Exoftalmos y dolor ocular como presentación clínica de linfoma de Hodgkin con afectación intracraneal al diagnóstico / Exophthalmos and ocular pain as clinical debut of intracranial Hodgkin's lymphoma at diagnosis

La afectación intracraneal del linfoma de Hodgkin (LH) es extremadamente rara, especialmente como forma de presentación de la enfermedad. Muestra un patrón radiológico inespecífico, pudiendo ser confundido con otras entidades de mayor frecuencia y pronóstico radicalmente distinto. Anatomopatológicamente se caracteriza por la presencia de células grandes binucleadas (células de Reed-Sternberg) eIntracranial involvement in Hodgkin's lymphoma (HL) is extremely unusual, especially at the time of diagnosis. Because of its non-specific radiological behaviour, it can be confused with more common entities with a radically different prognosis. Pathologically, large and bi-nucleated cells, called Reed-Sternberg cells, embedded in an inflammatory network.

In this report we describe the clinical case of a patient, with no medical history, with left ocular pain and exophthalmos as presentation of intracranial HL at diagnosis and review the most current literature. Intracranial involvement is often associated with extracranial disease. Therefore, a systemic study including body computed tomography, bone marrow biopsy and ophthalmological evaluation is necessary. Intracranial lesions respond favourably to treatment and the prognosis depends on the extracranial involvement. To date, there is no standardised management scheme for these patients. For us, the primary role of surgery in this context is to perform a biopsy to confirm the histological diagnosis (AU)mbebidas en un entramado inflamatorio. Presentamos el caso de una paciente con dolor ocular y exoftalmos izquierdo como presentación clínica de afectación intracraneal por LH al diagnóstico de su enfermedad y revisamos la literatura más reciente al respecto. En pacientes con LH intracraneal es necesario realizar un estudio de extensión con tomografía computarizada corporal, biopsia de médula ósea y examen oftalmológico. Se asocia con gran frecuencia a enfermedad extracraneal, que marca el pronóstico. La lesión intracraneal presenta buena respuesta al tratamiento, que no sigue un esquema estandarizado. El papel de la cirugía es la realización de una biopsia para confirmar el diagnóstico (AU)

Exophthalmos and ocular pain as clinical debut of intracranial Hodgkin's lymphoma at diagnosis.

Intracranial involvement in Hodgkin's Lymphoma (HL) is extremely unusual, especially at the time of diagnosis. Because of its non-specific radiological behaviour, it can be confused with more common entities with a radically different prognosis. Pathologically, large and bi-nucleated cells, called Reed-Sternberg cells, embedded in an inflammatory network. In this report we describe the clinical case of a patient, with no medical history, with left ocular pain and exophthalmos as presetation of intracranial HL at diagnosis and review the most current literature. Intracranial involvement is often associated with extracranial disease. Therefore, a systemic study including body computed tomography, bone marrow biopsy and ophthalmological evaluation is necessary. Intracranial lesions respond favourably to treatment and the prognosis depends on the extracranial involvement. To date, there is no standardised management scheme for these patients. For us, the primary role of surgery in this context is to perform a biopsy to confirm the histological diagnosis.

Beyond uncal herniation: An updated diagnostic reappraisal of ipsilateral hemiparesis and the Kernohan-Woltman notch phenomenon.

PURPOSE: This works comprehensively analyses a modern cohort of patients with ipsilateral hemiparesis (IH) and discusses the pathophysiological theories elaborated to explain this paradoxical neurological sign according to the findings from contemporary neuroimaging and neurophysiological techniques. METHODS: A descriptive analysis of the epidemiological, clinical, neuroradiological, neurophysiological, and outcome data in a series of 102 case reports of IH published on since the introduction of CT/MRI diagnostic methods (years 1977-2021) was performed. RESULTS: IH mostly developed acutely (75.8%) after traumatic brain injury (50%), as a consequence of the encephalic distortions exerted by an intracranial haemorrhage eventually causing contralateral peduncle compression. Sixty-one patients developed a structural lesion involving the contralateral cerebral peduncle (SLCP) demonstrated by modern imaging tools. This SLCP showed certain variability in its morphology and topography, but it seems pathologically consistent with the lesion originally described in 1929 by Kernohan & Woltman. The study of motor evoked potentials was seldom employed for the diagnosis of IH. Most patients underwent surgical decompression, and a 69.1% experienced some improvement of the motor deficit. CONCLUSIONS: Modern diagnostic methods support that most cases in the present series developed IH following the KWNP model. The SLCP is presumably the consequence of either compression or contusion of the cerebral peduncle against the tentorial border, although focal arterial ischemia may also play a contributing role. Some improvement of the motor deficit should be expected even in the presence of a SLCP, provided the axons of the CST were not completely severed.

