Early cortical atrophy in REM sleep behavior disorder. / Atrofia cortical precoz en el trastorno de conducta del sueño REM.

INTRODUCTION: The presence of cortical atrophy (focal or diffuse) prior to the development of symptoms of cognitive impairment could predict the earliest cases of neurodegenerative disease in patients with REM sleep behavior disorder (RSBD). We reviewed the usefulness of cranial CT and MRI as early markers of cortical atrophy in patients with RSBD at our center. PATIENTS AND METHODS: Retrospective observational descriptive analysis of patients diagnosed with RSBD from October 2012 to October 2022. All with cranial CT or MRI, evaluated by a neuroradiologist. RESULTS: 54 patients were included, 21 women (38.88%), 33 men (61.12%), mean age at diagnosis of RSBD: 69.04±12.625 years. Of the 54 patients, 44 (81.48%) had imaging tests consistent with their age, and 10 had atrophy greater than expected for their age. Of the 54 patients, 21 (38.88%) with a diagnosis of neurodegenerative disease, 33 (61.12%) persist as idiopathic, almost all with more than 5years of evolution (range of 1 to 10years of evolution without diagnosis). Of the 10 (18.52%) patients with greater atrophy, all were diagnosed with neurodegenerative disease (8 in 1year, 2 in 8years). CONCLUSIONS: Almost half of our series have developed a neurodegenerative disease in the first 10years of evolution. The majority of them presented global cortical atrophy measured by the GCA scale in the first year of diagnosis, without other neurological symptoms. Patients who did not show cortical atrophy at diagnosis have not yet developed the neurodegenerative disease in 10years of evolution. In our experience, the absence of cortical atrophy on cranial MRI or CT (measured by scales such as GCA) at the diagnosis of RSBD seems to predict slower progression cases. These data should be corroborated with larger series.

Use of Clamp-Like Devices to Fix Bifrontal Basal Craniotomies: Three Case Reports.

The three case reports presented here provide clinical evidence of a configuration to easily and successfully fix bifrontal basal craniotomies with a clamp-like fixation device (Cranial LOOP™, NEOS Surgery, Barcelona, Spain): A 72-year-old woman undergoing resection of a meningioma at the sphenoidal level, a 43-year-old man undergoing clipping of an anterior communicating artery aneurism, and a 55-year-old woman undergoing macroscopical resection of a meningioma on the olfactory groove had their surgery performed through a bifrontal basal craniotomy, and, once the intervention was completed, the fixation of the cranial flap was performed using Cranial LOOP™, with a configuration consisting of two Cranial LOOP™ L at the basal lateral level and two additional products at the posterior frontal level. The result of this fixation was very satisfactory with no settling or artifacts during the follow-up. These cases show that Cranial LOOP™ can be used to fixate bifrontal basal craniotomies.

Head and neck manifestations of an undiagnosed McCune-Albright syndrome: clinicopathological description and literature review.

Craniofacial fibrous dysplasia, characteristic of McCune-Albright syndrome (MAS), is usually present in patients with MAS-related acromegaly. We report here the first case of a patient with an undiagnosed MAS presenting with an acute hydrocephalus. A 21-year-old male with gigantism and craniofacial fibrous dysplasia consulted for rapidly progressive headache. An acute obstructive hydrocephalus due to a 39 × 35-mm cystic lesion in the third ventricle was discovered and operated, obtaining hydrocephalus resolution. Pathology described a colloid cyst material and a growth hormone-secreting pituitary adenoma. Genetic study revealed the mosaic GNAS R201H mutation in the pituitary tissue, confirming a MAS diagnosis. Adequate hormonal control was achieved postoperatively. Our results suggest that long-term untreated growth hormone excess in patients with MAS-related craniofacial fibrous dysplasia might end compromising cerebrospinal fluid flow. A prompt diagnosis and coordinated multidisciplinary treatment may help to avoid long-term deleterious impact of hyperfunctioning endocrinopathies in these patients.

Surgical outcomes of traumatic cervical fractures in patients with ankylosing spondylitis. / Resultados quirúrgicos de fracturas cervicales traumáticas en pacientes con espondilitis anquilosante.

OBJECTIVE: Ankylosing spondylitis is an inflammatory rheumatic disease mainly affecting the axial skeleton. The rigid spine may secondarily develop osteoporosis, further increasing the risk of spinal fracture. In this study, we reviewed fractures in patients with ankylosing spondylitis that had been clinically diagnosed to better define the mechanism of injury, associated neurological deficit, predisposing factors, and management strategies. METHODS: Between January 2004 and December 2014, 6 patients with ankylosing spondylitis and neurological complications after injuries were treated. Neuroimaging evaluation was obtained in all patients by using plain radiography, CT scan, and MR imaging. The ASIA Impairment Scale was used in order to evaluate the neurologic status of the patients. Surgical decision was based on relationship of neurological involvement and spinal instability. RESULTS: A total of 6 cervical injuries were identified in a review of patients in whom ankylosing spondylitis had been diagnosed. Of these, 2 patients were associated with a hyperextension mechanism and 4 cases by flexion mechanism. Posttraumatic neurological deficits were demonstrated in all 6 cases and neurological improvement after surgery was observed in 4 of these cases. The two cases were not improved by the surgery was on a case by presenting a degree of Asia A and another patient who initially improved with surgery but died of pneumonia in the postoperative. CONCLUSIONS: Patients with ankylosing spondylitis are highly susceptible to spinal fracture and spinal cord injury even after only mild trauma. Initial CT or MR imaging of the whole spine is recommended even if the patient's symptoms are mild. The patient should also have early surgical stabilization to correct spinal deformity and avoid worsening of the patient's neurological status.

