Syndrome of the third frontal convolution: Léon Ectors´ legacy on paradoxical ipsilateral hemiparesis.

Since the crossed control of sensitive-motor body functions by the contralateral cerebral hemispheres was recognized in the early 18th century, clinicians have been baffled by patients developing a motor deficit involving the extremities on the same side as an intracranial lesion. In the first third of the 20th century, three main hypotheses were proposed to explain this so-called ipsilateral or paradoxical hemiparesis: (1) the absence of decussation of the corticospinal tracts; (2) diaschisis, or blocking of the normal input to a brain region anatomically distant from the injured site; and (3) compression of the contralateral cerebral peduncle against the tentorial border, also known as the Kernohan-Woltman notch phenomenon. Here, we deal with the less widely known contributions of the Belgian neurosurgeon Léon Ectors, who included this paradoxical deficit within a neurological syndrome he considered highly specific for an early diagnosis of those meningiomas growing over the third frontal convolution. The present manuscript includes a systematic review of the cases of ipsilateral hemiparesis secondary to intracranial masses reported in ancient and modern scientific medical literature. We also address in-depth the physiopathological theories accounting for this syndrome and contrast them with Léon Ectors' observations.

Giant fronto-spheno-orbitary juvenile psammomatoid ossifying fibroma: Case report and literature review.

Juvenile psammomatoid ossifying fibroma (JPOF) is an osteofibrous neoplasm that originates in the craniofacial skeleton typically during the first three decades of life. JPOFs usually involve the orbit, paranasal sinuses or the jaws. Extensive involvement of the anterior cranial base with compromised visual function is a rare phenomenon. In such clinical context, a definite diagnosis can only be made on the basis of histopathological findings, given the absence of pathognomonic radiological features. Despite being considered a benign entity, JPOFs present a locally aggressive behavior. Therefore, these neoplasms must be included in the differential diagnosis in every patient harboring a skull base osteofibrous lesion, and, once diagnosed, gross total surgical removal should be attempted. In this study, we present our experience in the diagnosis and treatment of a patient diagnosed with a giant JPOF involving the cranial base.

Stroke secondary to leptomeningeal carcinomatosis with radiologic signs of arterial invasion.

Background: Compared to the general population, cancer patients are more likely to suffer from cerebral ischemia, either caused by the tumor itself or by the treatments applied. Case Description: We hereby present the clinical case of a patient treated for lung adenocarcinoma, who, years later, developed a case of the right frontal-temporal-insular ischemia secondary to leptomeningeal spread of the primary neoplasm, with an invasion of the walls of the right-middle cerebral artery and its branches. Conclusion: This should be considered an extremely rare form of recurrence of a primary solid tumor with clinical and radiological features that can mimic those of vascular inflammatory entities.

Diagnosis and Therapeutic Management of Ventricular Gangliogliomas: An Illustrated Review.

BACKGROUND: Gangliogliomas (GGs) are extremely rare benign neoplasms frequently located within the temporal lobe that usually present with seizures. GGs growing predominantly within the ventricular system (VGGs) are even more infrequent, so definite conclusions concerning their diagnosis and therapeutic management are lacking. METHODS: A retrospective review of case reports of VGGs was performed from the introduction of modern imaging techniques, including 4 new illustrative cases treated in our department. RESULTS: Thirty-four cases were collected. Ages ranged from 10 to 71 years (mean, 26.62 years), and 55.9% were male. Most patients developed symptoms related to high intracranial pressure. The lateral ventricles were predominantly involved (58.8%). Obstructive hydrocephalus was observed in 54.5% of patients. Cystic degeneration and calcification were frequently observed. Surgical treatment was carried out in all cases. Morbidity and mortality were 17.6% and 2.9%, respectively. Gross total tumor resection was achieved in 64.5% of patients. Four patients experienced tumor dissemination along the neural axis. More than 90% of patients maintained a good functional status at last follow-up. CONCLUSIONS: Despite their low incidence, a diagnosis of VGGs should be considered in young male adults who progressively develop intracranial hypertension, caused by a ventricular mass showing signs of cystic degeneration and calcification. Maximal and safe surgical resection represents the gold standard for the treatment of symptomatic VGGs, although total removal is frequently precluded by difficulties in defining appropriate tumor boundaries. Adjuvant radiotherapy should be considered if an incomplete resection was carried out, especially in World Health Organization grade III neoplasms.

