Guillain-Barré Syndrome and Hydrocephalus in an infant with Wiskott-Aldrich Syndrome. / Síndrome de Guillain Barré e hidrocefalia en un lactante con Síndrome de Wiskott Aldrich.
Rev Chil Pediatr
; 91(1): 105-110, 2020 Feb.
Article
em En, Es
| MEDLINE
| ID: mdl-32730420
INTRODUCTION: Guillain-Barre Syndrome (GBS) is rarely diagnosed in the first year of life. The association of GBS with Wiskott-Aldrich syndrome (WAS) is even less frequent and has been previously reported in only two children to our knowledge. Hydrocephalus is a known but rare complication of GBS. OBJECTIVE: To describe the case of an infant in which GBS, WAS and hydrocephalus appear clinically associated. CLINICAL CASE: A nine-months-old male infant with a history of WAS was admitted to our ICU with acute hypotonia and poor general condition. He developed flaccid paralysis, absent deep tendon reflexes, and respiratory failure. A lumbar puncture showed albuminocytologic dissociation. GBS was suspected and an electromyography was performed, showing a demyelinating polyneuropathy. He was successfully treated with intravenous immunoglobulins. During hospitalization, he presented intermittent bradycardia, so a brain CT scan was performed, showing acute hydrocephalus which was managed through an external ventricular drain achieving favorable response. In the long term, the patient underwent bone marrow transplant and had to be reoperated due to valve-related complications. However, his psychomotor development is normal, with no obvious neurological sequelae. CONCLUSION: We present the third case of GBS in a patient with WAS, which is the first infant younger than one year. Additionally, he presented acute hydrocephalus as a complication of GBS. We suggest considering these three comorbidities since their early diagnosis and prompt management allow bet ter neurological recovery and avoid potentially lethal complications.
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Síndrome de Wiskott-Aldrich
/
Síndrome de Guillain-Barré
/
Hidrocefalia
Tipo de estudo:
Screening_studies
Limite:
Humans
/
Infant
/
Male
Idioma:
En
/
Es
Revista:
Rev Chil Pediatr
Ano de publicação:
2020
Tipo de documento:
Article
País de afiliação:
Chile
País de publicação:
Chile