 children showed long T1 and T2 signals, mainly involving gray matter. Cerebrospinal fluid cell-protein separation was observed in 2 children. Pathogen detected in 1 child pharyngeal swab was enterovirus D68. Antibody IgM to adenovirus was positive in the blood of 1 child. Antibody IgG against Echo and Coxsackie B virus were positive in the blood of another child. After glucocorticoid, human immunoglobulin or simple symptomatic treatment and at the same time under later rehabilitation training, muscle strength recovered to different degrees, but there were disabilities left in 3 children. Conclusions: AFM should be considered in children with acute and asymmetrical flaccid paralysis accompanied by abnormal magnetic resonance imaging signal in the central region of spinal cord, especially post-infection. The effective treatment is limited and the prognosis is poor.

VP1 is the primary determinant of neuropathogenesis in a mouse model of enterovirus D68 acute flaccid myelitis.

Enterovirus D68 (EV-D68) is an emerging pathogen that can cause severe respiratory and neurologic disease [acute flaccid myelitis (AFM)]. Intramuscular (IM) injection of neonatal Swiss Webster (SW) mice with US/IL/14-18952 (IL52), a clinical isolate from the 2014 EV-D68 epidemic, results in many of the pathogenic features of human AFM, including viral infection of the spinal cord, death of motor neurons, and resultant progressive paralysis. In distinction, CA/14-4231 (CA4231), another clinical isolate from the 2014 EV-D68 outbreak, does not cause paralysis in mice, does not grow in the spinal cord, and does not cause motor neuron loss following IM injection. A panel of chimeric viruses containing sequences from IL52 and CA4231 was used to demonstrate that VP1 is the main determinant of EV-D68 neurovirulence following IM injection of neonatal SW mice. VP1 contains four amino acid differences between IL52 and CA4231. Mutations resulting in substituting these four amino acids (CA4231 residues into the IL52 polyprotein) completely abolished neurovirulence. Conversely, mutations resulting in substituting VP1 IL52 amino acid residues into the CA4231 polyprotein created a virus that induced paralysis to the same degree as IL52. Neurovirulence following infection of neonatal SW mice with parental and chimeric viruses was associated with viral growth in the spinal cord. IMPORTANCE: Emerging viruses allow us to investigate mutations leading to increased disease severity. Enterovirus D68 (EV-D68), once the cause of rare cases of respiratory illness, recently acquired the ability to cause severe respiratory and neurologic disease. Chimeric viruses were used to demonstrate that viral structural protein VP1 determines growth in the spinal cord, motor neuron loss, and paralysis following intramuscular (IM) injection of neonatal Swiss Webster (SW) mice with EV-D68. These results have relevance for predicting the clinical outcome of future EV-D68 epidemics as well as targeting retrograde transport as a potential strategy for treating virus-induced neurologic disease.

Magnetic Resonance Imaging/cerebrospinal Fluid-negative Myelitis Following COVID-19 with a Dramatic Response to Early Aggressive Immunosuppressive Therapy.

A 57-year-old man presented with subacute-onset paraparesis, bilateral dysesthesia in his lower extremities, and bladder/bowel disturbance six weeks after being infected with severe acute respiratory syndrome coronavirus 2 infection [coronavirus disease 2019 (COVID-19)]. A neurological examination suggested transverse myelitis at the level of the lower thoracic spinal cord. However, repeated spinal magnetic resonance imaging (MRI) showed no abnormalities in the spinal cord. Laboratory and cerebrospinal fluid (CSF) tests ruled out other etiologies of myelitis, eventually suggesting COVID-19-associated myelitis. Aggressive immunosuppressive therapy, started soon after hospitalization, dramatically improved his symptoms. Early aggressive immunosuppressive therapy should therefore be considered in cases of MRI/CSF-negative myelitis associated with COVID-19.

Dengue-associated longitudinally extensive transverse myelitis.