Sarcoma granulocítico del sistema nervioso central: reporte de 2 casos y revisión de la literatura / Central nervous system granulocytic sarcoma: Report of 2 cases and review of the literatura

Los sarcomas granulocíticos son neoplasias sólidas compuestas de células mieloides inmaduras, de localización extramedular, asociadas a síndromes mieloproliferativos. La afectación del sistema nervioso central es muy infrecuente y puede desarrollarse tras un periodo de remisión completa, coexistir con o preceder a la enfermedad sistémica, siendo esta el elemento fundamental que orienta el diagnóstico radiológico y marca el pronóstico. En este trabajo, presentamos 2 casos de sarcoma granulocítico intracraneal, verificados desde el punto de vista patológico tras ser tratados mediante cirugía, y discutimos sus características clínicas, diagnósticas, terapéuticas y pronósticas tras realizar una revisión bibliográfica de la literatura científica destacada publicada hasta la fecha

Granulocytic sarcomas are solid, extramedullary-located neoplasms composed of immature myeloid cells, associated with myeloproliferative syndromes. Central nervous system involvement is very rare and may develop either after complete remission, coexist with or precede the systemic disease, being the last one that guides the radiological diagnosis and marks the prognosis. In this work, we report 2 pathologically-verified cases of intracranial granulocytic sarcoma treated by surgical means. Their clinical, diagnostic, therapeutic and prognostic features are discussed in the light of the most relevant scientific literature published to date

Central nervous system granulocytic sarcoma: Report of 2 cases and review of the literature. / Sarcoma granulocítico del sistema nervioso central: reporte de 2 casos y revisión de la literatura.

Granulocytic sarcomas are solid, extramedullary-located neoplasms composed of immature myeloid cells, associated with myeloproliferative syndromes. Central nervous system involvement is very rare and may develop either after complete remission, coexist with or precede the systemic disease, being the last one that guides the radiological diagnosis and marks the prognosis. In this work, we report 2 pathologically-verified cases of intracranial granulocytic sarcoma treated by surgical means. Their clinical, diagnostic, therapeutic and prognostic features are discussed in the light of the most relevant scientific literature published to date.

Ipsilateral hemiparesis: the forgotten history of this paradoxical neurological sign.

OBJECTIVE: Establishing the neurological localization doctrine for the contralateral hemispheric control of motor functions in the second half of the 19th century, researchers faced the challenge of recognizing false localizing signs, in particular paradoxical or ipsilateral hemiparesis (IH). Despite tremendous progress in current methods of neuroradiological and electrophysiological exploration, a complete understanding of this phenomenon has yet to be attained. METHODS: The authors researched the well-described cases of hemiparesis/hemiplegia ipsilateral to an intracranial lesion published in the scientific literature in the pre-MRI era (before 1980). A comprehensive review of the physiopathological mechanisms proposed for paradoxical hemiparesis throughout this period, as well as the pathological evidence substantiating them, is provided. RESULTS: A collection of 75 patients with hemiparesis/hemiplegia ipsilateral to the primary intracranial lesion reported between 1858 and 1979 were eligible for analysis. Most cases occurred in adults with supratentorial, slowly developing, extraparenchymatous mass lesions, such as neoplasms (38%) or chronic subdural hematomas (36%). Physiopathological theories proposed by the neurologists who investigated IH can be grouped into 4 major concepts: 1) lack of anatomical decussation of the corticospinal tract; 2) impaired functional activation of the contralateral hemisphere by the lesioned dominant hemisphere through the callosal connections; 3) Kernohan's notch phenomenon, or mechanical injury of the contralateral cerebral peduncle against the free edge of the tentorium; and 4) cerebrovascular dysfunction involving the contralateral hemisphere owing to kinking and mechanical flattening of the carotid artery contralateral to the primary intracranial lesion. CONCLUSIONS: IH represents a still underdiagnosed paradoxical neurological phenomenon. With the aid of modern neuroradiological and neurophysiological methods, Kernohan's peduncle notch mechanism has been confirmed to cause IH in many of the cases reported in recent decades. Nevertheless, alternative functional and/or vascular mechanisms must be investigated further for unexplained IH cases, in particular for transitory IH without evidence of peduncle injury. The historical theories reviewed in this paper represent a conceptual framework that may be helpful for this purpose.

