[Paraneoplastic limbic encephalitis and epidermoid carcinoma of the pyriform]. / Encefalitis límbica paraneoplásica y carcinoma epidermoide del seno piriforme.

Paraneoplastic limbic encephalitis is a syndrome characterised by behaviour changes, short-term memory loss and seizures. We report on a case of a 62-year-old man, ex-smoker who was admitted to the Intensive Care Unit with seizures, and who had previously experienced behaviour changes. Tests revealed nothing notable, except for the CSF examination which revealed elevated protein and MRI findings indicating temporal lobe abnormalities. Once other diagnoses had been ruled out, neoplasm screening was initiated. Conventional imaging failed to identify the tumour. Antineuronal anti-Hu antibodies were negative. Finally, fluorodeoxyglucose (FDG) whole body -PET imaging was carried out revealing a pathologically increased glucose metabolism at the pharynx-larynx level. The biopsy showed an epidermoid carcinoma of the pyriform sinus. To the best of our knowledge, this is the first reported case of an epidermoid carcinoma of the pyriform sinus associated with paraneoplastic limbic encephalitis.

Atrofia muscular peroneal secundaria a hernia discal dorsal / Peroneal muscular atrophy secondary to a herniated dorsal disc

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Encefalitis límbica paraneoplásica y carcinoma epidermoide del seno piriforme / Paraneoplastic limbic encephalitis and epidermoid carcinoma of the pyriform sinus

La encefalitis límbica paraneoplásica es un síndrome que se caracteriza clínicamente por alteraciones de comportamiento, trastornos de memoria reciente y crisis epilépticas. Presentamos el caso clínico de un varón de 62 años, ex-fumador, que ingresa en UCI por un cuadro de crisis convulsivas, presentando anteriormente cambios en el carácter. Los estudios practicados resultaron negativos a excepción de LCR con hiperproteinorraquia y RNM que mostraba lesiones en el lóbulo temporal . Una vez descartadas otras posibles etiologías, iniciamos despistaje de neoplasia oculta. No se evidenció tumor en las pruebas de imagen habituales. Los anticuerpos anti-Hu resultaron negativos. Se realizó tomografía de emisión de positrones (TEP) de cuerpo entero que reveló un foco de hipercaptación a nivel faringolaríngeo. La biopsia fue diagnóstica de carcinoma epidermoide del seno piriforme. En nuestro conocimiento es el primer caso de ELP asociada a carcinoma epidermoide del seno piriforme

Paraneoplastic limbic encephalitis is a syndrome characterised by behaviour changes, short-term memory loss and seizures. We report on a case of a 62-year-old man, ex-smoker who was admitted to the Intensive Care Unit with seizures, and who had previously experienced behaviour changes. Tests revealed nothing notable, except for the CSF examination which revealed elevated protein and MRI findings indicating temporal lobe abnormalities. Once other diagnoses had been ruled out, neoplasm screening was initiated. Conventional imaging failed to identify the tumour. Antineuronal anti-Hu antibodies were negative. Finally, fluorodeoxyglucose (FDG) whole body -PET imaging was carried out revealing a pathologically increased glucose metabolism at the pharynx-larynx level. The biopsy showed an epidermoid carcinoma of the pyriform sinus. To the best of our knowledge, this is the first reported case of an epidermoid carcinoma of the pyriform sinus associated with paraneoplastic limbic encephalitis

Mielorradiculopatía lumbosacra como manifestación a distancia de fístula dural dorsal / Lumbosacral radiculomyelopathy as a remote symptom of a dural fistula at the dorsal level

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[Headache and fainting as initial symptoms of syringomyelia associated to Arnold-Chiari and facial angiomatous nevus]. / Cefalea y síncope como manifestaciones iniciales de siringomielia asociada a Arnold-Chiari y nevo angiomatoso facial.

INTRODUCTION: Syringomyelia and Arnold-Chiari malformation are two dysraphism that often appear together. Clinical manifestations are diverse although some patients remain asymptomatic for years. Syncope, supposedly due to a dysfunction of medulla baroceptors and spinal cord intermediolateral horn, is uncommon, particularly as the initial symptom. CASE REPORT: 34 year old woman, with a facial angiomatous nevus, presented with two episodes of headache followed by syncope; neurological examination was apparently normal. MRI showed mild supratentorial hydrocephalus, Arnold-Chiari malformation and cervical syringomyelia, with no vascular anomalies. CONCLUSION: This case, which shares some features with Sturge-Weber syndrome and PHACE syndrome, enhances the importance of the search for anomalies in patients with paroxistical symptoms and cutaneous lesions.

