Comparison of acute flaccid myelitis and transverse myelitis in children and evaluation of diagnostic criteria.

BACKGROUND AND PURPOSE: Acute flaccid myelitis (AFM) and transverse myelitis (TM) are serious conditions that may be difficult to differentiate, especially at onset of disease. In this study, we compared clinical features of pediatric AFM and TM and evaluated current diagnostic criteria, aiming to improve early and accurate diagnosis. METHODS: Two cohorts of children with enterovirus D68-associated AFM and clinically diagnosed TM were compared regarding presenting clinical features, additional investigations, and outcome. Current diagnostic criteria for AFM and TM were applied to evaluate their specificity. RESULTS: Children with AFM (n = 21) compared to those with TM (n = 36) were younger (median 3 vs. 10 years), more often had a prodromal illness (100% vs. 39%), predominant proximal weakness (69% vs. 17%), and hyporeflexia (100% vs. 44%), and less often had sensory deficits (0% vs. 81%), bowel and/or bladder dysfunction (12% vs. 69%), and hyperreflexia (0% vs. 44%). On magnetic resonance imaging, brainstem involvement was more common in AFM (74% vs. 21%), whereas supratentorial abnormalities were only seen in TM (0% vs. 40%). When omitting the criterion of a sensory level, 11 of 15 (73%) children with AFM fulfilled the diagnostic criteria for TM. Of children with TM, four of 33 (12%) fulfilled the diagnostic criteria for probable/definite AFM. CONCLUSIONS: Although there is considerable overlap between AFM and TM in children, we found important early differentiating clinical and diagnostic features. Meeting diagnostic criteria for AFM in children with TM and vice versa underlines the importance of thorough clinical examination and early and accurate diagnostic studies.

Characteristics of Upper Extremity Recovery in Acute Flaccid Myelitis: A Case Series.

BACKGROUND: Clinical characteristics and timing associated with nonsurgical recovery of upper extremity function in acute flaccid myelitis are unknown. METHODS: A single-institution retrospective case series was analyzed to describe clinical features of acute flaccid myelitis diagnosed between October of 2013 and December of 2016. Patients were consecutively sampled children with a diagnosis of acute flaccid myelitis who were referred to a hand surgeon. Patient factors and initial severity of paralysis were compared with upper extremity muscle strength outcomes using the Medical Research Council scale every 3 months up to 18 months after onset. RESULTS: Twenty-two patients with acute flaccid myelitis (aged 2 to 16 years) were studied. Proximal upper extremity musculature was more frequently and severely affected, with 56 percent of patients affected bilaterally. Functional recovery of all muscle groups (≥M3) in an individual limb was observed in 43 percent of upper extremities within 3 months. Additional complete limb recovery to greater than or equal to M3 after 3 months was rarely observed. Extraplexal paralysis, including spinal accessory (72 percent), glossopharyngeal/hypoglossal (28 percent), lower extremity (28 percent), facial (22 percent), and phrenic nerves (17 percent), was correlated with greater severity of upper extremity paralysis and decreased spontaneous recovery. There was no correlation between severity of paralysis or recovery and patient characteristics, including age, sex, comorbidities, prodromal symptoms, or time to paralysis. CONCLUSIONS: Spontaneous functional limb recovery, if present, occurred early, within 3 months of the onset of paralysis. The authors recommend that patients without signs of early recovery warrant consideration for early surgical intervention and referral to a hand surgeon or other specialist in peripheral nerve injury. CLINICAL QUESTION/LEVEL OF EVIDENCE: Risk, III.

JC Polyomavirus, progressive multifocal leukoencephalopathy and immune reconstitution inflammatory syndrome: a review.

