Bobble-head doll syndrome in an 80-year-old man, associated with a giant arachnoid cyst of the lamina quadrigemina, treated with endoscopic ventriculocystocisternotomy and cystoperitoneal shunt.

Bobble-head doll syndrome (BHDS) is a rare entity, characterized by antero-posterior head bobbing, which is of the type "yes-yes." Less frequently, having a head movement of the type "no-no" is described. We report an unusual case of an 80-year-old man with a cystic mass of the lamina quadrigemina, extending to the posterior fossa. We conclude that ventriculocystocisternotomy associated with a cystoperitoneal shunt is an effective treatment for a symptomatic giant arachnoid cyst in the lamina quadrigemina.

[Deep brain stimulation in Parkinson's disease. Preliminary outcomes]. / Estimulación cerebral profunda en la enfermedad de Parkinson. Resultados preliminares.

BACKGROUND: Parkinson's disease justifies the use of deep brain stimulation (DBS) in certain patients who suffer from this condition. We present mid-term and long-term post-surgical outcomes in a cohort of 60 patients, who underwent DBS in the Hospital de Especialidades at Centro Médico Nacional Siglo XXI, in Mexico City. METHODS: Patients underwent conventional stereotactic surgery with FrameLink software (Medtronics Inc). This technique consisted in the presurgical evaluation, the placement of stereotactic frame, imaging studies, preoperative planning procedure, microrecording, macrostimulation, as well as the placement of electrodes and generators in two phases. The variables were included in a data platform for Excel management. It was also included a variety of measurement instruments for data comparison. As a standard measure, it was used the Unified Parkinson Disease Rating Scale (UPDRS) before the surgery and at 3, 12, and 36 months. RESULTS: 60 patients underwent surgery: 41 men and 19 women, with an average age of 56.5 years (39-70). There were good results in the majority of patients with preoperative UPDRS and at 3, 12 and 36 months of 79.57, 66.85, 65.29 and 58.75, respectively (p < 0.0001). There were complications in nine patients (15 %) and they were managed in a conservative manner. CONCLUSIONS: Postsurgical outcomes were from good to excellent in the majority of patients. Complications were minimal and conservatively managed. We propose the use of this procedure in a selected group of Parkinson's patients.

Introducción: la enfermedad de Parkinson puede justificar un procedimiento quirúrgico que consiste en la estimulación cerebral profunda. Se presentan resultados a mediano y largo plazo de una cohorte de 60 pacientes del Hospital de Especialidades del Centro Médico Nacional Siglo XXI. Métodos: los pacientes fueron operados con una metodología estereotáctica convencional a través del protocolo FrameLink (Medtronics Inc.). La técnica consistió en la evaluación preoperatoria de los pacientes, la colocación de marco estereotáctico, la realización de estudios de imagen, la planeación preoperatoria, el microrregistro, la macroestimulación y la colocación de implantes, que estuvo conformada por electrodos y generador en dos fases. La escala unificada para la evaluación de la enfermedad de Parkinson (UPDRS) preoperatoria, a tres, 12, y 36 meses fue utilizada como medida estándar. Se analizaron los resultados y las complicaciones como variables de interés. Resultados: se operaron 60 pacientes (41 hombres y 19 mujeres), con edad promedio de 56.5 años (rango de 39-70). Se obtuvieron de buenos a excelentes resultados en la mayoría de los pacientes con UPDRS promedio en periodo preoperatorio, a 3, 12 y 36 meses de 79.57, 66.85, 65.29 y 58.75, respectivamente (p < 0.0001). Las complicaciones se presentaron en forma mínima (en nueve pacientes: 15 %) y fueron manejadas de forma conservadora. Conclusiones: hubo una mejoría progresiva en el UPDRS durante los 36 meses de seguimiento.

Plasma cell tumor of the clivus: report of two cases.

