Optic neuritis as sign presentation of acute disseminated encephalomyelitis following Mycoplasma pneumoniae infection

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Acute disseminated encephalomyelitis: A retrospective study of 20 children in a pediatrics department in Tunisia.

BACKGROUND: Acute disseminated encephalomyelitis (ADEM) is an inflammatory demyelinating disorder of the central nervous system. Little information is available about the clinical and neuroradiological profile or the follow-up of this disease in Tunisian children. AIM: To determine the clinical, laboratory, and radiological features and the outcome of ADEM in children admitted to the pediatrics department of a university hospital in Tunisia. METHODS: All children ≤ 18 years old presenting with ADEM and admitted to the tertiary referral center for pediatrics at Sahloul University Hospital from January 2000 to December 2020 were included in the study. The diagnosis of ADEM was confirmed according to the international pediatric multiple sclerosis study group criteria. RESULTS: A total of 20 patients (13 girls and 7 boys) fulfilled the diagnostic criteria for ADEM. The mean age at diagnosis was 5.6 years. The clinical presentation included polyfocal neurological signs such as cranial hypertension (45%), seizures (35%), and motor weaknesses (55%). Pyramidal tract signs and cranial nerve palsies were noted in 55% of cases. Brain magnetic resonance imaging showed particular features, namely, a relapsing tumor-like form in one case, and optic neuritis and demyelinating lesions of the white matter in the brain and the spinal cord with gadolinium cerebral ring-like enhancement in another case. The treatment consisted of intravenous immunoglobulin in 16 cases (80%) and corticosteroid in 19 cases (95%). Plasmapheresis was used for one patient. Complete recovery was observed in 12 patients (60%); 19 patients (95%) had a monophasic course of the disease while only one patient developed multiphasic ADEM. CONCLUSIONS: ADEM remains a difficult diagnosis in children. Nevertheless, after prompt diagnosis and adequate treatment, most children with ADEM have a favorable outcome with restitutio ad integrum.

Clinical time course of pediatric acute disseminated encephalomyelitis.

The detailed clinical time course in acute disseminated encephalomyelitis (ADEM) from initial symptoms, through exacerbation, to remission has not been widely reported. Hence, this study aimed to investigate the clinical time course of pediatric ADEM. This was a multicenter retrospective study based on registry data from medical chart reviews. The study included children who met the international consensus diagnostic criteria for ADEM. The patients comprised 18 boys and 6 girls, with a mean age of 5.5 ±â€¯3.3 years at onset. From onset, the time until peak neurological symptoms, time until initial improvement, and time until full recovery was 3.1 ±â€¯3.7 days, 6.0 ±â€¯4.5 days, and 26 ±â€¯34 days, respectively. Twenty-three (96%) patients were treated with high-dose methylprednisolone (mPSL) with a mean duration of 4.1 ±â€¯4.0 days from onset. The condition of 15 patients (65%) improved within 3 days of high-dose mPSL initiation, whereas, that of four patients began to improve after >5 days of high-dose mPSL initiation. Only one patient (4%) did not achieve full recovery despite treatment with high-dose mPSL, intravenous immunoglobulin, and plasma exchange. This study presents the detailed clinical time course in pediatric ADEM in Japan. Progression of neurologic deficits typically lasts a few days, with initial improvement in 1 week leading to full recovery within 1 month.

Transverse myelitis plus syndrome and acute disseminated encephalomyelitis plus syndrome: a case series of 5 children.