Kernohan-Woltman notch phenomenon: an exceptional neurological picture?

INTRODUCTION: Ipsilateral hemiparesis (IH) can be defined as a paradoxical dysfunction of the first motor neuron involving the extremities on the opposite side to that expected, given the location of the triggering intracranial pathology. Compression of the corticospinal tract (CSt) along its course through the contralateral cerebral peduncle against the free edge of the tentorium, known as the Kernohan-Woltman notch phenomenon (KWNP), represents the main cause of IH. METHODS: This retrospective study analyses a series of 12 patients diagnosed with IH secondary to KWNP treated at our institution, including a descriptive study of epidemiological, clinical, radiological, neurophysiological, and prognostic variables. RESULTS: In 75% of the cases, symptoms had an acute or subacute onset. Initial imaging studies showed signs of significant mass effect in half of the patients, whereas magnetic resonance imaging (MRI) identified a structural lesion in the contralateral cerebral peduncle in two thirds of them. Impairment of the motor evoked potentials (MEP) was verified in 4 patients. During follow-up 7 patients experienced improvement in motor activity, and near half of the cases were classified in the first three categories of the modified Rankin scale. CONCLUSIONS: In contrast to prior historical series, most of our patients developed a KWNP secondary to a traumatic mechanism. MRI represents the optimal method to identify both the classic cerebral peduncle notch and the underlying structural lesion of the CSt. The use of MEP can help to establish the diagnosis, especially in those cases lacking definite radiological findings.

Estimulación de nervio periférico: una alternativa terapéutica eficaz para el dolor refractario / Peripheral nerve stimulation: An effective treatment alternative for refractory pain

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Angiolipomas espinales epidurales: presentación de 7 casos y revisión de la literatura / Spinal extradural angiolipomas: 7 new cases and review of the literatura

Introducción: Presentamos nuestra experiencia en el manejo diagnóstico y terapéutico de los angiolipomas espinales. Métodos: Hemos realizado una revisión en nuestra base de datos quirúrgica correspondiente al diagnóstico patológico de angiolipoma, recogiendo las variables epidemiológicas, clínicas, diagnósticas, terapéuticas y de seguimiento de cada caso. Para la valoración funcional de los pacientes se utilizó la escala de mielopatía modificada de la Asociación Japonesa de Ortopedia (EAJO). Resultados: Obtuvimos un total de 7 casos, con edades comprendidas entre los 42 y 63 años; 4 eran mujeres. Todas las lesiones se localizaron en el espacio epidural torácico, con extensión extrarraquídea en 2 casos. La presentación más frecuente fue la combinación de dolor y mielopatía progresiva, con una puntuación entre 5 y 9 en la EAJO. Los hallazgos radiológicos fueron variables, claramente influenciados por la proporción de los componentes tumorales, identificándose un caso de variante celular, uno de predominio angiomatoso y otro mixoide. Cuatro estudios evidenciaban signos de afectación ósea. Tras el tratamiento quirúrgico se registraron puntuaciones óptimas en la EAJO, salvo en un caso (puntuación de 10 en la EAJO), sin evidenciarse recurrencias tras un periodo de seguimiento que varió entre uno y 5 años. Conclusiones: Los angiolipomas epidurales pueden dar lugar a manifestaciones clínicas y radiológicas muy variables. Su tratamiento quirúrgico debe diseñarse de acuerdo a la extensión de la tumoración, incluyendo en ocasiones un procedimiento reconstructivo espinal, y se traduce en resultados excelentes con independencia de la situación clínica previa. La posibilidad de recurrencia es baja, incluso en caso de resección incompleta

