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Classification of the Idiopathic Inflammatory Demyelinating Disease of CNS Idiopathic Inflammatory Demyelinating Disease of CNS Monofocal forms Acute hemorrhagic leukoencephalitis
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Inflammatory demyelinating diseases comprise a spectrum of disorders affecting the myelin of the central and peripheral nervous system. These diseases can usually be differentiated on the basis of clinical, radiological, laboratory and pathological findings.
Recent studies have contributed to current awareness that inflammatory demyelinating disea...
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... inflammatory-demyelinating diseases (IIDDs) constitute a heterogeneous group of CNS disorders thought *Address correspondence to this author at the Child Neurology, Paediatric Department, I Faculty of Medicine, "Sapienza University"c/o Policlinico Umberto I, Rome, Italy; Tel: 06-49979313; Fax: 06-49979312; E-mail: childneurology.sapienzaroma@live.it to be of autoimmune origin and including acute disseminated encephalomyelitis, multiple sclerosis, Devic's disease, trans- verse myelitis, and clinically isolated syndromes such as optic neuritis. This spectrum includes monophasic, multipha- sic, and progressive disorders ranging from highly localized forms to multifocal or diffuse variants (Table 1) [6,7]. ...
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Myelin oligodendrocyte glycoprotein (MOG) antibody disease (MOGAD) has been recognized as a distinct entity in CNS demyelination with specific clinical and paraclinical features. It usually occurs in fourth decade of life, with a slight female predominance with optic neuritis being the most frequent presentation. Disease course can be either monoph...
Citations
... The potential for physical and mental impairment brought on by these illnesses emphasizes the urgent requirement for therapeutic approaches for neurorehabilitation, neurodegeneration, and neuro repair. (5)(6)(7)(8)(9)(10)(11)(12)(13)(14)(15) A study was done on, the two most common demyelinating syndromes in children is examined. MS i.e. multiple sclerosis and ADEM i.e. acute disseminated encephalomyelitis are two diseases that affect the central nervous system. ...
Background: A neurological condition known as demyelinating illness causes damage to the myelin sheath, which protects nerve fibers, optic nerves, and the spinal cord. The damage weakens the conduction of transmission signals in affected nerves. This reduction cause deficiency in cognition, movement, sensation, or other neurological functions in which the nerves are involved. Here we are presenting a case of a 5-year-old male child who was brought by his parents to the emergency department with the chief complaints of difficulty in swallowing, slurred speech, weakness, stiffness of muscle, fever and cough, pain, and loss of coordination. He has a history of hospitalization for pneumonia at the age of one year. Physical examination shows weakness and dullness along with localized tenderness, muscle spasms, difficulty in movements, and slurring of speech. He has undergone a blood test, Magnetic resonance imaging (MRI). And then started on immunosuppressive therapy.
... The NMO is estimated to represent approximately 1% of CNS inflammatory demyelinating diseases in Europe, with an estimated prevalence of 1-2/100,000 [1]. NMO is more frequent in women than in men, and it usually begins in young adults even if an onset in childhood is possible [2,3]. ...
... The last two clinical manifestations required the simultaneous presence of NMOSD-typical brain lesions detected in MRIs [5]. The episodes of neuritis are more commonly unilateral than bilateral, but the presence of bilateral simultaneous optic neuritis is high specific for NMO [2]. In patients with positive AQP4-IgG, a clinical core is sufficient for diagnosis. ...
... The last two clinical manifestations required the simultaneous presence of NMOSDtypical brain lesions detected in MRIs [5]. The episodes of neuritis are more commonly unilateral than bilateral, but the presence of bilateral simultaneous optic neuritis is high specific for NMO [2]. In patients with positive AQP4-IgG, a clinical core is sufficient for diagnosis. ...
