L'incidence annuelle du syndrome de Guillain-Barre est de 1,5/100000 habitants La mortalite actuelle est estimee a environ 5 % d'apres des essais therapeutiques recents, bien conduits Dix pour cent des malades gardent des sequelles motrices tres invalidantes un an apres le debut des premiers signes neurologiques La prise en charge de ces malades necessite des equipes entrainees, multidisciplinaires, pouvant pratiquer l'ensemble des therapeutiques specifiques La corticotherapie per os'ou par voie intraveineuse est inefficace Les echanges plasmatiques sont le premier traitement dont l'efficacite a ete demontree par rapport a un groupe controle Les indications sont maintenant mieux connues Les formes benignes (marche possible) beneficient de 2 echanges plasmatiques; 2 echanges supplementaires sont realises en cas d'aggravation Dans les formes intermediaires (marche impossible) et les formes severes (recours a la ventilation mecanique), 4 echanges plasmatiques sont conseilles Il n'est pas utile d'augmenter leur nombre dans les formes severes ou en cas d'absence d'amelioration De fortes doses d'immunoglobulines donnees par voie intraveineuse (lq IV) [0,4 g/kg/j pendant 5 jours] sont aussi efficaces que les echanges plasmatiques dans les formes intermediaires et severes Dans ces formes, le choix entre Ig IV et echanges plasmatiques depend des contre-indications respectives de ces traitements et de leur faisabilite Les travaux en cours ont comme objectif de mieux preciser les indications respectives des echanges plasmatiques et des lq IV dans des formes de gravite differente, leur morbidite comparee, la dose optimale des lq IV

[Guillain-Barré syndrome].

Objectives To describe the characteristics of pediatric cases of eosinophilic granulomatosis with polyangiitis (EGPA), a systemic necrotizing vasculitis rarely diagnosed in children, retrieved from the French Reference Center for rare pediatric lung diseases and compared with adult cases included in the French Vasculitis Study Group cohort. Methods We collected information on pediatric EGPA disease presentation, management, and outcome. Cases met the Lanham criteria and/or American College of Rheumatology classification criteria. Results Fourteen cases of pediatric EGPA were included, from 1980 to 2012, with a median follow-up of 58.5 months. Median age at diagnosis was 12.3 years. All cases had respiratory involvement. The organ systems most frequently involved were the upper airway (85%), skin (71%), digestive tract (64%), and heart (57%). Neurological and renal involvement were rare. Four of the fourteen children were positive for ANCA (30.7%). During follow-up, three children required intensive care and one child died. The relapse rate was 64%. In comparison with an adult cohort, we found more ENT, heart, and digestive-tract involvement, and fewer neurological manifestations. In children, the delay between asthma onset and diagnosis was shorter, and biopsies showed fewer features of vasculitis. Conclusion This French cohort is the biggest pediatric EGPA series described to date, with a long follow-up period. The findings confirm that pediatric EGPA has specific clinical, radiological, and histological characteristics that differ from adult EGPA. Development of systemic symptoms, and consequently diagnosis, occur with a shorter delay in children, mainly during the eosinophilic phase and leading to a specific presentation.

Eosinophilic granulomatosis with polyangiitis in children: Data from the French RespiRare® cohort.

Churg-Strauss syndrome is an uncommon multisystem disorder characterized by asthma, eosinophilia and vasculitis. We report on a 12-year-old boy with asthma and deterioration of his general condition, who was eventually diagnosed with an ANCA-negative Churg-Strauss syndrome. The propositus included, 50 cases of childhood Churg-Strauss syndrome have been reported. The patient characteristics and clinical characteristics of these children are summarized. The respiratory tract is most frequently involved with pulmonary infiltrates, asthma and sinusitis. Early recognition of childhood Churg-Strauss syndrome is important as delayed diagnosis can lead to severe organ involvement, and possible fatal outcome.

https://repub.eur.nl/pub/65271/Repub_65271_O-A.pdf

Clinical presentation of Churg–Strauss syndrome in children: A 12-year-old-boy with ANCA-negative Churg–Strauss syndrome

Guillain-Barre syndrome (GBS) is an acquired disease of the peripheral nervous system which causes demyelination and leads to weakness, ataxia, and areflexia. There are a variety of forms of the syndrome, and although it is found in all age groups, it is rare in children less than two years of age. The present complaint of weakness, ataxia, or lower extremity pain in the pediatric population should cause the practitioner to consider GBS in the differential. We describe a case of a 14-month-old girl presenting with weakness and the inability to ambulate who was diagnosed with GBS. The purpose of this paper is to review the emergency medicine diagnosis and management of Guillain-Barre syndrome in children.

