Distonía focal del pie de inicio en el adulto / Adult-onset primary focal foot dystonia

Introducción. La distonía focal idiopática del pie de inicioen el adulto es una entidad rara de la que hasta la fechasólo se han encontrado siete casos publicados en la literatura.Dado que las formas idiopáticas en el adulto son excepcionales,su presentación obliga siempre a descartar formassecundarias. Caso clínico. Mujer de 51 años con distonía focal delpie de 3 años de evolución. La paciente presentaba posturaen flexión plantar e inversión del pie que empeoraba con laacción. Las exploraciones complementarias dirigidas a descartarcausas secundarias fueron negativas. A lo largo de suevolución el fenómeno distónico permaneció limitado al piey no desarrolló otra sintomatología neurológica. La pacientese benefició de tratamiento con toxina botulínica. Conclusiones. La distonía focal primaria idiopática delpie en el adulto es excepcional. El patrón más común es laflexión plantar y de los cinco dedos. El dolor asociado es unsíntoma relativamente frecuente. La patogenia es sólo parcialmenteconocida, los estudios neurofisiológicos y de resonanciafuncional demuestran que existe una pérdida delcontrol inhibitorio a nivel espinal y troncoencefálico, unaplasticidad cortical anómala y una integración sensitivomotoradefectuosa. La respuesta al tratamiento con fármacosorales es escasa, pero los pacientes pueden beneficiarsede la infiltración con toxina botulínica en los músculos implicados

Introduction. Adult-onset primary focal foot dystoniais a rare event. Up to now, only 7 cases have been reportedin the literature. Since the idiopathic-type fooddystonia is uncommon in adults, secondary types mustbe ruled out. Clinical report. We present the case of a 51 yearwoman with a 3 year history of focal food dystonia. Shehad abnormal posture of the foot with plantar flexionand inversion, which worsened with action. Complementarystudies aimed at ruling out secondary causes wereall negative. The dystonia remained limited to her foodand she did not develop any other neurological symptoms.She benefited from botulinum toxin treatment. Conclusions. Primary focal foot dystonia is uncommonin adults. The most common pattern is plantar andfive toes flexion. Associated pain is common. Its pathogenesisis only partially understood, the neurophysiologicstudies and functional resonance showing a loss ofinhibitory control at spine and brainstem levels, abnormalcortical plasticity and altered sensorimotor integration.The response of this disorder to drugs is poor, however,the patients may benefit from botulinum toxininfiltrations of the muscles involved

[Adult-onset primary focal foot dystonia]. / Distonía focal del pie de inicio en el adulto.

INTRODUCTION: Adult-onset primary focal foot dystonia is a rare event. Up to now, only 7 cases have been reported in the literature. Since the idiopathic-type food dystonia is uncommon in adults, secondary types must be ruled out. CLINICAL REPORT: We present the case of a 51 year woman with a 3 year history of focal food dystonia. She had abnormal posture of the foot with plantar flexion and inversion, which worsened with action. Complementary studies aimed at ruling out secondary causes were all negative. The dystonia remained limited to her food and she did not develop any other neurological symptoms. She benefited from botulinum toxin treatment. CONCLUSIONS: Primary focal foot dystonia is uncommon in adults. The most common pattern is plantar and five toes flexion. Associated pain is common. Its pathogenesis is only partially understood, the neurophysiologic studies and functional resonance showing a loss of inhibitory control at spine and brainstem levels, abnormal cortical plasticity and altered sensorimotor integration. The response of this disorder to drugs is poor, however, the patients may benefit from botulinum toxin infiltrations of the muscles involved.

[Cerebellar degeneration associated with anti-Tr antibodies without Hodgkin's disease. Four years follow-up]. / Degeneración cerebelosa con anticuerpos anti-Tr positivos sin linfoma de Hodgkin asociado. Seguimiento durante 4 años.

The presence of antineuronal anti-Tr antibodies is associated to paraneoplastic cerebellar degeneration due to Hodgkin's disease. The anti-Tr can become negative after successful and early treatment of the tumor, and there could even be remission of the cerebellar symptoms in some patients. There are few cases in which no tumor is found when there are anti-Tr. We report the case of a 66 year old man with a severe cerebellar syndrome and anti-Tr in serum detected by immunohistochemistry. After a 4 year follow-up, no underlying tumor has been found. In addition, anti-Tr spontaneously disappeared. The cerebellar degeneration persists and is incapacitating. This case suggests that in a few instances the origin of anti-Tr is not a tumor but another unknown cause. Alternatively the anti-Tr mediated immune response could have eradicated the underlying lymphoma.