BACKGROUND: Plasma cell tumor only rarely affects the cranium and may be found as an isolated lesion or as a part of multiple myeloma. In this review we present the clinical and radiological characteristics and analyze the evolution of two cases of this tumor located at the skull base, specifically in the clivus and sellar region. We also present a brief review of the literature. CLINICAL CASES: Case #1: The patient was a 66-year-old female with a solitary plasmacytoma of the bone (the isolated form of plasma cell tumor) that was totally removed. Case #2: The patient was a 61-year-old male with the diffuse form of this disease who was submitted to subtotal removal. In both patients, adjuvant treatment based on radiotherapy and chemotherapy was proposed; however, only one patient (Case #2) accepted adjuvant treatment and had a very favorable result. Most clinical symptoms disappeared and the patient is currently alive and with a very good quality of life (>3-year follow-up). The other patient (Case #1), despite the presence of the localized form of the disease, died 3 months after diagnosis. CONCLUSION: Early diagnosis and removal of as much of the tumor as possible, but mainly the opportune indication of adjuvant treatment with radiotherapy and chemotherapy, are the keys to management of these cases.

Diaphragma sellae: a surgical reference for transsphenoidal resection of pituitary macroadenomas.

OBJECTIVE: To classify patterns of descent of the diaphragma sellae (DS) to the sella turcica after transsphenoidal resection of pituitary macroadenomas and to determine whether there is any correlation between type of descent and volume or growth pattern of the tumor, as well as the presence of any postoperative hormone alteration, cerebrospinal fluid leak, and/or residual tumor. METHODS: One hundred patients with pituitary macroadenomas in which microsurgical transsphenoidal approach was indicated were prospectively included. We classified patterns of descent of the DS into four types: type A: symmetrical descent with a central fold corresponding to the pituitary stalk; type B: asymmetrical with a lateralized fold; type C: symmetrical and uniform descent without any fold; and type D: minimal or no descent in absence of visible residual tumor. A correlation was made between these types of descent and clinical and radiological findings. RESULTS: The largest tumors were types A and B; endocrine deficit was more frequent in types A and C, whereas the possibility of residual tumor was more elevated in types B and D. No statistically significant differences were found regarding tumor morphology and cerebrospinal fluid leakage. CONCLUSIONS: Our results suggest that pattern of descent of the DS may serve as a reference to determine the risk of leaving residual tumor as well as the possibility of developing postoperative endocrine deficit. It is apparent that tumor volume, more than morphology, is the main factor determining type of descent of the DS.

[Sellar remodeling: a surgical option for primary empty sella syndrome]. / Remodelación selar: una opción quirúrgica para el síndrome de la silla turca vacía primaria.

BACKGROUND: The empty sella is an entity that only rarely presents signs and symptoms. When noted, visual field deficits are an indication for surgical management. MATERIAL AND METHODS: We studied twenty patients with primary empty sella and visual field deficits surgically treated with a technique termed by us as "sellar remodeling." We treated 19 females and 1 male. Aside from visual deficits, all participants reported headache. We reported an increase in prolactin serum level in three cases. Patients with an increase in cerebrospinal fluid pressure were excluded. The surgical procedure involved placing through a transsphenoidal route an autologus graft formed by fat, aponeurosis and two bone lamina, with precise dimensions according to each patient's sella turcica. RESULTS: After surgery, visual deficits improved in 18 patients and headache in 17. Two patients displayed normal prolactin levels. No serious complications were reported during surgery. CONCLUSIONS: Sellar remodeling is a precise, simple, safe and inexpensive technique that significantly improves symptoms such as visual deficits and headache observed in primary empty sella syndrome.

