Mujer de 82 años con dolor abdominal, fiebre y lesiones cutáneas de larga evolución / Eighty-two year old female with long term abdominal pain, fever and skin lesions

Presentamos el caso de una mujer mayor, que consulta por cuadro de fiebre, dolor abdominal y síndrome constitucional de un año de evolución. El diagnóstico diferencial se realiza entre patología infecciosa, tumoral, o inflamatoria, que pudieran localizarse a nivel abdominal, realizándose exploraciones complementarias que descartaron proceso abdominal. La existencia de dolor en las piernas y la exploración a nivel de muslo izquierdo de una masa de consistencia dura, nos hace plantear otro diagnóstico. Finalmente se demuestran en la ecografía de partes blandas cambios inflamatorios en relación con paniculitis. A partir de este hallazgo se revisan las etiologías de la paniculitis. Se realiza la biopsia cutánea que nos demuestra el diagnóstico final (AU)

We report the case of an old woman, consulting for fever, abdominal pain and constitutional symptoms one year of evolution. The differential diagnosis is between infectious, tumoral, or inflammatory disease, which may be located at the abdominal level, performing additional tests to rule out abdominal process. The existence of pain in the legs and level scan left thigh of a mass of hard consistency, makes us raise another diagnosis. Finally show on ultrasound soft tissue inflammatory changes regarding panniculitis. From this finding aetiologies of panniculitis are reviewed. Skin biopsy that shows the final diagnosis is made (AU)

Eighty-two year old female with long term abdominal pain, fever and skin lesions. / Mujer de 82 años con dolor abdominal, fiebre y lesiones cutáneas de larga evolución.

We report the case of an old woman, consulting for fever, abdominal pain and constitutional symptoms one year of evolution. The differential diagnosis is between infectious, tumoral, or inflammatory disease, which may be located at the abdominal level, performing additional tests to rule out abdominal process. The existence of pain in the legs and level scan left thigh of a mass of hard consistency, makes us raise another diagnosis. Finally show on ultrasound soft tissue inflammatory changes regarding panniculitis. From this finding aetiologies of panniculitis are reviewed. Skin biopsy that shows the final diagnosis is made.

Aspergiloma cerebral en un paciente inmunocompetente: hallazgos radiológicos e histopatológicos / Cerebral aspergilloma in an immunocompetent patient: radiological and histopathological findings

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[Mitochondrial encephalopathy due to complex I deficiency. Brain tissue biopsy findings and clinical course following pharmacological]. / Encefalopatia mitocondrial por deficit del complejo I. Hallazgos en la biopsia cerebral y evolucion clinica tras tratamiento farmacologico.

INTRODUCTION: Mitochondrial encephalomyopathies belong to a heterogeneous group of diseases with a range of neurological symptoms caused by a dysfunction somewhere in the nervous system. They may arise from mutations of the mitochondrial DNA or nuclear DNA in the genes that code for the subunits of the respiratory chain. The results obtained from using different drugs to treat these diseases vary widely. CASE REPORT: A 33-year-old female with a history of migraine with aura, who was admitted to hospital because of epileptic seizures. Neuroimaging tests showed left-side occipital insult and a biopsy study of a sample of brain tissue revealed gliosis and vacuolisation of the white matter. Lactic acid levels in blood were normal. No ragged red fibres were seen in the muscle biopsy, but there was evidence of a complex I deficiency in the respiratory chain. After establishing treatment with coenzyme Q and riboflavin, the patient had no further episodes of neurological disorders. CONCLUSIONS: The absence of elevated levels of lactate, ragged red fibres in the muscle biopsy or the negative results for mutations in the genetic study do not rule out the possible existence of a mitochondrial disease. The gliosis and vacuolisation of the white matter with respect to the neurons that were found in the results of the brain tissue biopsy must lead us to consider a mitochondrial disease.

Encefalopatía mitocondrial por déficit del complejo I. Hallazgos en la biopsia cerebral y evolución clínica tras tratamiento farmacológico / Mitochindrial encephalopathy due to complex I. deficiency brain tissue biopsy findings and clinical course following pharmacological

Introducción. Las encefalomiopatías mitocondriales constituyen un grupo heterogéneo de enfermedades con sintomatología neurológica diversa por disfunción de cualquier parte del sistema nervioso. Pueden producirse por mutaciones del ADN mitocondrial o del ADN nuclear en los genes que codifican las subunidades de la cadena respiratoria. Los fármacos utilizados en estas enfermedades han tenido resultados muy dispares.Caso clínico. Mujer de 33 años con antecedentes de migraña con aura que ingresa por crisis epilépticas. En las pruebas de neuroimagen se observa una lesión occipital izquierda cuya biopsia cerebral muestra gliosis y vacuolización de la sustancia blanca. No presenta elevación del ácido láctico en sangre. Enla biopsia muscular no hay fibras rojo-rasgadas, pero se evidencia un déficit del complejo I de la cadena respiratoria. Tras iniciar tratamiento con coenzima Q y riboflavina, la paciente no vuelve a tener nuevos episodios de afectación neurológica.Conclusiones. La ausencia de lactato elevado, fibras rojo-rasgadas en la biopsia muscular o la negatividad de mutaciones en el estudio genético no excluyen la existencia de una enfermedad mitocondrial. Los hallazgos en la biopsia cerebral de gliosis y vacuolización de la sustancia blanca con relativo respeto de las neuronas nos deben hacer pensar en una enfermedad mitocondrial