Neuromyelitis optica (NMO) and neuromyelitis optica spectrum disorders (NMOSD) are a group of autoimmune inflammatory disorders of the central nervous system (CNS). Understanding of the molecular basis of these diseases in the last decades has led to an important improvement in the treatment of this disease, in particular, to the use of immunotherapeutic approaches, such as monoclonal antibodies and Hematopoietic Stem Cell Transplantation (HSCT). The aim of this review is to summarize the pathogenesis, biological basis and new treatment options of these disorders, with a particular focus on HSCT applications. Different HSCT strategies are being explored in NMOSD, both autologous and allogeneic HSCT, with the new emergence of therapeutic effects such as an induction of tolerance to auto-antigens and graft versus autoimmunity effects that can be exploited to hopefully treat a disease that still has prognosis.
... While many medications are increasingly approved for adults with MS, clinical trials on newer agents are still scant in pediatric age, and many drugs, particularly in refractory patients, are prescribed off-label. The first-line diseasemodifying agents for pediatric MS are IFN-β and glatiramer acetate; currently available data provide evidence on their efficacy and safety profiles in pediatric MS (44,45). Treatment options for pediatric patients who do not benefit from first-line therapy are less documented (44,45). ...
... The first-line diseasemodifying agents for pediatric MS are IFN-β and glatiramer acetate; currently available data provide evidence on their efficacy and safety profiles in pediatric MS (44,45). Treatment options for pediatric patients who do not benefit from first-line therapy are less documented (44,45). The newer agents approved for adults, as teriflunomide, fingolimod, and dimethyl fumarate have not been routinely used in pediatric patients, and clinical trials on the pediatric population are still ongoing. ...
Immune-mediated diseases of the central nervous system (CNS) in childhood are a heterogeneous group of rare conditions sharing the inflammatory involvement of the CNS. This review highlights the growing knowledge of childhood neuroimmune diseases that primarily affect the CNS, outlining the clinical and diagnostic features, the pathobiological mechanisms and genetics, current treatment options, and emerging challenges. The clinical spectrum of these conditions is increasingly expanded, and the underlying mechanisms of dysregulation of the immune system could vary widely. Cell-mediated and antibody-mediated disorders, infection-triggered and paraneoplastic conditions, and genetically defined mechanisms can occur in previously healthy children and can contribute to different stages of the disease. The careful evaluation of the clinical presentation and temporal course of symptoms, the specific neuroimaging and immunological findings, and the exclusion of alternative causes are mandatory in clinical practice for the syndromic diagnosis. A common feature of these conditions is that immunotherapeutic agents could modulate the clinical course and outcomes of the disease. Furthermore, specific symptomatic treatments and comprehensive multidisciplinary care are needed in the overall management. We focus on recent advances on immune-mediated demyelinating CNS disorders, autoimmune encephalitis, interferonopathies, and possible neuroimmune disorders as Rasmussen encephalitis. Better knowledge of these conditions could allow prompt diagnosis and targeted immunotherapy, to decrease morbidity and mortality as well as to improve clinical outcomes, reducing the burden of the disease due to possible long-term neuropsychiatric sequelae. Persisting controversies remain in the rigorous characterization of each specific clinical entity because of the relative rarity in children; moreover, in a large proportion of suspected neuroimmune diseases, the immune “signature” remains unidentified; treatment guidelines are mostly based on retrospective cohort studies and expert opinions; then advances in specific molecular therapies are required. In the future, a better characterization of specific immunological biomarkers may provide a useful understanding of the underlying pathobiological mechanisms of these conditions in order to individualize more tailored therapeutic options and paradigms. Multicenter collaborative research on homogeneous groups of patients who may undergo immunological studies and therapeutic trials could improve the characterization of the underlying mechanisms, the specific phenotypes, and tailored management.
... Damaged or degenerated myelin sheath leads to the impaired signal conduction towards nerve fibers resulting in neurological disorders (Nasrabady et al., 2018). Altered or deficient axonal signal conduction ability of neurons causes sensory, motor and cognitive disabilities leading to myelin degeneration disorders such as Guillain-Barré syndrome, Charcot-Marie-Tooth disease, Devic's disease and MS with differences in their etiology and phenotype in patient population (Spalice et al., 2010;Koros et al., 2013). Out of these demyelinating disorders, MS is the most prevalent pathological condition. ...