Introduction: We present our experience with the diagnosis and treatment of spinal angiolipomas (SAL). Methods: We used our database of surgical patients to gather epidemiological, clinical, diagnostic, treatment, and follow-up data from confirmed cases of SAL. Patients’ functional status was evaluated using the modified Japanese Orthopaedic Association (mJOA) score for thoracic myelopathy. Results: We included 7 patients with ages ranging between 42 and 63 years; 4 were women. Lesions in all patients were located in the thoracic epidural space; in 2 cases, lesions extended outside the spinal canal. The most frequent form of presentation was pain combined with progressive myelopathy; patients scored 5 to 9 points on the mJOA scale. Radiological findings varied; this variability clearly depended on the proportion of tumour components (cellular variant in one case, predominantly angiomatous in another, and myxoid in yet another case). Four cases displayed radiological signs of bone involvement. Surgery resulted in excellent mJOA scores except in one case (mJOA score of 10); no recurrences were detected during follow-up (periods ranging from 1 to 5 years). Conclusions: Extradural SAL can present with highly variable radiological and clinical features. Surgery must be tailored to each individual situation according to tumour extension; in some cases, it may include spinal reconstruction. Surgery results in excellent outcomes regardless of preoperative functional status and it is associated with low recurrence rates, even in case of incomplete resection

Clinical features, diagnosis and therapy of pituicytoma: an update.

BACKGROUND: Pituicytomas (PTs) are extremely rare, low-grade glial tumors closely related to the neurohypophyseal axis. Definite conclusions concerning the optimal diagnostic and therapeutic approach to these neoplasms are lacking to date, as most of this information has been presented as case reports. METHODS: Retrospective review of case reports published in the scientific literature to date, including a new illustrative example treated in our department. RESULTS: 116 cases were collected. PTs had a higher prevalence in the fifth and sixth decades of life, with a slight male predominance. Main symptoms, which tended to be progressive, included visual field defects and pituitary-hypothalamic dysfunction. Radiologically, PTs were found anywhere along the hypothalamic-pituitary axis mimicking other, more frequent tumors growing in this anatomical region. Surgical treatment included both transcranial or transsphenoidal approaches, and resulted in gross total resection and morbidity rates of 46.8 and 59%, respectively; the latter essentially consisted in anterior and posterior pituitary dysfunction, with limited impact on daily quality of life. CONCLUSIONS: Due to both low frequency and the absence of pathognomonic clinical and/or radiological features, formulating a suspicion diagnosis of PT represents a considerable challenge even for experienced professionals. The indication for treatment should be made on an individual basis, but it is inescapable in the presence of a visual field defect. The surgical approach has to be tailored according to the topography of the tumor and preoperative symptoms; the greatest challenges in accomplishing a gross total removal are represented by the degree of adherence and vascularization of the PT.

Spinal extradural angiolipomas: 7 new cases and review of the literature. / Angiolipomas espinales epidurales: presentación de 7 casos y revisión de la literatura.

INTRODUCTION: We present our experience with the diagnosis and treatment of spinal angiolipomas (SAL). METHODS: We used our database of surgical patients to gather epidemiological, clinical, diagnostic, treatment, and follow-up data from confirmed cases of SAL. Patients' functional status was evaluated using the modified Japanese Orthopaedic Association (mJOA) score for thoracic myelopathy. RESULTS: We included 7 patients with ages ranging between 42 and 63 years; 4 were women. Lesions in all patients were located in the thoracic epidural space; in 2 cases, lesions extended outside the spinal canal. The most frequent form of presentation was pain combined with progressive myelopathy; patients scored 5 to 9 points on the mJOA scale. Radiological findings varied; this variability clearly depended on the proportion of tumour components (cellular variant in one case, predominantly angiomatous in another, and myxoid in yet another case). Four cases displayed radiological signs of bone involvement. Surgery resulted in excellent mJOA scores except in one case (mJOA score of 10); no recurrences were detected during follow-up (periods ranging from 1 to 5 years). CONCLUSIONS: Extradural SAL can present with highly variable radiological and clinical features. Surgery must be tailored to each individual situation according to tumour extension; in some cases, it may include spinal reconstruction. Surgery results in excellent outcomes regardless of preoperative functional status and it is associated with low recurrence rates, even in case of incomplete resection.

