Miositis osificante en el triángulo de Petit. Caso clínico / Myositis ossificans on Petit triangle. Case report

La miositis osificante es una osificación heterotópica, autolimitada, que afecta a cualquier grupo de edad y suele presentarse en la musculatura de las extremidades, en su variante más frecuente, en zonas de traumatismo por su relación íntima con la lesión mecánica. Es considerada benigna y si la identificación por imágenes es clara, su tratamiento consiste en seguimiento clínico. Sin embargo, cuando existen dudas diagnósticas, conviene realizar biopsia para definir el tratamiento quirúrgico. Presentamos una paciente con tumoración dolorosa en región lumbar, a nivel del triángulo de Petit, de 18 meses de evolución. La biopsia de partes blandas era sugerente de tumor fibromixoide osificante cuyo análisis postoperatorio reveló miositis osificante. Se realizó extirpación local amplia y reconstrucción con malla biológica sin tensión y cierre por planos, ya que se han descrito hernias espontáneas y también secundarias a través del triángulo de Petit. Estas últimas, en su mayoría, son hernias postincisionales

Myositis ossificans is a heterotopic self-limited ossification that affects every age. It usually appears in limb musculature, commonly in exposed areas due to its intimate relationship with mechanical trauma. It is considered a benign disease and, if clinical and radiological identification results obvious, suggested treatment is clinical follow-up. But if there is a diagnostic doubt, a biopsy must be performed in order to define surgical treatment. We present a case report of a patient with an 18 month-evolution painful tumour in the lumbar area, at the level of Petit triangle. The initial biopsy of the soft tissue mass suggested fibromyxoid ossificans tumour, but after postoperative analysis, diagnosis of myositis ossificans was stated. Surgical treatment consisted of a wide local resection and immediate reconstruction with a biological mesh, achieving a non-tension closure in order to avoid spontaneous and secondary hernias, which are described specifically in the triangle of Petit triangle. These hernias are usually post-incisional

Perfil de consumo de antiepilépticos en los pacientes epilépticos del Área Oeste de Málaga / Consumption patterns of antiepileptic drugs among epileptic patients in the Western Málaga district

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Percheron artery syndrome: variability in presentation and differential diagnosis.

INTRODUCTION: Synchronous bilateral paramedian thalamic stroke (SBPTS), usually equated to Percheron artery infarction, is considered to be uncommon and difficult to diagnose clinically. Its characterization is based on the original description plus a few small series. AIM: To characterize SBPTS clinically by collecting cases and identifying the key difficulties for an early diagnosis. PATIENTS AND METHODS: Six cases at our centre plus another 115 located by systematic literature search and critical reading of articles fulfilled the criteria for SBPTS. An analysis was made of the variables age, gender, vascular risk factors, aetiology, alterations and fluctuations of consciousness, need for intubation, cognitive-behavioural disorders, pupillary changes, other neurological focal disorders and brainstem involvement on imaging studies. RESULTS: Of note in our series were disorders of consciousness (n=5), their fluctuations (n=3) and the diagnostic delay (seven days, with MRI in four patients). In only one case was a bilateral thalamic lesion seen on the initial CT. Joint analysis of all the cases showed a mean age of 61 years, a predominance of men (58%), the presence of vascular risk factors in 77%, a mainly cardioembolic aetiology (34% among those that were specified), sensory involvement in 75% (intubation in 7% and fluctuations in 16.5%), cognitive-behavioural disorders in 43%, oculomotor in 73%, pupillary in 31%, other in 67% and specified brainstem lesion in 37%. CONCLUSIONS: The SBPTS syndrome has a variable presentation with a low sensitivity on the initial CT, requiring brain MRI for typification. This explains the diagnostic difficulty and the fact that its frequency is probably underestimated.

Síndrome de hiperinsulinismo-hiperamoniemia y epilepsia mioclónica grave de la infancia / Hyperinsulinism and hyperammonaemia syndrome and severe myoclonic epilepsy of infancy

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Diseño del instrumento de ayuda para la toma de decisiones: “alternativas de tratamiento para el cáncer de mama: ¿qué opción prefiero? / Design a decision-making aid: “alternative treatment for breast cancer: What option do you prefer?

