Superficial siderosis of the central nervous system in a patient with asymptomatic sacral paraganglioma as source of chronic bleeding
Introduction: Superficial siderosis of the central nervous system is an infrequent pathology secondary to chronic bleeding into the cerebrospinal fluid. Spinal tumors are infrequent cause of superficial siderosis being ependymoma the most common etiology.
Case report: We report the case of a woman with sensorineural hearing loss and cerebellar ataxia, diagnosed of superficial siderosis on brain MRI. She had no previous history of axial back pain or radicular leg pain or bowel or bladder incontinence. On spine MRI an intradural lesion was found at the S1 level. No signs of intratumoral hemorrhage were observed on MRI gradient-echo images. At surgery, an intradural soft mass with signs of chronic bleeding was completely resected. Based on microscopic examination and immunohistochemistry of the specimen, a diagnosis of paraganglioma World Health Organization grade I was made.
Conclusions: Since the only proven treatment able to prevent further deterioration from superficial siderosis is to stop chronic bleeding into subarachnoid space, is of paramount importance to establish an early diagnosis of the source of bleeding. Cases of unexplained superficial siderosis of central nervous system should include routine spinal MRI to rule out bleeding of spinal tumor even in asymptomatic patients. Due to severity of potential deterioration caused by superficial siderosis, any tumoral lesion observed on spinal MRI even without documented sings of bleeding should be considered for resection.
Caso clínico Mujer con pérdida auditiva neurosensorial y ataxia cerebelosa, diagnosticada de siderosis superficial en la resonancia magnética cerebral. No tenía antecedentes de dolor raquídeo axial, dolor radicular ni incontinencia esfinteriana. En la resonancia magnética de la columna se encontró una lesión intradural en S1. No se observaron signos de hemorragia intratumoral en las secuencias de resonancia magnética en eco de gradiente. En la cirugía, se apreció una masa blanda intradural con signos de sangrado crónico que se resecó. Basado en el examen microscópico e inmunohistoquímico de la muestra, se alcanzó el diagnóstico de paraganglioma de grado I de la Organización Mundial de la Salud.
Conclusiones Dado que el único tratamiento probado capaz de prevenir un mayor deterioro por la siderosis superficial es detener el sangrado crónico en el espacio subaracnoideo, es importante establecer un diagnóstico temprano de la fuente de sangrado. Los casos no justificados de siderosis superficial del sistema nervioso central deben incluir una resonancia magnética de la columna rutinaria para descartar el sangrado de un tumor medular, incluso en pacientes asintomáticos. Debido a la gravedad del deterioro potencial causado por la siderosis superficial, cualquier lesión tumoral observada en una resonancia magnética del raquis, incluso sin presentar signos de sangrado, debería ser objeto de indicación quirúrgica.