Kikuchi disease complicated with aseptic meningitis following COVID-19 Vaccination: a case report.

BACKGROUND: Kikuchi Fujimoto disease is a rare self-limiting disorder mainly affecting young Asian females. The typical presentation is unexplained fever with associated cervical lymphadenopathy. It can mimic many sinister diseases such as lymphoma, tuberculosis, and systemic lupus erythematosus. Aseptic meningitis due to Kikuchi disease is extremely rare, and majority were reported from Japan. There have been no published cases of aseptic meningitis due to Kikuchi disease in Sri Lanka. CASE PRESENTATION: A 29 years old Sri Lankan female presented with a prolonged fever for three weeks with an associated headache for five days duration. She developed painful cervical lymphadenopathy during the hospital stay. She has been previously well and had been vaccinated against COVID-19 six weeks before. Her lumbar puncture showed lymphocytic pleocytosis with marginally elevated protein levels and reduced ratio of serum to CSF sugar. Lymph node biopsy was consistent with necrotizing lymphadenitis. She was subsequently diagnosed with Kikuchi disease complicated with aseptic meningitis. She responded to corticosteroids well and had an uneventful recovery. CONCLUSION: Kikuchi disease is a rare self-limiting disorder that can be complicated with aseptic meningitis on infrequent occasions. Other conditions such as tuberculosis, lymphoma, systemic lupus erythematosus, and adult-onset Still's disease should be considered as differential diagnoses. Knowledge of Kikuchi disease and its complications will prevent unnecessary investigations which delay the early diagnosis and treatment.

Aseptic meningitis and Fabry disease.

OBJECTIVE: Fabry disease is caused by enzymatic defects in alpha-galactosidase A that leads to the accumulation of glycosphingolipids throughout the body, resulting in a multisystemic disorder. The most common neurological manifestations are neuropathic pain, autonomic nervous system dysfunction and strokes, but some rarer neurological manifestations exist. Among these, aseptic meningitis is a possible complication. Our objectives were to measure the prevalence of this complication in a cohort of patients with Fabry disease, and to describe its clinical features. METHODS: We conducted a retrospective review of Fabry disease patients followed at our tertiary referral center between 1995 and September 2023 with at least one episode of meningitis, and performed a systematic review to identify similar published cases. RESULTS: Four patients out of 107 (3.7%) had at least one episode of aseptic meningitis. Our systematic review identified 25 other observations. The median age of these 29 patients was 29.0 years, the median cerebrospinal fluid leukocyte count was 24 cells/mm3 with a predominance of lymphocytes in 64.7% of cases. In 82.8% of the patients, the diagnosis of Fabry disease was unknown before the meningitis. Large artery stenosis was present in 17.2% of patients and 57.1% of patients had a recent stroke concomitant with the meningitis. Several differential diagnoses were evoked, such as multiple sclerosis or central nervous system vasculitis. INTERPRETATION: Our study suggests that Fabry disease should be considered as a cause of aseptic meningitis. The pathophysiological mechanisms underlying meningeal inflammation remain largely unknown but may reflect the dysregulation of pro-inflammatory signaling pathways.

Acute Aseptic Meningitis Temporally Associated with Intravenous Polyclonal Immunoglobulin Therapy: A Systematic Review.

An acute aseptic meningitis has been occasionally observed on intravenous polyclonal human immunoglobulin therapy. Since case reports cannot be employed to draw inferences about the relationships between immunoglobulin therapy and meningitis, we conducted a systematic review and meta-analysis of the literature. Eligible were cases, case series, and pharmacovigilance studies. We found 71 individually documented cases (36 individuals ≤ 18 years of age) of meningitis. Ninety percent of cases presented ≤ 3 days after initiating immunoglobulin therapy and recovered within ≤ 7 days (with a shorter disease duration in children: ≤ 3 days in 29 (94%) cases). In 22 (31%) instances, the authors noted a link between the onset of meningitis and a rapid intravenous infusion of immunoglobulins. Cerebrospinal fluid analysis revealed a predominantly neutrophilic (N = 46, 66%) pleocytosis. Recurrences after re-exposure were observed in eight (N = 11%) patients. Eight case series addressed the prevalence of meningitis in 4089 patients treated with immunoglobulins. A pooled prevalence of 0.6% was noted. Finally, pharmacovigilance data revealed that meningitis temporally associated with intravenous immunoglobulin therapy occurred with at least five different products. In conclusion, intravenous immunoglobulin may cause an acute aseptic meningitis. The clinical features remit rapidly after discontinuing the medication.

