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This article, published in the journal Neuropediatrics, discusses a case of acute unilateral mydriasis in a previously healthy 10-year-old girl. Anisocoria, or unequal pupil size, can be a sign of a serious condition and requires further investigation. The differential diagnosis for this condition includes intracranial aneurysm, tumor, trauma, or acute glaucoma. In this case, the girl had a sudden onset of a dilated right pupil, blurred vision, and conjunctival irritation. However, further investigations such as a cranial MRI, EEG, and visual evoked potentials were unremarkable, and the mydriasis resolved without treatment. The authors diagnosed the condition as a benign unilateral mydriasis after ruling out other underlying pathologies. [Extracted from the article]

Rostasy K, Fauth C, Gautsch K, Laimer I, Krabichler B, Wimmer K, Frühmesser A, Kotzot D, Moshir S. Modification of risk for cancer as a coincidental finding in DNA array investigation. The high resolution of modern DNA arrays has the implification of unintended coincidental detection of gene deletions predisposing to late-onset neurological and oncological disorders. Here, we report the case of an 18-year-old girl with mild intellectual disability, facial dysmorphisms, and a microdeletion of approximately 6.3 Mb on 22q12.1q12.3 including NF2, the gene for neurofibromatosis type 2, and CHEK2, a modifier gene for breast cancer. Subsequent magnetic resonance imaging of the brain showed she had already developed bilateral vestibular schwannomas. The challenge of DNA arrays and the consequences for genetic counselling and informed consent will be discussed in the light of this unique case with a microdeletion including both a high risk and a moderate risk cancer predisposition gene. [ABSTRACT FROM AUTHOR]

Background: Baló's concentric sclerosis (BCS) is a demyelinating disorder believed to be a rare variant of multiple sclerosis (MS). Human herpesvirus 6 (HHV-6) is a highly neurotropic virus causing severe central nervous system (CNS) infections predominantly following reactivation of latent HHV-6 in immunocompromised individuals. Primary infection with HHV-6 usually occurs in early childhood manifesting as exanthema subitum. The clinical spectrum of primary infection in adolescents or adults has not yet been evaluated.Case Report: A previously healthy 13 year old girl developed acute hemianopsia and anomia 5 days after an episode of fever and malaise of unknown origin. Cerebral MRI revealed three white matter lesions, one with ring-like contrast enhancement. Lumbar puncture showed mononuclear pleocytosis of 30 cells/microl, oligoclonal IgG, and a normal protein level. Follow up cerebral MRI scans revealed lamellar concentric hemispheric lesions characteristic of BCS. The first neurological symptoms of the patient coincided with primary HHV-6 CNS infection, diagnosed by a positive PCR test of the CSF together with seroconversion. Response to antiviral and corticosteroid treatment was only temporary, but immunoglobulin treatment has so far been followed by clinical stability for 30 months.Conclusions: To our knowledge, this is the first report both of an association between HHV-6 and BCS and of immunoglobulin treatment of BCS. A late primary infection with HHV-6 might be associated with BCS. Further studies in patients with this rare disease are needed to confirm this association and to evaluate the efficacy of antiviral and immunoglobulin treatment. [ABSTRACT FROM AUTHOR]

Background: Human immunodeficiency virus (HIV) leucoencephalopathy (HIVL) is an uncommon and rapidly progressive form of AIDS dementia complex (ADC) that has remained poorly understood. Tumour necrosis factor alpha (TNFalpha), which has been implicated in the pathogenesis of ADC, is predominantly localised in macrophages in the HIV infected brain, although in vitro studies indicate that neurones can express this cytokine.Objective: To examine the clinical/neuroradiological features of HIVL and the expression of TNFalpha in HIVL.Methods: Six patients who presented with rapidly progressive dementia within four to 12 weeks of the primary manifestation of their HIV infection were evaluated. Clinical history, treatment regimens, and imaging studies were reviewed, and brain samples from three of the patients were studied by means of immunohistochemistry.Results: Imaging studies showed diffuse bilateral deep white matter changes in all six patients. Clinical and imaging abnormalities improved in five of the six patients within weeks after initiation of antiretroviral treatment. Brain biopsies of two showed pronounced microglia/macrophage activation, but only scant viral protein (gp41) expression. Staining for TNFalpha was found in microglia/macrophages, and surprisingly, in neurones also. Postmortem analysis of a third patient also showed TNFalpha expression in neurones of the frontal cortex and basal ganglia.Conclusion: This study provides the first demonstration of staining for TNFalpha in the neurones of the HIV infected brain, and suggests that the process underlying this rapidly progressive form of ADC may reflect indirect mechanisms mediated by host factors, particularly TNFalpha. [ABSTRACT FROM AUTHOR]

