[Spinal cord compression: an unusual clinical manifestation of retroperitoneal fibrosis]. / Síndrome de compresión medular: una rara forma de presentación de la fibrosis retroperitoneal.

INTRODUCTION: Retroperitoneal fibrosis is a rare disorder consisted of a periaortic fibrous mass that compresses retroperitoneal organs. CASE REPORT: We report a case of retroperitoneal fibrosis diagnosed after a spinal cord compression. Magnetic resonance imaging scan showed a prevertebral mass which affected a vertebral body. It extended to the epidural space and compressed the spinal cord. The final diagnosis was established through the mass biopsy by means of mediastinoscopy. After prednisone treatment, both clinical remission and mass reduction in the follow-up MRI were observed. CONCLUSIONS: The spinal cord compression is an extremely unusual initial manifestation of retroperitoneal fibrosis. Nevertheless, it should be considered in the differential diagnosis of spinal cord compression syndrome, specially given the possibility of its medical treatment instead of aggressive surgical treatment.

Síndrome de compresión medular: una rara forma de presentación de la fibrosis retroperitoneal / Spinal cord copression: an unusual clinical manifetation of retroperitoneal fibrosis

Introducción. La fibrosis retroperitoneal es una rara enfermedad que se caracteriza por la presencia de una masa fibrosa periaórtica que comprime los órganos retroperitoneales. Los uré- teres resultan afectados a menudo, pero también pueden afectarse otras estructuras de forma menos frecuente. Caso clínico. Presentamos un caso en el que esta enfermedad se diagnosticó a partir de un síndrome medular subagudo. En las imágenes de resonancia magnética se observaba una masa prevertebral que afectaba a un cuerpo vertebral, se extendía al espacio epidural y comprimía el cordón medular. La biopsia de la lesión mostró un tejido fibroso con un infiltrado inflamatorio linfoplasmocitario compatible con fibrosis retroperitoneal. El paciente recibió tratamiento con prednisona y la clínica remitió, además de reducirse dicha lesión en el estudio radiológico. Conclusiones. El síndrome de compresión medular es una rara forma de presentación de la fibrosis retroperitoneal. A pesar de ello, esta enfermedad debe incluirse en su diagnóstico diferencial, sobre todo por la posibilidad de tratamiento médico (AU)

Introduction. Retroperitoneal fibrosis is a rare disorder consisted of a periaortic fibrous mass that compresses retroperitoneal organs. Case report. We report a case of retroperitoneal fibrosis diagnosed after a spinal cord compression. Magnetic resonance imaging scan showed a prevertebral mass which affected a vertebral body. It extended to the epidural space and compressed the spinal cord. The final diagnosis was established through the mass biopsy by means of mediastinoscopy. After prednisone treatment, both clinical remission and mass reduction in the follow-up MRI were observed. Conclusions. The spinal cord compression is an extremely unusual initial manifestation of retroperitoneal fibrosis. Nevertheless, it should be considered in the differential diagnosis of spinal cord compression syndrome, specially given the possibility of its medical treatment instead of aggressive surgical treatment (AU)

[Ischemic brain lesions following carotid revascularisation procedures: a comparative study using diffusion-weighted magnetic resonance imaging]. / Lesiones cerebrales isquémicas tras procedimientos de revascularización carotídea: estudio comparativo con resonancia magnética por difusión.