We diagnosed a patient with dengue fever who developed acute onset of sensorimotor quadriparesis with bladder involvement, and facial nerve involvement. Despite initial negative results in routine investigations and cerebrospinal fluid analysis, spinal MRI confirmed longitudinally extensive transverse myelitis. The aetiological workup was negative, prompting an investigation into the presence of dengue in the cerebrospinal fluid, which returned positive. This case underscores the importance of considering rare neurological complications in dengue, the value of advanced diagnostic techniques and the potential effectiveness of tailored interventions in challenging cases.

Síndrome de Brown-Séquard como forma de presentación de mielitis transversa idiopática / Brown-Séquard syndrome as a presentation of idiopathic transverse myelitis

El síndrome de Brown-Séquard es el conjunto de signos y síntomas causado por hemisección medular de diversos orígenes. Puede generarse por múltiples causas; las traumáticas son las más frecuentes. Las causas menos frecuentes son patología inflamatoria, isquémica, tumoral o infecciosa. Se presenta un niño de 12 años, con instauración aguda y progresiva de un síndrome de hemisección medular derecho, con parálisis hipo/arrefléctica homolateral y afectación de sensibilidad termoalgésica contralateral. En la resonancia magnética de médula espinal, se observó compromiso inflamatorio en hemimédula derecha a nivel de segunda y tercera vértebras torácicas. Con diagnóstico de mielitis transversa idiopática, inició tratamiento con corticoide intravenoso a altas dosis con evolución clínica favorable y restitución de las funciones neurológicas.

Brown-Séquard syndrome refers to a set of signs and symptoms caused by hemisection of the spinal cord from various sources. It may have multiple causes; traumatic injuries are the most frequent ones. The less common causes include inflammation, ischemia, tumors, or infections. This report is about a 12-year-old boy with an acute and progressive course of right hemisection of the spinal cord, with ipsilateral hypo/areflexic paralysis and contralateral loss of thermalgesic sensation. The MRI of the spinal cord showed inflammation in the right side of the spinal cord at the level of the second and third thoracic vertebrae. The patient was diagnosed with idiopathic transverse myelitis and was started on intravenous high-dose corticosteroids; he showed a favorable clinical course and recovered neurological functions.

Metabolic and Toxic Myelopathies.

OBJECTIVE: This article reviews the clinical presentation, diagnostic evaluation, and treatment of metabolic and toxic myelopathies resulting from nutritional deficiencies, environmental and dietary toxins, drugs of abuse, systemic medical illnesses, and oncologic treatments. LATEST DEVELOPMENTS: Increased use of bariatric surgery for obesity has led to higher incidences of deficiencies in nutrients such as vitamin B12 and copper, which can cause subacute combined degeneration. Myelopathies secondary to dietary toxins including konzo and lathyrism are likely to become more prevalent in the setting of climate change leading to drought and flooding. Although modern advances in radiation therapy techniques have reduced the incidence of radiation myelopathy, patients with cancer are living longer due to improved treatments and may require reirradiation that can increase the risk of this condition. Immune checkpoint inhibitors are increasingly used for the treatment of cancer and are associated with a wide variety of immune-mediated neurologic syndromes including myelitis. ESSENTIAL POINTS: Metabolic and toxic causes should be considered in the diagnosis of myelopathy in patients with particular clinical syndromes, risk factors, and neuroimaging findings. Some of these conditions may be reversible if identified and treated early, requiring careful history, examination, and laboratory and radiologic evaluation for prompt diagnosis.

Central nervous system immune-related disorders after SARS-CoV-2 vaccination: a multicenter study.