OBJECTIVE: To determine whether drawings can aid in the differential diagnosis of headaches in children. METHODS: Before taking any history, 226 children who were seen consecutively for the evaluation of headache were asked to draw a picture to show how their headache felt. The pictures were then scored as migraine or nonmigraine by pediatric neurologists who were blinded to the clinical history. A clinical diagnosis of headache type was determined independently by another pediatric neurologist using the usual history and examination. The diagnoses of headache type based on the pictures drawn and the clinical findings obtained were then compared to calculate the sensitivity, specificity, and predictive values of the drawings for the diagnosis of migraine. RESULTS: Children produced dramatic and insightful headache drawings. Compared with the clinical diagnosis (gold standard), headache drawings had a sensitivity of 93.1%, a specificity of 82.7%, and a positive predictive value (PPV) of 87.1% for migraine. That is, drawings that contained an artistic feature consistent with migraine (eg, pounding pain, nausea/vomiting, desire to lie down, periorbital pain, photophobia, visual scotoma) predicted the clinical diagnosis of migraine in 87.1% of cases. Predictive values were also calculated for specific migraine-associated features on drawings: artistic depiction of focal neurologic signs, periorbital pain, recumbency, visual symptoms (photophobia, scotomata), or nausea/vomiting had a PPV of >90% for migraine; severe or pounding pain had a PPV of >80% for migraine. Band-like pain was not predictive of migraine (PPV of 11.1%). Features on drawings such as sadness or crying did not differentiate migraine from nonmigraine headaches. CONCLUSIONS: Children's headache drawings are a simple, inexpensive aid in the diagnosis of headache type, with a very high sensitivity, specificity, and predictive value for migraine versus nonmigraine headaches. We encourage the use of drawings in the evaluation of any child with a headache, as an adjunct to the clinical history and physical examination. [ABSTRACT FROM AUTHOR]

Tamm-Horsfall protein (THP), a glycoprotein with a molecular weight of 95 kilodaltons, is produced and secreted in the ascending loop of Henle. To evaluate the measurement of THP in the assessment of fetal renal development and function, we stained fetal kidney sections for THP and measured THP concentrations in 129 amniotic fluid samples from healthy pregnancies, together with other parameters such as transferrin, albumin, α[sub 1] - and β[sub 2] -microglobulin. After the 16th week of gestation THP could be detected immunohistochemically in the distal tubular cells, but was not consistently detected by sandwich enzyme immunoassay until after the 20th week of gestation (detection limit 50 ng/ml). Between the 15th and 19th week of gestation THP was only detected occasionally, but after the 20th week of gestation the concentration increased significantly reaching levels of 0.4 – 4 mg/l at term. The THP concentration was lower in samples taken directly before birth than in the corresponding first urine after birth, indicating that THP is produced from the fetal kidney only and does not pass the placental barrier. This pattern was different from other proteins studied. Transferrin and albumin were significantly lower in the first urine voided, microglobulins remained unchanged, and the creatinine concentration increased. This indicates that maternal to fetal exchange or transport is likely for most of the other proteins. Measurement of THP concentrations, in addition to other proteins in the amniotic fluid, can improve fetal renal assessment, but because the range of THP concentrations is wide accurate predictions are still not possible. [ABSTRACT FROM AUTHOR]