Unusual non-infectious cause of meningitis.

Meningitis, though commonly caused by various infectious agents, may also have non-infectious aetiologies. The clinical presentation, however may be identical to infectious meningitis. We present a case of a female in her 50s who presented with fever, headache, vomiting and neck rigidity. She had features of inflammatory polyarthritis, cold sensitivity, puffy digits, alopecia and easy fatigability. No evidence of infection was found, and serological features consistent with mixed connective tissue disease (MCTD) were revealed, which led to the diagnosis of MCTD-related aseptic meningitis.

Aseptic meningitis following spinal anaesthesia: An uncommon and challenging diagnosis.

Drug-induced aseptic meningitis (DIAM) or chemical meningitis following spinal anaesthesia has rarely been reported. DIAM is caused by meningeal inflammation due to intrathecally administered drugs or secondary to systemic immunological hypersensitivity. We hereby present a case of a young adult with aseptic meningitis following neuraxial anaesthesia possibly provoked by bupivacaine. The initial cerebrospinal fluid (CSF) picture revealed neutrophilic pleocytosis and normal glycorrhachia. CSF culture was negative. The patient was put on invasive mechanical ventilation and started on intravenous antibiotics. There was a rapid improvement in clinical condition without any residual neurological deficit within the next few days. Aseptic meningitis following neuraxial anaesthesia can be prevented by strict aseptic protocols and careful inspection of visible impurities while administering the intrathecal drug. Detailed history taking, clinical examination, and focused investigations can distinguish between bacterial and chemical meningitis. Appropriate diagnosis of this entity may guide the treatment regimen, reducing hospital stay and cost.

Meningite asséptica e doença autoimune / Aseptic meningitis and autoimmune disease

A meningite asséptica (MA) é uma entidade anatomoclínica aguda, benigna, de ocorrência rara na população geral, com manifestações clínicas variáveis e ausência de patógenos identificáveis no líquido cefalorraquidiano. Sua prevalência é maior associada a administração ou presença de algum medicamento e/ou doença autoimune, respectivamente. Seu diagnóstico é dificultado devido à inespecificidade das manifestações clínicas e laboratoriais, o que resulta em hospitalizações, propedêutica invasiva e antibioticoterapia desnecessárias.

Aseptic meningitis (AM) is an acute and benign condition, rare in the general population, characterized by nonspecific clinical manifestations associated with changes on cerebrospinal fluid (CSF) sample analysis, including negative cultures. The subject of analysis is the relationship between medication intake and autoimmune diseases with the occurrence of AM. It has been concluded that this condition occurs more often in case both causes are associated. The diagnosis of AM is difficult because of the nonspecifity of the signs and symptoms and findings on CSF analysis, which results in unnecessary hospitalizations, invasive exams and antibiotic therapy.

Validación de una regla de predicción clínica para diferenciar meningitis bacteriana de meningitis aséptica / Validation of a clinical prediction rule to distinguish bacterial from aseptic meningitis