Introduction. Mitochondrial encephalomyopathies belong to a heterogeneous group of diseases with a range ofneurological symptoms caused by a dysfunction somewhere in the nervous system. They may arise from mutations of the mitochondrial DNA or nuclear DNA in the genes that code for the subunits of the respiratory chain. The results obtained from using different drugs to treat these diseases vary widely. Case report. A 33-year-old female with a history of migraine with aura, who was admitted to hospital because of epileptic seizures. Neuroimaging tests showed left-side occipital insult and a biopsy study of a sample of brain tissue revealed gliosis and vacuolisation of the white matter. Lactic acid levels in blood werenormal. No ragged red fibres were seen in the muscle biopsy, but there was evidence of a complex I deficiency in the respiratory chain. After establishing treatment with coenzyme Q and riboflavin, the patient had no further episodes of neurological disorders. Conclusions. The absence of elevated levels of lactate, ragged red fibres in the muscle biopsy or thenegative results for mutations in the genetic study do not rule out the possible existence of a mitochondrial disease. The gliosis and vacuolisation of the white matter with respect to the neurons that were found in the results of the brain tissue biopsy must lead us to consider a mitochondrial disease

Gliomatosis cerebri: evolución a glioblastoma multiforme / Gliomatosis cerebri: evolution to glioblastoma multiforme

Introducción. La gliomatosis cerebri es un trastorno neoplásico caracterizado por una infiltración difusa de células gliales con relativa conservación de las estructuras subyacentes. Las crisis comiciales, la cefalea y los trastornos del comportamiento suelen ser las manifestaciones iniciales.Caso clínico. Hombre de 38 años que presenta crisis parciales complejas y trastorno del comportamiento de 3 meses de evolución. En la resonancia magnética cerebral presenta lesiones hiperintesas en T2 sugerentes de gliomatosis cerebri, confirmándose con la biopsia cerebral. Varios meses después sufre un empeoramiento clínico rápido, evidenciándose el desarrollo de un glioblastoma multiforme sobre la lesión.Conclusiones. La gliomatosis cerebri, pese a su rareza, debe tenerse en cuenta en el diagnóstico diferencial de las lesiones infiltrativas difusas de la sustancia blanca. El empeoramiento clínico de rápida evolución y la aparición de lesiones focales que captan contraste nos deben hacer sospechar una trasformación a lesiones de mayor malignidad

Introduction. Gliomatosis cerebri is a neoplastic disorder characterized by diffuse infiltration of glial cells with relative conservation of the underlying structures. Seizures, headache and behavior disorders are generally the initial manifestations.Clínical case. A 38 year-old male who had complex partial seizures and behavior disorder of three months' evolution. The brain magnetic resonance imaging showed hyperintense lesions in T2 suggestive of gliomatosis cerebri, this being confirmed with the brain biopsy. Several months later, he suffered rapid clinical deterioration, observing the development of a glioblastoma multiforme over the lesion.Conclusions. In spite of its rareness, gliomatosis cerebri should be taken into account in the differential diagnoses of diffuse infiltrative lesions of the white matter. Rapid evolution clinical deterioration and the appearance of focal lesions that capture contrast should make us suspect a transformation to lesions of greater malignancy

[Gliomatosis cerebri: evolution to glioblastoma multiforme]. / Gliomatosis cerebri: evolución a glioblastoma multiforme.

INTRODUCTION: Gliomatosis cerebri is a neoplastic disorder characterized by diffuse infiltration of glial cells with relative conservation of the underlying structures. Seizures, headache and behavior disorders are generally the initial manifestations. CLINICAL CASE: A 38 year-old male who had complex partial seizures and behavior disorder of three months' evolution. The brain magnetic resonance imaging showed hyperintense lesions in T2 suggestive of gliomatosis cerebri, this being confirmed with the brain biopsy. Several months later, he suffered rapid clinical deterioration, observing the development of a glioblastoma multiforme over the lesion. CONCLUSIONS: In spite of its rareness, gliomatosis cerebri should be taken into account in the differential diagnoses of diffuse infiltrative lesions of the white matter. Rapid evolution clinical deterioration and the appearance of focal lesions that capture contrast should make us suspect a transformation to lesions of greater malignancy.