Remodelación selar: una opción quirúrgica para el síndrome de la silla turca vacía primaria / Sellar remodeling: a surgical option for primary empty sella syndrome

Antecedentes: La silla turca vacía es una entidad que sólo en raras ocasiones presenta signos y síntomas, dentro de los cuales la afección en el campo visual es una indicación para el manejo quirúrgico. Materiales y Metodos: Se analizaron 20 pacientes con silla turca vacía primaria y alteraciones en los campos visuales, que fueron manejados quirúrgicamente con una técnica que denominamos remodelación selar. Fueron 19 mujeres y un hombre; todos ellos, además del déficit visual presentaban cefalea y tres casos elevación en el nivel sérico de prolactina. Se excluyeron los que mostraron aumento en la presión del líquido cefalorraquídeo. El procedimiento quirúrgico consistió en la colocación, por vía transesfenoidal, de un injerto autólogo formado por grasa, aponeurosis y dos láminas de hueso, con dimensiones precisas de acuerdo al tamaño de la silla turca del paciente. Resultados: Con la cirugía se logró mejorar el déficit visual en 18 pacientes y la cefalea en 17; finalmente, dos de ellos normalizaron su nivel de prolactina. No se presentaron complicaciones serias. Conclusiones: La remodelación selar es una técnica precisa, sencilla, segura y barata que permite mejorar los síntomas del síndrome de la silla turca vacía primaria, en especial las alteraciones visuales y la cefalea.

BACKGROUND: The empty sella is an entity that only rarely presents signs and symptoms. When noted, visual field deficits are an indication for surgical management. MATERIAL AND METHODS: We studied twenty patients with primary empty sella and visual field deficits surgically treated with a technique termed by us as [quot ]sellar remodeling.[quot ] We treated 19 females and 1 male. Aside from visual deficits, all participants reported headache. We reported an increase in prolactin serum level in three cases. Patients with an increase in cerebrospinal fluid pressure were excluded. The surgical procedure involved placing through a transsphenoidal route an autologus graft formed by fat, aponeurosis and two bone lamina, with precise dimensions according to each patient's sella turcica. RESULTS: After surgery, visual deficits improved in 18 patients and headache in 17. Two patients displayed normal prolactin levels. No serious complications were reported during surgery. CONCLUSIONS: Sellar remodeling is a precise, simple, safe and inexpensive technique that significantly improves symptoms such as visual deficits and headache observed in primary empty sella syndrome.

[Cerebellar amygdalopexy in the management of Chiari I malformation]. / Amigdalopexia cerebelosa en el manejo de la malformación de chiari de tipo I.

BACKGROUND: The treatment of Chiari I malformation associated with syringomyelia is controversial. OBJECTIVE: We describe a series of patients with this disease treated during a twelve-year period. We also present clinical, surgical and radiological findings. MATERIAL AND METHODS: Forty eight patients were included; a non significant female predominance was found. Clinical course was characterized by headache, cerebellar signs and symptoms, neck pain and involvement of lower cranial nerves and pyramidal tract. The degree of tonsillar descent and syringomyelia size varied and a correlation between them was not found. Surgery consisted in an occipital craniectomy, C1 laminectomy and tonsillar elevation through bipolar coagulation and duraplasty. RESULTS: The best results were observed in pain and cerebellar symptoms, while a deficit of lower cranial nerves and pyramidal tract were observed. However, in most patients we were able to slow symptom progression. No adverse effects were documented. CONCLUSIONS: The surgicalprocedureproposed herein is an effective and safe treatment alternative for this malformation, and its results are comparable to other riskier procedures.