INTRODUCTION: The risk of cerebral embolism following angioplasty-stent placement (AGP) is higher than that observed after a carotid endarterectomy (CE) using transcranial Doppler ultrasonography. Nevertheless, no studies have been conducted to compare the two revascularisation procedures with the latest neuroimaging techniques. AIMS: To analyse the presence and repercussion of acute cerebral ischemia detected by diffusion-weighted magnetic resonance imaging (DMR) following carotid revascularisation procedures. PATIENTS AND METHODS: Our prospective study included a sample of 20 consecutive patients with atherosclerotic stroke and symptomatic carotid stenosis > 70% according to NASCET criteria and treated by CE (n = 10) or AGP (n = 10). Patients were submitted to a DMR during the 48 hours prior to revascularisation and another within the 72 hours following the intervention in order to evaluate the existence of new acute cerebral ischemic lesions. Patients were explored by a neurologist before and after the intervention. RESULTS: DMR allowed new areas of cerebral ischemia to be detected in 10% of the CE and in 40% of the AGP patients. 50% of the post-AGP ischemic lesions were multiple and 10% of the lesions in each therapeutic group presented some clinical correlation in the form of transient neurological focal signs. No significant differences were observed with respect to the pattern of risk of complications between the two therapeutic groups and no factors associated to a higher risk of new ischemic lesions were detected by DMR in patients submitted to AGP. CONCLUSIONS: Carotid AGP is linked to a higher frequency of new ischemic lesions in the brain than in the case of CE. Nevertheless, these ischemic lesions detected by DMR are usually silent. Symptomatic complications were similar in the two procedures.

Lesiones cerebrales isquémicas tras procedimientos de revascularización carotídea: estudio comparativo con resonancia magnética por difusión / Ischemic brain lesions following carotid revascularisation procedures: a comparative study using diffusion-weighted magnetic resonance imaging

Introducción. El riesgo de embolismo cerebral tras una angioplastia-endoprótesis (AGP) es superior al encontrado tras la endarterectomía carotídea (EC) mediante estudio Doppler transcraneal. Sin embargo, no existen estudios comparativos entre ambos procedimientos de revascularización con las técnicas recientes de neuroimagen. Objetivo. Analizar la presencia y repercusión de la isquemia cerebral aguda detectada mediante resonancia magnética por difusión (RMD) tras procedimientos de revascularización carotídea. Pacientes y métodos. Se incluyeron, de forma prospectiva, 20 pacientes consecutivos con ictus aterosclerótico y estenosis carotídea sintomática superior al 70 por ciento según los criterios NASCET y tratados con EC (n = 10) o AGP (n = 10). Se les practicó una RMD en las 48 horas previas a la revascularización y otra dentro de las 72 horas posteriores, para evaluar la existencia de nuevas lesiones isquémicas cerebrales agudas. El neurólogo exploró a los pacientes antes y después del procedimiento terapéutico. Resultados. Se detectaron nuevas áreas de isquemia cerebral con RMD en un 10 por ciento de las EC y en un 40 por ciento de las AGP. El 50 por ciento de las lesiones isquémicas tras la AGP fue múltiple. El 10 por ciento de las lesiones en cada grupo terapéutico presentó una correlación clínica en forma de focalidad neurológica transitoria. No se observaron diferencias significativas respecto al perfil de riesgo de complicaciones entre los grupos terapéuticos, ni se detectaron factores asociados a un mayor riesgo de lesiones isquémicas nuevas por RMD en los pacientes sometidos a AGP. Conclusión. La AGP carotídea se asocia a nuevas lesiones isquémicas cerebrales con más frecuencia que la EC; sin embargo, estas lesiones isquémicas detectadas mediante RMD son, habitualmente, silentes. Las complicaciones sintomáticas en ambos procedimientos fueron similares (AU)

Introduction. The risk of cerebral embolism following angioplasty-stent placement (AGP) is higher than that observed after a carotid endarterectomy (CE) using transcranial Doppler ultrasonography. Nevertheless, no studies have been conducted to compare the two revascularisation procedures with the latest neuroimaging techniques. Aims. To analyse the presence and repercussion of acute cerebral ischemia detected by diffusion-weighted magnetic resonance imaging (DMR) following carotid revascularisation procedures. Patients and methods. Our prospective study included a sample of 20 consecutive patients with atherosclerotic stroke and symptomatic carotid stenosis > 70% according to NASCET criteria and treated by CE (n = 10) or AGP (n = 10). Patients were submitted to a DMR during the 48 hours prior to revascularisation and another within the 72 hours following the intervention in order to evaluate the existence of new acute cerebral ischemic lesions. Patients were explored by a neurologist before and after the intervention. Results. DMR allowed new areas of cerebral ischemia to be detected in 10% of the CE and in 40% of the AGP patients. 50% of the post-AGP ischemic lesions were multiple and 10% of the lesions in each therapeutic group presented some clinical correlation in the form of transient neurological focal signs. No significant differences were observed with respect to the pattern of risk of complications between the two therapeutic groups and no factors associated to a higher risk of new ischemic lesions were detected by DMR in patients submitted to AGP. Conclusions. Carotid AGP is linked to a higher frequency of new ischemic lesions in the brain than in the case of CE. Nevertheless, these ischemic lesions detected by DMR are usually silent. Symptomatic complications were similar in the two procedures (AU)

