[Supratentorial endodermal cyst. Case report]. / Quiste endodérmico supratentorial: caso clínico y revisión de la literatura.

Endodermal cysts (EC) of the central nervous system are very uncommon lesions predominantly located in the spinal canal. Although rare, intracranial EC have been mainly described in the posterior fossa, with the supratentorial location considered exceptional. Apart from the low frequency of these lesions, their pathoembriology still remais unknown. We report a patient with a huge frontal EC and review the literature. A 62-year-old man presented with abnormal behaviour, disorientation and decreased level of consciousness after moderate head injury. Initial cranial CT scan revealed a large cyst in the left frontal region with marked midline shift. Emergency puncture and decompression of the cyst demonstrated a milky fluid with high protein levels. Cranial MRI after patient improvement confirmed the existence of the cystic lesion with less mass effect. Delayed surgery was performed with craniotomy and total removal of the cyst. Pathological examination confirmed the presence of a typical EC. Patient made a complete recovery on follow-up with no recurrence on postoperative MRIs. Differential diagnosis of EC based on radiological data is quite difficult. As aggresive behaviour of this condition has been described following incomplete resections, the treatment of choice is a radical removal of the cyst in one or two stages depending on patient clinical condition.

[Analysis of the clinical impact of the diagnostic reclassification of brain gliomas according to the World Health Organization classification (2016)]. / Análisis del impacto clínico de la reclasificación diagnóstica de gliomas cerebrales según la clasificación de la Organización Mundial de la Salud (2016).

INTRODUCTION: Since the introduction of genetic and molecular criteria in the 2016 World Health Organization (WHO) classification of brain tumours, there has been a diagnostic reclassification between certain astrocytomas and oligodendro-gliomas with histological and genetic discordances, the prognosis of which is unknown. AIM: To analyse the implications of the diagnostic reclassification of brain gliomas according to the 2016 WHO criteria, especially depending on isocitrate dehydrogenase (IDH) mutation and 1p19q codeletion. PATIENTS AND METHODS: We conducted a retrospective study of gliomas treated from 1 January 2012 to 31 December 2016, with analyses of clinicoradiological aspects and prognoses, and with available and complete follow-up until 31 March 2019. RESULTS: From a total of 147 brain gliomas, a molecular diagnosis and a diagnostic re-evaluation were carried out in 69 cases (grade II-IV astrocytomas or oligodendrogliomas). Twenty-four reclassified gliomas were detected, usually oligodendro-gliomas that became astrocytomas, and which showed greater survival, derived from their not being classified as grade IV. The reclassified gliomas, all grades II/III, mostly began with seizures, without focus, with single lesions, < 17 cm3 and with oedema, although with similar survival rates. The prognostic factors were: young age, focus, grade II and no contrast enhancement or necrosis, or multiplicity. No variations were detected according to the molecular pattern with IDH mutation or codeletion. CONCLUSION: The changes in diagnosis after the WHO classification of 2016 present specific clinical-radiological characteristics in this series, but no greater survival, although, due to the habitual survival in these cases, they would require a longer follow-up time.