IMPORTANCE: Classically, transverse myelitis and acute disseminated encephalomyelitis are considered central nervous system demyelinating conditions. In both conditions, the spinal cord is involved to varying degrees, and there is a variety of presentations, usually involving some degree of progressive paralysis of the upper and/or lower extremities. Treatment usually consists of high-dose intravenous steroids in addition to plasma exchange and/or intravenous immunoglobulin. In some cases, immunosuppressive medications, such as intravenous cyclophosphamide, have been used with variable success. Cases with atypical features on examination, imaging, or with neurophysiological studies may be helpful in shedding light on the etiology and/or pathophysiology because many of these patients have permanent disabilities despite appropriate treatment. OBSERVATIONS: This case series presents 5 pediatric cases observed from 2009-2012 at our medical center, Children's Medical Center Dallas. These cases were notable because they provided evidence of autoimmune events affecting the central nervous system but with additional peripheral axonal pathology. CONCLUSIONS AND RELEVANCE: We describe these cases with respect to findings that suggest a variant of these conditions that have concomitant nerve-root involvement. These patients had worse outcomes than typical patients with transverse myelitis/acute disseminated encephalomyelitis, and these observations build on previous work by other investigators that highlighted persistent flaccid paralysis and electrophysiological evidence of axonal loss portending a poorer prognosis. Furthermore, these cases suggest a potential role for approaching how we classify subtypes of transverse myelitis and acute disseminated encephalomyelitis.

Defining encephalopathy in acute disseminated encephalomyelitis.

The International Pediatric Multiple Sclerosis Study Group requires the presence of encephalopathy to diagnose acute disseminated encephalomyelitis. Clinical characteristics of encephalopathy are inadequately delineated in the pediatric demyelinating literature. The authors' purpose was to better define encephalopathy in pediatric acute disseminated encephalomyelitis by describing the details of the mental status change. A retrospective chart review was conducted for 25 children diagnosed with acute disseminated encephalomyelitis according to the International Pediatric Multiple Sclerosis Study Group guidelines. Frequency of encephalopathy-defining features was determined. Clinical characteristics, cerebrospinal fluid findings, and electroencephalography (EEG) findings were compared between patients with different stages of encephalopathy. The authors found irritability (36%), sleepiness (52%), confusion (8%), obtundation (20%), and coma (16%) as encephalopathy-defining features in acute disseminated encephalomyelitis. Twenty-eight percent had seizures, and 65% demonstrated generalized slowing on EEG. Approximately half of the patients in this study were diagnosed with encephalopathy based on the presence of irritability and/or sleepiness only. Such features in young children are often subtle and transient and thus difficult to objectively determine.

Immune-mediated CNS diseases: a review on nosological classification and clinical features.

The immune-mediated diseases of the central nervous system (CNS) cover a wide range of clinical manifestations. Over the last years, considerable efforts have been made to establish a nosologic concept based upon distinctive pathophysiological characteristics of the single diseases. We describe the historically defined entities of immune-mediated diseases that primarily, but not exclusively, are affecting myelin structures. These include very rare entities as Schilder's, Balo's and Marburg's disease or the chronic and relapsing types of optic neuritis, for which evidence based paradigms still are virtually missing. In other, slightly more frequent diseases as neuromyelitis optica (NMO), advances in the concepts of specific biological features have been achieved and are beginning to transform into changes in clinical concepts. Acute disseminated encephalomyelitis (ADEM) and multiple sclerosis (MS) are by far the most frequent entities in this group and thus the only ones for which extensive empirical data on disease biology and evidence based clinical management strategies exist by now. For the most important entities, clinical features and therapeutic approaches are reviewed on the basis of current evidence. The results of basic science studies are assessed for their implications in nosological classification.

Acute disseminated encephalomyelitis and its place amongst other acute inflammatory demyelinating CNS disorders.

The diagnosis of acute inflammatory demyelinating CNS conditions is complex and this is reflected in variations in how cohorts are defined across studies. For some conditions the diagnosis relies on whether it is monophasic or relapsing, in others the anatomical site of inflammation is used as a means of categorisation. Clinical features such as precipitants, gender and age may affect the probability of certain diagnoses, but are not highly accurate. Exclusive features for the pathology are identifiable for some but not all conditions, and are seldom available during life. Specific markers such as antibodies are informative and new developments in this area are likely in the near future. This review outlines the features and classification of acute disseminated encephalomyelitis and contrasts it with other related conditions before attempting to define a pragmatic organisation of these conditions based upon present evidence.

[Clinical re-evaluation of pediatric inflammatory disorders of the central nervous system:a study based on the new classification criteria proposed by the International Pediatric MS Study Group].