Bisphenol A (BPA) is a ubiquitously distributed endocrine disrupting chemical (EDC). Its exposure in humans has been of concern due to its increased application in the products of day to day use. BPA has been reported to cause toxicity in almost all the vital organ systems even at a very low dose level. It crosses the blood brain barrier and causes neurotoxicity. We studied the effect of BPA on the cerebral cortex of C57BL/6 J mice and examined whether BPA exposure alters the expression of axonal and myelin structural proteins. Male mice were dosed orally to 40 μg and 400 μg BPA/kg body weight for 60 days. BPA exposure resulted in memory loss, muscle coordination deficits and allodynia. BPA exposure also caused degeneration of immature and mature oligodendrocytes as evaluated by decreased mRNA levels of 2',3'-cyclic nucleotide 3' phosphodiesterase (CNPase), nestin, myelin basic protein (MBP) and myelin-associated glycoprotein-1 (MAG-1) genes revealing myelin related pathology. It was observed that subchronic BPA exposure caused neuroinflammation through deregulation of inflammatory cytokines mRNA and protein expression which further resulted into neurotoxicity through axonal as well as myelin degeneration in the brain. BPA also caused increased oxidative stress in the brain. Our study indicates that long term subchronic low dose exposure to BPA has potential to cause axonal degeneration and demyelination in the oligodendrocytes and neurons which may have implications in neurological and neuropsychological disorders including multiple sclerosis (MS), neuromyelitis optica and others.
... Idiopathic inflammatory demyelinating disorders of the central nervous system (IIDCDs) represent a broad spectrum of central nervous system (CNS) disorders thought to be of autoimmune origin. 1 The spectrum includes monophasic, multiphasic and progressive disorders ranging from highly localized forms to multifocal or diffuse variants. 2 Multiple sclerosis (MS) is the most common IIDCD worldwide 3 , while the ratio of Neuromyelitisoptica (NMO) to MS is much higher in the Asian countries compared to their western counterparts. 4 MS is a heterogeneous disorder with variable clinical and pathologic features reflecting different pathways to tissue injury. ...
... 2,3 Estos desórdenes se asocian a una morbilidad significativa, por lo que el diagnóstico precoz es esencial para un pronóstico funcional y neurológico más favorables. 4 La desmielinización suele ser secundaria a infecciones, desórdenes metabólicos, isquemia o trastornos ...
Inflammatory demyelinating diseases comprise a series of autoimmune disorders affecting myelin at the level of the central nervous system (CNS) and peripheral nervous system. They can be monophasic, multiphasic, progressive, monofocal or multifocal, and their diagnosis is usually of exclusion. We report the case of a 15-year-old female patient with headache, ataxia, hemiparesis, ophthalmoplegia and altered consciousness. Magnetic resonance imaging showed lesions compatible with demyelinating disease. Acute disseminated encephalomyelitis was diagnosed. The patient responded to treatment with corticosteroids.
... [1,2] Although these disorders are associated with significant morbidity, early diagnosis results in improved functional and neurological outcome. [3] ...
Objective:
This is a retrospective chart review of consecutive children with acquired demyelinating disorders presenting to a north Indian tertiary care hospital over 4 years. The aim of this review is to describe all the patients (with single event as well as those with recurrences) with detailed description of those who recurred.
Materials and methods:
Overall 35 cases were reviewed and their clinical presentations, diagnosis, management, and follow-up are being presented.
