Neuralgia del infraorbitario: un diagnóstico a considerar en pacientes con dolor malar / Infraorbital neuralgia: a diagnostic possibility in patients with zygomatic arch pain

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Hemorragia intracerebral recurrente en amiloidosis primaria / Recurrent intracereberal haemorrhage in primary amyloidosis

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Degeneración corticobasal / Corticobasal degeneration

La degeneración corticobasal (DCB) es un proceso neurodegenerativo lentamente progresivo, de inicio en la edad adulta que se presenta típicamente con parkinsonismo asimétrico y disfunción cognitiva. Actualmente, se clasifica como una taupatía. Los hallazgos neuropatológicos muestran una importante pérdida neuronal cortical que hasta fases muy avanzadas de la enfermedad es de predominio asimétrico y multifocal, más a menudo en las regiones frontalesparietales. Es un síndrome de una gran diversidad clínica cuya evolución estará marcada por la asimetría de los principales síntomas. El trastorno cognitivo puede ser una forma habitual de presentación identificándose un espectro de fenotipos clínicos dependiendo de la topografía del área degenerativa. Típicamente, se caracteriza por marcada rigidez e hipocinesia, distonía de predominio en una extremidad, mioclonías corticales reflejas, piramidalismo y temblor grosero postural o de acción. También destacan los déficits apráxicos, el déficit sensitivo cortical y el denominado fenómeno del miembro ajeno que aunque menos habitual cuando aparece es característico. El diagnóstico es fundamentalmente clínico, en base a los criterios diagnósticos del síndrome corticobasal propuestos en el año 2003 y con el apoyo de las pruebas las complementarias. El diagnóstico diferencial debería considerarse con los Parkinson-Plus así como con las demencias degenerativas primarias con predominio de síntomas frontales y/o temporales, en especial, con la PSP y la demencia fronto-temporal .La DCB progresa en 3-15 años hacia la muerte del individuo, normalmente como consecuencia de complicaciones derivadas de la inmovilidad (AU)

Corticobasal degeneration (CBD) is a slowly progressive neurodegenerative process, which begins in adulthood and typically presents with asymmetrical parkinsonism and cognitive dysfunction. It is currently classified as a tauopathy. Neuropathological findings show an important loss of cortical neurons that is predominantly asymmetrical and multifocal until very advanced phases of the disease, more often in the frontal-parietal regions. It is a syndrome with a wide clinical diversity and a progression that will be marked by the asymmetry of the main symptoms. Cognitive disorder may be a common presenting symptom and a range of clinical phenomena have been identified, depending on the topography of the degenerative area. It is typically characterised by a marked rigidity and hypokinesia, dystonia with predominance in one extremity, cortical reflex myoclonus, pyramidal signs and postural or action coarse tremor. Also prominent are apraxic deficits, cortical sensory deficit and the so-called alien limb phenomenon, which, although less frequent, is considered characteristic when it does appear. Diagnosis is fundamentally clinical and based on the diagnostic criteria of corticobasal syndrome put forward in the year 2003 and with the support of complementary tests. Differential diagnosis should be considered with Parkinson-Plus as well as with the primary degenerative dementias with predominance of frontal and/or temporal symptoms, more especially with PSP and fronto-temporal dementia. CBD progresses in 3-15 years towards the death of the individual, normally as a consequence of complications deriving from immobility (AU)

Epicrania fugax: características clínicas de una serie de 18 pacientes / Epicrania fugax: the clinical characteristics of a series of 18 patients

Introducción. La epicrania fugax es una entidad de reciente descripción, consistente en breves paroxismos dolorosos iniciados en regiones cefálicas posteriores, con irradiación hacia ojo, nariz o sien ipsilaterales. Objetivo. Presentar 18 casos de epicrania fugax de una consulta monográfica de cefaleas de un hospital terciario y analizar sus características demográficas y clínicas, así como la indicación y respuesta al tratamiento profiláctico. Pacientes y métodos. Entre marzo de 2008, momento en el que se describe la epicrania fugax y marzo de 2011, 18 pacientes (12 mujeres y 6 hombres), de entre 1.210 atendidos en dicha consulta (1,48%), recibieron dicho diagnóstico. Seis de estos casos se habían publicado con anterioridad. Resultados. Edad media al inicio de 42,5 ± 17,7 años (rango: 23-82 años). Presentaban paroxismos dolorosos iniciados en la región occipital (n = 11; 61,1%), parietal (n = 6; 33,3%) o parietooccipital (n = 1; 5,6%), e irradiados hacia el ojo (n = 12; 66,6%) o la sien (n = 6; 33,3%) ipsilaterales; todo el proceso duraba menos de 15 segundos. La mayoría describía su dolor como lancinante o punzante. En 10 casos (55,5%) persistía un dolor en la zona de origen de los paroxismos, que en 6 (33,3%) estaba circunscrito a una zona circular bien delimitada y reunía criterios de cefalea numular. En 12 casos (66,6%) se utilizó tratamiento profiláctico, sobre todo lamotrigina y gabapentina con respuesta variable. Conclusión. Pretendemos reforzar la propuesta de la epicrania fugax como un nuevo síndrome con un espectro clínico bien caracterizado. No parece una entidad excepcional, y su conocimiento dará lugar probablemente a la descripción de nuevas series. Con frecuencia es necesario tratamiento y, aunque se requiere mayor experiencia, la gabapentina y la lamotrigina tienen un papel prometedor (AU)