Amigdalopexia cerebelosa en el manejo de la malformación de chiari de tipo I / Cerebellar amygdalopexy in the management of Chiari I malformation

Antecedentes. El tratamiento de la malformación de Chiari de tipo I asociada a siringomielia es controvertido. En este trabajo se presenta un análisis clínico, quirúrgico y radiológico de los pacientes con esta afección que fueron manejados durante un periodo de doce años. Material y métodos. Se incluyeron 48 pacientes, donde se encontró un discreto predominio en el sexo femenino. El cuadro clínico estuvo dominado por cefalea, dolor cervical, signos y síntomas cerebelosos, afección de nervios craneales bajos y lesión de la vía piramidal. Resultados. Tanto el grado de descenso amigdalino como el tamaño de la siringomielia fueron muy variables, sin encontrar correlación entre ambos. La cirugía consistió en una craniectomía occipital, laminectomía de C1, ascenso de amígdalas cerebelosas mediante coagulación bipolar y plastía de duramadre. Los mejores resultados clínicos se obtuvieron en el dolor y los síntomas cerebelosos, mientras que los peores fueron en la afección de los nervios craneales bajos y de la vía piramidal, sin embargo, en la gran mayoría de los pacientes se logró detener la progresión de los síntomas. No se presentaron complicaciones serias en el presente estudio. Conclusión. El procedimiento propuesto ofrece una alternativa segura, efectiva y comparable con otros métodos más riesgosos para el manejo de esta malformación.

BACKGROUND: The treatment of Chiari I malformation associated with syringomyelia is controversial. OBJECTIVE: We describe a series of patients with this disease treated during a twelve-year period. We also present clinical, surgical and radiological findings. MATERIAL AND METHODS: Forty eight patients were included; a non significant female predominance was found. Clinical course was characterized by headache, cerebellar signs and symptoms, neck pain and involvement of lower cranial nerves and pyramidal tract. The degree of tonsillar descent and syringomyelia size varied and a correlation between them was not found. Surgery consisted in an occipital craniectomy, C1 laminectomy and tonsillar elevation through bipolar coagulation and duraplasty. RESULTS: The best results were observed in pain and cerebellar symptoms, while a deficit of lower cranial nerves and pyramidal tract were observed. However, in most patients we were able to slow symptom progression. No adverse effects were documented. CONCLUSIONS: The surgicalprocedureproposed herein is an effective and safe treatment alternative for this malformation, and its results are comparable to other riskier procedures.

[Neurocysticercosis associated with pituitary adenoma. Case report and literature review]. / Cisticercosis cerebral asociada con adenoma pituitario. Reporte de un caso y revisión de la literatura.

BACKGROUND: Given the high prevalence in Mexico, neurocysticercosis is frequently associated with all varieties of brain lesions. However, the clinical association between pituitary adenoma and suprasellar cysts is unknown. There are no case reports elsewhere in the literature. CLINICAL CASE: A 42-year-old female presented to the hospital complaining of increased headache and right-sided temporal hemianopsia. Imaging studies including CT and MRI revealed a moderate size pituitary adenoma along with multiple cysts located in the subarachnoid cisterns in the Sylvian fissure and around the suprasellar cistern. The patient subsequently underwent surgical exploration by means of a right frontotemporal craniotomy with pituitary adenoma resection and cyst removal. The histopathological exam was consistent with non-functioning pituitary adenoma and racemosus cysticercosis. CONCLUSIONS: There are no reports of a clinical association between pituitary adenoma and racemosus cysticercosis. A high suspicion index is indicated in order to avoid misdiagnoses.

[Primary central nervous system lymphoma in immunocompetent patients]. / Linfoma primario del sistema nervioso central en pacientes inmunocompetentes.

Primary central nervous system lymphoma has been traditionally described in patients with immunodeficiency syndromes; however, there is an increasing number of immunocompetent patients with this type of tumor that have been reported recently. In this paper we have retrospectively analyzed 22 immunocompetent patients with a confirmed diagnosis of primary lymphoma of the brain. The mean age in this group was 65 years with a similar male/female ratio. The time of evolution of the clinical course was 80.4 days and it was mainly characterized by headache and focal neurological deficit. In four patients multiple lesions were observed, while the remaining presented single lesions mainly located in the periventricular area of the cerebral hemispheres. All patients were initially administered steroids and a stereotactic biopsy was performed. The majority of tumors were histologically classified as diffuse large cells and all of them showed a positive reaction to B-cells antigens on immunohistochemistry. All patients were treated with radiotherapy and in 10 of them, chemotherapy with methotrexate was also indicated. The mean survival rate was II months among patients treated with radiotherapy alone and increased to 36 months when chemotherapy was added.