/pdf/weakness-and-the-inability-to-ambulate-in-a-14-month-old-4wk7wyzhnj.pdf

Weakness and the Inability to Ambulate in a 14-Month-Old Female: A Case Report and Concise Review of Guillain-Barre Syndrome

Background/objectives This study seeks to better define the clinical presentation and histopathology of cutaneous manifestations in childhood eosinophilic granulomatosis with polyangiitis (cEGPA). Methods Case reports were collected from Ovid Medline Database and PubMed using keyword identifiers from 1946 to 2017. Adult patients ≥ 18 years and cases not diagnosed with EGPA by the author were excluded. Sixty-five case reports of cEGPA were initially identified. These were reviewed individually, and fifty cases were determined to meet the American College of Rheumatology criteria for EGPA. No case series examining the cutaneous morphology and histopathology were identified. Cutaneous morphology, lesion location, and cutaneous histopathology results were recorded. Results were analyzed using summary statistics. Results Sixty-four percent (32/50) of cEGPA patients presented with cutaneous manifestations. Twenty-nine cases provided specific morphological descriptions and lesion location. Common manifestations included purpura (15/29), subcutaneous nodules (8/29), and a macular/papular/maculopapular rash (8/29). However, twelve different cutaneous morphologies were identified in this review. Lesions occurred most commonly on the extremities (26/29). Twenty-two cases reported corresponding cutaneous histopathology, which revealed extravascular eosinophils (15/22), vasculitis (13/22), and granulomas (5/22). Only one biopsy sample (1/22) had all three classical EGPA characteristics of granulomas, extravascular eosinophils, and vasculitis. Conclusion With nearly two-thirds of cEGPA patients presenting with cutaneous manifestation, this study highlights the importance of clinical recognition of this disease by dermatologists. While the varied morphology of skin lesions and rarity of this disease makes cEGPA a difficult diagnosis, prompt recognition and treatment will improve outcomes in this patient population.

Cutaneous manifestations of childhood Eosinophilic Granulomatosis with Polyangiitis (cEGPA): A case-based review.

Children frequently visit emergency departments with asthma exacerbations. Many of these asthmatic children may have fever and/or pneumonia, but when associated with eosinophilia or evidence of vasculitis, other diagnoses should be considered. Churg-Strauss syndrome is a rare form of systemic vasculitis, which usually occurs in patients with asthma in association with eosinophilia. The diagnosis of Churg-Strauss syndrome can be difficult because this syndrome may arise at first as a common association between asthma and allergic rhinitis. A delay in diagnosis and treatment may increase the morbidity from the complications of vasculitis. We report a young asthmatic adolescent who presented with vasculitis, eosinophilia, and peripheral neuropathy.

An asthmatic adolescent with a rash and eosinophilia.

Abnormal arm posture or movements in a neonate may cause significant concern in a pediatric emergency department. This can be secondary to osteomyelitis, which may rarely present with asymmetric arm movements in the neonatal period. The diagnosis of osteomyelitis is difficult to establish in a neonate because systemic signs may not be present at this age. We report an infant with upper limb weakness and asymmetric movements 3 weeks after delivery.

A neonate with asymmetric arm movements.

A n acute limp is a common reason for a child to present to the emergency department (ED). Limping takes many forms, and the cause is not always obvious. Limping is never normal, and both benign and life-threatening illnesses can present with limp. The provisional diagnosis can be a challenge to establish even after history, physical, and laboratory examinations. The few causes of limp in the weak pediatric patient help narrow the focus of the ED evaluation. A representative case is provided.

Emma Laureta

Papers

An asthmatic adolescent with a rash and eosinophilia.

A neonate with asymmetric arm movements.

Limping child with profound weakness.