Background: COVID-19 vaccines have been approved due to their excellent safety and efficacy data and their use has also permitted to reduce neurological complications of SARS-CoV-2. However, clinical trials were underpowered to detect rare adverse events. Herein, the aim was to characterize the clinical spectrum and immunological features of central nervous system (CNS) immune-related events following SARS-CoV-2 vaccination. Methods: Multicenter, retrospective, cohort study (December 1, 2020-April 30, 2022). Inclusion criteria were (1) de novo CNS disorders developing after SARS-CoV-2 vaccination (probable causal relationship as per 2021 Butler criteria) (2); evidence for an immune-mediated etiology, as per (i) 2016 Graus criteria for autoimmune encephalitis (AE); (ii) 2015 Wingerchuk criteria for neuromyelitis optica spectrum disorders; (iii) criteria for myelitis. Results: Nineteen patients were included from 7 tertiary referral hospitals across Italy and France (one of them being a national referral center for AE), over almost 1 year and half of vaccination campaign. Vaccines administered were mRNA-based (63%) and adenovirus-vectored (37%). The median time between vaccination and symptoms onset was 14 days (range: 2-41 days). CSF was inflammatory in 74%; autoantibodies were detected in 5%. CSF cytokine analysis (n=3) revealed increased CXCL-10 (IP-10), suggesting robust T-cell activation. The patients had AE (58%), myelitis (21%), acute disseminated encephalomyelitis (ADEM) (16%), and brainstem encephalitis (5%). All patients but 2 received immunomodulatory treatment. At last follow-up (median 130 days; range: 32-540), only one patient (5%) had a mRS>2. Conclusion: CNS adverse events of COVID-19 vaccination appear to be very rare even at reference centers and consist mostly of antibody-negative AE, myelitis, and ADEM developing approximately 2 weeks after vaccination. Most patients improve following immunomodulatory treatment.

Predictive Factors Associated With Radiation Myelopathy in Pediatric Patients With Cancer: A PENTEC Comprehensive Review.

PURPOSE: Radiation myelitis (RM) is a rare complication of radiation therapy (RT). The Pediatric Normal Tissue Effects in the Clinic spinal cord task force aimed to identify RT dose effects and assess risk factors for RM in children. Through systematic review, we analyzed RT dose, fraction size, latency between completion of RT and toxicity, chemotherapy use, age when irradiated, and sex. METHODS AND MATERIALS: We conducted literature searches of peer-reviewed manuscripts published from 1964 to June 2017 evaluating RM among children. Normality of variables was assessed with Kolmogorov-Smirnov or Shapiro-Wilk tests. Spearman's rank correlation coefficients were used to test correlations between RT dose/fraction size and latency between RT and development of toxicity. RESULTS: Of 1329 identified and screened reports, 144 reports were fully reviewed and determined to have adequate data for analysis; 16 of these reports had a total of 33 cases of RM with a median age of 13 years (range, 0.2-18) at the time of RT. The most common primary tumor histologies were rhabdomyosarcoma (n = 9), medulloblastoma (n = 5), and Hodgkin lymphoma (n = 2); the most common chemotherapy agents given were vincristine (n = 15), intrathecal methotrexate (n = 12), and intrathecal cytarabine (n = 10). The median RT dose and fraction size were 40 Gy (range, 24-57.4 Gy) and 1.8 Gy (range, 1.3-2.6 Gy), respectively. RT dose resulting in RM in patients who also received chemotherapy was lower than in those not receiving chemotherapy (mean 39.6 vs 49.7 Gy; P = .04). There was no association of age with RT dose. The median latency period was 7 months (range, 1-29). Higher RT dose was correlated with longer latency periods (P = .03) to RM whereas sex, age, fraction size, and chemotherapy use were not. Two of 17 patients with adequate follow-up recovered from RM; unfortunately, it was fatal in 6 of 15 evaluable patients. Complication probability modeling was not possible because of the rarity of events. CONCLUSIONS: This report demonstrates a relatively short latency from RT (with or without chemotherapy) to RM and a wide range of doses (including fraction sizes) associated with RM. No apparent association with age at the time of RT could be discerned. Chemotherapy appears to reduce spinal cord tolerance. Recovery from RM is rare, and it is often fatal.

Non-Polio Enteroviruses Isolated by Acute Flaccid Paralysis Surveillance Laboratories in the Russian Federation in 1998-2021: Distinct Epidemiological Features of Types.