Linfoma primario del sistema nervioso central en pacientes inmunocompetentes / Primary central nervous system lymphoma in immunocompetent patients

El linfoma primario del sistema nervioso central ha sido informado con frecuencia en pacientes que padecen síndromes de inmuno deficiencia. Sin embargo ésta no es una condición necesaria para su presentación, dado que existen informes de la enfermedad en sujetos inmunológicamente competentes. En el presente trabajo se analizaron en forma retrospectiva, los expedientes de 22 pacientes inmunocompetentes con diagnóstico confirmado de linfoma primario encefálico, se revisó la literatura mundial, con el fin de analizar objetivamente las manifestaciones clínicas, comportamiento radiológico, aspecto histopatológico, dificul tades diagnósticas y terapéuticas, así como las consideraciones pro nósticas. El promedio de edad fue de 65 años y con una relación equitativa hombre/mujer. El tiempo de evolución del cuadro clínico fue de 80.4 días y estuvo dominado por cefalea y déficit neurológico focal. En cuatro pacientes se encontraron lesiones múltiples, mientras que en el resto se trataba de lesiones únicas con localización predominante en la región periventricular de los hemisferios cerebrales. Todos los pacientes fueron manejados inicialmente con esteroides y sometidos a toma de biopsia por estereotaxia. La variedad histológica más frecuente fue la de células grandes difusas y la totalidad de los casos reaccionaron positivamente a antígenos de células B en la inmunohistoquímica. Los 22 pacientes fueron tratados con radio terapia y 10 de ellos además con quimioterapia con metotrexato. La supervivencia promedio fue de 11 meses en los pacientes radiados y de 36 meses en los que se agregó quimioterapia.

Primary central nervous system lymphoma has been traditionally described in patients with immunodeficiency syndromes; however, there is an increasing number of immunocompetent patients with this type of tumor that have been reported recently. In this paper we have retrospectively analyzed 22 immunocompetent patients with a confirmed diagnosis of primary lymphoma of the brain. The mean age in this group was 65 years with a similar male/female ratio. The time of evolution of the clinical course was 80.4 days and it was mainly characterized by headache and focal neurological deficit. In four patients multiple lesions were observed, while the remaining presented single lesions mainly located in the periventricular area of the cerebral hemispheres. All patients were initially administered steroids and a stereotactic biopsy was performed. The majority of tumors were histologically classified as diffuse large cells and all of them showed a positive reaction to B cells antigens on immunohistochemistry. All patients were treated with radiotherapy and in 10 of them, chemotherapy with methotrexate was also indicated. The mean survival rate was 11 months among patients treated with radiotherapy alone and increased to 36 months when chemotherapy was added.

[Soft palate transnasal retractor]. / Retractor transnasal del paladar blando.

INTRODUCTION: An anterior transoral approach allows the surgeon to access the lower portion of the clivus; to the first, second or third thoracic vertebra. It is indispensible that the teeth, lips, cheeks and tongue be retracted to avoid interposition on the surgical field. This retraction is carried out by means of a Dingman separator. Nevertheless, generally the soft palate is elevated with sutures through the nasal fossa. Because this invasive method could damage soft tissues, we have designed a basket to separate the soft palate from the surgical field, thus allowing the surgeon to maneuver with comfort and without damaging structures. TECHNIQUE: A 30-cm segment of 22 gauge wire is passed through each end of a thin sheet of silicon (4 x 3 x 1.5 cm) forming a basket. With the patient under general anesthesia, both free ends of the wire are introduced into the buccal cavity in the same manner nasal packing is used to control epistaxis. Each end of the wire is slid from the mouth through the nasal fossa to bring them outside the face. Traction is placed on both ends of the wire allowing the intraoral band of silicon to elevate and hold the soft palate. CLINICAL CASE: We present the case of a 64-year-old woman with spastic quadriparesis and hyperreflexia predominantly of the upper extremities, in addition to trophic changes due to arthritis of long duration. Imaging studies demonstrated nonreducible subluxation of the atlantoaxial joint with ventral compression of the bulbomedullar union by the odontoid apophysis. Resection of the anterior arch of the atlas and apophyses was made via the transoral route, where retraction of the soft palate by means of the described basket was employed. The patient did not experience significant sequelae as a result of the technique utilized.