The human neurotropic virus JC Polyomavirus, a member of the Polyomaviridae family, is the opportunistic infectious agent causing progressive multifocal leukoencephalopathy, typically in immunocompromised individuals. The spectrum of underlying reasons for the systemic immunosuppression that permits JCV infection in the central nervous system has evolved over the past 2 decades, and therapeutic immunosuppression arousing JCV infection in the brain has become increasingly prominent as a trigger for PML. Effective immune restoration subsequent to human immunodeficiency virus-related suppression is now recognized as a cause for unexpected deterioration of symptoms in patients with PML, secondary to a rebound inflammatory phenomenon called immune reconstitution inflammatory syndrome, resulting in significantly increased morbidity and mortality in a disease already infamous for its lethality. This review addresses current knowledge regarding JC Polyomavirus, progressive multifocal leukoencephalopathy, progressive multifocal leukoencephalopathy-related immune reconstitution inflammatory syndrome, and the immunocompromised states that incite JC Polyomavirus central nervous system infection, and discusses prospects for the future management of these conditions.

Revisión de la bibliografía. Coronavirus (COVID-19) y Sistema Nervioso Central. Un panorama para analizar en una situación de pandemia. Consideraciones sobre el neurotrofismo del Coronavirus / Literature review. Coronavirus (COVID-19) and Nervous System Central. A panorama to analyze in a pandemic situation. Considerations on coronavirus neurotrophy

En humanos, las infecciones virales del tracto respiratorio son una causa principal de morbilidad y mortalidad en todo el mundo. Varios agentes virales respiratorios reconocidos tienen una capacidad neuroinvasiva, ya que pueden propagarse desde el tracto respiratorio hasta el sistema nervioso central (SNC). Una vez allí, la infección de las células del SNC (neurotropismo) podría conducir a problemas de salud humana, como encefalitis y enfermedades neurológicas a largo plazo. Los coronavirus (HCoV) generalmente infectan el tracto respiratorio superior, donde se asocian principalmente con resfriados comunes. Sin embargo, en las poblaciones más vulnerables, como los recién nacidos, los bebés, los ancianos y las personas inmunocomprometidas, también pueden afectar el tracto respiratorio inferior y provocar neumonía, exacerbaciones del asma, síndrome de dificultad respiratoria o incluso síndrome respiratorio agudo severo (SRAS) . Se ha establecido claramente la afectación respiratoria del VHC desde la década de 1960. Además, durante casi tres décadas, la literatura científica también ha demostrado que los HCoV son neuroinvasivos y neurotrópicos y podrían inducir una activación excesiva del sistema inmune.

In humans, viral infections of the respiratory tract are a leading cause of morbidity and mortality worldwide. Several recognized respiratory viral agents have a neuroinvasive capacity since they can spread from the respiratory tract to the central nervous system (CNS). Once there, infection of CNS cells (neurotropism) could lead to human health problems, such as encephalitis and long-term neurological diseases. Coronaviruses (HCoV) usually infect the upper respiratory tract, where they are mainly associated with common colds. However, in more vulnerable populations, such as newborns, infants, the elderly and immune-compromised individuals, can also affect the lower respiratory tract, leading to pneumonia, exacerbations of asthma, respiratory distress syndrome, or even severe acute respiratory syndrome (SARS). It has been clearly established the respiratory involvement of HCoV since the 1960s. In addition, for almost three decades now, the scientific literature has also demonstrated that HCoV are neuroinvasive and neurotropic and could induce an over activation of the immune system

[Central Neurological Diagnosis in Patients Infected with HIV in the Infectious Diseases Unit of University Hospital of Casablanca, Morocco]. / Manifestations neurologiques centrales chez les patients infectés par le VIH dans le service des maladies infectieuses du CHU de Casablanca, Maroc.

The aim of this work is to study the epidemiology of central neurological system (CNS) diagnosed in the population of people living with HIV in the department of infectious diseases in UHC Ibn Rochd of Casablanca from January 2005 to May 2015. The demographic and clinical profile along with the outcome of these patients were studied. The data were collected from Nadis software. Three hundred and eighty-seven patients were admitted for CNS diagnosis, out of 3496 people living with HIV admitted during this time period, i.e., a prevalence of 11%. The sex ratio (M/F) was 1.27. The average age was 39 years (± 7). Neurological involvement was indicative of HIV infection in 225 cases (68.8%). Neurological disorders were dominated by headache (70%), focal neurological syndrome (35%), and meningeal syndrome (30%). CNS diagnosis noted were CNS tuberculosis (37%), cerebral toxoplasmosis (30%), and cryptococcal meningitis (20%). The median CD4 T-lymphocyte was 184 cells/mm3. Infection with severe immunosuppression was progressive multifocal leucoencephalitis, cryptococcal meningitis, and primary cerebral lymphoma. Lethality was 39%. In the department of infectious diseases of the UHC, the main cause of death among HIV-infected patients is tuberculosis. Collaboration between the national tuberculosis and AIDS programs has been established to improve the detection and management of these patients.