Meningiomas quísticos: correlación radiológica y patológica con implicaciones quirúrgicas / Cystic meningiomas: Radiological and pathological correlation with surgical implications

Introducción: Los meningiomas que asocian cambios quísticos prominentes (MQ) han representado un reto diagnóstico y terapéutico desde los orígenes de la neurocirugía moderna. Presentamos la experiencia en el manejo clínico y quirúrgico de esta entidad en nuestro Servicio. Material y métodos: En este estudio descriptivo se incluyen los MQ intervenidos en nuestro Servicio en los últimos 15 años, evaluando las variables clínicas, diagnósticas y terapéuticas de este subgrupo de meningiomas, con especial énfasis en la correlación de los hallazgos radiológicos y patológicos. Los tumores se clasificaron siguiendo el esquema propuesto por Nauta y sus colaboradores. Resultados: Se identificaron 11 casos, lo que supone un 1,8% de los meningiomas intervenidos en ese periodo. Todos los pacientes eran adultos y 8 de ellos, mujeres. Entre los síntomas, destacó la alta frecuencia de crisis epilépticas, mientras que solo un caso desarrolló hipertensión intracraneal aguda. La morfología predominante fue el tipo 2, seguida de los tipos 1, 3 y 4. En 5 casos pudo establecerse un diagnóstico radiológico inequívoco de meningioma. Todos los pacientes fueron tratados mediante cirugía, sin evidenciarse casos de recurrencia durante un seguimiento medio de 52,8 meses. Aunque la mayoría de las neoplasias correspondieron al grado i de la OMS, incluyendo subtipos variados, 4 de ellas fueron diagnosticadas como meningiomas atípicos. Conclusiones: De forma excepcional, los meningiomas pueden desarrollar cambios quísticos, intra- o extratumorales, en número y tamaño variable. Estos constituyen hallazgos poco típicos que pueden dificultar seriamente su diagnóstico diferencial radiológico. Los meningiomas con quistes periféricos de cápsula fina (tipos 2 y 3) suponen el escenario quirúrgico más complejo, pues el realce capsular en las pruebas de imagen no traduce necesariamente infiltración tumoral, mientras que el quiste puede contener células neoplásicas. Por lo tanto, recomendamos realizar al menos biopsia de la cápsula o seudocápsula, y lavado minucioso de la cavidad quirúrgica especialmente en estos subtipos

