Origen común de arterias carótidas y subclavias como variante anatómica excepcional del arco aórtico / Common origin of carotid and subclavian arteries as an exceptional anatomical variant of the aortic arch

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[Recurrent painful ophthalmoplegia secondary to polyostotic fibrous dysplasia of the maxillary sinuses with involvement of the superior orbital fissure]. / Oftalmoplejia dolorosa recurrente secundaria a displasia fibrosa poliostotica del seno maxilar con afectacion de la hendidura esfenoidal.

INTRODUCTION: Fibrous dysplasia is a bone disease that is usually accompanied by asymptomatic lesions but which may sometimes display neurological manifestations due to the involvement of the craniofacial bones. CASE REPORT: A 25-year-old female, with a history of migraines, who visited at the age of 18 due to pain in the right retro-ocular and maxillary region, although with characteristics that were unlike those of her usual migraines, and which was associated with ipsilateral ophthalmoparesis. The condition had a self-limiting course and responded well to corticoids, although it was recurring. Examination revealed intense pain on palpation of the right-hand maxillary sinus and incomplete paralysis of the ipsilateral oculomotor nerve with palpebral ptosis. Results of complementary studies were normal, except for magnetic resonance imaging of the head and computerised axial tomography of the face, which revealed an expansive lesion with involvement of the right superior maxillary sinus and the greater wing of the sphenoid bone, with probable compromise of the superior orbital fissure, consistent with the diagnosis of fibrous dysplasia, which was confirmed by means of a pathology study. CONCLUSIONS: Fibrous dysplasia is a benign bone disorder, of unknown causation, in which normal bone tissue is replaced by amorphous conjunctive tissue. There is sometimes craniofacial involvement and a hypertrophic bone mass is formed which can fill the paranasal sinuses and the orbit, resulting in exophthalmus and visual disorders. To date the scientific literature does not include any reports of this disease manifesting as bouts of recurrent painful ophthalmoparesis which responds to corticoids, as happened in the case of our patient.

Versión española del test perfil de síntomas autonómicos / Spanish version of the autonomic symptom profile test

Resumen. Introducción. El perfil de síntomas autonómicos (PSA) es un cuestionario autorrellenable, que ha sido validado en lengua inglesa para valorar los síntomas autonómicos y diferenciar entre pacientes y sujetos sanos según dicha sintomatología. Nuestro grupo ha estudiado su aplicación en lengua castellana. Sujetos y métodos. Después de traducir al castellano la versión inglesa y retrotraducirla, se evaluó la fiabilidad y la validez de criterio de la versión española del test PSA en una muestra de 50 sujetos (27 controles y 23 enfermos). A todos los pacientes con síntomas de disfunción autonómica se les estudió mediante la batería de Ewing y Clarke. Resultados. La fiabilidad de la versión española del PSA fue alta, y se obtuvo un coeficiente de correlación intraclase de 0,93. Con una puntuación en el test de 47 o superior, se obtiene una sensibilidad del 73,9% y una especificidad del 100% para detectar disfunción autonómica. Además, la puntuación de la versión española del test PSA mostró diferencias estadísticamente significativas entre el grupo de pacientes y el grupo control. Conclusiones. La versión española del PSA es fiable y permite distinguir entre pacientes con disfunción autonómica y sujetos control. En nuestro conocimiento, se trata del primer test que valora la sintomatología del sistema nervioso autónomo de forma global y en castellano (AU)

