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BACKGROUND: Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is an increasingly recognized neuroinflammatory syndrome that predominantly affects the pontine and cerebellar brain structures. Characteristically, patients will develop glucocorticoid-responsive brainstem disorders, demonstrate pontocerebellar contrast enhancement on magnetic resonance imaging (MRI), and exhibit an angiocentric, lymphocytic infiltrate in brain biopsies. We have presented and discussed 2 novel and challenging cases of CLIPPERS syndrome to highlight the clinical and radiological diversity of the syndrome. CASE DESCRIPTION: The first case was of a 66-year-old male patient who had presented with dizziness, headaches, gait disturbances, mild cognitive impairment, and visual field loss to the left side. MRI revealed 1 cerebellar and 2 occipital contrast-enhancing lesions that were suspicious for intracerebral metastases. The second case was of a 53-year-old male patient who had presented with temporal lobe epilepsy, anomic aphasia, and mild cognitive impairment. MRI demonstrated 4 contrast-enhancing lesions in the pons, temporal lobe, and thalamus that were suspicious for intracerebral lymphoma. Because of the radiological presentation, neoplastic disease was the most plausible diagnosis for both patients. However, repeated biopsies ruled out tumor manifestation, and the findings were finally consistent with CLIPPERS syndrome. The significant and long-lasting response to immunosuppressive treatment confirmed the diagnosis. CONCLUSIONS: In both cases, the characteristics of CLIPPERS syndrome imitated malignant tumor growth. This scenario can be challenging to clinicians and necessitates inclusion of this neuroinflammatory syndrome in the differential diagnosis of neuro-oncological disease.
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Cerebelo/patología , Encefalitis/tratamiento farmacológico , Encefalitis/patología , Inmunosupresores/uso terapéutico , Puente/patología , Corticoesteroides/uso terapéutico , Anciano , Humanos , Masculino , Persona de Mediana Edad , SíndromeRESUMEN
BACKGROUND: Anterior transarticular screw fixation (ATSF) of C1/C2 can be used for the treatment of unstable dens fractures. Here, we evaluated the feasibility of an anterior C1/C2 fixation in elderly patients with unstable dens fractures. Furthermore, we tried to analyze safe entry zones for ATSF surgery. METHODS: A consecutive cohort of 13 patients with unstable dens fractures were treated with ATSF of C1/C2 between January 2015 and October 2016. If necessary, an additional screw was placed into the odontoid process. The placement was radiographically analyzed using the 3D Arcadis Orbic (Siemens, Erlangen, Germany). Additionally, computed tomography scans of the cervical spine from 50 trauma patients were analyzed to evaluate safe entry zones for anterior odontoid screw fixation and for ATSF. RESULTS: ATSF was performed in 13 cases (7 female, 6 male; mean age 81.80 years). One screw had to be corrected intraoperatively due to initial malplacement. Neurological deficits or an injury of the vertebral artery were not observed. All patients suffered from swallowing difficulties during the postoperative course, without lesions of the esophagus or the trachea. In 4 patients (30.8%), an additional posterior fixation was offered to the patients due to progressive loosening of the screws.The anatomical-radiographic analyzes revealed a significantly shallower angle of trajectory for anterior odontoid screw fixation (24.9° ± 5.85°) than for ATSF (39.1° ± 6.44° (left); 40.5° ± 6.79° (right) P = 0.02). CONCLUSIONS: The ATSF of C1/C2 might be a valuable option in the treatment of instable C1/C2 fractures, especially in the elderly or in patients with short necks and/or high body mass index due to the steeper trajectory compared with odontoid screw placement. Yet available screws seem to be of inferior resistance compared with the biomechanical properties of a dorsal fixation. Further studies should focus on screws with better mechanical properties and probably additional cement augmentation. LEVEL OF EVIDENCE: 4.