TITLE: Análisis del impacto clínico de la reclasificación diagnóstica de gliomas cerebrales según la clasificación de la Organización Mundial de la Salud (2016).Introducción. Desde la introducción de los criterios genéticos y moleculares en la clasificación de la Organización Mundial de la Salud (OMS) de tumores cerebrales de 2016, se ha producido una reclasificación diagnóstica entre determinados astrocitomas y oligodendrogliomas con discordancias histológicas y genéticas, cuyo pronóstico se desconoce. Objetivo. Analizar las implicaciones de la reclasificación diagnóstica de los gliomas cerebrales según los criterios de la OMS de 2016, especialmente según la mutación de la isocitrato deshidrogenasa (IDH) y la codeleción 1p19q. Pacientes y métodos. Estudio retrospectivo de los gliomas tratados desde el 1 de enero de 2012 hasta el 31 de diciembre de 2016, con análisis de los aspectos clinicorradiológicos y pronósticos, y con seguimiento disponible y completo hasta el 31 de marzo de 2019. Resultados. De 147 gliomas cerebrales, en 69 (astrocitomas u oligodendrogliomas de grados II-IV) se realizaron un diagnóstico molecular y una reevaluación diagnóstica. Se detectaron 24 gliomas reclasificados, habitualmente oligodendrogliomas que pasaron a astrocitomas, y que mostraron mayores supervivencias, derivadas de la no reclasificación en grado IV. Los gliomas reclasificados, todos de grados II/III, comenzaron mayoritariamente con crisis, sin focalidad, con lesiones únicas, < 17 cm3 y con edema, aunque con similar supervivencia. Los factores pronósticos fueron: edad joven, focalidad, grado II y no captación de contraste o necrosis, o multiplicidad. No se detectaron variaciones según el patrón molecular con mutación en la IDH o codeleción. Conclusión. Los cambios diagnósticos tras la clasificación de la OMS de 2016 presentan características clinicorradiológicas específicas en esta serie, aunque no mayores supervivencias, si bien, por la supervivencia habitual en estos casos, precisarían un mayor tiempo de seguimiento.

[Radiation-induced cranial tumors: clinical series and literature review]. / Tumores craneales radioinducidos: serie clínica y revisión de la literatura.

Radiation induced tumors are well-known but rare complications of radiotherapy. Meningiomas are the most common radiation-induced (RI) cranial tumors, followed by gliomas and sarcomas, while other tumors as haemangioblastomas remain extremely exceptional. We present 7 patients with RI brain tumors diagnosed and treated at our institution between 1990 and 2006. Retrospective review of their clinical charts is supplied. All patients were irradiated during childhood as a treatment for another disease, and fulfilled the criteria of RI neoplasia. Four patients developed meningiomas and three developed other tumors (one glioblastoma, one softtissue sarcoma and one hemangioblastoma). In all cases a complete surgical removal was achieved. Preoperative assessment based on MRI supplied the correct diagnosis in six patients. The most important risks factors described in the literature for developing RI tumors are the age at which radiotherapy was administered and the dose of radiation applied. Differential diagnosis of RI tumors includes any tumor appearing after radiotheraphy, especially recurrences of the primary disease, as RI neoplasias are a rare complication. Even in cases with complete surgical resection, prognosis of this clinical entity is basically related to the histology of the RI tumor.

[Intraventricular tension pneumocephalus after transsphenoidal surgery: a case report and literature review]. / Neumoencéfalo intraventricular a tensión secundario a cirugía transesfenoidal: presentación de un caso y revisión de la literatura.

Tension pneumocephalus is a rare complication of transsphenoidal approaches. The case of a 37 year old woman with a transsphenoidal resection of a pituitary adenoma who presented self-limited rhinoliquorrhea postoperatively is reported. Three days later the patient developed progressive decreased consciousness, amnesia and headache, showing an intraventricular tension pneumocephalus on CT scan. Urgent treatment with bilateral external ventricular drainage and anterior nasal tamponade was performed with good clinical outcome. Later transsphenoidal sealing of the dural defect was achieved without recurrence. Tension pneumocephalus following transsphenoidal surgery usually occurs after the presentation of a cerebrospinal fluid leak due to an incomplete sealing of the sphenoid sinus. The postoperative insertion of a lumbar drainage seems to be a predisposing condition for this complication. The combined approach of tension pneumocephalus with external ventricular drainage and repair of the sphenoid sinus offers optimal results solving the acute neurological deterioration and avoiding recurrence.

[Familiar glioblastoma presenting as a true multicentric tumor: etiopathogenic and prognostic features]. / Glioblastoma familiar múltiple de aparición metácrona: implicaciones etiopatogénicas y pronósticas.