La mayoría de las meningitis en la infancia no son bacterianas, pero la dificultad en descartar tal etiología obliga con frecuencia a utilizar antibióticos inicialmente. El objetivo de este trabajo fue validar, en nuestro medio, una regla de predicción clínica para diferenciar la etiología de las meningitis en niños a su ingreso al hospital. Método. Estudio prospectivo que incluyó pacientes menores 19 años de edad con diagnósitco de meningitis. En todos se obtuvo líquido cefalorraquídeo (LCR) para análisis directo, citoquímico y cultivo, y hemograma. Se calculó el BMS (Bacterial Meningitis Score: puntaje de meningitis basteriana) descripto por Nigrovic (Pediatrics 2002; 110:712): tinción de Gram en LCR positiva = 2 puntos; proteinorraquia mayor o igual 80 mg/dl, neutrófilos en sangre periférica mayor o igual 10000/mn3, neutrofilos en LCR mayor o igual 1000/mm3, convulsiones, un punto cada uno. Se calculó sensibilidad (S), especificidad (E), valores predictivos positivo y negativo (VPP y VPN) y razones de verosimilitud positiva y negativa (RVP y RVN)del BMS para predecir meningitis bacteriana. Resultados. Se incluyeron 70 pacientes con meningitis (14 bacterianas) veinticinco pacientes presentaron BMS=0 puntos, 11 BMS=1 punto, y 34 BMS mayor o igual 2 puntos. Un BMS=0 evidenció S: 100 por ciento E: 44 por ciento, VPP; 31 por ciento, VPN: 100 por ciento, RVP: 1,81 RVN: 0 para predecir meningitis bacteriana. Un BMS mayor o igual 2 mostró S: 100 por ciento, E: 64 por ciento, VPP: 41 por ciento, VPN: 100 por ciento, RVP: 2,8 RVN: 0 para predecir meningitis bacteriana. Conclusión: La utilización del BMS fue simple y permitió identificar pacientes con bajo riesgo de meningitis bacteriana. Su empleo podría constituir una herramienta útil en la toma de decisiones clínicas.

Laboratorial diagnosis of lymphocytic meningitis

Meningitis is the main infectious central nervous system (CNS) syndrome. Viruses or bacteria can cause acute meningitis of infectious etiology. The term "Aseptic Meningitis" denotes a clinical syndrome with a predominance of lymphocytes in the cerebrospinal fluid (CSF), with no common bacterial agents identified in the CSF. Viral meningitis is considered the main cause of lymphocyte meningitis. There are other etiologies of an infectious nature. CSF examination is essential to establish the diagnosis and to identify the etiological agent of lymphocytic meningitis. We examined CSF characteristics and the differential diagnosis of the main types of meningitis.

Manifestaciones neurológicas de la granulomatosis de Wegener: Caso clínico / Neurological involvement in Wegener's granulomatosis: Report of one case

Wegener's granulomatosis is a systemic necrotizing vasculitis that affects medium size and small vessels. Neurological involvement occurs in 22 percent to 54 percent of patients, mainly in the form of mononeuritis multiplex. Central nervous system involvement is reported in only 2 percent to 8 percent of the cases. We report a 42-year-old male who presented with headache, diplopia, third and sixth cranial nerve palsies and íeft eye amaurosis associated to mass located in the íeft Meckel cavum and diffuse meningeal involvement. A biopsy of the mass disclosed a chronic granulomatous necrotizing inflammation with Langhans giant cells. A chest CAT scan showed three cavitated lung nodules and ANCA antibodies were positive in a titer of 1:80. Treatment with steroid and cyclophosphamide was started and cranial nerve palsies resolved and the number and size of lung nodules decreased. The patient was lost from follow up.

Manifestaciones neurológicas asociadas a infección por Mycoplasma pneumoniae / Neurological clinical manifestations associated to Mycoplasma pneumoniae infection

Mycoplasma pneumoniae, reconocido patógeno respiratorio, es también responsable de numerosas y variadas manifestaciones extrapulmonares, siendo las neurológicas las más frecuentes. Se presenta el caso de una escolar de 8 años, con un síndrome febril prolongado asociado a infección por M. pneumoniae que se complicó con encefalomielitis diseminada aguda. Se reportan otros seis casos con manifestaciones neurológicas: meningitis aséptica (1), meningoencefalitis (1), síndrome de Guillain Barré (1) y parálisis facial (3). La patogenia de las complicaciones neurológicas asociadas con infección por M. pneumoniae es aún desconocida, planteándose que algunas se deben a invasión directa del SNC y aquellas con pródromo prolongado, como encefalomielitis diseminada aguda, síndrome de Guillain Barré y mielitis transversa, obedecerían probablemente a fenómenos autoinmunes. En nuestro medio, en ausencia de RPC para confirmar el diagnóstico de esta infección, éste se fundamenta en la presencia de anticuerpos IgM o ascenso de IgG específica. La utilidad de los antimicrobianos en el control de estas manifestaciones neurológicas es discutida postulándose el uso de terapia inmunomoduladora.