Summary. Introduction. The Autonomic Symptom Profile (ASP) is a self-administered questionnaire that has been validated in English to assess autonomic symptoms and to differentiate between patients and healthy subjects according to said symptoms. Our group has studied its application in Spanish. Subjects and methods. After translating the English version into Spanish and then back-translating it, the reliability and validity of criteria in the Spanish version of the ASP test were evaluated in a sample of 50 subjects (27 controls and 23 patients). All the patients with symptoms of autonomic dysfunction were studied by means of the Ewing and Clarke battery. Results. The reliability of the Spanish version of the ASP was high and an interclass correlation coefficient of 0.93 was obtained. A score of 47 or over in the test yields a sensitivity of 73.9% and a specificity of 100% for the detection of autonomic dysfunction. Furthermore, the score in the Spanish version of the ASP test showed statistically significant differences between the group of patients and the control group. Conclusions. The Spanish version of the ASP is reliable and makes it possible to distinguish between patients with autonomic dysfunction and control subjects. As far as we are aware, this is the first test to evaluate the symptoms of the autonomic nervous system in a global manner and in Spanish (AU)

[Spanish version of the Autonomic Symptom Profile test]. / Versión española del test perfil de síntomas autonómicos.

INTRODUCTION: The Autonomic Symptom Profile (ASP) is a self-administered questionnaire that has been validated in English to assess autonomic symptoms and to differentiate between patients and healthy subjects according to said symptoms. Our group has studied its application in Spanish. SUBJECTS AND METHODS: After translating the English version into Spanish and then back-translating it, the reliability and validity of criteria in the Spanish version of the ASP test were evaluated in a sample of 50 subjects (27 controls and 23 patients). All the patients with symptoms of autonomic dysfunction were studied by means of the Ewing and Clarke battery. RESULTS: The reliability of the Spanish version of the ASP was high and an interclass correlation coefficient of 0.93 was obtained. A score of 47 or over in the test yields a sensitivity of 73.9% and a specificity of 100% for the detection of autonomic dysfunction. Furthermore, the score in the Spanish version of the ASP test showed statistically significant differences between the group of patients and the control group. CONCLUSIONS: The Spanish version of the ASP is reliable and makes it possible to distinguish between patients with autonomic dysfunction and control subjects. As far as we are aware, this is the first test to evaluate the symptoms of the autonomic nervous system in a global manner and in Spanish.

[Lepra: an uncommon cause of infectious neuropathy]. / Lepra: causa infrecuente de neuropatía potencialmente tratable.

INTRODUCTION: Lepra is an uncommon disease within our setting. However, it was considered the most frequent cause of polyneuropathy only 50 years ago. CASE REPORT: We present the case of a 37 year-old woman who consulted due to paresthesias in both hands and feet, livedo reticularis and complaints of frequent hand lesions. Examination of the skin detected nodular lesions and the neurophysiological study confirmed distal symmetric sensitive polyneuropathy with axonal predominance. The skin biopsy also showed histocytic infiltrate and mycobacterium lepra type intracytoplasmatic bacilli. This led to the diagnosis of Multibacillary lepromatous leprosy. Multiple treatment was begun, according to the World Health Organization recommendations, with good evolution. In spite of the attempts to eradicate lepra, its incidence continues to be elevated, especially in endemic areas, among which the south of Spain is included. It is generally associated to overcrowding and low social-economic level. CONCLUSIONS: This is the last endemic case in the Valencian Community. The possibility of lepra should be considered among the possible causes of sensitive polyneuropathy, above all in patients in endemic areas.

Lepra: causa infrecuente de neuropatía potencialmente tratable / Lepra: an uncommon cause of infectious neuropathy

Introducción. La lepra es una enfermedad poco frecuente en nuestro entorno; sin embargo, hace tan sólo 50 años era considerada la causa más frecuente de polineuropatía. Caso clínico. Presentamos el caso de una mujer de 37 años que consulta por parestesias en manos y pies, livedo reticularis y quejas de frecuentes ulceraciones en las manos. En la exploración cútanea se detectaron lesiones nodulares y el estudio neurofisiológico puso de manifiesto una polineuropatía sensitiva simétrica distal de predominio axonal. La biopsia cutánea mostró un infiltrado histocitario y bacilos intracitoplasmáticos de tipo Mycobacterium leprae, lo que condujo al diagnóstico de lepra lepromatosa multibacilar. Se instauró tratamiento con politerapia, según recomendaciones de la Organización Mundial de la Salud, con buena evolución. A pesar de los intentos de erradicación de la lepra, su incidencia continúa siendo elevada, especialmente en áreas endémicas, entre las que se incluye el sur de España y, generalmente, asociada a hacinamiento y bajo nivel socioeconómico. Conclusiones. Éste es el último caso autóctono comunicado en la Comunidad Valenciana. Entre las posibles causas de polineuropatía sensitiva debe tenerse en cuenta todavía la posibilidad de la lepra, sobre todo en pacientes procedentes de áreas endémicas (AU)