Introduction: Meningiomas associating prominent cystic changes (CM) have challenged neurosurgeons since the beginning of this surgical discipline. We present the experience in the diagnostic and therapeutic management of this entity in our institution. Material and methods: A review of our patient database was carried out, searching for those CM that were operated on in the last 15 years. Relevant clinical data were recorded and analyzed for each case, with special emphasis in the correlation of radiological and pathological findings. Cystic changes were classified according to the scheme proposed by Nauta et al. Results: A total of 11 patients were gathered, which represents 1.8% of the meningiomas operated on in our department during the period studied. All were adults, predominantly female patients (8 cases). Among the clinical symptoms a high rate of epileptic seizures was observed while only one patient developed acute intracranial hypertension. Morphologically, most tumors corresponded to type 2 CMs, followed by types 1, 3 and 4. In only five patients an unequivocal radiological diagnosis of meningioma could be made. All neoplasms were surgically removed and there were no records of recurrence (mean follow-up: 52.8 months). Microscopic findings were consistent with the pathological diagnosis of atypical meningioma in 4 cases, while the remaining tumors corresponded to OMS grade I neoplasms with variable microscopic patterns. Conclusions: Meningiomas can exceptionally associate cystic changes, both intra and/or extratumoral, in variable number and size. When cystic changes become too prominent (a large number or big-sized cysts), a serious preoperative diagnostic dilemma may arise. The surgical management of those CMs displaying a peripheral, thin-walled cyst (types 2 and 3) is especially complex, as contrast enhancement of the tumor wall did not correlate strictly with neoplastic invasion; even in the absence of this feature free floating islands of meningothelial cells intermixed with cyst fluid can be found. Consequently both surgical biopsy of every suspicious tissue and copious irrigation of the surgical cavity are strongly recommended for these CM types

Cystic meningiomas: Radiological and pathological correlation with surgical implications. / Meningiomas quísticos: correlación radiológica y patológica con implicaciones quirúrgicas.

INTRODUCTION: Meningiomas associating prominent cystic changes (CM) have challenged neurosurgeons since the beginning of this surgical discipline. We present the experience in the diagnostic and therapeutic management of this entity in our institution. MATERIAL AND METHODS: A review of our patient database was carried out, searching for those CM that were operated on in the last 15 years. Relevant clinical data were recorded and analyzed for each case, with special emphasis in the correlation of radiological and pathological findings. Cystic changes were classified according to the scheme proposed by Nauta et al. RESULTS: A total of 11 patients were gathered, which represents 1.8% of the meningiomas operated on in our department during the period studied. All were adults, predominantly female patients (8 cases). Among the clinical symptoms a high rate of epileptic seizures was observed while only one patient developed acute intracranial hypertension. Morphologically, most tumors corresponded to type 2 CMs, followed by types 1, 3 and 4. In only five patients an unequivocal radiological diagnosis of meningioma could be made. All neoplasms were surgically removed and there were no records of recurrence (mean follow-up: 52.8 months). Microscopic findings were consistent with the pathological diagnosis of atypical meningioma in 4 cases, while the remaining tumors corresponded to OMS grade I neoplasms with variable microscopic patterns. CONCLUSIONS: Meningiomas can exceptionally associate cystic changes, both intra and/or extratumoral, in variable number and size. When cystic changes become too prominent (a large number or big-sized cysts), a serious preoperative diagnostic dilemma may arise. The surgical management of those CMs displaying a peripheral, thin-walled cyst (types 2 and 3) is especially complex, as contrast enhancement of the tumor wall did not correlate strictly with neoplastic invasion; even in the absence of this feature free floating islands of meningothelial cells intermixed with cyst fluid can be found. Consequently both surgical biopsy of every suspicious tissue and copious irrigation of the surgical cavity are strongly recommended for these CM types.

Radiologic and Histopathologic Features in an Intracranial Localized Castleman Disease: A Case Report and Review of Literature.

INTRODUCTION: Castleman disease (CD) is a rare pathologic process of unknown etiology, characterized by non-neoplastic lymph node enlargement. Two distinct histologic patterns are recognized; the hyaline-vascular type and the less common plasma cells type. Another intermediate type has been described. The clinical features are classified into 2 categories, localized (unicentric) and generalized (multicentric), the later associated with systemic manifestations and poor prognosis. CD affecting the central nervous system is extremely rare. We report a new case of localized intracranial CD and we accomplish a review of the literature. CASE REPORT: A 30-year-old man presented with a generalized tonic-clonic seizure. Computerized tomography and magnetic resonance imaging showed a small mass in the right temporoparietal convexity with homogenous enhancement after contrast administration. Extensive vasogenic edema in comparison with the size of the mass was also identified and based on the neuroradiologic finding, a suspected diagnosis of meningoangiomatosis was formulated. The mass was completely resected and his histologic examination identified the hyaline-vascular type of CD. One year after surgery, the patient remains seizure free, without evidence of systemic involvement or recurrence of the mass. CONCLUSIONS: Our case and review of the literature show the value of the extensive brain edema on neuroimaging finding to the differential diagnosis for a solitary mass arising from the meninges. We emphasize on the need for histologic examination when the diagnosis of meningioma is not entirely clear.