It has been suggested that Chlamydia pneumoniae (C. pneumoniae) is involved in the pathogenesis of diverse diseases of the central nervous system (CNS), including multiple sclerosis. We report the case of a 12-year-old male with isolated recurrent optic neuritis and an associated CNS infection with C. pneumoniae. The patient presented with three attacks of optic neuritis within 5 months. A positive polymerase chain reaction for C. pneumoniae in the cerebrospinal fluid led to the diagnosis of a CNS infection with C. pneumoniae. After treatment with the antibiotic rifampicin, he experienced no further attacks during the follow-up period of 6 years. These findings suggest the possibility of a C. pneumoniae infection as a contributing factor or even causative event for the development of optic neuritis. [ABSTRACT FROM AUTHOR]

Background: Baló's concentric sclerosis (BCS) is a demyelinating disorder believed to be a rare variant of multiple sclerosis (MS). Human herpesvirus 6 (HHV-6) is a highly neurotropic virus causing severe central nervous system (CNS} infections predominantly following reactivation of latent HHV-6 in immunocompromised individuals. Primary infection with HHV-6 usually occurs in early childhood manifesting as exanthema subitum. The clinical spectrum of primary infection in adolescents or adults has not yet been evaluated. Case report: A previously healthy 13 year old girl developed acute hemianopsia and anomia 5 days after an episode of fever and malaise of unknown origin. Cerebral MRI revealed three white matter lesions, one with ring-like contrast enhancement. Lumbar puncture showed mononuclear pleocytosis of 30 cells/μl, oligoclonal lgG, and a normal protein level. Follow up cerebral MRI scans revealed lamellar concentric hemispheric lesions characteristic of ECS. The first neurological symptoms of the patient coincided with primary HHV-6 CNS infection, diagnosed by a positive PCR test of the CSF together with seroconversion. Response to antiviral and corticosteroid treatment was only temporary, but immunoglobulin treatment has so far been followed by clinical stability for 30 months. Conclusions: To our knowledge, this is the first report both of an association between HHV-6 and BCS and of immunoglobulin treatment of BCS. A late primary infection with HHV-6 might be associated with BCS. Further studies in patients with this rare disease are needed to confirm this association and to evaluate the efficacy of antiviral and immunoglobulin treatment. [ABSTRACT FROM AUTHOR]

This article, published in the journal Neuropediatrics, explores the effects of fingolimod treatment on the disease progression in children with CLN3, a neurodegenerative disorder. The study included ten children who were treated with fingolimod for at least one year. The treatment was well tolerated and did not result in any significant side effects. The results suggest that fingolimod may partially alleviate the disease progression in CLN3 patients by influencing secondary immunological reactions. [Extracted from the article]

This article, published in the journal Neuropediatrics, discusses the use of A20 (TNFAIP3) as a potential biomarker to distinguish relapse from remission in pediatric patients with monophasic MOGAD (MOG-IgG-associated disorders). MOGAD is a type of acquired demyelinating syndrome associated with antibodies against myelin oligodendrocyte glycoprotein. The study found that A20 levels were significantly lower during clinical events compared to remission in monophasic MOGAD patients. However, there was no significant difference in A20 levels between clinical events and remission in multiphasic MOGAD patients. The authors suggest that further research is needed to investigate the diagnostic, prognostic, and therapeutic implications of A20 in pediatric MOGAD patients. [Extracted from the article]

This article, published in the journal Neuropediatrics, discusses the treatment response in children with relapsing MOG-associated disease (MOGAD). The study evaluated the use of intravenous immunoglobulins (IVIG) compared to other therapies in children with relapsing disease course. The results showed that the annual relapse rate (ARR) was lower in all treatment groups compared to no treatment, with the lowest ARR observed in patients treated with IVIG alone. The study suggests that IVIG may have a favorable treatment effect in relapsing MOGAD, but further research is needed to determine the optimal treatment. [Extracted from the article]