Objetivo: Diseñar un Instrumento de Ayuda para la Toma de Decisiones (IATD) en el Proceso Asistencial Integrado ‘Cáncer de mama’ del Sistema Sanitario Público de Andalucía (SSPA) para el abordaje terapéutico de esta enfermedad en estadio inicial. Método: El diseño del IATD se realizó en cuatro fases: 1) Explorar la receptividad de las usuarias y los profesionales del SSPA sobre la incorporación de IATD en el proceso “Cáncer de mama”. 2). Seleccionar un IATD entre las experiencias internacionales.; 3) Adaptar transculturalmente del IATD seleccionado al entorno del SSPA. 4) Validar el IATD en el SSPA. Resultado: El IATD “Alternativas de tratamiento para el cáncer de mama: ¿Qué opción prefiero?” diseñado para el SSPA incluye contenidos innovadores frente a otras experiencias revisadas. Los resultados de la validación del IATD han mostrado que su diseño es atractivo para la paciente, su extensión y lenguaje idóneos, y la información clínica que contiene es de calidad. El Instrumento resuelve sus dudas (95%) y resume la información esencial para tomar la decisión (90%). El IATD ofrece información relevante que prepara a la paciente para la toma de decisiones (ausencia de conflicto decisional: 85,31), facilita la labor en consulta y la comunicación médico-paciente. Conclusiones: Pacientes y profesionales coinciden en recomendar la utilización del IATD y fomentar la participación en la toma de decisiones aunque reconocen que el factor tiempo es el principal obstáculo para incorporar su uso en el SSPA. (AU)

Purpose: To design a Decision-making Aid within the ‘Breast cancer’ healthcare process modelling of the Andalusian Public Health System (SSPA) for the therapeutic approach of early-stage disease. Methods: The Decision Aid design was conducted in four phases: 1) Explore the receptiveness of users and professionals in the mainstream of the SSPA Decision Aid “Breast Cancer” process. 2) Select a Decision Aid from international experiences. 3) Transcultural adaptation of above selected Decision Aid. 4) Decision Aid Validation in the SSPA. Results: The Decision Aid “Alternative treatment for breast cancer: What option do I prefer?” designed for the SSPA includes innovative contents compared to other reviewed experiences. The results of the validation of Decision Aid have shown that the design is attractive for the patient, ideal size and suitable language, and that the clinical information it contains is of quality. The Decision Aid an- swers your questions (95%) and summarizes the essential information to make the decision (90%). The Decision Aid offers relevant information that help the patient in the decision making process (lack of decisional conflict: 85.31), facilitates the work in the practice and doctor-patient communication. Results: The Decision Aid “Alternative treatment for breast cancer: What option do I prefer?” designed for the SSPA includes innovative contents compared to other reviewed experiences. The results of the validation of Decision Aid have shown that the design is attractive for the patient, ideal size and suitable language, and that the clinical information it contains is of quality. The Decision Aid an- swers your questions (95%) and summarizes the essential information to make the decision (90%). The Decision Aid offers relevant information that help the patient in the decision making process (lack of decisional conflict: 85.31), facilitates the work in the practice and doctor-patient communication. Conclusion: Patients and professionals agree to recommend the use of Decision Aid and to encourage participation in decision making while recognizing that the time factor is the main obstacle to incorporate its use in the SSPA (AU)

Síndrome de Déjerine-Roussy de origen isquémico en un adolescente con foramen oval permeable / Déjerine- Roussy Syndrome of an ischaemic origin in an adolescent with patent forament ovale

Resumen. Introducción. El síndrome de Déjerine-Roussy o síndrome talámico se caracteriza por hemiparesia leve transitoria, hemicoreoatetosis, hemihipoestesia, hiperalgesia, alodinia y hemiataxia con astereognosia de intensidad variable, y se presenta ante lesiones de los núcleos posteriores del tálamo. Puede producirse por infarto cerebral estratégico, descrito en pacientes de edad avanzada con factores de riesgo vascular. El foramen oval permeable se ha sugerido como factor de riesgo de ictus isquémico en jóvenes, especialmente cuando se asocia a ‘aneurisma’ del septo auricular y sobre todo a estado procoagulante. Caso clínico. Varón de 18 años de edad con antecedentes familiares de enfermedad de Behçet, que presenta infartos cerebrales talámico e hipocampal derechos, siendo un foramen oval persistente con ‘aneurisma’ septal el único factor de riesgo encontrado tras un estudio exhaustivo. En ese momento no cumplía los criterios de la enfermedad de Behçet, y tras un amplio rastreo sistémico no se hallaron signos directos ni indirectos de trombosis venosa. Se realizó cierre percutáneo del foramen. Conclusión. Se trata del primer caso publicado de síndrome de Déjerine-Roussy como manifestación de infarto cerebral criptogénico asociado a foramen oval permeable en un adolescente. El conjunto de datos clínicos y complementarios permiten realizar una reconstrucción de la secuencia fisiopatológica que sitúan al foramen oval con ‘aneurisma’ septal asociado como único factor de riesgo objetivable, lo que asociado al estrés del paciente y de la familia motivó su cierre (AU)