Síndrome de cauda equina secundario a neurolinfomatosis: presentación de un caso y revisión de la literatura / Cauda equina syndrome secondary to neurolymphomatosis: Case report and literature review

La neurolinfomatosis es una entidad rara caracterizada por la presencia de linfocitos neoplásicos que infiltran las estructuras del sistema nervioso periférico. Dada su naturaleza y localización puede desarrollar una gran variedad de síntomas, suponiendo un reto diagnóstico. El tratamiento se basa en el uso de metotrexato, aunque, a día de hoy, existen diversos esquemas de quimioterapia para los pacientes con enfermedad sistémica. Presentamos el caso de un varón con neurolinfomatosis a nivel de la cauda equina y agrupamos los casos descritos hasta el momento

Neurolymphomatosis is a rare disorder characterised by infiltration of neoplastic lymphocytes into the peripheral nervous system. A wide variety of symptoms can manifest depending on its nature and location, making its diagnosis a real challenge. Treatment is based on methotrexate, although various chemotherapy regimens are currently available for patients with systemic disease. We present the case of a male patient with neurolymphomatosis of the cauda equina, together with a review of all cases published to date

Cauda equina syndrome secondary to neurolymphomatosis: Case report and literature review. / Síndrome de cauda equina secundario a neurolinfomatosis: presentación de un caso y revisión de la literatura.

Neurolymphomatosis is a rare disorder characterised by infiltration of neoplastic lymphocytes into the peripheral nervous system. A wide variety of symptoms can manifest depending on its nature and location, making its diagnosis a real challenge. Treatment is based on methotrexate, although various chemotherapy regimens are currently available for patients with systemic disease. We present the case of a male patient with neurolymphomatosis of the cauda equina, together with a review of all cases published to date.

Pseudotumor inflamatorio gigante de la base del cráneo / Giant inflammatory pseudotumor of the cranial base

El pseudotumor inflamatorio (PTI) es una entidad no neoplásica, de etiología desconocida, caracterizada por la proliferación de un infiltrado inflamatorio policlonal sobre un estroma de tejido conjuntivo. La afectación del sistema nervioso es infrecuente y puede suponer un reto diagnóstico y terapéutico incluso para el clínico más experimentado. Presentamos el caso de una mujer de 56años diagnosticada de una masa gigante, infiltrante, centrada en el seno cavernoso izquierdo, que experimentó una rápida mejoría clínica y radiológica tras la instauración de tratamiento con esteroides. La biopsia de la lesión fue diagnóstica de PTI. Tras la retirada del tratamiento, se evidenció una progresión de un pequeño resto lesional orbitario. El inicio de tratamiento con ciclofosfamida indujo una remisión completa de la enfermedad, que ha permanecido estable durante 6años de seguimiento

The inflammatory pseudotumour (IPT) is a non-neoplastic entity of unknown origin, and is characterised by a proliferation of connective tissue and a polyclonal inflammatory infiltrate. Central nervous system involvement is uncommon, and usually represents a diagnostic and therapeutic challenge even for the experienced clinician. This reports deals with the case of a 56year-old woman diagnosed with a giant, infiltrating mass centred in the left cavernous sinus, who had a rapid clinical and radiological response to steroid therapy. Biopsy specimens were diagnostic for IPT. The progression of a small orbital residual lesion was detected after steroid withdrawal. Treatment with cyclophosphamide induced a complete response that remains stable after six years of follow-up