Familial glioblastoma multiforme is a rather uncommon entity, being in most cases associated to known genetic disorders (as Turcot syndrome, Li-Fraumeni syndrome, neurofibromatosis, etc.). However, familial gliomas have also been described, although less frequently, independently of these genetic syndromes showing some special features regarding its etiology and clinical manifestations. Less than 10% of gliomas may be considered as true multicentric tumours either synchronous or metachronous in clinical presentation. Metachronous glioblastomas have been associated to better prognosis in some studies, with genetic studies having found clear differences among the tumors within same patients. Familial glioblastoma with metachronous presentation is an exceptional disorder. These tumors show special therapeutic implications due to the limitations of radiotherapy once the patient has already irradiated. A variety of non-specific mutations have been found in these patients but true characterization of this disorder remains unclear and will be based on further genetic studies. We present a clinical report on a patient harbouring a familial and metachronous glioblastoma. The main aspects of this entity are reviewed.

[Immunotherapy in high grade astrocytomas: principles and current state]. / Immunoterapia en astrocitomas de alto grado: principios y estado actual

It is generally accepted that tumour development promotes a systemic response leading to protect the host against cancer. However, tumours may as well elicit a partial immunodeficiency to avoid the development of a complete and active immune response. Since Bloom's first studies on immunotherapy to treat high grade gliomas in 1960, many attempts have been made from different medical specialties to use the immune system as a weapon against a great diversity of cancers. Main objective of this study is to outline the basic features of the immune response inside the Central Nervous System, the strategies employed by astrocytic tumours to evade body defences, and to provide an extended literature review on research on immunotherapy, especially concerning its patho-physiology and the clinical results achieved till date.

[Cerebral cavernomas. A review and update of aetiological, clinical and therapeutic features]. / Cavernomas cerebrales. Revisión y actualización etiológica, clínica y terapéutica.

INTRODUCTION: Cavernous angiomas are uncommon lesions, with a reported incidence of 0.4 to 0.8%, presenting a controversial management especially regarding their surgical treatment. AIM: To update cavernous angiomas characteristics and management through a deep review of the literature concerning their aetiology, epidemiology, history, signs and symptoms, diagnosis, and surgical and radiosurgical treatment. DEVELOPMENT AND CONCLUSIONS: Most important advances found in the recent literature include the identification of the genetic basis responsible for the familial form of cavernomatosis (CCM1, CCM2 and CCM3), the identification of the dynamic pattern of these lesions based on their pathology and imaging features, the deeper knowledge on their natural history depending on their supra/infratentorial location, and the main indications for surgical treatment and radiosurgical therapy suggested by the recent series.

[Akinetic mutism related to hydrocephalus and cerebellar surgery treated with bromocriptine and ephedrine. A pathophysiological review]. / Mutismo acinético relacionado con hidrocefalia y cirugía cerebelosa tratado con bromocriptina y efedrina. Revisión fisiopatológica.

Akinetic mutism (AM) is a behavioral disorder characterized by impossibility to move or speak in awake patients. lt has been typically described as a transient disorder following posterior fossa tumour resection. Besides, AM may also appear after recurrent shunt failures in hydrocephalic patients, with no tendency towards improvement, either spontaneously or with shunt revisions. However successful treatment of this second type of AM has been achieved with bromocriptine. We present a patient who developed AM after a posterior fossa surgery complicated by ventriculitis and multiple hydrocephalic events. AM only improved with bromocriptine. We review AM pathophysiology. Although not well known, it appears to be quite different, depending on its cerebellar or hydrocephalic origin. Damage to dentate nucleus or its efferents (mainly of glutamate) should promote AM of cerebellar origin, while damage to paraventricular monoaminergic pathways could explain AM related to repeated shunt failures which has successful response to bromocriptine treatment. However, a more complete study of this disorder is required to ascertain its aetiology.

Trigeminal nucleus caudalis dorsal root entry zone radiofrequency thermocoagulation for invalidating facial pain.