Colagenopatia y meningitis recurrente en una paciente de 88 años / Collagen diseases and recurrent meningitis in an 88 year old patient

La meningitis aséptica recurrente (MAR) en ancianos es rara y generalmente es secundaria a drogas. Su asociación a colagenopatías en ancianos ha sido raramente informada. El Síndrome de Sjögren (SS) en ocasiones afecta el sistema nervioso central, pero la MAR asociada a SS es rara en este grupo etario. Se presenta una paciente de 88 años, autoválida, con antecedentes de xerostomía, xeroftalmos, Raynaud, disfagia y agrandamiento parotídeo recurrente autolimitado. En el año 2001 cursó una meningitis linfocítica y evolucionó con recuperación completa. Un año después volvió a presentar una meningitis linfocítica aséptica. Se descartaroncausas infecciosas. Se demostró FAN 1/160 con patrón nucleolar moteado, Ac anti Ro y anti RNP positivosy anticoagulante lúpico positivo. Se confirmó sequedad ocular y la biopsia de labio fue compatible con SS.Evolucionó con resolución completa en 10 días sin tratamiento. Se interpretó como una enfermedad mixta deltejido conectivo (EMTC) con síntomas predominantemente de SS, que cursó una MAR en su forma pura. La EMTC y el SS deberían ser considerados entre los diagnósticos diferenciales de la MAR, inclusive en ancianos.

Enfermedad mano-pie-boca: a propósito de un caso por enterovirus 71 / Hand-foot-mouth disease associated with enterovirus 71

Una niña de 10 meses fue derivada a la consulta dermatológica por exantema y enantema de 48 horas de evolución. Las vesículas de forma redondeada u ovalada asentaban sobre piel discretamente eritematosa y se extendían a muslos, manos, pies y boca tanto en labios como en lengua. El diagnóstico clínico e histopatológico de las lesiones resultó compatible con enfermedad mano-pie-boca y el diagnóstico virológico realizado en cultivo celular permitió identificar al agente etiológico como enterovirus 71 (EV71). Esta etiología ha sido asociada en epidermis de enfermedad mano-pie-boca con elevado compromiso neurológico. Nuestra paciente cursó sin complicaciones

Complicações neurológicas determinadas pela anestesia subaracnóidea / Spinal anesthesiaðinduced neurological complications

Justificativa e objetivos - Complicações neurológicas da anestesia subaracnóidea, apesar de raras, podem determinar seqüelas importantes. O objetivo deste estudo é apresentar estas complicações com a finalidade de esclarecer os fatores desencadeantes, o que facilita o diagnóstico das lesões. Conteúdo - Säo apresentadas as seguintes complicações: lesäo de nervo desencadeada pela agulha e cateter, cefaléia pós-punçäo, síndrome da artéria espinhal anterior, hematoma espinhal, meningite bacteriana, meningite asséptica, aracnoidite adesiva, síndrome da cauda eqüina e sintomas neurológicos transitórios. Conclusões - O conhecimento dos fatores desencadeantes de complicações neurológicas determinadas pela anestesia subaracnóidea pode prevenir as lesões, diagnosticar e tratar mais precocemente e, desse modo, mudar o prognóstico das mesmas

Polymerase chain reaction for the laboratory diagnosis of aseptic meningitis and encephalitis