Summary. Introduction. Déjerine-Roussy syndrome, or thalamic syndrome, is characterised by transient mild hemiparesis, hemichoreoathetosis, hemihypoesthesia, hyperalgesia, allodynia and hemiataxia with astereognosia that varies in intensity, and it appears in the presence of lesions in the posterior nuclei of the thalamus. It can be produced by strategic cerebral infarction, reported in elderly patients with vascular risk factors. Patent foramen ovale has been suggested as a risk factor for ischaemic stroke in young people, especially when associated to aneurysm of the auricular septum and above all to a procoagulating status. Case report. An 18-year-old male with a family history of Behçet’s disease, who presented right-side thalamic and hippocampal cerebral infarction; following an exhaustive study, patent foramen ovale with septal aneurysm was found as the only risk factor. At that time he did not satisfy criteria for Behçet’s disease, and thorough systemic screening did not reveal direct or indirect signs of venous thrombosis. Percutaneous closure of the foramen was performed. Conclusions. This is the first reported case of Déjerine-Roussy syndrome as a manifestation of cryptogenic cerebral infarction associated to patent foramen ovale in an adolescent. Taken as a whole, the clinical and complementary data enable us to reconstruct the pathophysiological sequence that position foramen ovale with an associated septal ‘aneurysm’ as the only detectable risk factor, which, when linked to the stress of the patient and the family, triggered its early closure (AU)

[Déjerine-Roussy syndrome of an ischaemic origin in an adolescent with patent foramen ovale]. / Síndrome de Déjerine-Roussy de origen isquémico en un adolescente con foramen oval permeable.

INTRODUCTION: Déjerine-Roussy syndrome, or thalamic syndrome, is characterised by transient mild hemiparesis, hemichoreoathetosis, hemihypoesthesia, hyperalgesia, allodynia and hemiataxia with astereognosia that varies in intensity, and it appears in the presence of lesions in the posterior nuclei of the thalamus. It can be produced by strategic cerebral infarction, reported in elderly patients with vascular risk factors. Patent foramen ovale has been suggested as a risk factor for ischaemic stroke in young people, especially when associated to aneurysm of the auricular septum and above all to a procoagulating status. CASE REPORT: An 18-year-old male with a family history of Behçet's disease, who presented right-side thalamic and hippocampal cerebral infarction; following an exhaustive study, patent foramen ovale with septal aneurysm was found as the only risk factor. At that time he did not satisfy criteria for Behçet's disease, and thorough systemic screening did not reveal direct or indirect signs of venous thrombosis. Percutaneous closure of the foramen was performed. CONCLUSIONS: This is the first reported case of Déjerine-Roussy syndrome as a manifestation of cryptogenic cerebral infarction associated to patent foramen ovale in an adolescent. Taken as a whole, the clinical and complementary data enable us to reconstruct the pathophysiological sequence that position foramen ovale with an associated septal 'aneurysm' as the only detectable risk factor, which, when linked to the stress of the patient and the family, triggered its early closure.

Infarto lacunar talámico con síndrome sensitivo puro como presentación de mixoma auricular / No disponible

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Síndrome de Guillain Barré asociado a anestesia epidural / No disponible

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[Miller Fisher syndrome as the presenting symptom of Wernicke's encephalopathy]. / Síndrome de Miller Fisher como presentación de una encefalopatía de Wernicke.

INTRODUCTION: Wernicke's encephalopathy is an acute neuropsychiatric syndrome resulting from a thiamine deficit, which is defined by the characteristic triad of confusion, ophthalmoparesis and ataxia, although rare presentations have been reported that delay its diagnosis. Miller Fisher syndrome is characterised by the triad ophthalmoparesis, ataxia and areflexia and is considered to be a variant of Guillain-Barré syndrome; its differential diagnosis includes Wernicke's encephalopathy. CASE REPORT: A 75-year-old female with chronic digestive disorders, who developed an acute picture of bilateral internuclear ophthalmoplegia, ataxia and areflexia, with proteinocytologic dissociation in cerebrospinal fluid; accordingly, an initial diagnosis of Miller Fisher syndrome was proposed. Results of the neurophysiological studies were normal; anti-GQ1b antibodies were negative; and magnetic resonance imaging of the brain suggested Wernicke's encephalopathy. The response to thiamine was spectacular. CONCLUSIONS: The similarities in the distribution of the lesions of the two conditions, in the signs and symptoms and the lab findings, as well as the influence of certain misleading factors (hyponatremia, advanced age), went to make up a typical syndrome that favoured a wrong presumptive aetiological diagnosis. This was corrected at an early stage, however, in light of the results of certain diagnostic tests and after observing the therapeutic response. In addition to being an atypical presentation for Wernicke's encephalopathy, this case highlights the fact that for there to be an agreement between the syndromic and aetiological diagnoses it is necessary to carry out a correct differential diagnosis based on details from the patient's history, on appropriate complementary tests and on the follow-up study of how the patients progress, even when we come across typical syndromes that are usually related to a predominant aetiopathogenesis.