INTRODUCTION: Facial pain syndromes occasionally result in desperate clinical settings completely unresponsive to any known therapy. Trigeminal nucleus caudalis dorsal root entry zone (DREZ) lesion is reported to be of benefit in such cases. In 1982 Nashold performed the first DREZ caudalis lesion in a patient with anaesthesia dolorosa. PATIENTS AND METHODS: From 1994 to 2002 we have performed six DREZ caudalis lesions on five patients with extremely invalidating facial pain resistant to multiple pharmacological and surgical therapies. Pain was secondary to previous craniofacial surgery in all but one case. Pain presented as anaesthesia dolorosa or atypical facial pain so severe as to interfere with personal hygiene and even to prevent patients from oral feeding. A midline suboccipital approach was used and radiofrequency lesions (at the trigeminal nucleus caudalis in the cervicomedullary junction) were made at 1-mm intervals, 75 (o)C for 15 seconds each along the ipsilateral posterolateral sulcus from the cervical DREZ up to the obex. RESULTS: Pain relief was complete and permanent in two patients. Three patients experienced significant improvement but pain recurred in two (weeks to a few months after the procedure). No patient's pain was made worse. A patient with persistent postoperative nasolabial pain was re-operated on (improving again but ultimately remaining unchanged). Air venous embolism related to the sitting position (3 patients) during surgery and bradycardia due to manipulation in medulla (2 patients) occurred during some of the procedures without any cardiovascular or neurological repercussion. Postoperative complications included mild and transient ataxia and monoparesia (3 patients). DISCUSSION: Facial pain secondary to craniofacial surgery is known to be among the least responsive to treatment and a true challenge for pain clinicians. Actual indications for this procedure, operative technical details and the results of our series compared to previous reports are reviewed. CONCLUSION: Trigeminal nucleus caudalis radiofrequency thermocoagulation is an effective neurosurgical procedure for the treatment of chronically debilitating and desperate facial pain syndromes with acceptable morbidity.

[Decompressive craniectomy in malignant infarction of the middle cerebral artery]. / Craniectomía descomprensiva en ictus isquémico maligno de arteria cerebral media.

INTRODUCTION: Medically managed malignant infarction of the middle cerebral artery (MCA) is associated with an 80% mortality rate. However, several studies report a 40-50% increase in survival rates when decompressive craniectomy is performed. We present our experience with such surgical treatment and a literature review. PATIENTS AND METHODS: Seven decompressive craniectomies were performed on five patients for spaceoccupying MCA infarctions. Age ranged from 33 to 57 years-old (three males and two females). Preoperative GCS score was 9-13. Cranial CT was performed within the first 12 hours. Intracranial pressure (ICP) was continuously measured in four patients. Two infarcts occurred in the dominant hemisphere and three in the non-dominant side. Wide fronto-parieto-temporal craniectomies were performed. The duramater was opened and a large heterologous dura graft was placed. RESULTS: Surgery was performed on the second day after the onset of symptoms (median: 47 hours). Preoperative ICP ranged from 27 to 50 mmHg (median: 30.5 mmHg), with immediate postoperative ICP under 15 mmHg in all patients. Two patients (both non-dominant side) survived with good (after reoperation) and excellent functional outcome. They remain stable after ten and five months of follow-up. Three patients died five, five and thirteen days after admission due to uncontrollable high ICP. In our experience, bone removal itself was more relevant than dural opening for ICP control. Initial wide craniectomies may spare reoperations. CONCLUSION: The significant mortality rate reduction, a wide therapeutic window (2-3 days) and a low incidence of intraoperative complications make decompressive craniectomy a relevant treatment in malignant cerebral MCA infarction.

[Ruptured aneurysms presenting with subdural hematoma: management without angiography]. / Hematomas subdurales de causa aneurismática: estrategia terapéutica sin angiografía diagnóstica.