[Anatomical variations of the middle cerebral artery: duplication and accessory artery. Implications in the treatment of acute stroke]. / Variantes anatomicas de la arteria cerebral media: duplicidad y arteria accesoria. Implicaciones en el tratamiento del ictus agudo.

INTRODUCTION: Less than half of all subjects display a normal configuration of the Circle of Willis, according to anatomical studies. Variations of the middle cerebral artery (MCA) such as duplication or accessory MCA are infrequent but nevertheless have important clinical implications. We report the case of two patients with these variations of the MCA and their repercussions in the management of acute stroke are discussed. CASE REPORTS: Case 1: a 53 year old male with a 2 hour history of sensory motor syndrome; a transcranial Doppler (TCD) scan revealed asymmetrical speeds in the MCA. Spontaneous perforation of the MCA was suspected and we therefore decided to perform a magnetic resonance angiography scan before administering fibrinolytics. The magnetic resonance angiography scan showed an accessory MCA lying ipsilateral to the lesion. We interpreted the anomalies in blood flow detected in the TCD recording as being secondary to this anatomical variation and not due to reperfusion. Following the magnetic resonance angiography study, the possibility of fibrinolysis was ruled out. The patient recovered the neurological deficit in a matter of hours. Case 2: a 21 year old female with headaches and transient hemiparesis, who was diagnosed as suffering from migraine with aura. Later, following another stroke, it was shown that the previous clinical symptoms had been secondary to intracranial dissection with embolism in the lenticulostriate arteries and ischemic infarction in that territory. A magnetic resonance angiography scan showed duplication of the ipsilateral MCA. CONCLUSIONS: The double vascularisation of the hemisphere in cases of anatomical duplication can give rise to strokes with a better progression and prognosis, despite the occlusion of one of the MCA. The presence of anatomical variations of the MCA can lead to mistaken interpretations of the transcranial Doppler scan and may affect decision making as regards the therapy to be employed in patients with acute stroke.

Variantes anatómicas de la arteria cerebral media: duplicidad y arteria accesoria. Implicaciones en el tratamiento del ictus agudo / Anatomical variations of the middle cerebral artery: duplication and accessory artery. Implications in the treatment of acute stroke

Introducción. La conformación normal del polígono de Willis aparece en menos de la mitad de los sujetos, según estudios anatómicos. Las variantes de la arteria cerebral media (ACM) como la duplicidad o la ACM accesoria son infrecuentes, aunque con implicaciones clínicas importantes. Se presentan dos pacientes con estas variantes de la ACM y se discute su repercusión en el manejo del ictus agudo. Casos clínicos. Caso 1: varón de 53 años, con síndrome sensitivo motor de dos horas de evolución, cuya ecografía Doppler transcraneal (EDTC) mostró una asimetría de velocidades en la ACM. Ante la sospecha de reperfusión espontánea de la ACM, se decidió practicar una angiorresonancia antes de administrar fibrinolíticos. La angiorresonancia mostró una ACM accesoria ipsilateral a la lesión. Se interpretó que las anomalías de flujo detectadas en la EDTC eran secundarias a esta variante anatómica y no a reperfusión. Tras el estudio de angiorresonancia se desestimó la fibrinólisis. El paciente recuperó el déficit neurológico en el plazo de horas. Caso 2: mujer de 21 años, con cefalea y hemiparesia transitoria diagnosticada de migraña con aura. Posteriormente, a raíz de otro ictus, se demostró que el cuadro clínico previo había sido secundario a disección intracraneal con embolismo de las arterias lenticuloestriadas e infarto isquémico en dicho territorio. La angiorresonancia demostró una duplicidad de la ACM ipsilateral. Conclusiones. La doble vascularización hemisférica en casos de duplicidad anatómica puede ocasionar ictus de mejor evolución y pronóstico, a pesar de la oclusión de una de las ACM. La presencia de variantes anatómicas de la ACM puede dar lugar a interpretaciones erróneas del estudio Doppler transcraneal y afectar a la decisión terapéutica en pacientes con ictus agudo (AU)

