Encephalopathy in children: an approach to assessment and management

Davies, Emily; Connolly, Daniel; Mordekar, Santosh R.

doi:10.1136/adc.2011.300998

Cited by 35 publications

(19 citation statements)

References 26 publications

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“…Clinical observation, investigation and treatment often need to be carried out simultaneously. 1 Antibody-mediated encephalopathies, in which patients present with neurological syndromes associated with serum and/or cerebrospinal fluid (CSF) antibodies directed against ion channels, receptors and associated proteins, are now well recognised in adults. [4][5][6] Although the antigenic targets are generally expressed throughout the nervous system, the clinical picture can localise to specific areas of the central nervous system (CNS).…”

Section: Introductionmentioning

confidence: 99%

Paediatric autoimmune encephalopathies: clinical features, laboratory investigations and outcomes in patients with or without antibodies to known central nervous system autoantigens

Hacohen

Wright

Waters

et al. 2012

J Neurol Neurosurg Psychiatry

238

209

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ObjectiveTo report the clinical and investigative features of children with a clinical diagnosis of probable autoimmune encephalopathy, both with and without antibodies to central nervous system antigens.MethodPatients with encephalopathy plus one or more of neuropsychiatric symptoms, seizures, movement disorder or cognitive dysfunction, were identified from 111 paediatric serum samples referred from five tertiary paediatric neurology centres to Oxford for antibody testing in 2007–2010. A blinded clinical review panel identified 48 patients with a diagnosis of probable autoimmune encephalitis whose features are described. All samples were tested/retested for antibodies to N-methyl-D-aspartate receptor (NMDAR), VGKC-complex, LGI1, CASPR2 and contactin-2, GlyR, D1R, D2R, AMPAR, GABA(B)R and glutamic acid decarboxylase.ResultsSeizures (83%), behavioural change (63%), confusion (50%), movement disorder (38%) and hallucinations (25%) were common. 52% required intensive care support for seizure control or profound encephalopathy. An acute infective organism (15%) or abnormal cerebrospinal fluid (32%), EEG (70%) or MRI (37%) abnormalities were found. One 14-year-old girl had an ovarian teratoma. Serum antibodies were detected in 21/48 (44%) patients: NMDAR 13/48 (27%), VGKC-complex 7/48(15%) and GlyR 1/48(2%). Antibody negative patients shared similar clinical features to those who had specific antibodies detected. 18/34 patients (52%) who received immunotherapy made a complete recovery compared to 4/14 (28%) who were not treated; reductions in modified Rankin Scale for children scores were more common following immunotherapies. Antibody status did not appear to influence the treatment effect.ConclusionsOur study outlines the common clinical and paraclinical features of children and adolescents with probable autoimmune encephalopathies. These patients, irrespective of positivity for the known antibody targets, appeared to benefit from immunotherapies and further antibody targets may be defined in the future.

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Section: Introductionmentioning

confidence: 99%

Paediatric autoimmune encephalopathies: clinical features, laboratory investigations and outcomes in patients with or without antibodies to known central nervous system autoantigens

Hacohen

Wright

Waters

et al. 2012

J Neurol Neurosurg Psychiatry

238

209

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“…Given the fact that encephalopathies comprise disorders of hereditary or non-hereditary nature, i.e. congenital or acquired conditions which are primarily characterised by brain damage [16,17] and may affect the head size, it seems justified that the present findings show significant correlations of head size in both assessment systems (dysmorphology versus traditional) to the classification of the subjects based on etiopathogenesis, presence and character of encephalopathy.…”

Section: Discussionmentioning

confidence: 73%

“…The present study shows that cranium growth defects can be observed in children and Encephalopathies are all kinds of conditions affecting brain structure. Depending on the patients' age and the causes, it is possible to distinguish various types of encephalopathy [16,17]. Taking into account the character and expected presence of encephalopathy, the current study identified subjects with progressive and non-progressive encephalopathy.…”

Section: Discussionmentioning

confidence: 99%

Abnormal cranium development in children and adolescents affected by syndromes or diseases associated with neurodysfunction: a retrospective analysis

Guzik¹,

Perenc²,

Drużbicki³

et al. 2019

Preprint

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Background: Microcephaly and macrocephaly are cranial growth defects and at the same time clinical symptoms. There are two assessment criteria, one applied in dysmorphology and the other conventionally used in clinical practice. Which one should be used on a daily basis and what is the relationship between microcephaly or macrocephaly and the syndromes or diseases associated with neurodysfunction?. The study was designed to investigate the associations between abnormal cranium development (head size) and diseases or syndromes linked with neurodysfunction, based on essential data collected upon admission of the patients to the Neurological Rehabilitation Ward for Children and Adolescents.Methods: Retrospective analysis took into account 327 children and adolescents with medical conditions associated with neurodysfunction. In order to identify the subgroups of subjects with microcephaly, normal head size, and macrocephaly two assessment criteria were applied, one system commonly used in clinical practice and the other -applied in dysmorphology. To enable assessment based on all the above criteria, z-score for head circumference (z-score hc) was computed for each study participant. Normative values published earlier were used as reference. Results:The subjects were divided into seven subgroups -six with diseases/syndromes usually associated with encephalopathy (90.2%), and one with diseases commonly occurring without encephalopathy, i.e. neuromuscular disorders (9.8%). The findings showed more/less common cooccurrence of as well as statistically significant relationships between: the size of the headdysmorphology classification (hc), and diseases or syndromes associated with neurodysfunction (p=0.026), classification with regard to etiopathogenesis, presence and character of encephalopathy (p=0.010), epilepsy (p<0.001), hypothyroidism p=0.024, Cp=0.105); and the size of the headtraditional classification (hc) and entities or syndromes associated with neurodysfunction (p<0.001), classification with regard to etiopathogenesis, presence and character of encephalopathy (p<0.001), encephalopathy in neural tube defects (p=0.004, Cp=0.564), type of spasticity (p=0.034, Cp=0.211), epilepsy (p<0.001), as well as hypothyroidism (p<0.001). Conclusions: Children and adolescents with syndromes or diseases associated with neurodysfunction

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“…Inflammatory markers would be expected to be raised in meningitis, encephalitis and cerebral abscesses, though not invariably 9. One should not be reassured by unremarkable values when there is good clinical suspicion of central nervous system infection.…”

Section: Other Common Aetiologiesmentioning

confidence: 99%

Fifteen-minute consultation: Approach to the child with an acute confusional state

Prasad

Seal

Mordekar

2016

Arch Dis Child Educ Pract Ed

Self Cite

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Acute confusional state (ACS) refers to sudden impairment of cognitive function and represents a major medical emergency. The impairment may be global or confined specifically to a particular faculty of higher mental function, such as memory. This review highlights the importance of relevant medical history and clinical signs and symptoms in reaching the correct diagnosis. In this review, we have presented a diagnostic approach to a child presenting with ACS and described commonly encountered causes, their treatments and outcomes. We have also presented an algorithm for the diagnostic approach to the child with ACS.

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Encephalopathy in children: an approach to assessment and management

Cited by 35 publications

References 26 publications

Paediatric autoimmune encephalopathies: clinical features, laboratory investigations and outcomes in patients with or without antibodies to known central nervous system autoantigens

Paediatric autoimmune encephalopathies: clinical features, laboratory investigations and outcomes in patients with or without antibodies to known central nervous system autoantigens

Abnormal cranium development in children and adolescents affected by syndromes or diseases associated with neurodysfunction: a retrospective analysis

Fifteen-minute consultation: Approach to the child with an acute confusional state

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