INTRODUCTION: Ruptured aneurysms on rare occasions cause subdural hematomas as described in literature. Sudden deterioration and coma is a common feature in those patients and a emergent surgical attitude is prompt required, even without confirmation with angiography. CASE REPORTS: We described three cases with acute subdural hematomas and little or no subarachnoid hemorrhage caused by ruptured aneurisms who presented with rapid neurologic deterioration. Urgent craniotomy and evacuation of the hematoma was performed without previous angiography in the three patients. In two patients the aneurysm was found during surgical exploration and subsequently clipped; in the remaining patient the aneurysm was embolized postoperatively. CONCLUSIONS: The occurrence of a subdural hematoma caused by the rupture of an intracranial aneurysm must be suspected in spontaneous subdural hematomas, especially in association with disproportioned conscious deterioration. All the three patients we report debuted with sudden conscious deterioration. If a ruptured aneurysm causing subdural hematoma is suspected, early surgical intervention is required even if angiography is not available. Severe neurological deficit and uncal herniation might still be reversible if provided decompression can be carried out in promptly. Angiography availability should not postpone surgery. Aneurysm presence should be ruled out whether by surgical exploration or by delayed angiography. Posterior communicating aneurysm are related to formation of subdural hematoma.

[Massive intratumoral haemorrhage as a clinical presentation of acoustic neuroma]. / Hemorragia intratumoral masiva como presentación de neurinoma del acústico.

INTRODUCTION: Acoustic neurinoma presenting as massive intratumoural hemorrhage is quite an uncommon entity, with less than 40 clinical reports in the literature. Secondary mass effect compressing the brain stem may be a life threatening condition. CASE REPORT: A 38 years old male was admitted at our hospital with several cranial nerve palsies on the right side and decreased level of consciousness (GCS 5) of acute onset. On cranial computed tomography a great haematoma was found in the right cerebello pontine angle, appearing to be an intratumoural bleeding. Emergency surgery was performed with complete removal of the haematoma and of the tumour related to the VIII cranial nerve, which was diagnosed as hypervascularized schwannoma. Clinical outcome was good. CONCLUSIONS: Intratumoural hemorrhage in acoustic neurinomas is rare, appearing mostly in those tumours measuring more than 3 cm and with increased vascularity. In general they manifest with subtle neurological deficits. Acute neurological deterioration with decrease in consciousness level although exceptional may be associated to great mortality rates. The treatment of choice is the complete removal of the haematoma and the tumour, in one surgery if possible. Prognosis is good in most cases, with morbidity consisting on cranial nerve palsies. Hypoglossal facial anastomosis may restore at least partial facial function. Neurophysiology tests give a clear measurement of cranial nerve lesions and their evolution with treatments.

Surgical activity by Spanish Residents in Neurosurgery under the Training Programme in place since 1984 and changes to be implemented under the New Training Programme.

The Spanish National Committee for Neurosurgery has recently approved a new training programme which will shortly replace the former programme, which dates from 1984. The object of this report was to evaluate training in neurosurgery in Spain under the extant programme as perceived by the residents themselves. Assessment of any educational shortcomings might help in designing and evaluating the new one. Secondary objects were to present and discuss the principal changes to be implemented by the new programme.A questionnaire was circulated to the entire population of Spanish neurosurgical residents nation-wide (N = 62) in 2000, and this was repeated using the same group in 2001 and 2002. Residents were to answer questions concerning their experience during the reporting year and the preceding years, the results compiling information for 1996 to 2002. The questionnaire covered 72 surgical procedures listed in ascending order of complexity, with a five-point scale of surgical responsibility for each type. Responses were received from 48 residents. Spanish residents appeared to uniformly fulfil the general requirements of the former programme. The main shortcomings identified were low levels of surgical activity for certain special operations, especially functional and skull base approaches, and the absence of a log book specific to neurosurgery. The new training programme developed by the Spanish National Committee for Neurosurgery has taken these shortcomings into consideration and has introduced major changes in order to ensure that training will be improved on an ongoing basis.