ABSTRACT
Erdheim-Chester disease is a non-Langerhans histiocytosis. Until 2014 at least 550 cases have been reported. According to European Rare Disease Organization and National Organization for Rare Disorders it is a rare disease. The most common symptom is bone pain in the lower extremities and it usually appears between the 5th and 7th decades of life. The diagnostic is based on immunohistochemical results: S100(+/-), CD68(+), and CD1a(-), the latter 2 are mandatory. The best treatment nowadays is alpha-interferon or pegylated alpha-2. The overall survival is 96% at one year and 68% at 5 years. Central nervous system involvement is associated with a worse outcome. Two cases are presentedwith central nervous system lesions in the absence of lesions in other organs on their onset. Very few cases have been reported with this kind of presentation. We also noted that these patients had recurrences or new lesions at 8 months. A follow-up is proposed with brain MRI and thoraco-abdominal PET every 3-4 months.
Subject(s)
Erdheim-Chester Disease/diagnosis , Child, Preschool , Erdheim-Chester Disease/complications , Humans , Magnetic Resonance Imaging , Pain/etiologyABSTRACT
Cavernous hemangiomas, also known as deep hemangiomas are benign tumors of blood vessels, including normal and abnormal vascular structures, that develop in skin tissue and sometimes even in deep tissues. Its intraneural development in the peripheral nerve is very rare with less than 50 cases reported in the literature. We present a case of a cavernous hemangioma of the medial sural nerve in a patient with symptoms of severe pain and allodynia with complete resolution of symptoms with microsurgery.
Subject(s)
Hemangioma, Cavernous , Humans , Hemangioma, Cavernous/complications , Hemangioma, Cavernous/diagnostic imaging , Hemangioma, Cavernous/surgery , Peripheral Nerves/pathologyABSTRACT
Spinal cord stimulation (SCS) consists of the application of electrical stimuli to the dorsal columns of the spinal cord or to the posterior nerve roots in order to modulate the pain signals carried by the ascending pain pathways to the brain. Two cases of SCS in patients with cauda equina syndrome after lumbar surgery are presented. They were treated for persistent neuropathic pain but also experienced improvement in their motor and urinary symptoms after this treatment. Although the primary indication for SCS is neuropathic pain control, its application can also lead to improvement of motor deficits, sensory disorders, and urinary incontinence, as shown in these two cases. SCS will likely play a fundamental role in rehabilitative therapies in different neurological diseases. Further investigation in the field is needed.
Subject(s)
Cauda Equina Syndrome , Cauda Equina , Spinal Cord Stimulation , Cauda Equina Syndrome/etiology , Cauda Equina Syndrome/therapy , Humans , Spinal Cord , Spinal Nerve RootsABSTRACT
Meralgia paresthetica is a neurological disorder caused by a neuropathy of the lateral femoral cutaneous nerve. Its aetiology can be spontaneous or iatrogenic. It is characterized by pain, paresthesia, and numbness in the anterolateral aspect of the thigh. Diagnosis is based on clinical examination, although image and neurophysiological tests can be useful as well. Despite conservative measures use to be effective in most of patients, refractory cases can benefit from alternative treatments. Available surgical procedures are: nerve decompression (neurolysis) or section (neurectomy) and radiofrequency ablation. We present a case of refractory meralgia paresthetica where spinal cord stimulation was used as a possible effective technique in pain relief and to avoid the neurectomy of the lateral femoral cutaneous nerve.
Subject(s)
Femoral Neuropathy , Nerve Compression Syndromes , Spinal Cord Stimulation , Humans , Femoral Neuropathy/therapy , Femoral Neuropathy/complications , Spinal Cord Stimulation/adverse effects , Nerve Compression Syndromes/etiology , Nerve Compression Syndromes/therapy , Paresthesia/etiology , Pain/complicationsABSTRACT
Ependymomas are well defined glial tumours composed of uniform small cells with round nuclei in a fibrillar matrix. They have characteristic perivascular acellular areas (pseudorosettes) and, in some cases, ependymal rosettes. The three most well-known histological phenotypes are papillary, clear-cell and tanycytic. The WHO classification includes rare cases of ependymoma with lipomatous metaplasia. Lipomatous ependymomas of the posterior fossa are extremely rare; we only found 7reports of cases in adults. They usually arise in the fourth ventricle and may extend into the cerebellum, when they often show extensive vacuolization, pushing the nucleus to the periphery and giving rise to a signet-ring cell appearance. Radiologically, there are few findings characteristic of these tumours. Immunohistochemistry is essential to differentiate this subtype from other more common lesions, such as metastatic adenocarcinoma, especially from breast, intestine and kidney.