This article, published in the journal Neuropediatrics, examines the outcome and brain volume changes over time in children with MOG-encephalitis (MOG-E), a rare clinical phenotype of MOGAD. The study included 21 children with MOG-E, who presented with symptoms such as encephalopathy, headache, focal neurological signs, seizures, or CSF pleocytosis. The results showed that the majority of children had a good clinical outcome, with resolution of MRI changes and decreasing MOG antibodies over time. However, brain volumetry measurements indicated a decrease in whole brain volume, particularly in gray matter, after onset. [Extracted from the article]

This article, published in the journal Neuropediatrics, discusses the use of MRI imaging in 10 children with enterovirus-associated acute flaccid myelitis (AFM). AFM is characterized by limb weakness, decreased muscle tone, and cranial nerve involvement, and is predominantly caused by enterovirus infections. The MRI findings in these patients showed longitudinal spinal cord lesions mainly affecting the gray matter, as well as lesions in the brainstem and cerebellum. Supratentorial involvement was uncommon. The study confirms previous findings about the MRI imaging findings in enterovirus-associated AFM. [Extracted from the article]

This article, published in the journal Neuropediatrics, examines the frequency of antibody-mediated autoimmune encephalitis (AE) in children with suspected encephalitis. The researchers reviewed medical records of 1,341 children treated in Hannover and Oldenburg between 2011 and 2020. They found that antibody-mediated AE was not frequent in children, but suggested that further testing for serum MOG antibodies should be included in diagnostic criteria. The study highlights the need for continued research to identify other potential antibodies involved in AE in children. [Extracted from the article]

This article, published in the journal Neuropediatrics, discusses the clinical and imaging features of children with autoimmune encephalitis (AE) and glial fibrillary acidic protein (GFAP) antibodies. The study included 16 children who met the diagnostic criteria for possible AE and tested positive for GFAP antibodies in their serum and/or cerebrospinal fluid. The children presented with symptoms such as encephalopathy, headache, focal seizures, and ataxia. MRI imaging revealed abnormalities in 13 out of 16 children, including freckling lesions in the pons and nucleus caudate, signal abnormalities around the aqueduct, and myelin indicative of transverse myelitis. The study recommends including GFAP antibody testing in the work-up of children with suspected AE, particularly in cases involving brainstem involvement. [Extracted from the article]

The chemotherapeutic agent mitoxantrone is approved for the treatment of aggressive multiple sclerosis (MS) in adults, Its use, however, is limited by the risk of severe adverse events including cardiotoxicity, myelosuppression, liver toxicity and secondary leukemia. The aim of this retrospective study is to present data on the safety, tolerability and efficacy of mitoxantrone in a small cohort of children with MS. 4 pediatric MS patients with a high relapse rate or severe, disabling relapses were treated with mitoxantrone and followed for 3.8-18 years. The cumulative dose of mitoxantrone was 36, 68, 84 and 120 mg/m2, respectively. The frequency and severity of relapses as well as disability scores, decreased in the year after treatment onset. Short-term adverse events were transient in all cases. Cardiac monitoring by transthoracic echocardiography (IrE) showed asymptomatic left ventricular dysfunction during treatment in I patient, which was again normal at the next assessment. Long-term adverse events, including late-onset mitox-antrone-induced cardiotoxicity or secondary leukemia did not occur during the follow-up period. In our cohort of pediatric MS patients, mitoxantrone showed short-term reduction of disease activity and no long-term adverse events on follow-up. However, the risk of mitoxantrone-induced cardiotoxicity or toxic leukemia remains a life-long threat. [ABSTRACT FROM AUTHOR]

