[Abnormalities in the central nervous system and alterations in the upper limbs in patients with myelomeningocele]. / Anormalidades del sistema nervioso central y alteraciones de los miembros superiores en pacientes con mielomeningocele.

INTRODUCTION: Myelomeningocele is a neural tube defect resulting in motor and sensory deficit below the level of the lesion, bowel and neurogenic bladder, sexual dysfunction, cognitive dysfunction, neurodevelopment delay and motor skills disability. During the past decades, hand function impairment has been described in myelomeningocele patients, probably due to central nervous system abnormalities. AIM. To determine the occurrence of upper limb impairment and additional central nervous system abnormalities in patients with myelomeningocele. SUBJECTS AND METHODS: A transversal study including 33 patients with myelomeningocele, 6 years and older, referred to pediatric rehabilitation program at Sarah Network of Rehabilitation Hospital (Fortaleza, Brazil), and 33 control subjects. All patients underwent brain and spinal cord magnetic resonance imaging. RESULTS: Hydrocephalus, Chiari type II malformation with or without kinking of the medullocervical junction, hydrosyringomyelia, spinal cord and brain atrophy were the most common central nervous system abnormalities. Dysmetria, evaluated by the Index-Index Test, was exhibited by 87.9% of the patients and 21.2% of them exhibited dysdiadochokinesia. The mean hand grip strength of the myelomeningocele group was significantly lower compared with the control group (p < 0,001). CONCLUSIONS: High occurrence of upper limb dysfunction and additional central nervous system abnormalities was detected in patients with myelomeningocele, supporting previous studies. Further investigation is still necessary to elucidate the upper limb impairment impact on the daily live activities of the patient with myelomeningocele.

Anormalidades del sistema nervioso central y alteraciones de los miembros superiores en pacientes con mielomeningocele / Abnormalities in the central nervous and alterations in the upper limbs in patients with myelomeningocele

El mielomeningocele es un defecto del cierre del tubo neural cuyo cuadro clínico incluye déficit defuerza muscular y sensibilidad por debajo del nivel de la lesión, vejiga e intestino neurogénicos, disfunción sexual, déficit cognitivo, retraso en el desarrollo neuropsicomotor y lentitud en la realización de actos motores. En las últimas décadas se ha documentadoel perjuicio de la función manual, que puede deberse principalmente a anormalidades asociadas al sistema nervioso central. Objetivo. Describir las alteraciones de los miembros superiores y anormalidades del sistema nervioso central en pacientes con mielomeningocele. Sujetos y métodos. Estudio transversal donde participaron 33 pacientes con mielomeningocele,de edad superior a 6 años, en seguimiento en el Centro de Rehabilitación Sarah Fortaleza, Brasil, junto con 33 sujetos control. Todos los pacientes se sometieron a resonancia magnética de encéfalo y médula. Resultados. Hidrocefalia, malformación de Chiari tipo II con o sin kinking cervicomedular, hidrosiringomielia y atrofia medular y cerebral fueron las anormalidadesmás encontradas. Se halló dismetría, evaluada a través del test índice-índice, en el 87,9% de los pacientes, y disdiadococinesia en el 21,2%. La media de la fuerza de prensión de los pacientes con mielomeningocele fue significativamente inferior a la del grupo de control (p < 0,001). Conclusiones. Se detectó una alta ocurrencia de anormalidades del sistema nerviosocentral y alteraciones de los miembros superiores en los pacientes con mielomeningocele, corroborando estudios previos. Se precisan investigaciones futuras para evaluar el impacto de las alteraciones de los miembros superiores en las actividades de la vida diaria

Myelomeningocele is a neural tube defect resulting in motor and sensory deficit below the level of thelesion, bowel and neurogenic bladder, sexual dysfunction, cognitive dysfunction, neurodevelopment delay and motor skills disability. During the past decades, hand function impairment has been described in myelomeningocele patients, probably dueto central nervous system abnormalities. Aim. To determine the occurrence of upper limb impairment and additional central nervous system abnormalities in patients with myelomeningocele. Subjects and methods. A transversal study including 33 patients with myelomeningocele, 6 years and older, referred to pediatric rehabilitation program at Sarah Network ofRehabilitation Hospital (Fortaleza, Brazil), and 33 control subjects. All patients underwent brain and spinal cord magnetic resonance imaging. Results. Hydrocephalus, Chiari type II malformation with or without kinking of the medullocervical junction,hydrosyringomyelia, spinal cord and brain atrophy were the most common central nervous system abnormalities. Dysmetria, evaluated by the Index-Index Test, was exhibited by 87.9% of the patients and 21.2% of them exhibited dysdiadochokinesia. The mean hand grip strength of the myelomeningocele group was significantly lower compared with the control group (p < 0,001).Conclusions. High occurrence of upper limb dysfunction and additional central nervous system abnormalities was detected in patients with myelomeningocele, supporting previous studies. Further investigation is still necessary to elucidate the upper limb impairment impact on the daily live activities of the patient with myelomeningocele