A retrospective analysis of the clinical and MRI features in 20 Japanese children diagnosed with central nervous system inflammatory demyelinating disorders was performed. Using the new criteria proposed by International Pediatric MS Study Group, half of children were reclassified into clinical isolated demyelinating syndrome (CIS). Presence of seizures and a pattern of diffuse bilateral lesions on brain MRIs are more frequent in children with ADEM than in CIS. However we suggest these features and encephalopathy may be associated with the age of patients. Furthermore, though persistence of abnormal MRI lesions is significantly more likely in the group of CIS, none of these patients had a subsequent recurrence or developed MS during the follow-up period. The prediction of patient prognosis seems to be difficult even based on the new criteria, and the nationwide multicenter analysis may be necessary in Japan for acquiring the definite conclusion.

[Background of the new definitions for pediatric multiple sclerosis].

The characteristics of pediatric multiple sclerosis (MS), especially with the onset below ten years of age, are different from those of adult-onset MS. Polysymptomatic and encephalopathic features are frequently observed in pediatric MS. The MRI findings in a half of pediatric MS do not fulfill the McDonald criteria. There are different opinions on the prognosis of pediatric MS. Pediatric patients with the biphasic inflammatory demyelination and the good prognosis has been reported, which was previously classified in MS. For the purpose of distinguishing transient demyelinating syndromes from the lifelong disease, the new definitions for pediatric MS and related disorders were proposed. The surveillance of pediatric MS using the new definitions has been started in Japan.

Encefalomielitis aguda diseminada en pediatría: presentación de la casuística (2000-2008): Hospital de Niños J. M de Los Ríos, Caracas-Venezuela: (primer premio como póster con mejor contenido científico: LIV Congreso Nacional de Pediatría septiembre 2008): [revisión] / Acute disseminated encephalomyelitis in pediatrics: presentation of the series (2000-2008): HospitaL de Niños JM de Los Ríos Caracas-Venezuela: (first prize poster presentation LIV Congreso Nacional de Pediatria september 2008): [review]

La Encefalomielitis Aguda Diseminada (EMAD) es una leucoencefalopatía adquirida, de naturaleza inflamatoria, autoinmune, de presentación predominante en la edad pediátrica; generalmente es monofásica, polisintomática y con frecuente compromiso del estado de conciencia. No hay marcadores biológicos para su reconocimiento, por lo que la presentación clínica aunada a los hallazgos en la Resonancia Magnética cerebral son los que permiten realizar una adecuada aproximación diagnóstica. Su curso es generalmente favorable, sin embargo, el tratamiento con esteroides puede resultar beneficioso. Se presenta la experiencia con 16 pacientes atendidos en el Hospital de Niños “J.M de Los Ríos”, Caracas-Venezuela, en un periodo de 8 años (abril 2000-abril 2008), a la luz de los últimos criterios propuestos para la clasificación de la EMAD.

Acute Disseminated encephalomyelitis (ADEM) is an acquired inflammatory autoimmune nature leucoencephalopathy, which occurs more frequently in the pediatric age group. It is usually monophasic, polysymptomatic and with frequent deteriorating consciousness. There are no biological markers for diagnosis, therefore the clinical presentation coupled with findings in the brain Magnetic Resonance Imaging allow a proper diagnostic approach. Its course is generally favorable; however treatment with brain steroids can be beneficial. We present the experience with 16 patients treated at children’s Hospital “J.M de Los Ríos”, Caracas-Venezuela, in a period of 8 year (April 2000–April 2008), in the light of recent proposed criteria for the classification of ADEM.

Severe steroid-resistant post-infectious encephalomyelitis: general features and effects of IVIg.

