[Unusual variants of Guillain-Barré syndrome in infancy]. / Variantes inusuales del síndrome de Guillain-Barré en la infancia.

AIMS: To identify unusual variants within a paediatric population of patients with Guillain-Barré syndrome (GBS), to determine the frequency of their occurrence and to describe their clinical and electrophysiological characteristics. PATIENTS AND METHODS: The medical records of 179 patients diagnosed with GBS were reviewed. Those who strictly satisfied the Asbury criteria were excluded. RESULTS: Twenty patients, with a mean age of 7.6 years at the onset of symptoms, presented the following clinical variants: multiple cranial polyneuropathy (4 cases), Miller Fisher syndrome (MFS) (3 cases), pharyngeal-cervical-brachial palsy (PCBP) (2 cases), combined MFS and PCBP (3 cases), paraparesis (4 cases), palpebral ptosis without ophthalmoplegia (1 case), ophthalmoplegia without ataxia (1 case), paresis of the abducent nerve with paresthesias (1 case), and saltatory (1 case). Albuminocytologic dissociation was reported in 77.8% of patients and neuroconduction with a demyelinating pattern was noted in 75%. Patients progressed favourably in 94.4% of cases. CONCLUSIONS: In our study, 11.2% of a population of children with GBS presented unusual variants and did not meet the criteria established by Asbury et al. Recognising these variants of GBS in patients with complex pictures such as those described here enables therapy to be established quickly. The question remains as to whether these variants represent a clinical response to different antibodies or to a regional susceptibility of the host.

Variantes inusuales del síndrome de Guillain-Barré en la infancia / Unusual variants of Guillain-Barré syndrome in infancy

Objetivos. Identificar variantes inusuales en una población pediátrica de pacientes con síndrome de Guillain-Barré (SGB), establecer la frecuencia y describir las características clínicas y electrofisiológicas. Pacientes y métodos. Revisión de las historias clínicas de 179 pacientes con diagnóstico de SGB. Se excluyeron los que cumplían estrictamente con los criterios de A sbury. Resultados.20 pacientes, con edad media de comienzo de los síntomas de 7,6 años, presentaron las siguientes variantes clínicas: polineuropatía craneal múltiple (4 casos), síndrome de Miller Fisher (SMF)(3 casos), parálisis faringocervicobraquial (PFCB) (2 casos), combinación de SMF y PFCB (3 casos), paraparesia (4 casos), ptosispalpebral sin oftalmoplejía (1 caso), oftalmoplejía sin ataxia (1 caso), paresia del VI par con parestesias (1 caso), saltatoria (1 caso).Documentamos disociación albuminocitológica en el 77,8% y neuroconducción con patrón desmielinizante en el 75%. La evolución fue favorable en el 94,4% de los pacientes. Conclusiones. El 11,2%de una población de niños con SGB presentaron variantes inusuales y no cumplieron con los criterios de Asbury et al. Reconocerestas variantes de SGB en pacientes con cuadros complejos como los descritos permite un rápido abordaje terapéutico. Queda por resolver si estas variantes son la respuesta clínica a diferentes anticuerposo a una susceptibilidad regional del huésped (AU)

Aims. To identify unusual variants within a paediatric population of patients with Guillain-Barré syndrome (GBS),to determine the frequency of their occurrence and to describe their clinical and electrophysiological characteristics. Patients and methods. The medical records of 179 patients diagnosed with GBS were reviewed. Those who strictly satisfied the Asbury criteria were excluded. Results. Twenty patients, with a mean age of 7.6 years at the onset of symptoms, presented the following clinical variants: multiple cranial polyneuropathy (4 cases), Miller Fisher syndrome (MFS) (3 cases), pharyngeal-cervical brachial palsy (PCBP) (2 cases), combined MFS and PCBP (3 cases), paraparesis (4 cases), palpebral ptosis without ophthalmoplegia(1 case), ophthalmoplegia without ataxia (1 case), paresis of the abducent nerve with paresthesias (1 case), and saltatory (1 case). Albuminocytologic dissociation was reported in 77.8% of patients and neuro conduction with a demyelinating pattern was noted in 75%. Patients progressed favourably in 94.4% of cases. Conclusions. In our study, 11.2% of a population of children with GBS presented unusual variants and did not meet the criteria established by Asbury et al.Recognising these variants of GBS in patients with complex pictures such as those described here enables therapy to beestablished quickly. The question remains as to whether these variants represent a clinical response to different antibodies orto a regional susceptibility of the host (AU)