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OBJECTIVE: Demographic changes have led to a higher incidence of C-2 fractures, especially in elderly patients. For patients with type II fractures, treatment remains controversial, as discussed by Anderson and D'Alonzo, due to the rising morbidity and mortality rates for any treatment. The aim of this study was to compare conservative and surgical management in patients with type II C-2 fractures regarding outcomes, complications, and the mortality rate. METHODS: A retrospective analysis was performed of the medical records, X-rays, and/or computed tomography scans of patients ≥80 years of age with type II fractures who were admitted to our Department of Neurosurgery between January 1990 and December 2017. The success of treatment was evaluated 3 months after surgery. RESULTS: In total, 125 patients were included, of whom 98 were treated surgically and 27 were treated conservatively. Surgical treatment was successful in 90.8% of cases, while conservative treatment was successful in 70.0%. The in-hospital mortality was 14.29% and the 3-month mortality was 27.8% in the surgical group, compared to 3.7% and 20% in the conservatively treated group. The in-hospital complication rate was 22.4% in the surgically treated patients and 7.4% in the conservatively treated patients. CONCLUSION: Surgical treatment of type II fractures seemed to be associated with higher success and complication rates than conservative treatment. Nevertheless, 3-month mortality was comparable in both groups. Therefore, we conclude that surgical treatment for type II fractures in elderly patients is superior to conservative management, although conservative treatment remains a valuable option in elderly patients with severe comorbidities.
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OBJECTIVE: Liponeurocytoma is a rare benign tumor of the central nervous system, which develops mainly in adult patients within the posterior fossa. The World Health Organization has categorized this entity in its last classification of 2016 as a benign grade II tumor. Histopathologic characteristics contain neuronal and variable astrocytic differentiation with foci of lipomatous distinction. Only a few case reports and case series have been reported and the knowledge of this tumor is limited. General treatment guidelines do not exist. The aim of this study was to analyze the literature to create treatment guidelines. METHODS: PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) guidelines were followed to search existing online databases between January 1, 1978 and May 15, 2018. RESULTS: A systematic review of the literature found 73 patients (40 female, 33 male) with liponeurocytoma, in 59 of whom (80.8%) the disease occurred in the posterior fossa. The Ki-67/MIB-1 proliferation index was described in 58 patients, showing a mean value of 3.73% ± 4.01%. Follow-up was performed in 49 patients, with a median length of 52.02 ± 50.52 months, showing tumor recurrence in 14 patients (28.57%). Tumor recurrence was observed in only 1 patient (1/12, 8.33%) after adjuvant radiotherapy, whereas tumor recurrence was seen in 13/29 patients (44.83%) who did not receive adjuvant radiotherapy. CONCLUSIONS: Liponeurocytomas are rare benign tumors, occurring mainly in the cerebellum. The therapy of choice is surgery. Postoperative radiotherapy seems to decrease the risk of tumor recurrence and should be offered to the patient.
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Neoplasias Cerebelosas/cirugía , Lipoma/cirugía , Neurocitoma/cirugía , Astrocitos/patología , Biopsia , Encéfalo/patología , Encéfalo/cirugía , Diferenciación Celular/fisiología , Neoplasias Cerebelosas/diagnóstico , Neoplasias Cerebelosas/patología , Terapia Combinada , Fosa Craneal Posterior/patología , Fosa Craneal Posterior/cirugía , Estudios de Seguimiento , Humanos , Lipoma/diagnóstico , Lipoma/patología , Recurrencia Local de Neoplasia/diagnóstico , Recurrencia Local de Neoplasia/patología , Recurrencia Local de Neoplasia/cirugía , Neoplasia Residual/diagnóstico , Neoplasia Residual/patología , Neoplasia Residual/cirugía , Neurocitoma/diagnóstico , Neurocitoma/patología , Neuronas/patología , Guías de Práctica Clínica como Asunto , Radioterapia AdyuvanteRESUMEN
BACKGROUND: Cerebellar liponeurocytoma is a rare tumor of the central nervous system occurring mainly in the posterior fossa, which shows neuronal and variable astrocytic differentiation with foci of lipomatous differentiation. Liponeurocytoma develops in adult patients and is defined in the World Health Organization classification of 2016 as a rare benign grade II tumor. CASE PRESENTATION: A 39-year-old Italian man presented to our department suffering from headache and nausea. Magnetic resonance imaging revealed a right-sided cerebellar lesion showing poor contrast enhancement without an obstructive hydrocephalus. Surgery was indicated and total tumor resection was achieved. He was discharged without any neurological deficits. Histopathological examinations revealed a cerebellar liponeurocytoma. A neurological follow-up examination revealed no neurological deficit directly after surgery and 1 year later. Radiotherapy was recommended at the neurooncological board despite the total removal of the tumor, but our patient refused adjuvant radiotherapy. Magnetic resonance imaging of his neurocranium with and without contrast enhancement 48 hours after surgery and 15 months after surgery showed no residual tumor. CONCLUSIONS: Liponeurocytomas are rare benign tumors occurring in the majority of cases in the cerebellum. The therapy of choice is surgery. Postoperative radiotherapy has to be discussed individually, but seems to be sufficient if complete tumor resection is not achieved or in cases of a tumor recurrence.