Introduction: Lepra is an uncommon disease within our setting. However, it was considered the most frequent cause of polyneuropathy only 50 years ago. Case Report: We present the case of a 37 year-old woman who consulted due to paresthesias in both hands and feet, livedo reticularis and complaints of frequent hand lesions. Examination of the skin detected nodular lesions and the neurophysiological study confirmed distal symmetric sensitive polyneuropathy with axonal predominance. The skin biopsy also showed histocytic infiltrate and mycobacterium lepra type intracytoplasmatic bacilli. This led to the diagnosis of Multibacillary lepromatous leprosy. Multiple treatment was begun, according to the World Health Organization recommendations, with good evolution. In spite of the attempts to eradicate lepra, its incidence continues to be elevated, especially in endemic areas, among which the south of Spain is included. It is generally associated to overcrowding and low social-economic level. Conclusions: This is the last endemic case in the Valencian Community. The possibility of lepra should be considered among the possible causes of sensitive polyneuropathy, above all in patients in endemic areas (AU)

[Statins and neuromuscular pathology]. / Estatinas y patología neuromuscular.

INTRODUCTION: Statins represent a group of pharmaceutical agents with proven effectiveness and scarce side effects; however, their widespread use increases the probability of the appearance of such adverse effects. DEVELOPMENT: We reviewed the articles that have been published on myotoxicity, neurotoxicity and other cases of disorders affecting the peripheral nervous system, both in reviews and in clinical trials and isolated cases. The frequency of appearance, clinical repercussion, proposed physiopathogenetic mechanisms were analysed in order to draw conclusions regarding the therapeutic attitude that should be adopted. CONCLUSIONS: Generally speaking they are safe pharmaceuticals, but the physician must pay attention to the appearance of possible side effects, given their potential reversibility and lower degree of severity if they are diagnosed at an early stage.

Estatinas y patología neuromuscular / Statins and neuromuscular pathology

Introducción. Las estatinas representan un grupo de fármacos con eficacia probada y escasos efectos secundarios; sin embargo, su amplia difusión aumenta la probabilidad de su aparición. Desarrollo. Revisamos los artículos publicados sobre miotoxicidad, neurotoxicidad y otros casos de afectación del sistema nervioso periférico, tanto en revisiones como en ensayos clínicos y casos aislados, analizando la frecuencia de aparición, la repercusión clínica, los mecanismos fisiopatogénicos propuestos y extrayendo la actitud terapéutica que debe tomarse.Conclusiones. En general son fármacos seguros, pero el facultativo debe prestar atención a la aparición de posibles efectos adversos dada su potencial reversibilidad y menor gravedad si se diagnostican de forma precoz

Introduction. Statins represent a group of pharmaceutical agents with proven effectiveness and scarce side effects; however, their widespread use increases the probability of the appearance of such adverse effects. Development. We reviewed the articles that have been published on myotoxicity, neurotoxicity and other cases of disorders affecting the peripheral nervous system, both in reviews and in clinical trials and isolated cases. The frequency of appearance, clinical repercussion, proposed physiopathogenetic mechanisms were analysed in order to draw conclusions regarding the therapeutic attitude that should be adopted. Conclusions. Generally speaking they are safe pharmaceuticals, but the physician must pay attention tothe appearance of possible side effects, given their potential reversibility and lower degree of severity if they are diagnosed at an early stage