Background Cerebral palsy (CP) is the most common motor impairment in childhood and often accompanied by a broad spectrum of comorbidities. Data are sparse concerning visual impairment (VI) and functional classification among CP children. Objective The objective of this study was to analyze the prevalence of VI among children with CP and to investigate a possible association between VI and Gross Motor Function Classification System (GMFCS) and the Bimanual Fine Motor Function (BFMF). Methods In this hospital-based study, records of 200 children with CP aged 2 to 17 years were reviewed. Results Overall, VI was found in 59.5% of children with CP. Prevalence of VI was higher when compared with non-CP children. A correlation between GMFCS as well as BFMF and severity of VI was found. Children with severe CP were at greater risk for severe VI, especially cerebral VI compared with children with mild CP. Conclusion VI is a significant problem in children with CP and is correlated with motor function. Children with CP should undergo detailed ophthalmologic and orthoptic assessment to enable early intervention. [ABSTRACT FROM AUTHOR]

Introduction: While monophasic and relapsing forms of myelin oligodendrocyte glycoprotein antibody associated disorders (MOGAD) are increasingly diagnosed world-wide, consensus on management is yet to be developed. Objective: To survey the current global clinical practice of clinicians treating MOGAD. Method: Neurologists worldwide with expertise in treating MOGAD participated in an online survey (February–April 2019). Results: Fifty-two responses were received (response rate 60.5%) from 86 invited experts, comprising adult (78.8%, 41/52) and paediatric (21.2%, 11/52) neurologists in 22 countries. All treat acute attacks with high dose corticosteroids. If recovery is incomplete, 71.2% (37/52) proceed next to plasma exchange (PE). 45.5% (5/11) of paediatric neurologists use IV immunoglobulin (IVIg) in preference to PE. Following an acute attack, 55.8% (29/52) of respondents typically continue corticosteroids for ≥ 3 months; though less commonly when treating children. After an index event, 60% (31/51) usually start steroid-sparing maintenance therapy (MT); after ≥ 2 attacks 92.3% (48/52) would start MT. Repeat MOG antibody status is used by 52.9% (27/51) to help decide on MT initiation. Commonly used first line MTs in adults are azathioprine (30.8%, 16/52), mycophenolate mofetil (25.0%, 13/52) and rituximab (17.3%, 9/52). In children, IVIg is the preferred first line MT (54.5%; 6/11). Treatment response is monitored by MRI (53.8%; 28/52), optical coherence tomography (23.1%; 12/52) and MOG antibody titres (36.5%; 19/52). Regardless of monitoring results, 25.0% (13/52) would not stop MT. Conclusion: Current treatment of MOGAD is highly variable, indicating a need for consensus-based treatment guidelines, while awaiting definitive clinical trials. [ABSTRACT FROM AUTHOR]

Pediatric multiple sclerosis (MS) is one of the most important acquired neurological disorders in childhood and adolescence. A timely recognition, diagnosis and treatment are of utmost importance. This article highlights the current state of knowledge on the etiology, pathogenesis, diagnosis, clinical presentation and treatment in childhood. Although the rate of progression of disability in the early years is slower in younger patients compared to adults, a disease-modifying therapy should be started once MS is diagnosed. [ABSTRACT FROM AUTHOR]

Epilepsy is a common finding in patients with chromosomal macro- and micro-rearrangements but only few aberrations show a constant pattern of seizures. DNA array-based studies have reported causative copy number variations ( CNVs) in 5-30% of patients with epilepsy with or without co-morbidities. The interpretation of many of the detected CNVs remains challenging. In order to identify CNVs carrying epilepsy-related genes we investigated 43 children with various patterns of epileptic seizures, intellectual disability (ID), and minor dysmorphism, using the Illumina® Infinium Human1M-DuoV1 array. In three patients we found likely causative de novo CNVs, i.e. deletions in 1q41q42.12 (3.4 Mb) and 19p13.2 (834 kb), and a mosaic two-segment duplication in 17p13.2 (218 kb) and 17p13.1 (422 kb). In six additional patients there were aberrations (a deletion in one and duplications in five patients) with uncertain clinical consequences. In total, the finding of causative chromosomal micro-rearrangements in 3 out of 43 patients (7%) and potentially causative CNVs in 6 additional patients (14%) with epilepsy and ID but without major malformations confirms the power of DNA arrays for the detection of new disease-related genetic regions. [ABSTRACT FROM AUTHOR]