Introduction. Epicrania fugax is a recently reported condition consisting in brief painful paroxysms that begin in the posterior regions of the brain and irradiate towards the ipsilateral eye, nose or temple. Aims. To present 18 cases of epicrania fugax from a monographic headache centre in a tertiary hospital and to analyse their demographic and clinical features, as well as the indication and response to prophylactic treatment. Patients and methods. Between March 2008, when epicrania fugax was first reported, and March 2011, of a total of 1210 patients who were attended in that service (1.48%), 18 (12 females and 6 males) were diagnosed as suffering from this condition. Six of these cases had been published earlier. Results. The mean age at onset was 42.5 ± 17.7 years (range: 23-82 years). They presented painful paroxysms that began in the occipital (n = 11; 61.1%), parietal (n = 6; 33.3%) or parieto-occipital (n = 1; 5.6%) regions and irradiated towards the ipsilateral eye (n = 12; 66.6%) or temple (n = 6; 33.3%); the whole process lasted less than 15 seconds. Most of them described the pain as lancinating or stabbing. In 10 cases (55.5%) a pain remained in the area where the paroxysms began, which in 6 cases (33.3%) was limited to a well-defined circular area and met the criteria for classification as nummular headache. In 12 cases (66.6%), prophylactic treatment was used, above all lamotrigine and gabapentin, with varying results. Conclusion. Our aim is to back the proposal of epicrania fugax as a new syndrome with a well-defined clinical spectrum. It does not appear to be an exceptional condition and further knowledge about it will probably give rise to the description of new series. Treatment is often necessary and, although further information and experience are needed, gabapentin and lamotrigine both play a promising role (AU)

[Nummular headache: clinical features and therapeutic experience in a series of 30 new cases]. / Cefalea numular: caracteristicas clinicas y experiencia terapeutica en una serie de 30 nuevos casos.

INTRODUCTION: Nummular headache (NH) is a mild or moderate pain, located in a small, well circumscribed, rounded or elliptical area. Temporal pattern is variable and pain exacerbations have been described. AIM. To analyze clinical characteristics and therapy requirement and response in a series of patients attended due to NH in a headache outpatient office. PATIENTS AND METHODS: 30 patients (18 females, 12 males) diagnosed as NH. We considered demographic and nosological characteristics, temporal pattern, presence and intensity of exacerbations, and requirement of symptomatic or preventive therapies. RESULTS: Age at onset 49.2 ± 18.1 years (range: 21-79 years). Two of the patients presented a bifocal NH and we analyzed 32 areas, 28 rounded and 4 elliptical. Diameter: 4.7 ± 1.1 cm. Regarding locations occipital (10 areas, 31.3%), parietal (9 areas, 28.1%) and frontal (6 areas, 18.8%). Pain intensity of 5.2 ± 1.8 on a ten-point visual analogical scale. Regarding temporal profile, in 18 areas (56.3%) was chronic, in 5 (15.6%) episodic and undefined due to a scarce time from onset in 9 (28.1%). In 16 areas (50%) pain exacerbations lasting from 3 seconds to 15 minutes occurred. Nine (30%) patients did not improve with symptomatic drugs, and at least one preventative was prescribed in 23 (76.6%) patients without consistent effectiveness. CONCLUSIONS: NH is not an uncommon diagnosis in an outpatient headache office. In our series, basal pain intensity is moderate and symptomatic drugs commonly provide no relief. So, patients frequently need a preventive therapy.

Neuralgia del nervio intermediario y parálisis facial con o sin herpes / Neuralgia of the intermediate nerve and facial palsy with or without herpes

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Alteración de la mirada vertical y retracción palpebral como únicos síntomas de infarto en el territorio de la comisura posterior / Paralysis of upward gaze and eyelid retraction as isolated symptoms of posterior commissure infarction

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Sexsomnia: descripción de un nuevo caso / Sexsomnia: a new case description

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Estudio descriptivo de pacientes diagnosticados de esteatosis hepática no alcohólica relacionada con la obesidad / Descriptive study of patients diagnosed with obesity-related hepatic steatosis unrelated to alcohol

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Maltrato en el anciano. Posibilidades de intervención desde la atención primaria (II) / Mistreatment of the elderly. Possibilities of intervention from primary care (II)

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Maltrato en el anciano. Posibilidades de intervención desde la atención primaria (I) / Mistreatment of the elderly. Possibilities of intervention from primary care (I)

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