Papilitis unilateral como presentación de neurosífilis / Unilateral papillitis as the presenting symptom of neurosyphilis

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Edema de papila bilateral como manifestación inicial de un síndrome de POEMS / Bilateral papilloedema as the initial symptom of POEMS syndrome

Introducción. El edema de papila suele ser secundario a hipertensión intracraneal, pero en ocasiones puede ser la manifestación inicial de enfermedades sistémicas como síndrome de Guillain-Barré, neurosarcoidosis, enfermedad de Leber, enfermedad de Lyme, síndrome de POEMS y otros. Presentamos un caso ocurrido en el Hospital General Universitario de Alicante, en el que el edema de papila fue la manifestación inicial de un síndrome de POEMS. Caso clínico. Mujer de 36 años, sin antecedentes de interés, que consulta por cefalea opresiva frontal izquierda, de cinco días de evolución, de inicio brusco, sin náusea ni vómitos acompañantes, sin exacerbación con las maniobras de Valsalva y sin otra sintomatología acompañante. En la exploración destaca borramiento nasal del ojo derecho y papiledema en el ojo izquierdo, hiperpigmentación cutánea en cara y escote que respetaba pliegues, hipoestesia táctil dolorosa distal en miembros inferiores y arreflexia aquílea e hiporreflexia rotuliana bilateral. Tras realizar distintas pruebas complementarias se descartó lesión ocupante de espacio intracraneal e hipertensión intracraneal, y se llegó al diagnóstico de polineuropatía crónica sensitivomotora de tipo mixto en miembros superiores e inferiores con afectación predominantemente proximal, edema de papila bilateral y fotosensibilidad cutánea a estudio. Tras dos años, la paciente presentó hepatomegalia, hipotiroidismo subclínico y alteraciones cutáneas esclerodermiformes, con lo que se diagnosticó como probable síndrome de POEMS, confirmado por la presencia de componente monoclonal en sangre y orina. Conclusión. El edema de papila como manifestación inicial de un síndrome de POEMS es infrecuente, pero debe estar presente en el diagnóstico diferencial de esta patología por las implicaciones diagnósticas y terapéuticas que conlleva

INTRODUCTION. Oedema of the papilla, or papilloedema, is usually due to intracranial hypertension, but can sometimes be the initial symptom of systemic diseases like Guillain-Barre syndrome, neurosarcoidosis, Leber’s disease, Lyme disease or POEMS syndrome, among others. We report on a case that occurred at the Hospital General Universitario in Alicante, in which papilloedema was the initial symptom of POEMS syndrome. CASE REPORT. A 36-year-old female, with no relevant past history, who visited after suffering a left-side frontal oppressive headache for five days; onset was sudden, there was no concomitant nausea or vomiting, it did not get worse when Valsalva manoeuvres were performed and there were no other accompanying symptoms. The examination revealed nasal blurring in the visual field of the right eye and papilloedema in the left eye, hyperpigmentation of the skin on the face and upper chest area that respected creases in the skin, painful distal tactile hypaesthesia in the lower limbs and bilateral areflexia of Achilles’ tendons and patellar hyperreflexia. After conducting a number of different complementary tests intracranial space-occupying lesions and intracranial hypotension were both ruled out and the patient was diagnosed with mixed-type sensory-motor chronic polyneuropathy in the upper limbs, and in the lower extremities with predominantly distal involvement, bilateral oedema of the papilla and skin photosensitivity. Two years later the patient had hepatomegaly, subclinical hypothyroidism and sclerodermiform alterations of the skin, which suggested a probable diagnosis of POEMS syndrome that was later confirmed by the presence of monoclonal component in blood and urine. CONCLUSIONS. Papilloedema is rarely seen as the initial symptom of POEMS syndrome, but it must be included in the differential diagnosis of this pathology due to the diagnostic and therapeutic implications it entails

[Bilateral papilloedema as the initial symptom of POEMS syndrome]. / Edema de papila bilateral como manifestación inicial de un síndrome de POEMS.