Desmoplastic astrocytoma: new insights into its clinical profile, diagnosis, and treatment.

BACKGROUND: Desmoplastic astrocytoma (DA) is a rare intracranial tumor which usually affects pediatric patients. The aim of this study is to describe the clinical features and management of DA based on a joint analysis of the cases reported in the scientific literature. MATERIAL AND METHODS: A thorough review was carried out, gathering those pathologically proven DAs reported since the first description of this entity. Two new own cases were included in order to illustrate this review. Epidemiological, clinical, radiological, therapeutic, and follow-up data were analyzed with the software SPSS version 20. RESULTS: A total of 52 DAs were recorded. Most cases occurred in the first 2 years of life, although older patients were also reported. Patients mainly presented symptoms and signs of elevated intracranial pressure. According to their radiological features, we were able to classify DAs in four main groups, with distinct differential diagnosis and prognosis. After treatment, 14.2 % of patients presented persistent neurological impairment and the mortality rate was close to 10 %. CONCLUSION: DAs can be diagnosed at any age from birth to adolescence. These neoplasms can show up a wider range of radiological morphologies than previously thought. Surgery represents the treatment of choice for DAs, although chemotherapy can also be useful in the setting of recurrence or progression of the disease. Those DAs lacking classic radiological features, especially type 4 tumors, were linked with a poorer clinical outcome.

Fenómeno de Kernohan-Woltman / Kernohan-Woltman notch phenomenon

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[Cavernous haemangioma of the skull]. / Hemangioma cavernoso intraóseo craneal.

INTRODUCTION: Cavernous haemangiomas are benign tumours that rarely affect the skull. A correct suspicion diagnosis is seldom obtained when typical radiological signs are lacking. In this way a definite diagnosis is only obtained after a surgical procedure in most cases. CASE REPORT: A 52-year-old female presented a painless, slow-growing tumoration in her right forehead. Skull CT showed an osteolytic lesion located within the right frontal bone. On suspicion of a metastatic origin of the lesion, a systemic research for a primary tumour was performed without significative findings. Finally, en bloc resection of the lesion was performed followed by cranioplasty. Microscopically, the lesion proved to be a cavernous haemangioma of the frontal bone. CONCLUSION: Despite their low frequency, cavernous haemangiomas must be included in the differential diagnosis of slow-growing osteolytic lesions located within the skull. The elective treatment of this tumours includes a complete resection by craniectomy, with safe bony margins.

Hemangioma cavernoso intraóseo craneal / Cavernous haemangioma of the skull

Introducción. Los hemangiomas cavernosos sontumores benignos que excepcionalmente afectan loshuesos craneales. En ausencia de signos radiológicostípicos, frecuentemente son intervenidos bajo la sospechade otro tipo de neoplasia ósea, obteniéndose el diagnósticoúnicamente tras el procedimiento quirúrgico.Caso clínico. Presentamos el caso de una pacientede 52 años con una tumoración indolora del huesofrontal derecho, de lento crecimiento y característicasosteolíticas desde el punto de vista neurorradiológico.Para descartar un origen metastásico, se llevó a cabo unestudio oncológico sistémico, sin hallazgo de neoplasiaprimaria. Finalmente, la lesión fue extirpada en bloquemediante craniectomía, seguido de craneoplastia.El diagnóstico anatomo-patológico fue hemangiomacavernoso intraóseo.Conclusión. A pesar de su baja frecuencia, el diagnósticode hemangioma cavernoso intraóseo debe serconsiderado ante la presencia de una tumoración cranealde lento crecimiento y características osteolíticasen las pruebas de neuroimagen. El tratamiento deelección consiste en la resección completa de la lesiónmediante craniectomía, incluyendo márgenes de seguridadde hueso sano (AU)