A protocol for testing cerebrospinal fluid specimens using a range of PCR assays for the diagnosis of central nervous system infection was developed and used to test prospectively 383 specimens. PCR assays were used for the detection of adenovirus, Borrelia burgdorferi, enteroviruses, Epstein Barr virus, cytomegalovirus, herpes simplex virus, human herpes virus type 6, JC virus, Leptospira interrogans, Listeria monocytogenes, lymphocytic choriomeningitis virus, measles virus, mumps virus, Mycobacterium sp., Mycoplasma pneumoniae, Toxoplasma gondii and varicella zoster virus. Of the 383 specimens tested in this study, 46 (12.0 percent) were found to be positive. The microorganisms detected were CMV, enterovirus, Epstein Barr virus, herpes simplex virus, human herpes virus type 6, JC virus, L. monocytogenes, Mycobacterium genus, Toxoplasma gondii and varicella zoster virus. The introduction of the PCR protocol described has improved the diagnosis of a range of central nervous system infections in our laboratory. We believe however that further evaluation of these assays in immunocompromised patients is necessary to better determine the predictive value of positive PCR results in these patient groups

Magnetic resonance imaging in the evaluation of patients with aseptic meningoencephalitis and connective tissue disorders

OBJECTIVE: To describe the role of magnetic resonance imaging (MRI) in the evaluation of patients with chronic and recurrent aseptic meningitis.METHOD: A retrospective study of five patients with aseptic meningoencefalitis diagnosed by clinical and CSF findings. CT scans showed without no relevant findings. RESULTS: MRI showed small multifocal lesions hyperintense on T2 weighted images and FLAIR, with mild or no gadolinium enhancement, mainly in periventricular and subcortical regions. Meningoencephalitis preceded the diagnosis of the underlying disease in four patients (Behçet's disease or systemic lupus erythematosus). After the introduction of adequate treatment for the rheumatic disease, they did not present further symptoms of aseptic meningoencephalitis. CONCLUSION: Aseptic meningoencephalitis can be an early presentation of an autoimmune disease. It is important to emphasize the role of MRI in the diagnosis and follow-up of these patients.

Caracterización etiológica de las meningoencefalitis asépticas en la provincia Ciego de Avila, Cuba / Etiologic characterization of aseptic meningoencephalitis in the province of Ciego de Avila, Cuba

Se realiza análisis prospectivo aleatorio a 198 pacientes con meningoencefalitis aséptica (MEA) ingresados en el servicio de medicina del hospital provincial ®Dr. Antonio Luaces Iraola¼ de Ciego de Avila, Cuba. Se les efectuó estudios con sueros pareados para determinar anticuerpos neutralizantes a enterovirus, pruebas de hemoglutinación, neutralización viral para arbovirus, hemolítica para leptospira y estudio virológico de heces fecales buscando enterovirus. En 36.8 por ciento de los pacientes se encontró como agente etiológico un enterovirus. El segundo agente en frecuencia fue la leptospira en 14.6 por ciento. Se determinó el agente etiológico en 51.4 por ciento de los pacientes estudiados

Caxumba e infecçåo aguda do sistema nervoso central (SNC) / Mumps and infections of Central Nervous System (C.N.S.)

Dentre 916 casos de "Meningite Asséptica" internados no Hospital de Doenças Tropicais de Goiânia no período de 1982-1993, o vírus da caxumba foi o mais provável agente etiológico dde 157 (17,14 pôr cento) casos, pois havia concomitância de parotidite ou história recente de contágio intradomiciliar. Foram levantados dados sobre a idade dos pacientes, o tempo de permanência hospitalar, o tempo de duraçåo da doença e em que dia da caxumba surgiram os primeiros sinais do acometimento do S.N.C.. Os exames de rotina do L.C.R. foram solicitaddos e, no sangue a amilasemia. O tipo de acometimento nervoso, a concomitância de pancreatite e os anos e meses de maiores e menores incidências såo citados. O autor eenfatiza que o acometimento cerebral nåo deve ser considerado uma complicaçåo e sim forma clínica da parotidite epidêmica do mesmo modo que a pancreatite e a orquite