Introduction. Less than half of all subjects display a normal configuration of the circle of Willis, according to anatomical studies. Variations of the middle cerebral artery (MCA) such as duplication or accessory MCA are infrequent but nevertheless have important clinical implications. We report the case of two patients with these variations of the MCA and their repercussions in the management of acute stroke are discussed. Case reports. Case 1: a 53-year-old male with a 2-hour history of sensory-motor syndrome; a transcranial Doppler (TCD) scan revealed asymmetrical speeds in the MCA. Spontaneous perforation of the MCA was suspected and we therefore decided to perform a magnetic resonance angiography scan before administering fibrinolytics. The magnetic resonance angiography scan showed an accessory MCA lying ipsilateral to the lesion. We interpreted the anomalies in blood flow detected in the TCD recording as being secondary to this anatomical variation and not due to reperfusion. Following the magnetic resonance angiography study, the possibility of fibrinolysis was ruled out. The patient recovered the neurological deficit in a matter of hours. Case 2: a 21-year-old female with headaches and transient hemiparesis, who was diagnosed as suffering from migraine with aura. Later, following another stroke, it was shown that the previous clinical symptoms had been secondary to intracranial dissection with embolism in the lenticulostriate arteries and ischemic infarction in that territory. A magnetic resonance angiography scan showed duplication of the ipsilateral MCA. Conclusions. The double vascularisation of the hemisphere in cases of anatomical duplication can give rise to strokes with a better progression and prognosis, despite the occlusion of one of the MCA. The presence of anatomical variations of the MCA can lead to mistaken interpretations of the TCD scan and may affect decision-making as regards the therapy to be employed in patients with acute stroke (AU)

[Chloroquine-induced myopathy and neuropathy: progressive tetraparesis with areflexia that simulates a polyradiculoneuropathy. Two case reports]. / Miopatía y neuropatía inducida por cloroquina: tetraparesia progresiva con arreflexia que simula una polirradiculoneuropatía. A propósito de dos casos.

INTRODUCTION: Chloroquine is a drug that is widely used in rheumatology and occasionally prescribed in dermatology. From a neurotoxicological point of view, chloroquine can have effects on the peripheral nerves, muscles, neuromuscular junctions and the central nervous system. In this study we analyse the clinical, neurophysiological and anatomopathological findings in two patients with chloroquine induced neuromyopathy, which took the form of a polyradiculoneuropathy. CASE REPORTS: Case 1: a 75 year old female with rheumatoid arthritis treated with daily doses of 250 mg of chloroquine for four years. The patient visited because of several months history of predominantly proximal progressive tetraparesis with areflexia. Analytical tests and lumbar puncture were normal. Electromyogram (EMG): proximal myopathic and distal neuropathic patterns. Muscular biopsy: vacuolar myopathy with accumulations of phagolysosomes, lipids, lipofuscin, myelinic curvilinear bodies. Case 2: a 74 year old female with arthropathy treated with daily doses of 250 mg of chloroquine for nine months. The patient presented a progressive proximal paraparesis with generalised areflexia. Analytical tests and lumbar puncture were normal. EMG: mixed sensory motor polyneuropathy, myogenic pattern with high frequency discharges in the iliac psoas and a neurogenic pattern in the distal muscles. Muscular biopsy: vacuolar myopathy suggesting a myopathy due to chloroquine. After stopping treatment with this drug the patients progressed favourably. CONCLUSION: Chloroquine can induce a clinical pattern that suggests a polyradiculoneuropathy. It is important to establish a history of having taken this drug. If this is indeed the case, then an electromyographic study of the most proximal muscles should be performed in order to detect a myogenic pattern and the same exploration should be applied to the distal muscles to reveal a neurogenic pattern. The final diagnosis will be established by muscular biopsy.