More than 100 types of non-polio enteroviruses (NPEVs) are ubiquitous in the human population and cause a variety of symptoms ranging from very mild to meningitis and acute flaccid paralysis (AFP). Much of the information regarding diverse pathogenic properties of NPEVs comes from the surveillance of poliovirus, which also yields NPEV. The analysis of 265 NPEV isolations from 10,433 AFP cases over 24 years of surveillance and more than 2500 NPEV findings in patients without severe neurological lesions suggests that types EV-A71, E13, and E25 were significantly associated with AFP. EV-A71 was also significantly more common among AFP patients who had fever at the onset and residual paralysis compared to all AFP cases. In addition, a significant disparity was noticed between types that were common in humans (CV-A2, CVA9, EV-A71, E9, and E30) or in sewage (CVA7, E3, E7, E11, E12, and E19). Therefore, there is significant evidence of non-polio viruses being implicated in severe neurological lesions, but further multicenter studies using uniform methodology are needed for a definitive conclusion.

Brown-Séquard syndrome as a presentation of idiopathictransverse myelitis. / Síndrome de Brown-Séquard como forma de presentación de mielitis transversa idiopática.

Brown-Séquard syndrome refers to a set of signs and symptoms caused by hemisection of the spinal cord from various sources. It may have multiple causes; traumatic injuries are the most frequent ones. The less common causes include inflammation, ischemia, tumors, or infections. This report is about a 12-year-old boy with an acute and progressive course of right hemisection of the spinal cord, with ipsilateral hypo/areflexic paralysis and contralateral loss of thermalgesic sensation. The MRI of the spinal cord showed inflammation in the right side of the spinal cord at the level of the second and third thoracic vertebrae. The patient was diagnosed with idiopathic transverse myelitis and was started on intravenous high-dose corticosteroids; he showed a favorable clinical course and recovered neurological functions.

El síndrome de Brown-Séquard es el conjunto de signos y síntomas causado por hemisección medular de diversos orígenes. Puede generarse por múltiples causas; las traumáticas son las más frecuentes. Las causas menos frecuentes son patología inflamatoria, isquémica, tumoral o infecciosa. Se presenta un niño de 12 años, con instauración aguda y progresiva de un síndrome de hemisección medular derecho, con parálisis hipo/arrefléctica homolateral y afectación de sensibilidad termoalgésica contralateral. En la resonancia magnética de médula espinal, se observó compromiso inflamatorio en hemimédula derecha a nivel de segunda y tercera vértebras torácicas. Con diagnóstico de mielitis transversa idiopática, inició tratamiento con corticoide intravenoso a altas dosis con evolución clínica favorable y restitución de las funciones neurológicas.

Neurological and neuromuscular manifestations in patients with West Nile neuroinvasive disease, Belgrade area, Serbia, season 2022.

INTRODUCTION: We aimed to describe neurological manifestations and functional outcome at discharge in patients with West Nile neuroinvasive disease. METHODS: This retrospective study enrolled inpatients treated in the University Clinic for Infectious and Tropical Diseases in Belgrade, Serbia, from 1 June until 31 October 2022. Functional outcome at discharge was assessed using modified Rankin scale. RESULTS: Among the 135 analyzed patients, encephalitis, meningitis and acute flaccid paralysis (AFP) were present in 114 (84.6%), 20 (14.8%), and 21 (15.6%), respectively. Quadriparesis/quadriplegia and monoparesis were the most frequent forms of AFP, present in 9 (6.7%) and 6 (4.4%) patients, respectively. Fourty-five (33.3%) patients had cerebellitis, 80 (59.3%) had rhombencephalitis, and 5 (3.7%) exhibited Parkinsonism. Ataxia and wide-based gait were present in 79 (58.5%) patients each. Fifty-one (37.8%) patients had tremor (41 (30.3%) had postural and/or kinetic tremor, 10 (7.4%) had resting tremor). Glasgow coma score (GCS) ≤ 8 and respiratory failure requiring mechanical ventilation developed in 39 (28.9%), and 33 (24.4%) patients, respectively. Quadriparesis was a risk factor for prolonged ventilator support (29.5 ± 16.8 vs. 12.4 ± 8.7 days, p = 0.001). At discharge, one patient with monoparesis recovered full muscle strength, whereas 8 patients with AFP were functionally dependent. Twenty-nine (21.5%) patients died. All of the succumbed had encephalitis, and 7 had quadriparesis. Ataxia, tremor and cognitive deficit persisted in 18 (16.9%), 15 (14.2%), and 22 (16.3%) patients at discharge, respectively. Age, malignancy, coronary disease, quadriparesis, mechanical ventilation, GCS ≤ 8 and healthcare-associated infections were risk factors for death (p = 0.001; p = 0.019; p = 0.004; p = 0.001; p < 0.001; p < 0.001, and p < 0.001, respectively).