Síndrome de Miller Fisher como presentación de una encefalopatía de Wernicke / Miller fisher syndrome as the presenting symptom of wernicke’s encephalopathy

La encefalopatía de Wernicke es un síndrome neuropsiquiátrico agudo secundario a un déficit de tiamina, definido por la tríada característica de confusión, oftalmoparesia y ataxia, aunque se han descrito presentaciones raras que retrasan el diagnóstico. El síndrome de Miller Fisher se caracteriza por la tríada de oftalmoparesia, ataxia y arreflexia,y se considera una variante del síndrome de Guillain-Barré; su diagnóstico diferencial incluye la encefalopatía de Wernicke. Caso clínico. Mujer de 75 años con trastorno digestivo crónico, que desarrolla un cuadro agudo de oftalmoplejía internuclearbilateral, ataxia y arreflexia, con disociación proteinocitológica en el líquido cefalorraquídeo, por lo que se propuso el diagnóstico inicial de síndrome de Miller Fisher. Los estudios neurofisiológicos fueron normales, los anticuerpos anti-GQ1b,negativos, y la resonancia magnética cerebral sugirió una encefalopatía de Wernicke; la respuesta a la tiamina fue espectacular. Conclusiones. Las similitudes en la distribución lesional de ambas entidades, en la semiología y los resultados analíticos, así como la influencia de ciertos factores de confusión (hiponatremia, edad avanzada), conformaron un síndrome típico que favoreció un diagnóstico de sospecha etiológico erróneo, que pudo modificarse precozmente a la luz de ciertas pruebas diagnósticasy después de la respuesta terapéutica. Además de tratarse de una presentación atípica para una encefalopatía de Wernicke, este caso recuerda que la conjunción del diagnóstico sindrómico con el etiológico pasa por realizar un correcto diagnóstico diferencial apoyándonos en detalles de la anamnesis, en las pruebas complementarias necesarias y en el seguimientoevolutivo de los pacientes, incluso cuando nos encontramos ante síndromes típicos habitualmente relacionados con una etiopatogenia predominante

Wernicke’s encephalopathy is an acute neuropsychiatric syndrome resulting from a thiamine deficit,which is defined by the characteristic triad of confusion, ophthalmoparesis and ataxia, although rare presentations have been reported that delay its diagnosis. Miller Fisher syndrome is characterised by the triad ophthalmoparesis, ataxia and areflexiaand is considered to be a variant of Guillain-Barré syndrome; its differential diagnosis includes Wernicke’s encephalopathy. Case report. A 75-year-old female with chronic digestive disorders, who developed an acute picture of bilateral internuclear ophthalmoplegia, ataxia and areflexia, with proteinocytologic dissociation in cerebrospinal fluid; accordingly, an initial diagnosis of Miller Fisher syndrome was proposed. Results of the neurophysiological studies were normal; anti-GQ1b antibodies were negative; and magnetic resonance imaging of the brain suggested Wernicke’s encephalopathy. The response to thiamine was spectacular. Conclusions. The similarities in the distribution of the lesions of the two conditions, in the signs and symptoms and the lab findings, as well as the influence of certain misleading factors (hyponatremia, advanced age), wentto make up a typical syndrome that favoured a wrong presumptive aetiological diagnosis. This was corrected at an early stage, however, in light of the results of certain diagnostic tests and after observing the therapeutic response. In addition to being anatypical presentation for Wernicke’s encephalopathy, this case highlights the fact that for there to be an agreement between the syndromic and aetiological diagnoses it is necessary to carry out a correct differential diagnosis based on details from thepatient’s history, on appropriate complementary tests and on the follow-up study of how the patients progress, even when we come across typical syndromes that are usually related to a predominant aetiopathogenesis

Réplica. Ausencias y ‘pseudoausencias’ frontales / Frontal absences and ‘pseudo-absences’. Reply

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Ausencias frontales: presentación de un caso / Frontal absences: a case report

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