Management of Inflammatory Neuropathies

Hadden, Robert DM; Hughes, Richard

doi:10.1136/jnnp.74.suppl_2.ii9

Cited by 31 publications

(19 citation statements)

References 7 publications

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“…Although children with CIDP have a favorable response to immunomodulation, the overall prognosis is variable; many patients improve and even recover, but a subset remains with significant neurologic deficits that may lead to death. [4][5][6][7][8][9][10][11] Although childhood CIDP is a distinctive entity, there are clinical variants in which CIDP research criteria cannot be met but which do respond to immune modulating therapy. In the present study, we detected CD59 deficiency in 5 infants from 4 unrelated families of North-African Jewish origin with chronic hemolysis accompanied by sensory-motor, demyelinating, or axonal peripheral polyneuropathy.…”

Section: Introductionmentioning

confidence: 99%

CD59 deficiency is associated with chronic hemolysis and childhood relapsing immune-mediated polyneuropathy

Nevo

Ben‐Zeev

Tabib

et al. 2013

Blood

127

105

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Section: Introductionmentioning

confidence: 99%

CD59 deficiency is associated with chronic hemolysis and childhood relapsing immune-mediated polyneuropathy

Nevo

Ben‐Zeev

Tabib

et al. 2013

Blood

127

105

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“…There was a non-significant trend toward a better outcome noted in the group receiving longer treatment of 6 days, and this trend reached significance when only ventilated patients were considered, but the shorter course such as 3 days was proven to be significantly less effective 57,58,59 .…”

Section: High-dose Immunoglobulinmentioning

confidence: 87%

Untitled

2012

IJPSR

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Guillain-Barré syndrome is a rare but important disease that can lead to life threatening respiratory failure. This review summarises the verified consideration of Guillain-Barré syndrome (GBS) which are thought to be caused by direct autoimmune attack on peripheral nerves. Aim-Nerve conduction study helps differentiate the heterogeneous subtypes of GBS. Patients exhibit a progressive paralysis that reaches a plateau phase. Treatment and Result-Treatment with both intravenous immunoglobulin and plasma exchange reduces the time taken for recovery to occur, although mortality remains around 8%, with about 20% of patients remaining disabled. Though no significant differences were found between patients with Guillain Barre syndrome treated with plasma exchange and patients treated with intravenous immune globulins either alone or in combination with high dose methylprednisolone. Conclusion-It can be concluded that tentative and the gap in current research should not be interpreted as proof that multidisciplinary care is ineffective. Further research is needed into appropriate study designs; outcome measurement; caregiver needs; and the evaluation of optimal settings, type, intensity or frequency and costeffectiveness of multidisciplinary care in the Guillain-Barré syndrome population.

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“…Rarely, an LP may be helpful in differential diagnosis from GBS, when EMG is not available. However it should always be kept in mind that the CSF protein levels may be normal during the first week of GBS [14]. Therefore an LP is generally not indicated at the initial presentation of acute ataxia [1].…”

Section: Discussionmentioning

confidence: 99%

Postinfectious Acute Cerebellar Ataxia in Childhood

Ünver¹

2016

Ann Clin Anal Med

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ÖzetAmaç: Postenfeksiyöz akut serebellar ataksi çocukluk çağı ataksilerinin en sık sebebidir. Olgular çocuk acil servis veya çocuk nöroloji polikliniklerine ani başlangıçlı ataksi ile başvururlar. Varisella virüsü en sık ilişkilendiren virüs-tür. Bu çalışmanın amacı postenfeksiyöz akut serebellar ataksi nedeniyle izlenen çocuklarda klinik özellikleri, etiyoloji ve prognozu belirlemek ve çocukluk çağında akut ataksiye genel bir yaklaşım önermektir. Gereç ve Yöntem: Ocak 2011-Haziran 2015 tarihleri arasında başvurmuş 16 çocuğun dosyaları geriye dönük olarak incelendi. Bulgular: Dokuz olgu erkekti (%56,2). Olguların ço-ğunluğunun yaşı 2-5 yaş aralığındaydı (%62,5). Olguların %87,5'inde bulguların ortaya çıkmasından önce viral enfeksiyon öyküsü mevcuttu: Çocukların ikisinde suçiçeği enfeksiyonu, birinde Epstein Barr enfeksiyonu geri kalanlarda nonspesifik ateşli hastalık saptandı. Ateşli hastalık ve bulguların ortaya çıkışı arasındaki ortalama süre 7,4 (±5) gündü. Ortalama hastane yatış süresi 4,37 (± 1,4) gündü. Ortanca iyileşme süresi 7 gündü. En uzun iyileşme süresi 4 aydı. Tartışma: Çocukluk çağında postenfeksiyöz akut serebellar ataksi, ani başla-yan aylar içinde iyileşme gösteren benign bir durumdur. Ancak akut serebellar ataksinin bir dışlama tanısı olduğu unutulmamalı; bu çocuklarda laboratuar testleri ve görüntüleme incelemelerinin uygun kullanımı ile santral sinir sistemi enfeksiyonu ve kitle lezyonu gibi daha ciddi medikal durumların ayı-rıcı tanısı mutlaka yapılmalıdır. Anahtar KelimelerPostenfeksiyöz Akut Serebellar Ataksi; Çocuk; Serebellit; Varisella Enfeksiyonu; Serebellar Ataksi Abstract Aim: Postinfectious acute cerebellar ataxia is the most common cause of childhood ataxia. Cases present with acute onset of ataxia to pediatric emergency and pediatric neurology clinics. Varicella zoster is the most commonly associated virus. The aim of this study is to assess the clinical features, etiology, and prognosis of children with postinfectious acute cerebellar ataxia and to propose a diagnostic approach to acute cerebellar ataxia in children. Material and Method: Files of 16 children admitted between January 2011 and June 2015 were retrospectively evaluated. Results: Nine patients were male (56.2%). The majority of the cases were in the 2-5 years age group (62.5%). A history of a preceding febrile infection was noted in 87.5% of the cases: Two children had varicella infection, one Epstein Barr infection, and the rest nonspecific febrile illness. The mean time interval between the prodromal febrile illness and the onset of the symptoms was 7.4 (±5) days. The mean time of hospitalization was 4.37 (± 1.4) days. The median time for recovery was 7 days. The longest time for recovery was 4 months. Discussion: Postinfectious acute cerebellar ataxia in childhood is the most common cause of childhood ataxia, which presents abruptly and requires recovery over weeks. However, it should be kept in mind that it is a diagnosis of exclusion. Appropriate utilization of laboratory tests and imaging studies is necessary fo...

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Management of Inflammatory Neuropathies

Cited by 31 publications

References 7 publications

CD59 deficiency is associated with chronic hemolysis and childhood relapsing immune-mediated polyneuropathy

CD59 deficiency is associated with chronic hemolysis and childhood relapsing immune-mediated polyneuropathy

Untitled

Postinfectious Acute Cerebellar Ataxia in Childhood

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