Miopatía y neuropatía inducida por cloroquina: tetraparesia progresiva con arreflexia que simula una polirradiculoneuropatía. A propósito de dos casos / Chloroquine-induced myopathy and neuropathy: progressive tetraparesis with areflexia that simulates a polyradiculoneuropathy. Two case reports

Introducción. La cloroquina es un fármaco ampliamente utilizado en reumatología y, ocasionalmente, en dermatología. Desde el punto de vista neurotoxicológico, la cloroquina puede afectar a los nervios periféricos, a los músculos, a la unión neuromuscular y al sistema nervioso central. En el presente trabajo se analizan los hallazgos clínicos, neurofisiológicos y anatomopatológicos de dos pacientes con una neuromiopatía inducida por cloroquina, que se manifestaron como una polirradiculoneuropatía. Casos clínicos. Caso 1. Mujer de 75 años con artritis reumatoide tratada con cloroquina 250 mg/día durante cuatro años. Consultó por tetraparesia progresiva de predominio proximal de meses de evolución con arreflexia. Las pruebas analíticas y la punción lumbar fueron normales. Electromiograma (EMG): patrones miopáticos proximales y neuropáticos distales. Biopsia muscular: miopatía vacuolar con acúmulo de fagolisosomas, lípidos, lipofucsina, cuerpos mielínicos y curvilíneos. Caso 2. Mujer de 74 años con artropatía tratada con cloroquina 250 mg/día durante nueve meses. Presentó una paraparesia proximal progresiva con arreflexia universal. Las pruebas analíticas y la punción lumbar fueron normales. EMG: polineuropatia mixta sensitivomotora, patrón miógeno con descargas de alta frecuencia en psoas ilíaco y patrón neurógeno en los músculos distales. Biopsia muscular: miopatía vacuolar sugestiva de miopatía por cloroquina. Tras la retirada de la medicación presentaron una evolución favorable de la clínica. Conclusión. La cloroquina puede inducir un cuadro clínico sugestivo de polirradiculoneuropatía. Es importante interrogar sobre el antecedente de ingesta del fármaco. En caso positivo tiene interés el estudio electromiográfico de los músculos más proximales para detectar patrón miógeno y de los músculos distales para evidenciar patrón neurógeno. La biopsia muscular establecerá el diagnóstico definitivo (AU)

Chloroquine is a drug that is widely used in rheumatology and occasionally prescribed in dermatology. From a neurotoxicological point of view, chloroquine can have effects on the peripheral nerves, muscles, neuromuscular junctions and the central nervous system. In this study we analyse the clinical, neurophysiological and anatomopathological findings in two patients with chloroquine-induced neuromyopathy, which took the form of a polyradiculoneuropathy. Case reports. Case 1: a 75-year-old female with rheumatoid arthritis treated with daily doses of 250 mg of chloroquine for four years. The patient visited because of several months’ history of predominantly proximal progressive tetraparesis with areflexia. Analytical tests and lumbar puncture were normal. Electromyogram (EMG): proximal myopathic and distal neuropathic patterns. Muscular biopsy: vacuolar myopathy with accumulations of phagolysosomes, lipids, lipofuscin, myelinic curvilinear bodies. Case 2: a 74-year-old female with arthropathy treated with daily doses of 250 mg of chloroquine for nine months. The patient presented a progressive proximal paraparesis with generalised areflexia. Analytical tests and lumbar puncture were normal. EMG: mixed sensory-motor polyneuropathy, myogenic pattern with high frequency discharges in the iliac psoas and a neurogenic pattern in the distal muscles. Muscular biopsy: vacuolar myopathy suggesting a myopathy due to chloroquine. After stopping treatment with this drug the patients progressed favourably. Conclusion. Chloroquine can induce a clinical pattern that suggests a polyradiculoneuropathy. It is important to establish a history of having taken this drug. If this is indeed the case, then an electromyographic study of the most proximal muscles should be performed in order to detect a myogenic pattern and the same exploration should be applied to the distal muscles to reveal a neurogenic pattern. The final diagnosis will be established by muscular biopsy (AU)