New therapies are being evaluated by clinical trials and, if efficacious, introduced for the treatment of adult MS. The role of these new and existing agents in the management of pediatric MS has yet to be defined. Pediatric investigation plans are now required by the Food and Drug Administration and European Medicines Agency for approval of new biological agents, providing an important opportunity to gather much-needed data for clinicians caring for children and adolescents with MS. However, challenges include the small number of patients, and the need for efficient yet comprehensive study designs incorporating factors necessary to inform the clinical care of children with MS. The elected Steering committee of the International Pediatric MS Study Group (IPMSSG) conducted a structured review of existing data on the disease-modifying therapies in pediatric MS and developed a consensus statement, which was further modified by the IPMSSG general membership, using an online survey tool. Fifty-one IPMSSG members from 21 countries responded to the survey, and 50 approved the final statement. Consensus recommendations regarding use of existing first- and second-line therapies, as well as a proposed definition for inadequate treatment response, are presented. Recommendations for the use and evaluation of emerging therapies (currently in phase III clinical trials or recently approved for adult MS) are discussed. The IPMSSG endorses the inclusion of pediatric MS patients in trials evaluating appropriate new and emerging therapies. Mechanisms for conducting high-impact, multicenter studies, including long-term follow-up in pediatric MS, are required to ensure that all MS patients, irrespective of age, benefit from advances in MS therapeutics. [ABSTRACT FROM AUTHOR]

We investigate common pathophysiology in paediatric and adult multiple sclerosis (MS) by comparison of cerebrospinal fluid (CSF) data.We compared cerebrospinal fluid (CSF) data from eight patient groups with onset of MS at 7 to 29 years (n=184). A new statistics program allows sensitive detection, quantifies the mean amount of intrathecal Ig synthesis in groups based on the 96% reference range of 4100 non-inflammatory controls, corrects for age-related increase of bloodderived albumin and immunoglobulins in CSF, and presents graphical data interpretation in Reibergrams. Already at onset of MS before puberty (≤10 years) the frequency of intrathecal IgG synthesis (oligoclonal IgG) was 100% like in adults with 98%, but the amount of intrathecal IgG increases twofold during puberty. Intrathecal IgM synthesis is most frequent before and during puberty (in 57-67% of patients) compared with 41% in adults. The amount of intrathecal IgM synthesis before puberty is only 30% of that in adults. IgG and IgM Index are biased evaluations not suitable for characterizing agerelated dynamics. A twofold age-related increase of the albumin quotient, QAlb, as a measure of the blood-CSF barrier function, represents normal physiological growth. Cell counts in CSF are low. The pre-puberty gender ratio is about 1:1. Intrathecal antibodies against measles, rubella and/or varicella zoster virus are detected in 73% of patients before puberty compared with 89% of adults. Individual paediatric patients (n=17), with sequential punctures over 2-5 years, show constant quantities of intrathecal IgM and specific antibodies. In conclusion, paediatric MS already at first clinical manifestation shows the complete, neuroimmunological data pattern in CSF, i.e. inflammatory signs are not gradually evolving. Paediatric and adult MS differ quantitatively but not qualitatively in neuroimmunological patterns which does not allow for discrimination between 'early' and 'late' onset MS. CSF analysis may help to discriminate between acute and monosymptomatic chronic inflammatory disease already at earliest clinical manifestation. [ABSTRACT FROM AUTHOR]