Based on their presumed immuno-mediated etiology, post-infectious CNS disorders are commonly treated with high-dose steroids. Factors influencing treatment effectiveness, possible alternative options for steroid-resistant cases, and their outcome profiles, remain unclear. We here describe the clinical features, the prognosis and the efficacy of i. v. immunoglobulins (IVIg) in a series of severe ADEM refractory to steroids. We performed an inception cohort study on inpatients of the Neurologic and Infectious Disease Clinics, consecutively admitted over eight years, with a minimum two-year follow-up. Nineteen patients affected by classic and site-restricted ADEM were treated with IVIg after steroid failure. Five other patients received IVIg as first-line treatment due to steroids contraindications: although not included in the analysis, they were monitored for anecdotal comparison. Steroids were administered as IV 6-methylprednisolone (6-MP) 500/1000 mg daily until a maximum dose of 6-8 g; IVIg were administered at 0.4 g/kg/day for 5 days. The outcome was assessed by the Scripps Neurological Rating Scale (SNRS) score with determined periodicity. We observed that steroid-resistant patients showed high prevalence of PNS damage (89%) and myelitis (95 %). Other features were old age, severe disability at onset, and moderate to severe blood-brain-barrier (BBB) damage on CSF. In 10/19 patients (53 %) IVIg were effective, the clinical improvement beginning within the end of the five-day cycle,without relapses. Prominent effects of IVIg were detectable on motor dysfunction. Milder onset disability (p = 0.013) and lower CSF albumin (p = 0.006) were the predictors of IVIg response. Among steroid-free patients, 3/5 were responsive to IVIg. We conclude that IVIg can be useful in a portion of patients with severe steroid-resistant ADEM and prominent motor dysfunction. Unsolved issues regard the usefulness of IVIg in less selected groups, and the spectrum of their clinical effects.

Postinfectious inflammatory disorders: subgroups based on prospective follow-up.

BACKGROUND: Acute disseminated encephalomyelitis (ADEM) refers to a monophasic acute multifocal inflammatory CNS disease. However, both relapsing and site-restricted variants, possibly associated with peripheral nervous system (PNS) involvement, are also observed, and a systematic classification is lacking. OBJECTIVE: To describe a cohort of postinfectious ADEM patients, to propose a classification based on clinical and instrumental features, and to identify subgroups of patients with different prognostic factors. METHODS: Inpatients of a Neurologic and Infectious Disease Clinic affected by postinfectious CNS syndrome consecutively admitted over 5 years were studied. RESULTS: Of 75 patients enrolled, 60 fulfilled criteria for ADEM after follow-up lasting from 24 months to 7 years. Based on lesion distribution, patients were classified as encephalitis (20%), myelitis (23.3%), encephalomyelitis (13.3%), encephalomyeloradiculoneuritis (26.7%), and myeloradiculoneuritis (16.7%). Thirty patients (50%) had a favorable outcome. Fifteen patients (25%) showed a relapsing course. Poor outcome was related with older age at onset, female gender, elevated CSF proteins, and spinal cord and PNS involvement. All but two patients received high-dose steroids as first-line treatment, with a positive response in 39 (67%). Ten of 19 nonresponders (53%) benefited from high-dose IV immunoglobulin; 9 of 10 had PNS involvement. The data were not controlled. CONCLUSIONS: A high prevalence of "atypical variants" was found in this series, with site-restricted damage or additional peripheral nervous system (PNS) involvement. Prognosis and response to steroids were generally good, except for some patient subgroups. In patients with PNS involvement and steroid failure, a favorable effect of IV immunoglobulin was observed.

Postinfectious encephalomyelitis.

Postinfectious forms of encephalomyelitis, also termed acute disseminated encephalomyelitis (ADEM), form one of several categories of inflammatory demyelinating disorders of the central nervous system (CNS). Recent large, retrospective case series have refined our understanding of the clinical, laboratory, and neuroimaging characteristics of ADEM. The differences between childhood and adult ADEM, risks of development of multiple sclerosis, and the contributions of recent studies to refining the nosology of CNS demyelinating syndromes are discussed.

[Borderline forms of multiple sclerosis]. / Les formes frontières de sclérose en plaques.

Multiple sclerosis (MS) has been described for more than a century, but its cause remains unknown and no simple diagnostic marker is available. Therefore, it is not surprising that numerous articles were written on closely related diseases, borderline forms of multiple sclerosis. Different forms have been distinguished: a clinical form of MS (Devic's neuromyelitis optica), pathological forms (Balo, Schilder, Maburg), forms associated with MS (peripheral neuropathy, autoantibodies) and closely related disorders (acute disseminated encephalomyelitis).