Dataset Information

Nodding syndrome in Ugandan children--clinical features, brain imaging and complications: a case series.

ABSTRACT:

Objectives

Nodding syndrome is a devastating neurological disorder of uncertain aetiology affecting children in Africa. There is no diagnostic test, and risk factors and symptoms that would allow early diagnosis are poorly documented. This study aimed to describe the clinical, electrophysiological and brain imaging (MRI) features and complications of nodding syndrome in Ugandan children.

Design

Case series.

Participants

22 children with nodding syndrome brought to Mulago National Referral Hospital for assessment.

Outcome measures

Clinical features, physical and functional disabilities, EEG and brain MRI findings and a staging system with a progressive development of symptoms and complications.

Results

The median age of symptom onset was 6 (range 4-10) years and median duration of symptoms was 8.5 (range 2-11) years. 16 of 22 families reported multiple affected children. Physical manifestations and complications included stunting, wasting, lip changes and gross physical deformities. The bone age was delayed by 2 (range 1-6) years. There was peripheral muscle wasting and progressive generalised wasting. Four children had nodding as the only seizure type; 18 in addition had myoclonic, absence and/or generalised tonic-clonic seizures developing 1-3 years after the onset of illness. Psychiatric manifestations included wandering, aggression, depression and disordered perception. Cognitive assessment in three children demonstrated profound impairment. The EEG was abnormal in all, suggesting symptomatic generalised epilepsy in the majority. There were different degrees of cortical and cerebellar atrophy on brain MRI, but no hippocampal changes. Five stages with worsening physical, EEG and brain imaging features were identified: a prodrome, the development of head nodding and cognitive decline, other seizure types, multiple complications and severe disability.

Conclusions

Nodding syndrome is a neurological disorder that may be characterised as probably symptomatic generalised epilepsy. Clinical manifestations and complications develop in stages which might be useful in defining treatment and rehabilitation. Studies of risk factors, pathogenesis, management and outcome are urgently needed.

SUBMITTER: Idro R

PROVIDER: S-EPMC3646179 | biostudies-literature | 2013 May

REPOSITORIES: biostudies-literature

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Publications

Nodding syndrome in Ugandan children--clinical features, brain imaging and complications: a case series.

Idro Richard R Opoka Robert Opika RO Aanyu Hellen T HT Kakooza-Mwesige Angelina A Piloya-Were Theresa T Namusoke Hanifa H Musoke Sarah Bonita SB Nalugya Joyce J Bangirana Paul P Mwaka Amos Deogratius AD White Steven S Chong Kling K Atai-Omoruto Anne D AD Mworozi Edison E Nankunda Jolly J Kiguli Sarah S Aceng Jane Ruth JR Tumwine James K JK

BMJ open 20130503 5

<h4>Objectives</h4>Nodding syndrome is a devastating neurological disorder of uncertain aetiology affecting children in Africa. There is no diagnostic test, and risk factors and symptoms that would allow early diagnosis are poorly documented. This study aimed to describe the clinical, electrophysiological and brain imaging (MRI) features and complications of nodding syndrome in Ugandan children.<h4>Design</h4>Case series.<h4>Participants</h4>22 children with nodding syndrome brought to Mulago Na ...[more]

PMID: 23645924

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Project description:ObjectiveNodding syndrome is a poorly understood epileptic encephalopathy characterized by a unique seizure type-head nodding-and associated with Onchocerca volvulus infection. We hypothesized that altered immune activation in the cerebrospinal fluid (CSF) and plasma of children with nodding syndrome may yield insights into the pathophysiology and progression of this seizure disorder.MethodWe conducted a case-control study of 154 children (8 years or older) with long-standing nodding syndrome and 154 healthy age-matched community controls in 3 districts of northern Uganda affected by nodding syndrome. Control CSF samples were obtained from Ugandan children in remission from hematological malignancy during routine follow-up. Markers of immune activation and inflammation (cytokines and chemokines) and complement activation (C5a) were measured in plasma and CSF using ELISA or Multiplex Luminex assays. O volvulus infection was assessed by serology for anti-OV-16 IgG levels.ResultsThe mean (SD) age of the population was 15.1 (SD: 1.9) years, and the mean duration of nodding syndrome from diagnosis to enrollment was 8.3 (SD: 2.7) years. The majority with nodding syndrome had been exposed to O volvulus (147/154 (95.4%)) compared with community children (86/154 (55.8%)), with an OR of 17.04 (95% CI: 7.33, 45.58), P < .001. C5a was elevated in CSF of children with nodding syndrome compared to controls (P < .0001). The levels of other CSF markers tested were comparable between cases and controls after adjusting for multiple comparisons. Children with nodding syndrome had lower plasma levels of IL-10, APRIL, CCL5 (RANTES), CCL2, CXCL13, and MMP-9 compared with community controls (P < .05 for all; multiple comparisons). Plasma CRP was elevated in children with nodding syndrome compared to community children and correlated with disease severity.SignificanceNodding syndrome is associated with exposure to O. volvulus. Compared to controls, children with long-standing symptoms of nodding syndrome show evidence of complement activation in CSF and altered immune activation in plasma.

