SciCombinator

Discover the most talked about and latest scientific content & concepts.

Journal: European journal of paediatric neurology : EJPN : official journal of the European Paediatric Neurology Society

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OBJECTIVE: To identify the clinical findings of Hashimoto’s encephalopathy (HE) in children and assess their neurological outcome. METHODS: In this retrospective observational study of 42 children with encephalitis dominated by acute neuro-behavioral features, eight met the diagnostic criteria of HE. Their biological, EEG and brain MRI characteristics were compared to those of the other 34 children. Their clinical outcome was also compared to that of 14 children with Hashimoto’s thyroiditis (HT). RESULTS: All eight HE children were girls and had high levels of anti-thyroid peroxidase (TPO) antibodies at onset (4043.3 ± 2969.8 IU/mL, inclusion criteria: TPO > 60 IU/mL) despite normal T4 and TSH levels in six of them. All HE children had abnormal EEG and brain MRI was abnormal in four of them. Relapses were observed in five children with a second relapse, despite steroid therapy, occurring sooner after the previous episode (median 18 days (range 17-188) vs 213 days (range 14-518)). Immunosuppressive therapy was started in all five children and two developed sequelae by the last follow-up visit (after 4 ± 1.3 years). Mean anti-TPO antibody titers were significantly higher in HE children than in those with Hashimoto’s thyroiditis (HT) (4043.3 ± 2969.8 IU/mL vs 1980.9 ± 3449.9 IU/mL, p = 0.03). Four HE children subsequently developed hypothyroidism whereas only one HT patient presented encephalitis. CONCLUSION: HE is characterized by suggestive clinical symptoms with high levels of anti-TPO antibodies and, in most cases, normal T4 and TSH titers. Despite steroid treatment, relapses and sequelae are frequent. HE may evolve toward HT, but the reverse appears to be rare.

Concepts: Observational study, Thyroid-stimulating hormone, Electroencephalography, Hashimoto's thyroiditis, Hyperthyroidism, Thyroid, Immunosuppressive drug, Hashimoto's encephalopathy

28

Neurological complications in paediatric patients with inflammatory bowel disease (IBD) are rare. Most previous reports involve cerebral venous thrombosis, central nervous system vasculitis, or peripheral nerve inflammation. We report a child with active inflammatory bowel disease complicated by acute disseminated encephalomyelitis (ADEM). The child presented with acute neurological deficits following an exacerbation of colitis with evidence of lesions in the central nervous system white matter on magnetic resonance imaging. Ancillary investigations did not provide evidence of systemic infection, coagulation disorders, or vasculitis. In our case the colitis improved with immunosuppressive therapy, with a similar improvement in the white matter lesions showing almost complete resolution of the MR scan changes and no evidence of infarction. This case suggests that ADEM may be another extra intestinal manifestation of inflammatory bowel disease, probably associated with an autoimmune pathogenic mechanism.

Concepts: Immune system, Inflammation, Central nervous system, Nervous system, Brain, Ulcerative colitis, Inflammatory bowel disease, Acute disseminated encephalomyelitis

27

BACKGROUND: Communication is often impaired in cerebral palsy (CP). Tools are needed to describe this complex function, in order to provide effective support. AIM: To study communication ability and the relationship between the Communication Function Classification System (CFCS) and CP subtype, gross motor function, manual ability, cognitive function and neuroimaging findings in the CP register of western Sweden. METHODS: Sixty-eight children (29 girls), 14 with unilateral spastic CP, 35 with bilateral spastic CP and 19 with dyskinetic CP, participated. The CFCS, Gross Motor Function Classification System (GMFCS) and Manual Ability Classification System (MACS) levels, cognitive impairment and neuroimaging findings were recorded. RESULTS: Half the children used speech, 32% used communication boards/books and 16% relied on body movements, eye gaze and sounds. Twenty-eight per cent were at the most functional CFCS level I, 13% at level II, 21% at level III, 10% at level IV and 28% at level V. CFCS levels I-II were found in 71% of children with unilateral spastic CP, 46% in bilateral spastic CP and 11% in dyskinetic CP (p = 0.03). CFCS correlated with the GMFCS, MACS and cognitive function (p < 0.01). Periventricular lesions were associated with speech and more functional CFCS levels, while cortical/subcortical and basal ganglia lesions were associated with the absence of speech and less functional CFCS levels (p < 0.01). CONCLUSION: Communication function profiles in CP can be derived from the CFCS, which correlates to gross and fine motor and cognitive function. Good communication ability is associated with lesions acquired early, rather than late, in the third trimester.