[Lumbar spinal angiolipoma]. / Angiolipoma epidural lumbar.

Spinal angiolipomas are fairly infrequent benign tumours that are usually located in the epidural space of the thoracic column and represent 0.14% to 1.3% of all spinal tumours. Lumbar angiolipomas are extremely rare, representing only 9.6% of all spinal extradural angiolipomas. We report the case of a woman who complained of a lumbar pain of several months duration with no neurological focality and that had intensified in the last three days without her having had any injury or made a physical effort. The MR revealed an extradural mass L1-L2, on the posterior face of the medulla, decreasing the anteroposterior diameter of the canal. The patient symptoms improved after surgery. Total extirpation of the lesion is possible in most cases, and the prognosis is excellent even if the lesion is infiltrative. For this reason, excessively aggressive surgery is not necessary to obtain complete resection.

[Hemangioblastoma of the right L4 nerve root with radicular extraforaminal involvement]. / Hemangioblastoma radicular extraforaminal de la raíz L4 derecha.

Hemangioblastomas are benign hypervascular tumours, which are frequently located at the posterior fossa or the spinal cord. Nerve root hemangioblastomas account for less than 2% of total cases. Sometimes the initial diagnosis can be inaccurate and the final diagnosis is not reached until the surgical procedure is performed. Given the high vascularisation of this particular kind of tumour, preoperative embolisation of the tumour is recommended to reduce surgical bleeding and minimise the risk of injuries to motor nerve roots. In this particular case, the patient presented with radicular pain in the right L4 nerve root territory originated by a radicular extraforaminal hemangioblastoma. After tumour devascularization and under neurophysiological control, total excision of lesion was achieved without posterior neurological deficit.

[Erythropoietin as a protective factor in rat CNS cells receiving radiotherapy -an in vitro study]. / La eritropoyetina como factor de proteccion de la radioterapia sobre celulas del sistema nervioso central. Estudio in vitro.

AIM: To investigate the effect of erythropoietin in cultured rat cerebral cortex cells receiving radiotherapy. MATERIALS AND METHODS: Cerebral cortex was taken from 17-day-old Wistar rat embryos and placed in culture. At 72 hours, the cultures were divided into two groups, one receiving 30 pM erythropoietin alpha and the other was the control group. Both groups received 6 Gy from a Phoenix apparatus and were incubated for another 24 hours before fixation in 4% formaldehyde. TUNEL technique was employed to calculate the number of apoptotic cells in the irradiated cultures. RESULTS: Apoptosis affected 25.22% of the cells cultured without erythropoietin and 15.5% in the group receiving erithropoyetin. Student's t-test was used to analyse quantitative variables and showed a significant difference in apoptosis between the two groups (p < 0.0001). CONCLUSION: Our in vitro experimental model demonstrated that erythropoietin effectively prevents apoptosis in irradiated rat SNC cells, opening new fields for investigation into protective agents for the nervous system.

TITLE: La eritropoyetina como factor de proteccion de la radioterapia sobre celulas del sistema nervioso central. Estudio in vitro.Objetivo. Investigar el efecto de la eritropoyetina en cultivos celulares de corteza cerebral de ratas cuando se administra radioterapia. Materiales y metodos. El estudio se desarrolla con la obtencion de corteza cerebral de embriones de 17 dias de preñez de ratas Wistar. Las celulas cultivadas despues de 72 horas de la extraccion de la corteza se dividieron en dos grupos, a uno de ellos se le administro eritropoyetina alfa a una concentracion de 30 pM y el otro era el grupo control. A los dos grupos de celulas se les radio con 6 Gy mediante un aparato Phoenix. Tras la radioterapia permanecieron 24 horas en la incubadora antes de fijarlas. Las celulas fueron fijadas con formaldehido al 4%. A continuacion, con la tecnica de TUNEL, se valoro el numero de celulas apoptoticas en los cultivos radiados. Resultados. Se observo un porcentaje de apoptosis del 25,22% del grupo de cultivo sin eritropoyetina, mientras que en el grupo de celulas radiadas con eritropoyetina fue del 15,5%. Las variables cuantitativas se analizaron mediante el test t de Student y el resultado de la comparacion entre los dos grupos fue estadisticamente significativo (p < 0,0001). Conclusion. En nuestro modelo experimental in vitro se comprobo que la eritropoyetina es eficaz en la prevencion de la apoptosis en celulas del sistema nervioso central de ratas por radiacion. Esto abre nuevos campos para la investigacion del efecto protector del sistema nervioso.

[Tumours of the upper cervical spine]. / Tumores vertebrales de la región cervical alta.

OBJECTIVE: Vertebral tumours arising in the upper cervical spine are rare. We present our experience in managing these neoplasms. MATERIAL AND METHODS: We retrospectively reviewed the case histories of patients treated at our institution between January 2000 and June 2011. RESULTS: There were 9 patients with tumours in C1-C2-C3: 2metastases, 3chordomas, 2plasmocytomas, 1chondrosarcoma and 1osteochondroma. All patients complained of neck pain at the time of diagnosis. Three patients underwent an anterior and posterior approach, 3 an exclusively posterior approach and 3 an exclusively anterior surgical approach. Tumour resection was intralesional in 7 cases. Chemo-radiotherapy was used as adjuvant therapy in patients with malignant tumours. CONCLUSIONS: Vertebral tumours in the upper cervical spine are usually malignant. Achieving en bloc resection is particularly challenging and is technically unfeasible in many cases. This worsens the prognosis and makes adjuvant treatment very important.