Project description:BackgroundHIV infection has been associated with a non-erosive inflammatory arthritis in children, although few published reports exist. This study describes the clinical, laboratory and imaging features of this noncommunicable disease in a series of HIV-infected children in South Africa.MethodsA database search was conducted to identify HIV-infected children enrolled in a Paediatric Rheumatology service in Cape Town, South Africa between 1 January 2010 and 31 December 2020. Retrospective data were collected from individuals classified with HIV arthropathy, based on a predefined checklist. Demographic, clinical, laboratory, sonographic, therapeutic, and outcomes data were extracted by chart review. Descriptive statistical analysis was performed using R (v4.0.3).ResultsEleven cases of HIV arthropathy were included in the analysis. Cases predominantly presented in older boys with low CD4+ counts. Median age at arthritis onset was 10.3 years (IQR 6.9 - 11.6) and the male-female ratio was 3.0. The median absolute CD4+ count was 389 cells/uL (IQR 322 - 449). The clinical presentation was variable, with both oligoarthritis and polyarthritis being common. Elevated acute phase reactants were the most consistent laboratory feature, with a median ESR of 126 mL/h (IQR 67 - 136) and median CRP of 36 mg/L (IQR 25 - 68). Ultrasonography demonstrated joint effusions and synovial hypertrophy. Response to therapy was slower than has generally been described in adults, with almost all cases requiring more than one immunosuppressive agent. Five children were discharged in established remission after discontinuing immunotherapy, however outcomes data were incomplete for the remaining six cases.ConclusionsIn this case series, HIV arthropathy was associated with advanced immunosuppression. Therapeutic modalities included immunomodulators and antiretroviral therapy, which consistently induced disease remission although data were limited by a high rate of attrition. Prospective studies are needed to define and understand this HIV-associated noncommunicable disease.

Project description:Abstract Nodding syndrome is an uncommon disorder of childhood onset and unknown cause, presenting with nodding seizures, and which appears to occur exclusively in clusters in sub-Saharan Africa. An endemic pattern of disease was initially described in Tanzania and in Liberia; epidemic occurrences were later reported in South Sudan and northern Uganda. Not the least significant of the many questions remaining about nodding syndrome concerns the common presence or otherwise of neurological features other than seizures—clearly relevant to the core issue of whether this is a focal, primary epileptic disease, or a multi-system CNS disorder, with, in turn implications for its aetiology. We had the opportunity to interview and clinically to examine 57 affected individuals in rural northern Uganda some 10 years after onset. In this observational cross-sectional study, nodding onset was invariably between the ages of 5 and 14, presenting with food-triggered nodding attacks in over 75% of cases; 86% went on to develop other seizure types. In 53 of 57 nodding syndrome individuals (93%), there was a definite history of the child and his or her family having resided in or been fed from an internally displaced person camp for some time prior to the onset of nodding. A half of nodding syndrome sufferers (28/57) had focal neurological abnormalities—mainly pyramidal signs (92%), often asymmetric, some with extrapyramidal abnormalities. Many individuals (28/57) were severely functionally disabled, ranging from ‘sometimes can dig’ to ‘can do nothing at home’ or ‘cannot even feed herself’. Such sufferers tended more frequently to have significant burns, and clear cognitive impairment. We conclude that nodding syndrome is a unique multisystem CNS disorder of childhood onset and then slow progression over several years often followed by spontaneous stabilisation, consistent with an underlying self-limiting neurodegenerative process. We discuss the possibility that this might be triggered by food-related mycotoxins, within a fixed window of CNS vulnerability during childhood. Nodding syndrome is a poorly understood, predominantly epileptic disorder identified almost exclusively in rural East Africa. Focal neurological signs have not been studied. Scolding et al. examined 57 individuals with chronic nodding syndrome in rural settings in northern Uganda and found pyramidal and/or extrapyramidal signs in almost half. Graphical Abstract Graphical Abstract

Dataset Information

Nodding syndrome in Ugandan children--clinical features, brain imaging and complications: a case series.

Objectives

Design

Participants

Outcome measures

Results

Conclusions

Publications

Nodding syndrome in Ugandan children--clinical features, brain imaging and complications: a case series.

Similar Datasets

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