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Neoplasias Cerebelosas/patología , Lipoma/patología , Neurocitoma/patología , Adulto , Neoplasias Cerebelosas/diagnóstico por imagen , Neoplasias Cerebelosas/cirugía , Humanos , Lipoma/diagnóstico por imagen , Lipoma/cirugía , Imagen por Resonancia Magnética , Masculino , Neurocitoma/diagnóstico por imagen , Neurocitoma/cirugíaRESUMEN
OBJECTIVE: The demographic change in the population leads to a rising number of patients presenting with fractures of the cervical spine, especially C-2, due to falls. With an increase of co-morbidities in these elder patients, the risk for intra- and postoperative complications is increased likewise. Thus, an alternative strategy instead of operative management of these fractures should be taken into account whenever possible. Conservative management of dens fractures in the elderly is still a subject of great controversy despite numerous studies on this topic. The aim of this study was to analyze the rate of successful osseous consolidation of C-2 fractures with conservative treatment by wearing a semi-rigid collar or halo thoracic vest without further surgical intervention. PATIENTS AND METHODS: We analyzed the medical records and CT-scan of the cervical spine of 254 patients with C-2 fractures retrospectively, who were admitted to our department between January 1990 and September 2015. Fractures were diagnosed by CT-scan of the cervical spine and classified according to the Anderson - D`Alonzo classification. 183 patients were submitted to surgery as treatment of choice. In 71 patients a conservative management with external immobilization was chosen. The latter group was subjected to study analysis. RESULTS: 71 patients (mean age 74.08 years⯱â¯16.06 years) were diagnosed with C-2 fractures (Typ I: 4; Typ II 36; Typ III: 31) and treated conservatively using a Philadelphia collar (nâ¯=â¯57), or a halo-thoracic vest (nâ¯=â¯14), respectively. 12 patients were lost to follow up and excluded from further analysis. Conservative treatment of the fractures was deemed successful when a bony consolidation of the fracture in follow-up CT scans was seen (45 of 59 patients; 76.3%). 20 patients with a Type II fracture (20/28, 71.4%) showed a successful ossification. In 14 patients (overall 23.7%; Typ I: 1 (7.14%), Typ II: 8 (57.14%), Typ III: 5 (35.71%)) external immobilization failed to achieve primary stability. These patients were submitted to consecutive surgery. CONCLUSION: From our data it can be concluded that elderly patients, presenting with non-dislocated Type II fractures of the axis without accompaining neurological deficits, will have a more than 70% chance for a bony consolidation by conservative management. Consolidation rates may be estimated even higher in Type I and III fractures. Therefore, we suggest that external immobilization might be a valuable option to treat elderly patients with these fractures under certain circumstances.