Subject(s)
Ependymoma , Lipoma , Ependymoma/pathology , Humans , Immunohistochemistry , MetaplasiaABSTRACT
Spinal cord stimulation (SCS) consists of the application of electrical stimuli to the dorsal columns of the spinal cord or to the posterior nerve roots in order to modulate the pain signals carried by the ascending pain pathways to the brain. Two cases of SCS in patients with cauda equina syndrome after lumbar surgery are presented. They were treated for persistent neuropathic pain but also experienced improvement in their motor and urinary symptoms after this treatment. Although the primary indication for SCS is neuropathic pain control, its application can also lead to improvement of motor deficits, sensory disorders, and urinary incontinence, as shown in these two cases. SCS will likely play a fundamental role in rehabilitative therapies in different neurological diseases. Further investigation in the field is needed.
ABSTRACT
The coronavirus disease 2019 (COVID-19) has amazed by its distinct forms of presentation and severity. COVID-19 patients can develop large-scale ischemic strokes in previously healthy patients without risk factors, especially in patients who develop an acute respiratory distress syndrome (SARS-CoV-2). We hypothesize that ischemic events are usually the result of the combined process of a pro-inflammatory and pro-coagulant state plus vascular endothelial dysfunction probably potentiated by hypoxia, hemodynamic instability, and immobilization, as reported in other cases. To the best of our knowledge, we report the first case of partial obstruction of a vertebral artery in a patient with COVID-19. Decompressive surgery remains a life-saving maneuver in these patients (as in other non-COVID-19 strokes) and requires further investigation.
ABSTRACT
Ischemic stroke (IS) is the leading cause of disability in the western world, assuming a high socio-economic cost. One of the most used strategies in the last decade has been biomaterials, which have been initially used with a structural support function. They have been perfected, different compounds have been combined, and they have been used together with cell therapy or controlled release chemical compounds. This double function has driven them as potential candidates for the chronic treatment of IS. In fact, the most developed are in different phases of clinical trial. In this review, we will show the ischemic scenario and address the most important criteria to achieve a successful neuroreparation from the point of view of biomaterials. The spontaneous processes that are activated and how to enhance them is one of the keys that contribute to the success of the therapeutic approach. In addition, the different routes of administration and how they affect the design of biomaterials are analyzed. Future perspectives show where this broad scientific field is heading, which advances every day with the help of technology and advanced therapies.
ABSTRACT
Los ependimomas son tumores gliales bien circunscritos compuestos de células pequeñas uniformes con un núcleo redondo en una matriz fibrilar. Se caracterizan por zonas anucleadas perivasculares (pseudorrosetas) y, en algunos casos, rosetas ependimarias. Tres fenotipos histológicos son los más reconocidos: papilar, de células claras y tanicítico. La OMS reconoce casos raros de ependimomas con metaplasia lipomatosa. Los ependimomas lipomatosos de fosa posterior son extremadamente infrecuentes, y en nuestra búsqueda hemos encontrado 7casos reportados en la literatura (excluyendo niños). Se originan habitualmente del 4.° ventrículo y podrían presentar extensión cerebelosa. Estos suelen presentar extensa vacuolización empujando el núcleo a la periferia y simulando la apariencia de «signet-ring cells» (células en anillo de sello). Radiológicamente hay pocos hallazgos que sean característicos de este tipo de tumores. La inmunohistoquímica es decisiva para no confundir esta variante con lesiones más comunes, como el adenocarcinoma metastásico, especialmente los de mama, intestino y renal.(AU)
Ependymomas are well defined glial tumours composed of uniform small cells with round nuclei in a fibrillar matrix. They have characteristic perivascular acellular areas (pseudorosettes) and, in some cases, ependymal rosettes. The three most well-known histological phenotypes are papillary, clear-cell and tanycytic. The WHO classification includes rare cases of ependymoma with lipomatous metaplasia. Lipomatous ependymomas of the posterior fossa are extremely rare; we only found 7reports of cases in adults. They usually arise in the fourth ventricle and may extend into the cerebellum, when they often show extensive vacuolization, pushing the nucleus to the periphery and giving rise to a signet-ring cell appearance. Radiologically, there are few findings characteristic of these tumours. Immunohistochemistry is essential to differentiate this subtype from other more common lesions, such as metastatic adenocarcinoma, especially from breast, intestine and kidney.(AU)
Subject(s)
Humans , Ependymoma/pathology , Metaplasia , Neoplasm Metastasis , Brain Neoplasms , Immunohistochemistry , LipomaABSTRACT
