Tortícolis recurrente como forma de presentación de un tumor de la fosa posterior del cerebro / Recurrent torticollis as a form of presenting a tumor in the posterior fossa brain

La tortícolis se presenta como una manifestación focal del sistema nervioso central, y es una afección poco frecuente, aunque puede ser el síntoma inicial en patologías de la fosa posterior del cerebro. Se presenta el caso de un paciente masculino de cuatro años de edad, con antecedentes de dolor, lateralización y torsión del cuello con nueve meses de evolución, cuyos síntomas se mantuvieron progresando. Gradualmente se asocian cefalea, náuseas e inestabilidad a la marcha. Al realizar el examen físico se encontraron elementos clínicos de una tortícolis y un síndrome cerebeloso de hemicuerpo derecho. Se realizó una resonancia magnética nuclear de cráneo, la cual evidenció una lesión sólida que ocupaba todo el piso del IV ventrículo, y se extendía hasta el segundo segmento medular cervical. Se efectuó abordaje quirúrgico de la fosa posterior del cerebro y exeresis de la lesión. El diagnóstico histológico fue el de un ependimoma. Aunque la tortícolis la mayoría de las ocasiones es una condición benigna, cuando se presenta secundariamente en los niños, uno de los diagnósticos diferenciales que deben tenerse en cuenta, son los tumores de la fosa posterior del cerebro, como ocurrió en este caso

Torticollis is presented as a focal demonstration of the central nervous system, and it is a rare condition, but it may be the initial symptom in diseases of the posterior fossa brain. We report the case of a male patient aged four, with a history of pain, lateralization and twisted neck with nine months of evolution, whose symptoms were progressing. Gradually, headache, nausea and gait instability are associated. When performing the physical examination, we found clinical elements of a stiff neck and a right hemisphere cerebellar syndrome. We performed a cranial MRI, which showed a solid lesion occupying the entire floor of the fourth ventricle, and it extended to the second cervical spinal segment. Surgical approach was performed in the posterior fossa and brain tumor excision. Ependymoma was the histological diagnosis. Though most occasions, torticollis is a benign condition, when it occurs secondarily in children, one of the differential diagnoses that should be considered is the posterior fossa tumors of the brain, as in this case

Surgical Outcome and Prognostic Factors of Spinal Intramedullary Ependymomas in Adults

Ependymoma is the most common spinal intramedullary tumor in adults. The authors reviewed clinical data of 31 patients with spinal ependymoma who underwent operations between 1979 and 1996. The ages of the patients ranged from 15 to 62 years with a mean of 36.9. We analyzed clinical manifestations, radiologic findings, extents of surgical removal, histologic subtypes and follow-up results. Most patients were presented with sensory symptoms as initial symptoms which had lasted for 36.5 months on the average. The most frequent location was conus region(10 cases, 32%) followed by cervical, thoracic and cervico-thoracic spinal level. All cases were divided into two groups histologically, 12 myxopapillary subtypes and 19 non-myxopapillary subtypes. Operative results were dependent on the locations and the histologic subtypes of the tumor. Total removal was achieved in 4 cases out of 10 cases with masses around the conus and in 19 cases out of 21 cases with masses at other regions(p=0.003). Tumors were totally removed in 97% of 19 non-myxopapillary subtypes, but in 42% of 12 myxopapillary subtypes(p=0.001). From the follow-up data, we found that mean progression free interval was 83 months and 5 year progression free rate was 70%. Extent of removal was the only significant prognostic factor on multivariate analysis. Other factors such as tumor location, histologic subtype and radiation therapy were not significant. Disease progression was noted in 2 cases out of 23 cases of total removal group, but in 4 cases out of 8 cases of incomplete removal group (p=0.008). Postoperative radiation therapy was done in 4 cases in incomplete removal group and tumor regrowth was noted more frequently in non-radiation group than in radiation group without statistic significance. We concluded that disease progression can be determined by the extent of removal which is related to the tumor location and histologic subtypes.