Resección completa de neurocitoma extraventricular hipervascularizado tras embolización preoperatoria / Complete resection of hypervascularised extraventricular neurocytoma after preoperative embolisation

Una mujer de 13 años de edad presenta clínica de cefalea de 15 días de evolución y solo edema de papila bilateral en la exploración. El estudio inicial de tomografía computarizada y RM mostró una gran masa multiquística frontal izquierda con calcificación rodeada de edema periférico, sangrado intralesional subagudo y múltiples importantes vasos asociados. Se interviene en otro centro, encontrando cavidad con hematoma subagudo que se evacua con múltiples vasos y venas arteriolizadas. Ante la sospecha de malformación arteriovenosa (MAV) a pesar de los hallazgos de la neuroimagen realizada previamente, se deriva a nuestro centro para seguir tratamiento. Realizamos arteriografía, angio-RM y RM con secuencias avanzadas que muestran masa intraaxial hipervascularizada que se emboliza previo a la interviene quirúrgica definitiva con resultado anatomopatológico de neurocitoma extraventricular (NEV). Los NEV son lesiones extremadamente raras que no se han descrito previamente en la literatura como lesiones hipervascularizadas que en nuestro caso requirió la realización de angiografía y embolización previa para su correcto diagnóstico y adecuado manejo

A 13-year-old female arrived at the Emergency Department with a two-week history of headache, and bilateral papilloedema on examination. The initial study with CT and MRI showed a large multicystic left frontal mass with calcification surrounded by peripheral oedema, subacute intralesional bleeding and association of multiple large vessels. She was initially operated on in another centre where a subacute haematoma was found, evacuating to multiple vessels and arteriolised veins. Despite the earlier neuroimaging findings, arteriovenous malformation (AVM) was suspected, so she was referred to our centre for further treatment. We performed angiography, MR angiography and MRI with advanced sequences, diagnosing a highly vascularised intra-axial tumour which was embolised. The patient was then definitively operated on, with the resulting finding of extraventricular neurocytoma (EVN). EVN are extremely rare lesions, not previously described in the literature as hypervascularised lesions, which in our case required prior angiography and embolisation for proper diagnosis and adequate management

Complete resection of hypervascularised extraventricular neurocytoma after preoperative embolisation. / Resección completa de neurocitoma extraventricular hipervascularizado tras embolización preoperatoria.

A 13-year-old female arrived at the Emergency Department with a two-week history of headache, and bilateral papilloedema on examination. The initial study with CT and MRI showed a large multicystic left frontal mass with calcification surrounded by peripheral oedema, subacute intralesional bleeding and association of multiple large vessels. She was initially operated on in another centre where a subacute haematoma was found, evacuating to multiple vessels and arteriolised veins. Despite the earlier neuroimaging findings, arteriovenous malformation (AVM) was suspected, so she was referred to our centre for further treatment. We performed angiography, MR angiography and MRI with advanced sequences, diagnosing a highly vascularised intra-axial tumour which was embolised. The patient was then definitively operated on, with the resulting finding of extraventricular neurocytoma (EVN). EVN are extremely rare lesions, not previously described in the literature as hypervascularised lesions, which in our case required prior angiography and embolisation for proper diagnosis and adequate management.

Síndrome de twiddler en paciente con trastorno obsesivo compulsivo tratado mediante estimulación cerebral profunda / Twiddler’s syndrome in a patient with obsessive-compulsive disorder treated with deep brain stimulation

El síndrome de twiddler es una extraña complicación relacionada con los dispositivos implantables de estimulación eléctrica. Descrito por primera vez en un paciente portador de marcapasos, es una complicación conocida en el ámbito de la cardiología, aunque no lo es tanto en el mundo de la neurocirugía, donde se ha descrito en relación con dispositivos de estimulación cerebral profunda. Se caracteriza por la manipulación, ya sea consciente, ya inconsciente, del generador de tales dispositivos, lo que origina el giro de este sobre sí mismo, lo que ocasiona el trenzado del cableado de estos sistemas, pudiendo provocar su rotura o bien el desplazamiento de los electrodos intracraneales. Describimos un caso de síndrome de twiddler en un paciente tratado mediante estimulación cerebral profunda para el trastorno obsesivo compulsivo que, tras una buena respuesta inicial, presenta un deterioro clínico, apreciándose en las radiografías de control del sistema el giro del cableado y el desplazamiento de los electrodos intracraneales

Twiddler's syndrome is a rare complication associated with implantable electrical stimulation devices. First described in a patient with a pacemaker, it is a known complication in the field of cardiology. However, it is not so recognised in the world of neurosurgery, in which it has been described in relation to deep brain stimulation (DBS) devices. Characterised by manipulating either consciously or unconsciously the generator of such devices, which causes it to rotate on itself, the syndrome causes the coiling of the wiring of these systems and can lead to their rupture or the displacement of intracranial electrodes. We describe a case of twiddler's syndrome in a patient treated with DBS for obsessive-compulsive disorder, in which clinical deterioration presented after a good initial response. Control radiographs revealed rotation of the wiring system and displacement of the intracranial electrodes

[Twiddler's syndrome in a patient with obsessive-compulsive disorder treated with deep brain stimulation]. / Síndrome de twiddler en paciente con trastorno obsesivo compulsivo tratado mediante estimulación cerebral profunda.

Twiddler's syndrome is a rare complication associated with implantable electrical stimulation devices. First described in a patient with a pacemaker, it is a known complication in the field of cardiology. However, it is not so recognised in the world of neurosurgery, in which it has been described in relation to deep brain stimulation (DBS) devices. Characterised by manipulating either consciously or unconsciously the generator of such devices, which causes it to rotate on itself, the syndrome causes the coiling of the wiring of these systems and can lead to their rupture or the displacement of intracranial electrodes. We describe a case of twiddler's syndrome in a patient treated with DBS for obsessive-compulsive disorder, in which clinical deterioration presented after a good initial response. Control radiographs revealed rotation of the wiring system and displacement of the intracranial electrodes.