Parche sanguíneo epidural como tratamiento de un cuadro de hipotensión intracraneal espontánea / Epidural blood patch for the management of a patient with spontaneous intracranial hypotension

Una paciente de 75 años con clínica de cefalea postural incapacitante, secundaria a un cuadro hipotensión intracraneal espontánea (HIE), es remitida para la realización de parche sanguíneo epidural autólogo, tras fracaso del tratamiento conservador inicial. Aunque la etiología de la HIE no está suficientemente aclarada, se sospecha la existencia de un defecto anatómico meníngeo intracraneal o espinal, a través del cual se producirían pérdidas de líquido cefalorraquídeo (LCR) suficientes para provocar el síndrome. Usualmente el cuadro se resuelve de manera espontánea o con tratamiento sintomático. Sin embargo, si la cefalea postural persiste y sobre todo si tiene síntomas neurológicos asociados, los tratamientos epidurales son de elección, siendo el parche sanguíneo epidural el que ha demostrado ser más efectivo. Si los parches epidurales de sangre autóloga fracasan se considerará el tratamiento quirúrgico. Las inyecciones epidurales de pegamento de fibrina están teniendo últimamente unos resultados prometedores (AU)

[Alien hand syndrome in Creutzfeldt-Jakob disease]. / Síndrome de la mano alienígena en la enfermedad de Creutzfeldt-Jakob.

Alien hand syndrome is defined by uncontrolable actions of the arm and hand that seem to have a purpose. It is usually associated with acute focal lesions after a stroke or surgery of the corpus callosum. It has been described in chronic dementiating diseases such as cortico-basal degeneration, Alzheimer's disease, orthochromatic leukodystrophy and Marchiafava-Bignami disease. We now report a patient with Creutzfeldt-Jakob disease and alien hand syndrome, which appeared after the cognitive alterations and had characteristics of the frontal type of alien hand affecting the dominant upper limb. In the three cases described previously in the literature the alien hand affected the non-dominant hand and the abnormal movement appeared before dementia was clinically obvious. The non specific nature and poorly localising sign of alien hand is remarked. Alien hand should be added the list of features of Creutzfeldt-Jakob disease.

Síndrome de la mano alienígena en la enfermedad de Creutzfeldt-jakob / Alien hand syndrome in CreutzfeldtJakob disease

El síndrome de la mano alienígena se caracteriza por acciones incontroladas del brazo y la mano que parecen tener un propósito. La extrañeza que causa al sujeto esa extremidad y sus acciones define a este síndrome. Se asocia habitualmente a lesiones focales agudas tras ictus y a tumores o cirugía sobre el cuerpo calloso. También se ha descrito en enfermedades crónicas demenciantes como la degeneración corticobasal, la enfermedad de Alzheimer, la leucodistrofia ortocromática y la enfermedad de Marchiafava-Bignami. Describimos el caso de un paciente con enfermedad de Creutzfeldt-Jakob y síndrome de la mano alienígena que aparece con posterioridad a la alteración cognitiva y que reúne características de mano alienígena en su variante frontal con afección de mano dominante. En los tres casos previamente descritos en la bibliografía la afección fue de la mano no dominante y este movimiento anormal precedió al deterioro cognitivo. Se señala la inespecifidad de este síndrome en cuanto a etiología y valor localizador. Sugerimos que la mano alienígena debe considerarse como una de las posibles manifestaciones de la enfermedad de Creutzfeldt-Jakob (AU)

Paciente de 45 años con pérdida de la conciencia / A 45 years old patient with loss of consciousness

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[Spinal cord ischemia as a consequence of dissection of an abdominal aortic aneurysm]. / Isquemia medular como consecuencia de un aneurisma de aorta abdominal.

INTRODUCTION: Ischemic spinal cord infarct is the most frequent vascular lesion, but although aortic aneurysms are a possible cause, it is unusual for such cases to be seen. Clinical case. We present a case of spinal ischemia as the first sign of the dissection of an aneurysm of the abdominal aorta. A 58 year old man was seen in the hospital Emergency Department complaining of lumbar pain and the sudden onset of paraplegia of the legs, associated with pain in the middle of his back but with no history of previous trauma or effort. The only relevant personal history was of smoking. Whilst he was in the Neurology Department, the anomaly was diagnosed after dorsal, and lumbar gadolinium magnetic resonance (MR), when a zone of ischaemia at T9-T10 was seen and, as a casual observation, an image compatible with an aneurysm of the abdominal aorta. The relationship between the dissection of the aorta and the neurological complications may be explained by a clear understanding of the vascular supply to the spinal cord. In this case, both the clinical findings and the MR were clearly indicative of an anterior spinal artery syndrome. CONCLUSIONS: In spite of its rarity, aortic aneurysm should be included in the differential diagnosis of a clinical picture of ischemic myelopathy, especially when there is lumbar and/or abdominal pain before the appearance of neurological symptoms. Spinal MR is important for this diagnosis.