INTRODUCTION: Oedema of the papilla, or papilloedema, is usually due to intracranial hypertension, but can sometimes be the initial symptom of systemic diseases like Guillain-Barre syndrome, neurosarcoidosis, Leber's disease, Lyme disease or POEMS syndrome, among others. We report on a case that occurred at the Hospital General Universitario in Alicante, in which papilloedema was the initial symptom of POEMS syndrome. CASE REPORT: A 36-year-old female, with no relevant past history, who visited after suffering a left-side frontal oppressive headache for five days; onset was sudden, there was no concomitant nausea or vomiting, it did not get worse when Valsalva manoeuvres were performed and there were no other accompanying symptoms. The examination revealed nasal blurring in the visual field of the right eye and papilloedema in the left eye, hyperpigmentation of the skin on the face and upper chest area that respected creases in the skin, painful distal tactile hypaesthesia in the lower limbs and bilateral areflexia of Achilles' tendons and patellar hyperreflexia. After conducting a number of different complementary tests intracranial space-occupying lesions and intracranial hypotension were both ruled out and the patient was diagnosed with mixed-type sensory-motor chronic polyneuropathy in the upper limbs, and in the lower extremities with predominantly distal involvement, bilateral oedema of the papilla and skin photosensitivity. Two years later the patient had hepatomegaly, subclinical hypothyroidism and sclerodermiform alterations of the skin, which suggested a probable diagnosis of POEMS syndrome that was later confirmed by the presence of monoclonal component in blood and urine. CONCLUSIONS: Papilloedema is rarely seen as the initial symptom of POEMS syndrome, but it must be included in the differential diagnosis of this pathology due to the diagnostic and therapeutic implications it entails.

Meningitis por Escherichia coli como complicación tardía de bomba intratecal de baclofeno / Meningitis due to Escherichia coli as a delayed complication of intrathecal de baclofeno

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[Neuropathic pain as the reason for visiting Neurology: an analysis of its frequency]. / El dolor neuropático como motivo de consulta en Neurología: análisis de su frecuencia.

INTRODUCTION: Neuropathic pain (NP) is defined as pain that begins with or is caused by a primary injury or by a dysfunction in the nervous system. AIMS: Our aim was to evaluate how often patients visit Neurology as outpatients with NP as the main reason for referral. PATIENTS AND METHODS: A descriptive, cross sectional study was carried out on the use of the health care services; patients attended for the first time in a Neurology Screening visit were included consecutively. The variables studied were the following: the number of first visits and the total number of patients attended per visiting session, rate of patients with NP per visiting day, the topography and probable causation of the NP, and the rate of patients referred to the monographic NP clinic; the different quantitative variables are expressed in terms of their mean and standard deviation (SD), whereas the qualitative variables are given as their absolute value and the percentage. A total of 1,972 patients were attended, of whom 1,422 (72.1%) were first visits, with an average of 17.5 (SD: 2.5) new patients per visiting session. RESULTS: In all 113 patients clinically diagnosed with NP were identified, which represents a rate of 7.95% of the first visits. CONCLUSIONS: NP may be among the most frequent causes of the demand for neurological ambulatory care. The most common causes of NP were found to be trigeminal neuralgia, post-herpes neuralgia and diabetic polyneuropathy.

El dolor neuropático como motivo de consulta en Neurología: análisis de su frecuencia / Neuropathic pain as the reason for visiting neurology: an analysis of its frequency