[Pyridoxine dependence: the importance of the clinical diagnosis and early treatment]. / Dependencia de piridoxina: valor del diagnóstico clínico y del tratamiento precoz.

OBJECTIVE: We described the electroclinical features, evolution and family history of two patients with definitive diagnosis of pyridoxine dependency. CASE REPORTS: The first patient is a 15-month-old girl who at 1 month of age started with seizures and irritability. At two months of age, pyridoxine was prescribed with a good control of seizures. At five months of age withdrawal response provoked 7 days after seizures recurrence. Pyridoxine was reintroduced and seizures disappeared. Her sister, at two months of age, started with refractory seizures. This sister also had mental retardation and at four years, she died. Her brother, 16 years old, presents mental retardation, refractory epilepsy and progressive motor and cognitive impairment. At 3 months of age, he started with seizures and at 15 years of age, pyridoxine was prescribed with a significative improvement the number of seizures and a better visual connection. The second patient is a 4-month-old girl who started with clonic seizures at 3 days of age and she had a good response to pyridoxine. Withdrawal response provoked seizure recurrence at 48 hours. Pyridoxine was introduced immediately with total control of seizures. She had two cousins with seizures who died at 3 months and 3 years of age respectively. CONCLUSION: When dealing with an infant with refractory seizures which start in the first two years of life and without etiology, we should consider the diagnosis of pyridoxine dependency. Early diagnosis and treatment with pyridoxine is crucial to avoid high risk morbidity and mortality. All infants in the two first years of life with refractory seizures without etiology must be prescribed oral pyridoxine (50-200 mg per day).

[Posterior reversible encephalopathy in infancy]. / Encefalopatía posterior reversible en la infancia.

INTRODUCTION: Posterior reversible encephalopathy (PRE) is a radiological clinical syndrome that was initially reported in adults and which is characterised by seizures, headache, visual disorders, decreased awareness and anomalous neuroimages. It is an acute transient disorder. The most frequent causes are arterial hypertension (AHT) and immunosuppressive therapy. AIMS: The aim of this study is to describe the characteristics of PRE in patients of paediatric age. PATIENTS AND METHODS: From a total of 109,267 patients admitted to hospital between 1/1/1999 and 1/8/2002, we reviewed the case histories of seven patients who satisfied PRE criteria. RESULTS: Seven patients presented AHT associated with kidney disease (6) and with an undetermined causation (1). The initial symptoms were vomiting in seven and headaches in five patients, followed by seizures in six cases, which were partial (4), generalised (1), motor status (1). Five of them suffered visual disorders. All of them had decreased awareness and one of them required the assistance of mechanical ventilation. The condition cleared within 3 to 12 days after antihypertensive therapy. The anomalies that showed up in the neuroimaging studies (hypodensities in CAT, hyperintensities in T2 and hypointensities in T1 in MR) revealed the involvement of cortical and parieto occipital cortico subcortical regions asymmetrically. Frontal (2), temporal (2) and cerebellous (1) involvement was also observed. These disorders disappeared in patients from whom control images were obtained. CONCLUSIONS: PRE must be considered in the presence of the symptoms and the anomalous neuroimages described above, when associated with acute AHT. With correct management of arterial hypertension it is possible to clear the neurological symptoms and normalise the neuroimages that confirm this diagnosis.