L'objectif de ce travail est d'étudier l'épidémiologie des manifestations neurologiques centrales (MNC) des patients vivant avec le VIH (PvVIH) suivis dans le service des maladies infectieuses du CHU Ibn Rochd de Casablanca entre janvier 2005 et mai 2015. La source des données était le logiciel Nadis. Trois cent quatre-vingt-sept patients ont été hospitalisés pour une MNC sur 3 496 PvVIH, soit une prévalence de 11 %. Le sex-ratio (H/F) était de 1,27. L'âge moyen des patients était de 39 ans (± 7). L'atteinte neurologique était révélatrice de l'infection à VIH dans 266 cas (69 %). Les troubles neurologiques étaient dominés par les céphalées (70 %), le syndrome neurologique focal (35 %) et le syndrome méningé (35 %). Les étiologies étaient dominées par la méningoencéphalite tuberculeuse (37 %), la toxoplasmose cérébrale (30 %) et la cryptococcose neuroméningée (CNM) (20 %). La médiane des lymphocytes T CD4 était de 184 cellules/mm3. Les atteintes survenues en cas d'immunodépression sévère étaient la leucoencéphalite multifocale progressive, la CNM et le lymphome cérébral primitif. Le taux de létalité global était de 39 %. Dans le service des maladies infectieuses du CHU prenant en charge les PvVIH, la tuberculose est la première étiologie des MNC au cours de l'infection au VIH. Une collaboration conjointe du programme national de lutte contre la tuberculose et de celui de lutte contre le sida a été mise en place pour améliorer le dépistage et la prise en charge de ces patients.

The Olfactory Bulb: An Immunosensory Effector Organ during Neurotropic Viral Infections.

In 1935, the olfactory route was hypothesized to be a portal for virus entry into the central nervous system (CNS). This hypothesis was based on experiments in which nasophayngeal infection with poliovirus in monkeys was prevented from spreading to their CNS via transection of olfactory tracts between the olfactory neuroepithelium (ONE) of the nasal cavity and the olfactory bulb (OB). Since then, numerous neurotropic viruses have been observed to enter the CNS via retrograde transport along axons of olfactory sensory neurons whose cell bodies reside in the ONE. Importantly, this route of infection can occur even after subcutaneous inoculation of arboviruses that can cause encephalitis in humans. While the olfactory route is now accepted as an important pathway for viral entry into the CNS, it is unclear whether it provides a way for infection to spread to other brain regions. More recently, studies of antiviral innate and adaptive immune responses within the olfactory bulb suggest it provides early virologic control. Here we will review the data demonstrating that neurotropic viruses gain access to the CNS initially via the olfactory route with emphasis on findings that suggest the OB is a critical immunosensory effector organ that effectively clears virus.

Infection of the central nervous system with dengue virus 3 genotype I causing neurological manifestations in Brazil

Abstract: A case of dengue virus 3 (DENV-3) genotype I infection with neurological manifestations occurred in Belo Horizonte, Minas Gerais in October 2012. The serotype was detected by PCR, and the genotype was assessed by sequencing and phylogenetic analysis of the C-prM region. The virus causing neurological manifestations clustered with other sequences of DENV-3 genotype I. Because neurological manifestations of DENV are possibly misdiagnosed in Brazil, this study serves as an alert of the importance of DENV diagnoses in CNS infections.

Clinical profile of Monomelic Amyotrophy (MMA) and role of persistent viral infection.