Introduction. Cavernous haemangiomas are benigntumours that rarely affect the skull. A correct suspiciondiagnosis is seldom obtained when typical radiologicalsigns are lacking. In this way a definite diagnosis is onlyobtained after a surgical procedure in most cases.Case report. A 52-year-old female presented a painless,slow-growing tumoration in her right forehead.Skull CT showed an osteolytic lesion located within theright frontal bone. On suspicion of a metastatic originof the lesion, a sistemic research for a primary tumourwas performed without significative findings. Finally,en bloc resection of the lesion was performed followedby cranioplasty. Microscopically, the lesion proved to bea cavernous haemangioma of the frontal bone.Conclusion. Despite their low frequency, cavernoushaemangiomas must be included in the differentialdiagnosis of slow-growing osteolytic lesions locatedwithin the skull. The elective treatment of this tumoursincludes a complete resection by craniectomy, with safebony margins (AU)

Hemangioblastoma quístico de la unión bulbomedular asociado a enfermedad de von Hippel-Lindau. Presentación de dos casos y revisión de la bibliografía / Cystic hemangioblastoma of the junction of the medulla and upper spinal cord associated to von hippel-lindau disease. Two case reports and a review of the literature

Introducción. Los hemangioblastomas son tumores benignos muy vascularizados que pueden presentarse de forma esporádica o bien asociados al síndrome de von Hippel-Lindau en un 20% de los casos. Sólo un 5-20% de los hemangioblastomas se localizan en el tronco cerebral, y los de la unión bulbomedular son los diagnosticados con menor frecuencia. Casos clínicos. Dos pacientes jóvenes fueron diagnosticados de enfermedad de von Hippel-Lindau tras comenzar con un hemangioblastoma quístico de la unión bulbomedular como primera manifestación patológica. Ambos fueron intervenidos mediante craneotomía suboccipital, y se les extirpó completamente ambas lesiones sin presentar déficit neurológicos posquirúrgicos. Uno de los pacientes requirió una reintervención años después por una nueva recidiva de hemangioblastoma sintomático en la médula dorsal. El estudio genético llevado a cabo en ambos pacientes confirmó la presencia de una mutación en el gen VHL, responsable del cuadro sindrómico que padecían. Conclusión. La presencia de hemangioblastomas quísticos de la unión bulbomedular asociados a la enfermedad de von Hippel-Lindau es muy infrecuente. A pesar de la dificultad técnica existente para su extirpación, la incorporación de las últimas técnicas de neuroimagen y microcirugía ha permitido una reducción significativa de la morbimortalidad asociada al tratamiento quirúrgico de estos pacientes (AU)

Introduction. Hemangioblastomas are very highly vascularised benign tumours that can present either sporadically or in association with von Hippel-Lindau syndrome in 20% of cases. Only 5-20% of hemangioblastomas are located in the brainstem, and those that occur at the junction of the medulla and upper spinal cord are the ones that are less commonly diagnosed. Case report. Two young patients were diagnosed with von Hippel-Lindau disease after beginning with a cystic hemangioblastoma at the junction of the medulla and upper spinal cord as the first pathological manifestation. Both of them underwent a suboccipital craniotomy and the two lesions were completely removed without any kind of post-operative neurological deficits. One of the patients required a reintervention some years later due to a new recurrence of a symptomatic hemangioblastoma in the dorsal medulla. The genetic study conducted in both patients confirmed the presence of a mutation in the VHL gene, which is responsible for the syndrome they were suffering from. Conclusions. The presence of cystic hemangioblastomas of the junction of the medulla and upper spinal cord associated to von Hippel-Lindau disease is very rare. Despite the technical difficulties involved in removing them, the incorporation of the latest neuroimaging and microsurgery techniques has made it possible to significantly reduce the morbidity and mortality rate associated to the surgical treatment of such patients (AU)

[Cystic hemangioblastoma of the junction of the medulla and upper spinal cord associated to von Hippel-Lindau disease. Two case reports and a review of the literature]. / Hemangioblastoma quistico de la union bulbomedular asociado a enfermedad de von Hippel-Lindau. Presentacion de dos casos y revision de la bibliografia.