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Vértebra Cervical Axis/diagnóstico por imagen , Vértebra Cervical Axis/lesiones , Manejo de la Enfermedad , Fracturas de la Columna Vertebral/diagnóstico por imagen , Fracturas de la Columna Vertebral/terapia , Anciano , Anciano de 80 o más Años , Femenino , Estudios de Seguimiento , Humanos , Masculino , Persona de Mediana Edad , Estudios RetrospectivosRESUMEN
The transsphenoidal approach to the sellar region has been introduced more than a 100 years ago. It is the accepted standard operative corridor to pathologies of the pituitary gland and surrounding structures. There are anatomical landmarks within the sphenoid sinus that are used for orientation directing to the sella floor or the cavernous sinus. Yet, little data can be found on the consistency of these landmarks. It is the aim of this study to evaluate the reliability of these anatomical landmarks for the surgeon's orientation. A total of 245 computed tomography (CT) volume data sets of the cranium performed according to a standardized protocol were analyzed for study purposes. CT scans of the cranium of 125 patients admitted to the emergency room of our hospital receiving a trauma spiral according to the local protocol were employed as a control group when no pathology in the sellar region was observed. In addition, preoperative CT scans of a group of 120 patients diagnosed with pituitary adenomas between 2009 and 2013 were analyzed. Image analysis of the anatomical landmarks included the minimal intercarotid distance (ICD), diameter of the sphenoid sinus (DSS), direction of the septum sinuum sphenoidalium (SSS), and the distance between vomer and clivus (VCD). The overall mean ICD was 16.2 mm, with patients suffering from adenomas showing a mean ICD of 15.8 mm compared with an average 16.5 mm in the control group. DSS was equal for both groups (adenoma group: mean 31.5 mm; controls: mean 31.3 mm). Mean VCD was 27.9 mm in patients with pituitary adenomas compared with 26.7 mm in controls. A septum of the sphenoid sinus located in the midline was found in overall 23 % only. SSS was directed into the bony shield of the internal carotid artery in 28 % of underlying tumors and in 37 % of the control group. This is the first detailed description of landmarks of the sphenoid sinus based on a large radiologic-anatomical analysis of CT scans yielding a wide variation and high inconsistency of these landmarks. From out data, we suggest that the surgeon approaching the sphenoid sinus should handle the anatomical landmarks with care bearing their inconsistency in mind. A thorough planning of the procedure up front is highly recommended. Usage of a navigation system considering the bony structures might as well facilitate as steady the approach to the sellar region in some cases for the patient's sake.
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Adenoma/diagnóstico por imagen , Puntos Anatómicos de Referencia/diagnóstico por imagen , Neoplasias Hipofisarias/diagnóstico por imagen , Seno Esfenoidal/diagnóstico por imagen , Tomografía Computarizada por Rayos X , Adenoma/patología , Adenoma/cirugía , Adulto , Arteria Carótida Interna/diagnóstico por imagen , Estudios de Casos y Controles , Femenino , Humanos , Masculino , Neoplasias Hipofisarias/patología , Neoplasias Hipofisarias/cirugía , Reproducibilidad de los Resultados , Base del Cráneo/diagnóstico por imagen , Hueso Esfenoides/diagnóstico por imagen , Seno Esfenoidal/cirugíaRESUMEN
OBJECTIVE: Dens fractures are common cervical injuries in advanced aged patients. The presented study was undertaken to analyze the clinical results and risks of surgically treated patients with dens fractures over 70 years. METHODS: Data of 28 patients (17 female, 11 male) over 70 years treated from September 2004 to October 2009 were recorded. Clinical and radiological parameters were obtained including type of fracture, associated cervical and/or other injuries, comorbidities, symptoms, neurological condition, surgical strategy, postoperative course and complications. RESULTS: 89% were in a good neurological condition before surgery (ASIA E or D). In most cases, surgery was performed at an early stage after trauma (21 patients within 5 days). Ventral screw fixation was the preferred surgical strategy (64%). A slight worsening of neurological functions immediately after operation was only seen in one patient. Five patients died in the early and 2 in the late postoperative course which means a treatment mortality of 25%. Among the surviving patients two had general medical complications. CONCLUSION: Type II dens fractures are a common fracture of elderly patients. Our results are good concerning the neurological functions. Surgical and general medical complications were acceptable. However, the study also underlines that mortality rate is high and therefore treatment options should be well-considered in this high risk group.