OBJECTIVES: The objective of our study was to describe the clinical characteristics, electrophysiology, MRI features and conduct viral assays in patients with Monomelic Amyotrophy (MMA) and follow them up over one year. METHODS: Consecutive patients with MMA who attended the Neurology services from April 2013 to March 2014 were included. Age and sex matched controls were taken for the purpose of viral assay analysis. The clinical evaluation was repeated at six months and one year. RESULTS: 109 cases and 109 controls were included in the study. The patients were predominantly males (98.2%; n=107/109) and had involvement of upper limbs (83.5%; n=91/109). 26 (23.8%) patients with clinically unilateral involvement had bilateral neurogenic changes in the electromyography. Serological assays of Japanese E, West Nile Virus, and Poliovirus 1, 2 and 3, HIV 1 and 2 were negative in all the cases and controls. CONCLUSIONS: Patients with MMA are predominantly young males with upper limb wasting and weakness. MRI of the cervical cord is normal in most of the patients (67.9%). The present study did not find any evidence of the association of viral infection in MMA.

Viral-induced intracranial hypertension mimicking pseudotumor cerebri.

BACKGROUND: Pseudotumor cerebri or idiopathic intracranial hypertension is characterized by normal spinal fluid composition and increased intracranial pressure in the absence of a space-occupying lesion. METHODS: This study describes a subgroup of 10 patients with the same typical presenting symptoms (headache, vomiting, and papilledema) but without nuchal rigidity, meningeal signs, or change in mental status. Patients had normal neuroimaging studies and intracranial hypertension but also pleocytosis in the cerebrospinal fluid, suggesting central nervous system infection. From the results it can be hypothesized that those children represent a unique subgroup of viral-induced intracranial hypertension when comparing their risk factors, clinical course, treatment, and outcome with 58 patients who had idiopathic intracranial hypertension. RESULTS: All patients with viral-induced intracranial hypertension presented with papilledema but none had reduced visual acuity or abnormal visual fields, compared with 20.7% of patients who had idiopathic intracranial hypertension. They also responded better to treatment with acetazolamide, needed a shorter duration of treatment (7.7 ± 2.6 months vs 12.2 ± 6.3 months, P = 0.03), and had no recurrences. CONCLUSIONS: The results suggest that children who fulfill the typical presenting signs and symptoms and all diagnostic criteria for pseudotumor cerebri other than the normal cerebrospinal fluid component may represent a unique subgroup of viral-induced intracranial hypertension and should be managed accordingly. The overall prognosis is excellent.

The human polyomavirus BK (BKPyV): virological background and clinical implications.

Polyomavirus BK (BKPyV) infects most people subclinically during childhood and establishes a lifelong infection in the renourinary tract. In most immunocompetent individuals, the infection is completely asymptomatic, despite frequent episodes of viral reactivation with shedding into the urine. In immunocompromised patients, reactivation followed by high-level viral replication can lead to severe disease: 1-10% of kidney transplant patients develop polyomavirus-associated nephropathy (PyVAN) and 5-15% of allogenic hematopoietic stem cell transplant patients develop polyomavirus-associated haemorrhagic cystitis (PyVHC). Other conditions such as ureteric stenosis, encephalitis, meningoencephalitis, pneumonia and vasculopathy have also been associated with BKPyV infection in immunocompromised individuals. Although BKPyV has been associated with cancer development, especially in the bladder, definitive evidence of a role in human malignancy is lacking. Diagnosis of PyVAN and PyVHC is mainly achieved by quantitative PCR of urine and plasma, but also by cytology, immunohistology and electron microscopy. Despite more than 40 years of research on BKPyV, there is still no effective antiviral therapy. The current treatment strategy for PyVAN is to allow reconstitution of immune function by reducing or changing the immunosuppressive medication. For PyVHC, treatment is purely supportive. Here, we present a summary of the accrued knowledge regarding BKPyV.

Post-infectious opsoclonus and reversible magnetic resonance imaging changes: a case report and review of the literatures.