Introducción. El dolor neuropático (DN) se define como aquel dolor iniciado o causado por una lesión primaria o por una disfunción en el sistema nervioso. Objetivo. Evaluar la frecuencia con la que los pacientes con DN como motivo principal de derivación se remiten a una consulta ambulatoria de Neurología. Pacientes y métodos. Se trata de un estudio descriptivo, transversal sobreutilización de los servicios sanitarios; se incluyeron consecutivamente los pacientes atendidos por primera vez en una consulta de Neurología de selección; variables estudiadas: número de primeras visitas y número total de pacientes atendidos por sesión de consulta, tasa de pacientes con DN por día de consulta, la topografía y probable etiología del DN, y la tasa de pacientes remitidos a la consulta monográfica de DN; las diferentes variables cuantitativas se expresarán con su media y desviación estándar (DE), mientras que en las variables cualitativas esto se hará con su valor absoluto y el porcentaje. Se atendieron 1.972 pacientes, de los cuales 1.422(72,1%) fueron primeras visitas, con una media de 17,5 (DE: 2,5) pacientes nuevos por sesión de consulta. Resultados. Se identificaron113 pacientes con diagnóstico clínico de DN, lo que supone una tasa del 7,95% de las primeras visitas. Conclusiones. El DN puede estar entre las causas más frecuentes de demanda de asistencia neurológica ambulatoria. Las causas más frecuentes de DN fueron la neuralgia del trigémino, la neuralgia posherpética y la polineuropatía diabética (AU)

Introduction. Neuropathic pain (NP) is defined as pain that begins with or is caused by a primary injury or by a dysfunction in the nervous system. Aims. Our aim was to evaluate how often patients visit Neurology as outpatients with NPas the main reason for referral. Patients and methods. A descriptive, cross sectional study was carried out on the use of the health care services; patients attended for the first time in a Neurology Screening visit were included consecutively. The variables studied were the following: the number of first visits and the total number of patients attended per visiting session, rate of patients with NP per visiting day, the topography and probable causation of the NP, and the rate of patients referred to the monographic NP clinic; the different quantitative variables are expressed in terms of their mean and standard deviation (SD), whereas the qualitative variables are given as their absolute value and the percentage. A total of 1,972patients were attended, of whom 1,422 (72.1%) were first visits, with an average of 17.5 (SD: 2.5) new patients per visiting session. Results. In all 113 patients clinically diagnosed with NP were identified, which represents a rate of 7.95% of the first visits. Conclusions. NP may be among the most frequent causes of the demand for neurological ambulatory care. The most common causes of NP were found to be trigeminal neuralgia, post-herpes neuralgia and diabetic polyneuropathy (AU)

Intoxicación accidental por alcohol metílico: secuelas neurológicas agudas y crónicas / Accidental methyl alcohol poisoning: acute and chronic neurological sequelae

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[Binocular diplopy as the presentation of a haematoma in the rectus inferior]. / Diplopía binocular como presentación de un hematoma del recto inferior.

INTRODUCTION: The presence of quick onset binocular diplopy makes it essential to carry out a comprehensive differential diagnosis. The most frequent causes in adults include vascular, post-traumatic, tumorous and myopathic pathologies. Yet, to perform a differential diagnosis we also have to take into account less common aetiologies such as demyelinating disease, carotid-cavernous fistulas and Tolosa Hunt syndrome, among others. We report the case of a patient who was admitted to our hospital because she presented an acute onset diplopy secondary to a spontaneous haematoma of the left rectus inferior. CASE REPORT: We describe the case of a 54-year-old female with a history of hypercholesterolemia, who was a smoker and allergic to iodine contrasts and who visited because of an acute onset diplopy accompanied by instability and pain in the left eye. The neurological examination revealed paresis of the extraocular muscles dependent on the left oculomotor nerve with left palpebral ptosis, paresis of the rectus inferior, with no involvement of the pupils. Other results of the exploration were within normal limits. A computerised axial tomography scan of the head showed a haemorrhage in the rectus inferior. Magnetic resonance images of the head revealed an increase in the volume of the left-side rectus inferior with signs of bleeding, and the rest of the complementary explorations were normal. The patient progressed favourably and the diplopy gradually disappeared. CONCLUSIONS: Spontaneous bleeding of the extraocular muscles, although infrequent, must be taken into account in the differential diagnosis of painful ophthalmoplegia with diplopy in patients with no underlying pathology, especially in the absence of other neurological disorders.