Síndrome de Bruns: descripción de un caso de neurocisticercosis con estudio anatomopatológico / Bruns’ syndrome: description of a case of neurocysticercosis with pathological study

El síndrome de Bruns se caracteriza por cefalea súbita e intensa que se acompaña de vómitos, acúfenos y vértigos, precipitada por los movimientos bruscos de la cabeza y que puede acarrear coma profundo y la muerte. Puede deberse a neurocisticercosis por quiste a nivel del cuarto ventrículo, el cual contiene la larva enquistada de la Taenia solium. En los últimos años se están volviendo a ver en España casos de esta parasitosis debido a la inmigración desde zonas endémicas, pero las formas clínicas de esta infección no han cambiado. Se trata de un varón de 44 años de nacionalidad ecuatoriana que presenta sintomatología compatible con síndrome de Bruns secundario a quiste en el cuarto ventrículo con mala evolución pese haberse sometido a derivación ventrículoperitoneal y tratamiento con albendazol. Finalmente se sometió a excisión quirúrgica de la lesión mediante craniectomía suboccipital. Concluimos que este síndrome debe llevar a descartar la presencia de una lesión, quística o no, a nivel intraventricular y que el adecuado manejo de los quistes ventriculares cisticercóticos conduce a un pronóstico más favorable

Bruns’ syndrome is characterized by sudden and strong headache, accompanied by vomiting, acuphenos and vertigo, triggered by abrupt movement of the head and can produce deep coma and death. This can be due to neurocysticercosis by cyst in the fourth ventricle, which contains the larval stage of Taenia solium. In the last years, new cases of this parasitosis are being seen in Spain because of the immigration from endemic areas, but the clinical forms of this infestation are not varied. We present a 44 year old man Ecuadorian who has clinical symptoms consistent with Bruns’ syndrome secondary to cyst in the fourth ventricle that progressed badly despite undergoing surgery with ventriculoperitoneal shunt and treatment with albendazol. Finally, he was operated with the excision of the lesion by means of suboccipital craniectomy. We concluye that this syndrome must rule out a cystic or non-cystic, intraventricular lesion and that the appropriate manipulation of the cysticerotic ventricular cysts carries a more favourable prognosis

[Bruns' syndrome: description of a case of neurocysticercosis with pathological study]. / Síndrome de Bruns: descripción de un caso de neurocisticercosis con estudio anatomopatológico.

Bruns' syndrome is characterized by sudden and strong headache, accompanied by vomiting, acuphenos and vertigo, triggered by abrupt movement of the head and can produce deep coma and death. This can be due to neurocysticercosis by cyst in the fourth ventricle, which contains the larval stage of Taenia solium. In the last years, new cases of this parasitosis are being seen in Spain because of the immigration from endemic areas, but the clinical forms of this infestation are not varied. We present a 44 year old man Ecuadorian who has clinical symptoms consistent with Bruns' syndrome secondary to cyst in the fourth ventricle that progressed badly despite undergoing surgery with ventriculoperitoneal shunt and treatment with albendazol. Finally, he was operated with the excision of the lesion by means of suboccipital craniectomy. We conclude that this syndrome must rule out a cystic or non-cystic, intraventricular lesion and that the appropriate manipulation of the cysticerotic ventricular cysts carries a more favourable prognosis.

[Gas gangrene due to Clostridium septicum in a patient with an occult colonic neoplasm. A case report and review of the literature]. / Gangrena gaseosa por Clostridium septicum en un paciente con neoplasia de colon oculta. Presentación de un caso y revisión de la literatura.

A case of gaseous gangrene by Clostridium septicum associated with colorectal cancer is presented. The patient evolved rapidly towards septic shock and death. Autopsy showed occult neoplasm and pelvic and retroperitoneal myonecrosis. An exceptional finding was that of myocarditis in which thick gram-positive bacilli were identified. A review of the literature was carried out regarding the pathogenesis and clinical manifestations of this disease. The association of colonic neoplasm and Clostridium septicum may be related with the sensitivity of the cells of this neoplasm to the toxins of the microorganisms. The usefulness of this cytotoxicity is being tested in the therapeutic reduction of tumoral mass. With respect to clinical attitude, all the authors agree on the need for clinical suspicion as to the possible existence of occult colon neoplasm in individuals with septic shock by gaseous gangrene with no obvious entry site. Diagnosis is performed by imaging techniques with barium enema and if this is normal colonoscopy is carried out. Emergency treatment consists in laparotomy with resection of the neoplasm and debridement of the area accompanied by hyperbaric oxygen and antibiotics.

[Deteriorating natural killer activity in familial erythrophagocytic lymphohistiocytosis]. / Actividad "natural killer" deteriorada en la linfohistiocitosis eritrofagocítica familiar.

We report the occurrence of familial erythrophagocytic lymphohistiocytosis disease in a child who had greater involvement of the brain than of visceral organs. The diagnosis was made during life, allowing us to carry out biological, histological and immunological studies. The natural Killer activity was abolished in this patient. The combination chemotherapy--including systemic administration of VP 16-213, steroids and intrathecal methotrexate--, failed to stem the rapid course of the disease.