Transverse Myelitis in the Setting of Enterobius vermicularis (Pinworm) Infection: Case Report.

Myelitis is a rare inflammatory myelopathy, and known associated etiologies only account for a small number of causes. A significant percentage of cases have an unknown etiology and are considered idiopathic. With 64% to 68% of cases fitting into the idiopathic category, helminth infections, and specifically pinworm parainfections, should be considered in cases that would otherwise be classified as idiopathic. This case report outlines a pediatric patient diagnosed with myelitis given her progressive weakness, fussiness, refusal to bear weight as well as magnetic resonance imaging (MRI) demonstrating T2-hyperintense signal and/or T1 gadolinium enhancement, and/or positive cerebrospinal fluid (CSF) inflammatory markers. This patient had a negative evaluation for typical known etiologies for myelitis including no signs of multiple sclerosis and neuromyelitis optica spectrum disorder on brain MRI, oligoclonal banding and aquaporin-4 autoantibodies, and no evidence of bacterial or viral meningitis given normal cell counts and cultures in CSF. She was found to have a pinworm infection, suggesting a parasitic parainfectious etiology of her myelitis. This case outlines the first case noting the correlation between myelitis and pinworm infection in a pediatric patient.

Sarcoidosis with myelitis and autonomic neuropathy treated with infliximab.

In this case report, a 55-year-old man presented with back pain, urinary retention, sensory disturbances, erectile dysfunction, leg paresis and orthostatism. Spinal MRI showed longitudinal extensive myelitis. Lymph node biopsy was compatible with sarcoidosis and a diagnosis of probable neurosarcoidosis (NS) was made. The patient benefited from prednisolone but relapsed during withdrawal. Infliximab resulted in almost complete remission. In conclusion, relapse is often seen when phasing out prednisolone, whereas infliximab appears to have a lasting effect and should be considered in the early stages of severe NS.

Iagnosis and treatment of myelitis after lumbar reoperation: A case report.

RATIONALE: At present, acute myelitis (AM) is a great challenge to diagnosis and treatment because of its complicated etiology, critical condition, and poor prognosis, and it is easy to leave different degrees of limb motor dysfunction. The report of this case is helpful to improve the understanding of AM after lumbar surgery, reduce misdiagnosis and provide reference for clinical treatment. PATIENTS CONCERN: This study reported a case of AM after lumbar reoperation. Before the patient was diagnosed as AM, we gave high-dose hormone anti-inflammatory and detumescence symptomatic treatment according to empirical treatment, and the effect was ideal and rehabilitation treatment was actively carried out at the right time. After 10 months of follow-up, the patient recovered well. DIAGNOSIS: Because lumbar surgery is a contraindication of lumbar puncture, the patient's diagnosis was confirmed by thoracic magnetic resonance imaging. Magnetic resonance imaging of thoracic vertebra on the 17th day after lumbar operation showed that small round T1W1 signal, slightly higher T2W1 signal and T2-fat suppression imaging equal signal were seen in the horizontal spinal cord of thoracic vertebra 10. INTERVENTION: According to the empirical treatment, patients have been given high-dose hormone therapy after operation, and comprehensive treatment such as comprehensive training of paraplegic limbs, joint loosening training, electric massage and other rehabilitation training will be carried out when the general condition of patients improves. OUTCOMES: After 10 months of follow-up, there were no major sequelae such as limb paralysis. CONCLUSION: Due to the rarity of AM in clinical work, it is easy for doctors to ignore the disease and miss the best treatment stage, which will lead to serious sequelae.