PURPOSE: Opsoclonus is a rare neurological disorder in adult. The etiology of opsoclonus includes parainfectious, paraneoplastic, toxic, and metabolic disorders. We reported an old female with post-infectious opsoclonus who had a benign clinical course and reversible brain MRI lesions, and its review of the literature. CASE REPORT: A 67-year-old woman presented with opsoclonus and truncal ataxia for two weeks. The magnetic resonance imaging (MRI) showed the hyperintensity lesions in bilateral medial thalamus, hypothalamus, and tegmentum of pons on Fluid-attenuated inversion recovery (FLAIR) imaging. Investigations of neoplasm and autoimmune disorders showed negative findings. Clinical symptoms subsided in two-week duration and MRI abnormalities also disappeared one month later. CONCLUSION: A benign clinical course and reversible MRI lesions could be found in the patients with postinfectious opsoclonus such as our case. However, detailed investigations and long-term follow-up are needed to exclude paraneoplastic or other systemic and immunological disorders.

[Epidemiology, diagnostics, and treatment of complications after neuroinfections: chronic fatigue syndrome].

Epidemiology information which testify to prevalence syndrome of chronic ustalostti (SV) is resulted in the article, and from some data this diagnosis is covered at more than 20% patients which carried neyroinfection. SV meets more frequent only in age 40-59, thus for women a disease is marked in 4 times more frequent, than for men. Today etiology of disease remains unknown, but the value of genetic, immunological factors, pathogens, neurogenic violations and features of feed is examined. Possibility of infectious etiology SV causes considerable interest of researchers, but at first this syndrome was examined as a sharp viral infection, where the most reliable exciter is consider the virus of Epshteyna-barr. Using of intravenous introduction of globulin for SV carries experimental character and grounded on a hypothesis about immunological or infectious etiology of this disease.

Viral infection of central nervous system in children: one year prospective study.

Viral infection of central nervous system (CNS) is a common problem worldwide, especially in children. The clinical manifestations of viral CNS infection are the most important clues for diagnosis and treatment. The 1-year prospective study to explore the prevalence, clinical manifestations, and laboratory findings of viral CNS infection in children, including human herpes virus (HHV) type 1, 2, 3, 4, 5, 6A, 6B, 7, enterovirus B, mumps virus, measles virus, Japanese encephalitis virus, JC virus, BK polyomavirus, Nipha virus and influenza virus (H1N1, H3N2) were performed. Total of 71 children suspected CNS infection, aged between 2 days to 12.9 years were enrolled from May 2009 to April 2010. Total 4 children with non CNS infection, 5 bacterial meningitis, 2 tuberculous meningitis CNS infection were excluded. The HHV2 (50.0%) was the most common viral CNS infection. Other viral CNS infection included HHV1 (11.60%), VZV (6.7%), HHV6 (3.3%), HHV7 (3.3%), enterovirus B (1.67%) and H3N2 (1.67%). Diarrhea, irritability and CSF pleocytosis may helpful for differentiation between subtype of viral CNS infection.

Negative association of Epstein-Barr virus or herpes simplex virus-1 with tumefactive central nervous system inflammatory demyelinating disease.

Central nervous system (CNS) demyelination has been suggested to be associated with infections caused by the Epstein-Barr virus (EBV) or herpes simplex virus (HSV)-1. CNS inflammatory demyelinating disease (IDD) rarely presents as a large lesion. We evaluated samples of serum and cerebrospinal fluid (CSF) by enzyme-linked immunosorbent assay to detect recent infection with these viruses and analyzed CSF and brain specimens by polymerase chain reaction (PCR) or immunohistochemical studies for evidence of these viruses in three patients with biopsy-proven CNS IDD. The results of PCR tests for EBV and HSV in CSF or brain specimens were negative. Elevated anti-EBV or -HSV antibody levels were not found in serum or CSF in any patient. Immunohistochemical studies showed that IDD lesions were negative for latent membrane protein (LMP)-1, Epstein-Barr nuclear antigen (EBNA)-2, and EBNA noncoding RNA (EBER)-1. These results suggest a negative association between CNS IDD and EBV or HSV.

Frequency and prognosis of convulsive status epilepticus of different causes: a systematic review.