[Hypophyseal macroadenomas. A neurosurgical challenge]. / Macroadenomas de hipófisis. Un reto neuroquirúrgico.

Management of pituitary adenoma is a real challenge because of neurovascular structures that surround the sellar region. In this paper we included 336 patients with pituitary tumors greater than 10 mm in diameter to make an analysis of surgical results. In this group, a discrete female predominance was found, with an average of 43.2 years of age and 21.5 mm in diameter of lesions. The clinical course was dominated by headache and visual deficit and only one quarter of patients had functioning tumors. The majority of the tumors were operated on by a transsphenoidal approach; in 72% of cases, the lesion was completely removed. Visual recovery was observed in 68% of patients, better in those who had a shorter clinical course; on the other hand, only 34 of 84 patients with functioning tumors could be cured. Complications were specially related to growing pattern of adenoma and its consistency. Even though higher morbidity and mortality was found in patients operated on by craniotomy, they also had the most complex lesions. Based on our experience, we are proposing here some recommendations to chose correctly the best surgical option and to avoid complications. Finally, we designed a diagram showing the critical route for optimal treatment of these lesions.

Separador intermaxilar en el acceso a la base craneal / Intermaxillar retractor in the skull base

Durante el acceso transmaxilar a la base craneal, es necesario estabilizar y mantener en una posición inferior el segmento distal del maxilar que ha sido osteotomizado por técnica Le Fort I.Con este fin, hace tiempo propusimos el uso de un abrebocas de goma que se insertaba entre los bordes óseos remanentes.Ahora usamos un instrumento similar, obtenido de un bloque de silastic (Dow Corning, Inc Midland MI) en el cual se perfeccionó su tamaño y retención.

Uso de abrebocas de goma en el acceso transmaxilar a la base del cráneo / Use of rubber mouth props for transmaxillar approach to skull base

El abordaje transmaxilar es muy útil en pacientes con tumores extradurales el tercio medio e inferior del clivus. Uno de los puntos más críticos en este procedimiento es mantener separados los maxilares osteotomizados; en una cirugía habitual de este tipo, se utiliza un separador de Dingman modificado (Codman & Shurtleff, Inc. Randolph M.A.). En lugar de este instrumento, hemos empleado un abrebocas de goma (Markel, mounth prop. adult/child) que es más barato, fácil de obtener, usar y permite al cirujano un amplio espacio para trabajar.

Cirugía de la base del cráneo / Surgical of the skull base

La cirugía de la base del cráneo ha tenido un gran avance en los últimos años debido principalmente a un mejor entendimiento de la anatomía, el desarrollo de nuevas tecnologías en estudios radiológicos y la participación multidisciplinaria de varios expertos en dicho campo; en cada uno de los abordajes, el principal objetivo es lograr la máxima exposición con un mínimo de retracción cerebral. En esta revisión se presentan las diferentes opciones que actualmente existen en el tratamiento de las lesiones en esta área. Se discuten las ventajas y desventajas de cada una de ellas y se incluyen algunos ejemplos clínicos ilustrativos. Para este análisis se dividió la base del cráneo en piso anterior, seno cavernoso, clivus, región petroclival, agujero mango, unión cráneo-vertebral, y foramen yugular