La meralgia parestésica es un desorden neurológico causado por una neuropatía del nervio femorocutáneo lateral. Su etiología puede ser idiopática o iatrogénica. Se caracteriza por dolor, parestesias y entumecimiento en la cara anterolateral del muslo. Su diagnóstico es básicamente clínico, aunque pueden ser útiles pruebas de imagen o neurofisiológicas. A pesar de que el tratamiento conservador suele ser eficaz en la mayoría de los pacientes, existen casos refractarios que pueden precisar de otras formas de tratamiento. Los procedimientos quirúrgicos disponibles son la descompresión nerviosa (neurólisis) o la sección (neurectomía) y las ablaciones por radiofrecuencia. Presentamos un caso de meralgia parestésica invalidante refractaria en el cual empleamos la estimulación medular como posible técnica eficaz en el alivio del dolor y poder evitar la realización de una neurectomía del nervio femorocutáneo lateral (AU)
Meralgia paresthetica is a neurological disorder caused by a neuropathy of the lateral femoral cutaneous nerve. Its etiology can be spontaneous or iatrogenic. It is characterized by pain, paresthesia, and numbness in the anterolateral aspect of the thigh. Diagnosis is based on clinical examination, although image and neurophysiological tests can be useful as well. Despite conservative measures use to be effective in most of patients, refractory cases can benefit from alternative treatments. Available surgical procedures are: nerve decompression (neurolysis) or section (neurectomy) and radiofrequency ablation. We present a case of refractory meralgia paresthetica where spinal cord stimulation was used as a possible effective technique in pain relief and to avoid the neurectomy of the lateral femoral cutaneous nerve (AU)
Subject(s)
Humans , Female , Adult , Femoral Neuropathy/diagnosis , Nerve Compression Syndromes/diagnosis , Spinal Cord Stimulation , Femoral Neuropathy/therapy , Nerve Compression Syndromes/therapy , Treatment OutcomeABSTRACT
The coronavirus disease 2019 (COVID-19) has amazed by its distinct forms of presentation and severity. COVID-19 patients can develop large-scale ischemic strokes in previously healthy patients without risk factors, especially in patients who develop an acute respiratory distress syndrome (SARS-CoV-2). We hypothesize that ischemic events are usually the result of the combined process of a pro-inflammatory and pro-coagulant state plus vascular endothelial dysfunction probably potentiated by hypoxia, hemodynamic instability, and immobilization, as reported in other cases. To the best of our knowledge, we report the first case of partial obstruction of a vertebral artery in a patient with COVID-19. Decompressive surgery remains a life-saving maneuver in these patients (as in other non-COVID-19 strokes) and requires further investigation (AU)
La enfermedad por coronavirus 2019 (COVID-19) ha sorprendido por sus distintas formas de presentación y gravedad. Los pacientes con COVID-19 pueden desarrollar accidentes cerebrovasculares isquémicos a gran escala, incluso aquellos previamente sanos, sin factores de riesgo, y especialmente los que desarrollan un síndrome de dificultad respiratoria aguda (SARS-CoV-2). Presumimos que los eventos isquémicos son generalmente el resultado del proceso combinado de un estado proinflamatorio y procoagulante, más una posible disfunción endotelial vascular, probablemente potenciada por hipoxia, inestabilidad hemodinámica e inmovilización, como se ha reportado en otros casos. Hasta nuestro conocimiento reportamos el primer caso de una obstrucción parcial de una arteria vertebral en un paciente con COVID-19. La cirugía descompresiva sigue siendo una maniobra que salva vidas (como en otros accidentes cerebrovasculares que no están relacionados con la COVID-19) y requiere más investigación (AU)
Subject(s)
Humans , Male , Adult , Middle Aged , Coronavirus Infections/complications , Stroke/diagnostic imaging , Stroke/virology , Tomography, X-Ray ComputedABSTRACT
La enfermedad de Erdheim-Chester es una histiocitosis no-Langerhans. Hasta 2014 han sido reportados alrededor de 550 casos, y está catalogada como enfermedad rara según la European Rare Disease Organization y la National Organization for Rare Disorders. La clínica más frecuente es dolor óseo en miembros inferiores y generalmente aparece entre la 5.a -7.a década. El diagnóstico se basa en inmunohistoquímica S100(+/-), CD68(+) y CD1a(-), estos 2 últimos suficientes y mandatorios para el diagnóstico. El mejor tratamiento consiste en administrar interferón-alfa o interferón-alfa2-pegilado. La supervivencia es del 96% al año y del 68% a los 5 años, siendo menor en casos con afectación del sistema nervioso central. Presentamos 2 casos con afectación aislada del sistema nervioso central al diagnóstico, siendo muy pocos los casos publicados con esta forma de presentación. También observamos que estos pacientes presentaron recidivas o nuevas lesiones a los 8 meses por lo que proponemos seguimiento con RMN cerebral y PET toracoabdominal cada 3-4 meses
Erdheim-Chester disease is a non-Langerhans histiocytosis. Until 2014 at least 550 cases have been reported. According to European Rare Disease Organization and National Organization for Rare Disorders it is a rare disease. The most common symptom is bone pain in the lower extremities and it usually appears between the 5 th and 7 th decades of life. The diagnostic is based on immunohistochemical results: S100(+/-), CD68(+), and CD1a(-), the latter 2 are mandatory. The best treatment nowadays is alpha-interferon or pegylated alpha-2. The overall survival is 96% at one year and 68% at 5 years. Central nervous system involvement is associated with a worse outcome. Two cases are presented with central nervous system lesions in the absence of lesions in other organs on their onset. Very few cases have been reported with this kind of presentation. We also noted that these patients had recurrences or new lesions at 8 months. A follow-up is proposed with brain MRI and thoraco-abdominal PET every 3-4 months