Concepts: Brain, Cerebral cortex, Basal ganglia, Language, Cerebral palsy, Spastic diplegia, Complex analysis, Wilson's disease

27

Alternating hemiplegia of childhood (AHC) is rare disorder characterised by recurrent attacks of hemiplegia followed by developmental delay. We investigated the parental perceptions and psychosocial issues of AHC. Using a questionnaire sent to the French AHC association, we investigated families' concerns and needs of support. Additionally, we evaluated the impact of this disease. RESULTS: We analysed 47 questionnaires from 30 families. At time of diagnosis, the concern of the parents was highest for the hemiplegic spells and abnormal eye movements. These concerns decreased over time. The highest concern at the time of the study was the outcome of the patients with an emphasis on cognitive consequences and the level of autonomy. The results showed that AHC has a significant impact on families. INTERPRETATION: Our data enhance how the explanation of the disease by healthcare professional is important. This study also highlights the need for family support over time.

Concepts: Family, Psychology, Parent, Disability, Mother, Father, Hemiplegia, Alternating hemiplegia

26

Epilepsy affects up to 90% of TSC patients and majority of them have seizure at the age of 3-5 months, after a period of latent epileptogenesis, but some develop epilepsy earlier.

Concepts: The Age, Neurology, Autism, Epilepsy, Seizure, Tuberous sclerosis, Post-traumatic epilepsy, Epileptogenesis

24

The current study evaluated the relationship between tic, sleep disorders and specific psychiatric symptoms (anxiety, depression, obsessive compulsive symptoms).

Concepts: Tourette syndrome

23

Pediatric stroke has the potential for long term impact on the lives of children and their families. Child-centred intervention depends on understanding of needs from diagnosis onwards. However, little is known about the health and care support self-reported needs of this population.

21

In recent years advances in the field of pharmacogenomics have expanded the concept for more individualized treatments. Our aim is to provide literature data about the relationship between genetic polymorphisms and efficacy of antiepileptic drugs in children.

5

We investigated the safety, feasibility, and efficacy of transcranial direct current stimulation (tDCS) combined with constraint-induced movement therapy (CIMT) in children and young adults with unilateral cerebral palsy. Twenty participants were randomized to receive active or sham tDCS. The intervention consisted of 10 consecutive weekday sessions of tDCS applied to the non-lesioned hemisphere (20 min) concurrently with CIMT (120 min). Participants, caregivers, and interventionists were blinded to group assignment. The primary safety outcome investigated adverse events. The primary behavioral outcome was the Assisting Hand Assessment. All 20 participants (mean age = 12.7 yrs, range = 7.4-21.6 years) were evaluated for the primary outcomes. No serious adverse events occurred, and the most commonly reported minor adverse events were headache and itchiness. Both groups demonstrated a significant improvement in hand function after the intervention, although no significant effect of tDCS was observed (between-group difference = -2.18, 95% CI = [-6.48, 2.12], p = 0.30). Although hand function improved overall, no significant differences between intervention groups were found. Children with preserved corticospinal tract circuitry from the lesioned hemisphere, compared to those without, showed greater improvement in hand function (mean difference = 3.04, 95% CI = [-0.64, 6.72], p = 0.099). Our study demonstrates the safety and feasibility of serial sessions of tDCS, and presents preliminary evidence for the effect of CST circuitry on outcomes following tDCS/CIMT. Future work in children with unilateral cerebral palsy should focus on the optimal dosing and consider individual brain circuitry when describing response to combined interventions.

Concepts: Clinical trial, Improve, Effectiveness, Demonstration, Direct current, Cerebral palsy, Outcome, Transcranial direct current stimulation

2

Hand, foot and mouth disease (HFMD) caused by enterovirus A71 (EV-A71) is associated with acute neurological disease in children. This study aimed to estimate the burden of long-term sequelae and death following severe HFMD.