Results:
Out of 35 cases, 24 did not show any recurrences (seven acute disseminated encephalomyelitis (ADEM) and 17 clinically isolated syndromes). Amongst the 11 patients with recurrent demyelination, majority were multiple sclerosis (8/11, 72.7%) followed by neuromyelitis optica (NMO; 2/11), and multiphasic ADEM (1/11). The median disease duration and follow-up since onset for those with recurrent episodes is 4 years (2.5-4.5 years). Steroids caused significant improvement in acute episodes of demyelination. However, recurrent demyelinating disorders like multiple sclerosis and NMO required long-term immunomodulation. Azathioprine currently is the most favored long-term immunomodulator used in NMO. Interferon-β and glatiramer acetate are currently recommended for multiple sclerosis. However, azathioprine may be a suitable alternative in a resource-limited setting.
Conclusion:
The consensus definitions for these groups of disorders need further validation in the pediatric age group. Studies with larger population size are required to characterize features that predict future recurrences.
Background:
Brain tumors are frequent in clinical practice and associated with high morbidity and mortality. However, many diseases can present as tumefactive lesions and mimic neoplastic lesions. We aimed to determine the frequency of pseudotumoral central nervous system lesions referred to an oncology center and the frequency of the tumor mimickers.
Methods:
This was a retrospective study at the National Institute of Cancer, Rio de Janeiro, Brazil. Medical charts of patients admitted to the Neurosurgery and Pediatrics services from 2007 to 2011 were reviewed. Clinical and radiologic features of cases initially diagnosed with primary central nervous system tumors but received a final diagnosis of pseudotumoral disease were recorded.
Results:
Among 891 patients referred as primary brain tumors, 38 cases had pseudotumoral lesions (4.3%). Most were adults (63%), with mean age of 29.4 years, and women (60.5%). Most frequent symptoms were headache (28.9%), motor signs (23.7%), and seizures (15.8%). Mean time from initial symptoms to diagnosis was 12.2 months. Lesions were single in 84.2% of patients, had contrast enhancement in 45.6%, and surrounding edema in 17.4%. Twenty patients (52,6%) underwent biopsy. Systemic autoimmune diseases were the most frequent etiologies (28.9%), followed by idiopathic inflammatory demyelinating diseases, infections, and vascular abnormalities (15.8% each). Good outcome with no major deficits was observed in 60.5% cases.
Conclusions:
The frequency of pseudotumoral lesions in an oncology reference center was low. Young women were most affected, and lesions were associated more frequently with systemic autoimmune diseases. Prompt recognition is important to avoid unnecessary treatment, because most patients had a good outcome.
Immunotherapeutic strategies to treat neurodegenerative disorders have inspired the scientific community. The aim of our review is to address the translational aspects of neuroimmunology to describe the efficacy of immunotherapy in the treatment of pediatric neurodegenerative disorders. In the studies we analysed IVIG were found to be efficient in the treatment of post-streptococcal neurodegenerative disorders, even if in PANDAS, plasma-exchange (PE) showed a higher efficiency. IVIG were also successfully used in ADEM and Guillan-Barré syndrome. In Sydenham Chorea the use of methylprednisolone was found in most cases as efficient as IVIG, while in Tourette's Syndrome, Colecoxib was successfully used in one patient. Paediatric Multiple Sclerosis seems to respond better to immunosuppressant agents (Mitoxantrone, Cyclophosphamide, Natalizumab), as well as Neuromyelitis optica (Rituximab, Mycofenolate). The importance of this review relies in the attempt to draw standardized guidelines for immunotherapy in pediatric neurodegeneratve disorders.
Guillain-Barré Syndrome (GBS) is an immune-mediated peripheral neuropathy characterized by rapidly progressive symmetrical ascending weakness and sensory loss. The disease can progress rapidly and be fatal if diagnosis and treatment interventions are delayed. In most patients, the neuropathic symptoms are preceded by an antecedent infection. The patient may present with initial symptoms of upper respiratory tract infection or gastroenteritis. This article presents a case report of GBS, followed by a detailed discussion of the pathophysiology, diagnostic studies, differential diagnosis, types, prognosis, and management of patients with this condition.