Cefalea y síncope como manifestaciones iniciales de siringomielia asociada a Arnold-Chiari y nevo angiomatoso facial / Headache and fainting as initial symptoms of syringomyelia associated to arnold-chiari and facial angiomatous nevus

Introducción. La siringomielia y la malformación de Arnold-Chiari son dos malformaciones disráficas que frecuentemente se presentan asociadas; sus manifestaciones clínicas son muy variadas y algunos pacientes permanecen asintomáticos durante años. El síncope, atribuido a una disfunción de los baroceptores bulbares y del asta intermediolateral medular es una manifestación paroxística poco habitual, especialmente como forma de inicio. Caso clínico. Una mujer de 34 años, portadora de un nevo angiomatoso facial, consultó por presentar dos episodios de cefalea y síncope. La exploración neurológica resultó normal. El estudio de resonancia magnética demostró una moderada hidrocefalia supratentorial, malformación de Arnold-Chiari y siringomielia cervical, sin observarse alteraciones vasculares. Conclusión. Este caso, que comparte algunas características del síndrome de Sturge-Weber y el síndrome PHACE, es un ejemplo de la importancia de investigar alteraciones del sistema nervioso central en pacientes con síntomas paroxísticos y alteraciones cutáneas (AU)

Introduction. Syringomyelia and Arnold-Chiari malformation are two dysraphism that often appear together. Clinical manifestations are diverse although some patients remain asymptomatic for years. Syncope, supposedly due to a dysfunction of medulla baroceptors and spinal cord intermediolateral horn, is uncommon, particularly as the initial symptom. Case report. 34 year old woman, with a facial angiomatous nevus, presented with two episodes of headache followed by syncope; neurological examination was apparently normal. MRI showed mild supratentorial hydrocephalus, Arnold-Chiari malformation and cervical syringomyelia, with no vascular anomalies. Conclusion. This case, which shares some features with Sturge-Weber syndrome and PHACE syndrome, enhances the importance of the search for anomalies in patients with paroxistical symptoms and cutaneous lesions (AU)

[Rapidly progressing dementia as the presenting symptom of Waldenstrom's macroglobulinemia: findings from magnetic resonance imaging of the brain in Bing Neel syndrome]. / Demencia rapidamente progresiva como presentacion de macroglobulinemia de Waldenstrom: hallazgos de la resonancia magnetica cerebral en el sindrome de Bing Neel.

CASE REPORT: A 68 year old male with no relevant clinical history was admitted to hospital because of symptoms of cognitive impairment (attentional deficit, short term memory disorders and behavioural disorders), accompanied by apraxia of gait and rectal and urinary incontinence. Results of a general clinical exploration were normal. In the MRI study of the brain numerous areas of hypersignal were observed in different arterial territories, which stood out in the T2, FLAIR and, above all, in diffusion weighted sequences. Analytical studies showed hypergammaglobulinemia with monoclonal IgM k and a bone marrow biopsy revealed infiltration by plasmatic cells. Bing Neel syndrome was diagnosed. CONCLUSIONS: We underline this exceptional form of presentation of Waldenstrom's macroglobulinemia and highlight the findings of diffusion weighted MRI, which suggested multiple infarcts for which a neoplastic vascular obstruction mechanism similar to that involved in malignant angioendotheliomatosis is posited.

Demencia rápidamente progresiva como presentación de una macroglobulinemia de Waldenström: hallazgos de la resonancia magnética cerebral en el síndrome de Bing-Neel / Rapidly progressing dementia as the presenting symptom of Waldenström's macroglobulinemia: findings from magnetic resonance imaging of the brain in Bing-Neel syndrome

Caso clínico. Un varón de 68 años, sin antecedentes relevantes, ingresó por un cuadro subagudo de deterioro cognitivo (déficit atencional, alteración de la memoria a corto plazo y trastornos de la conducta), acompañado de apraxia de la marcha e incontinencia de esfínteres. La exploración clínica general resultó normal. En el estudio de RM cerebral se observaron múltiples áreas de hiperseñal, localizadas en distintos territorios arteriales, que brillaban en las secuencias T2, FLAIR y, sobre todo, en la difusión. Los estudios analíticos demostraron hipergammaglobulinemia con IgM- monoclonal, y la biopsia de médula ósea, infiltración por células plasmáticas. Se diagnosticó de síndrome de Bing-Neel. Conclusión. Se subraya esta excepcional forma de presentación de la macroglobulinemia de Waldenström y se destacan los hallazgos de la RM-difusión, sugestivos de infartos múltiples, para los que se postula un mecanismo de obstrucción vascular neoplásica similar al de la angioendoteliomatosis maligna (AU)