Nd:YAG, argon and diode lasers have been used in neurosurgical procedures including neuroendoscopy. However, many neurosurgeons are reluctant to use these lasers because of their inappropriate wavelength and uncontrollable tissue interaction, which has the potential to cause serious complications. Recently, a 2.0-μm near infrared laser with adequate wavelength and minimal tissue penetration became available. This laser was developed for endoscopic neurosurgical procedures. It is the aim of the study to report the initial experiences with this laser in neuroendoscopic procedures. We have performed 43 laser-assisted neuroendoscopic procedures [multicompartmental congenital, posthaemorrhagic or postinfectious hydrocephalus ( n = 17), tumour biopsies ( n = 6), rescue of fixed and allocated ventricular catheters ( n = 2), endoscopic third ventriculostomy (ETV, n = 17) and aqueductoplasty ( n = 1)] in 41 patients aged between 3 months and 80 years. The laser beam was delivered through a 365-µm bare silica fibre introduced through the working channel of a rigid endoscope. It was used for the opening of cysts, perforating the third ventricular floor, and for coagulation prior to and after biopsy. The therapeutic goals [creating unhindered cerebrospinal fluid (CSF) flow between cysts, ventricles and cisterns, sufficient tissue samples for histopathological diagnosis and catheter rescue] were achieved in 40 patients by the first and in 2 patients by a second neuroendoscopic operation. In one child, a CSF shunt was later required despite patency of the created stoma proven by magnetic resonance imaging (MRI). In another patient ETV was abandoned due to a tiny third ventricle. There was neither mortality nor transient or permanent morbidity. The authors conclude that the use of the 2.0-µm near infrared laser enables safe and effective procedures in neuroendoscopy. [ABSTRACT FROM AUTHOR]

Abstract: Opsoclonus–myoclonus syndrome (OMS) in children is a rare disorder including a severe eye movement disturbance, myoclonia, ataxia and often developmental retardation. Both OMS forms, idiopathic or neuroblastoma-associated (paraneoplastic), have been suspected to be autoimmune. Recently, autoantibodies have been found in OMS sera. We here show that autoantibodies in OMS, both intracellular and surface binding, belong mainly to the IgG3 subclass, although the total serum IgG3 level is normal. These results support the autoimmune hypothesis and point to a protein autoantigen as antigenic target. [Copyright &y& Elsevier]

The article offers a look whether autoantibodies that bind properly folded oligodendrocyte glycoprotein (MOG) are present in the serum and central nervous system (CNS) parenchyma of patients with multiple sclerosis (MS)and controls. Results showed that autoantibodies recognizing MOG are rarely present in the serum of patients with MS. Collectively, the data also demonstrated that autoantibodies recognizing folded MOG are present in the CNS parenchyma of patients.

An abstract of the study "Limbic encephalitis in children and adolescents," by E. Haberlandt et al, published in the October 19, 2010 online issue of the journal "Archives of Disease in Childhood" is presented.

Objective Limbic encephalitis is rare in people <18 years of age and rarely given a formal diagnosis. Design Retrospective study on presentation and outcome of children and adolescents with the clinico-radiological syndrome of limbic encephalitis tested for specific neuronal autoantibodies (Abs) over 3.5 years. Setting Assessment, diagnosis, treatment and follow-up at 12 neuropaediatric and neurological departments in Europe, with Abs determined in Bonn, Germany and Oxford, UK. Patients Ten patients <18 years of age who presented with a disorder mainly affecting the limbic areas of <5 years' duration with MRI evidence of mediotemporal encephalitis (hyperintense T2/FLAIR signal, resolving over time). Results Median age at disease onset was 14 years (range 3-17). Eight patients had defined Abs: one each with Hu or Ma1/2 Abs, four with high titre glutamic acid decarboxylase (GAD) Abs, two of whom had low voltage-gated potassium channel (VGKC) Abs and two with only low titre VGKC Abs. A tumour was only found in the patient with Hu Abs (a neuroblastoma). After a median follow-up of 15 months with corticosteroid or intravenous immunoglobulin treatment, starting after a median of 4 months, two patients recovered, eight remained impaired and one died. Conclusions Limbic encephalitis is a disease that can occur in childhood or adolescence with many of the hallmarks of the adult disorder, suggesting that both result from similar pathogenic processes. Since most of the cases were non-paraneoplastic, as now also recognised in adults, more systematic and aggressive immunotherapies should be evaluated in order to improve outcomes. [ABSTRACT FROM AUTHOR]