INTRODUCTION: Hemangioblastomas are very highly vascularised benign tumours that can present either sporadically or in association with von Hippel-Lindau syndrome in 20% of cases. Only 5-20% of hemangioblastomas are located in the brainstem, and those that occur at the junction of the medulla and upper spinal cord are the ones that are less commonly diagnosed. CASE REPORTS: Two young patients were diagnosed with von Hippel-Lindau disease after beginning with a cystic hemangioblastoma at the junction of the medulla and upper spinal cord as the first pathological manifestation. Both of them underwent a suboccipital craniotomy and the two lesions were completely removed without any kind of post-operative neurological deficits. One of the patients required a reintervention some years later due to a new recurrence of a symptomatic hemangioblastoma in the dorsal medulla. The genetic study conducted in both patients confirmed the presence of a mutation in the VHL gene, which is responsible for the syndrome they were suffering from. CONCLUSIONS: The presence of cystic hemangioblastomas of the junction of the medulla and upper spinal cord associated to von Hippel-Lindau disease is very rare. Despite the technical difficulties involved in removing them, the incorporation of the latest neuroimaging and microsurgery techniques has made it possible to significantly reduce the morbidity and mortality rate associated to the surgical treatment of such patients.

[Unilateral subthalamic stimulation in the treatment of advanced Parkinson's disease]. / Estimulación subtalámica unilateral en el tratamientode la enfermedad de Parkinson avanzada.

INTRODUCTION: The use of unilateral subthalamic stimulation (USTS) in the treatment of advanced Parkinson's disease can be effective and offer a series of advantages on comparing this technique with bilateral subthalamic stimulation (BSTS). PATIENTS AND METHODS: We studied 35 consecutive patients: 22 with BSTS and 13 with USTS (six left and seven right). The epidemiological features and the scores on the functional assessment scales -Hoehn and Yahr, Schwab and England, and the Unified Parkinson's Disease Rating Scale (UPDRS) I to IV- were not significantly different in the two groups, except for the Hoehn and Yahr in off (USTS: 3.3 +/- 0.3; BSTS: 4.1 +/- 0.2; p = 0.004). RESULTS: The overall percentages of improvement six months after surgery were UPDRS I: 12%; II: 21.6%; III-medication off/stimulator on: 30.6% (with respect to the baseline off state); III-on/on: 8.8% (with respect to the baseline on state); IV: 48.9%. Reduction in the levodopa equivalent dose: USTS: 26.3%; BSTS: 17%. These percentages of improvement were not significantly different in the two groups. If we consider the motor scale in medication-off and stimulator-on in the sixth month and we compare it with the same in the off state before surgery, the axial motor symptoms improved by 17.1% (USTS) and 25% (BSTS); in the extremities, USTS: 39.1% in the limbs contralateral to the implanted electrode, and 14.5% in the ipsilaterals; BSTS: right extremities, 32.6%; left extremities, 31.5%. No significant differences were found on comparing the electrical power consumed by the electrodes in the two groups of patients in the sixth month of treatment. CONCLUSIONS: USTS was effective in improving the axial symptoms. In our series, the reduction in medication following surgery was similar to that of the patients with BSTS.

[Refractory epilepsy as the presenting symptom of a brucellar brain abscess]. / Epilepsia refractaria como presentacioón de absceso cerebral brucelósico.

INTRODUCTION: Brucellosis is a zoonotic disease that is occasionally transmitted to human beings from infected animal reservoirs. It is an important condition in endemic areas. One infrequent complication of systemic brucellosis is the infection of the central or the peripheral nervous systems. CASE REPORT: A 54-year-old male who was being studied prior to surgery for refractory epilepsy, with clinical expression in the form of complex partial seizures. Neuroimaging findings revealed an expansive lesion in the right temporal lobe, which direct serological, histopathological and microbiological evidence showed to be a chronic brucellar abscess. After combined treatment involving complete surgical resection followed by a cycle of standard antimicrobial therapy, the patient was seizure-free at one year of follow-up. CONCLUSIONS: Despite its low frequency, infection by Brucella must be considered in the differential diagnosis of intracranial expansive lesions, as well as in the case of patients whose presenting symptoms are epileptic seizures. To perform the diagnosis it is especially important to be aware of the wide range of clinical and radiological manifestations that can be produced, and which do not always correlate. Identification of risk factors on the patient record is also a crucial step.