We conducted a systematic review of all studies of status epilepticus (SE) with more than 30 patients published between January 1, 1990, and December 31, 2008, to determine the frequencies of the common underlying causes and the extent to which the underlying causes affect the prognosis of an episode of SE. The frequencies of underlying causes vary among studies and show marked geographic differences, but in most studies, the most common underlying causes were cerebrovascular disease and low antiepileptic drug levels. A relatively good prognosis of SE is found when the underlying cause is associated with low antiepileptic drug levels or alcohol abuse, and a relatively poor outcome occurs when the underlying cause is cerebrovascular disease, particularly in the case of SE due to acute cerebral anoxia, but in most conditions, the reported prognosis is variable. Also, when SE occurs in the context of an acute cerebral insult, such as cerebral infection or cerebrovascular disease, the prognosis of the acute cerebral event is worsened.

Chronic viral infection and primary central nervous system malignancy.

Primary central nervous system (CNS) tumors cause significant morbidity and mortality in both adults and children. While some of the genetic and molecular mechanisms of neuro-oncogenesis are known, much less is known about possible epigenetic contributions to disease pathophysiology. Over the last several decades, chronic viral infections have been associated with a number of human malignancies. In primary CNS malignancies, two families of viruses, namely polyomavirus and herpesvirus, have been detected with varied frequencies in a number of pediatric and adult histological tumor subtypes. However, establishing a link between chronic viral infection and primary CNS malignancy has been an area of considerable controversy, due in part to variations in detection frequencies and methodologies used among researchers. Since a latent viral neurotropism can be seen with a variety of viruses and a widespread seropositivity exists among the population, it has been difficult to establish an association between viral infection and CNS malignancy based on epidemiology alone. While direct evidence of a role of viruses in neuro-oncogenesis in humans is lacking, a more plausible hypothesis of neuro-oncomodulation has been proposed. The overall goals of this review are to summarize the many human investigations that have studied viral infection in primary CNS tumors, discuss potential neuro-oncomodulatory mechanisms of viral-associated CNS disease and propose future research directions to establish a more firm association between chronic viral infections and primary CNS malignancies.

Neurological aspects of HIV/human T lymphotropic virus coinfection.

Human T lymphotropic virus type 1 is associated with some neurologic diseases, mainly human T lymphotropic virus type 1-associated myelopathy/tropical spastic paraparesis. Human T lymphotropic virus type 2 has also been associated with similar cases of human T lymphotropic virus type 1-associated myelopathy/tropical spastic paraparesis, but evidences for a definitive relationship are less clear. On the other hand, neurologic manifestations of HIV infection are quite common, affecting more than one third of patients in HIV clinics. Seroepidemiologic studies show that HIV-infected individuals are at an increased risk for human T lymphotropic virus infection and vice versa in comparison with the general population. Furthermore, HIV/human T lymphotropic virus coinfection has been associated with distinctive immunophenotypes and an increased risk for development of neurodegenerative conditions. Thus, studies on HIV/human T lymphotropic virus coinfection have a practice clinical importance. In this review, we aim to discuss clinical and laboratorial data focusing on neurologic diseases in HIV/human T lymphotropic virus coinfection.

NeuroAIDS: characteristics and diagnosis of the neurological complications of AIDS.

The neurological complications of AIDS (NeuroAIDS) include neurocognitive impairment and HIV-associated dementia (HAD; also known as AIDS dementia and HIV encephalopathy). HAD is the most significant and devastating central nervous system (CNS) complications associated with HIV infection. Despite recent advances in our knowledge of the clinical features, pathogenesis, and neurobiological aspects of HAD, it remains a formidable scientific and therapeutic challenge. An understanding of the mechanisms of HIV neuroinvasion, CNS proliferation, and HAD pathogenesis provide a basis for the interpretation of the diagnostic features of HAD and its milder form, HIV-associated minor cognitive/motor disorder (MCMD). Current diagnostic strategies are associated with significant limitations, but it is hoped that the use of biomarkers may assist researchers and clinicians in predicting the onset of the disease process and in evaluating the effects of new therapies.