RESUMEN
We describe a 69-year-old patient with superior altitudinal hemianopia who contentiously denied having any visual impairment after stroke in the lower banks of both calcarine fissures. Although the patient did not produce intentional responses to visual stimuli in the blind fields, he showed reduced reaction times to stimuli presented in the inferior visual fields when they were primed by identical stimuli in the superior blind fields. Furthermore he showed left extinction to the double stimulation and delayed reaction times for left unprimed stimuli in the inferior fields. Based on these findings we discuss the possibility that blindsight and right hemisphere damage might be both necessary conditions for denying bilateral blindness.
Asunto(s)
Atención , Ceguera Cortical/psicología , Deluciones/psicología , Percepción Visual , Anciano , Ceguera Cortical/diagnóstico , Deluciones/complicaciones , Humanos , Masculino , Estimulación Luminosa/métodos , Tiempo de Reacción , Campos VisualesAsunto(s)
Trastornos del Conocimiento/etiología , Colgajos Quirúrgicos/efectos adversos , Trastornos del Conocimiento/diagnóstico , Craneotomía/efectos adversos , Lóbulo Frontal/diagnóstico por imagen , Lóbulo Frontal/cirugía , Humanos , Masculino , Escala del Estado Mental , Persona de Mediana Edad , Tomografía Computarizada por Rayos XAsunto(s)
Anticuerpos Monoclonales de Origen Murino/uso terapéutico , Seropositividad para VIH/tratamiento farmacológico , Factores Inmunológicos/uso terapéutico , Miastenia Gravis/tratamiento farmacológico , Anticuerpos/sangre , Femenino , Seropositividad para VIH/complicaciones , Seropositividad para VIH/inmunología , Humanos , Miastenia Gravis/complicaciones , Miastenia Gravis/inmunología , Proteínas Tirosina Quinasas Receptoras/inmunología , Receptores Colinérgicos/inmunología , Rituximab , Adulto JovenRESUMEN
Neurology is a polymorphic discipline, with several subspecialties. In 2006, as in the previous years, a huge amount of scientific work focusing on treatment has been published. However, there has not been a true revolution in any of the current therapeutic strategies; rather, we experienced an improvement in the knowledge about several specific "details". This allows to consider more and more variables when administering a specific treatment, therefore, in each subspecialty a move towards a better patient's care has been made. In this contribution, several specialists analyse and interpret new facts about their respective neurological domain.
Asunto(s)
Enfermedades del Sistema Nervioso/terapia , Trastornos Cerebrovasculares/terapia , Demencia/terapia , Epilepsia/terapia , Humanos , Esclerosis Múltiple/terapia , Enfermedades Neuromusculares/terapia , Enfermedad de Parkinson/terapiaRESUMEN
Neurodegenerative and cerebrovascular diseases have been related for more than a century. Epidemiological data show that main vascular risk factors are also risk factors for Alzheimer disease. Experimental evidences demonstrate that some of those risk factors accelerate the progress of Alzheimer lesions, mainly by acting on the amyloid cascade. Recent advances in understanding the basic mechanisms of CADASIL and familial amyloid angiopathy reveal that these forms of vascular dementias are degenerative disease of brain vessels. Modern neuroimaging techniques will allow to better understand relations between symptomatic strokes, silent infarcts, leukoaraiosis, microbleeds and degenerative pathology before the stage of dementia.
Asunto(s)
Trastornos Cerebrovasculares/complicaciones , Enfermedades Neurodegenerativas/etiología , Humanos , Factores de RiesgoRESUMEN
INTRODUCTION: Panarteritis nodosa (PAN) is a systemic vasculitis affecting small and medium-sized arteries. Neuro-ophthalmological complications of PAN are rare but numerous, and may affect the eye, the visual and the oculomotor pathways. Such complications occur mainly in patients previously diagnosed with PAN. OBSERVATION: A 51-year-old woman presented with an isolated right trochlear (IV) palsy, in the setting of headaches and fluctuating fever of unknown etiology. Erythrocyte sedimentation rate was 13 mm and full blood cell count was normal. Previous chest X-ray and blood studies were negative for an infection or inflammation. Orbital and cerebral CT scan was normal. Spontaneous recovery of diplopia ensued over four days. Two days later, paresthesia and sensory paresis of the dorsal portion of the left foot were present. Lumbar puncture revealed 14 leucocytes (76 percent lymphocytes) with elevated proteins, but blood studies and serologies were negative. A diagnosis of undetermined meningo-myelo-radiculoneuritis was made. Because of a possible tick bite six weeks previously the patient was empirically treated with 2 g intravenous ceftriaxone for 3 weeks. Fever rapidly dropped. Six weeks after the onset of diplopia, acute onset of blindness in her right eye, diffuse arthralgias and fever motivated a new hospitalization. There was a central retinal artery occlusion of the right eye. Blood studies now revealed signs of systemic inflammation (ESR 30 mm, CRP 12 mg/L, ANA 1/80, pANCA 1/40, leucocytosis 12.4 G/L, Hb 111 g/L, Ht 33 percent). Biopsy of the left sural nerve revealed arterial fibrinoid necrosis. A diagnosis of PAN was made. CONCLUSIONS: Transient diplopia can be the heralding symptom of a systemic vasculitis such as PAN, giant cell arteritis and Wegener granulomatosis. In this patient the presence of accompanying systemic symptoms raised a suspicion of systemic inflammation, but the absence of serologic and imaging abnormalities precluded a specific diagnosis initially. A few weeks later, the presence of a second ischemic event (retinal) and positive blood studies led to a further diagnostic procedure. Oculomotor and abducens palsies have rarely been reported in association with PAN. We report the first case of trochlear nerve paresis as the inaugural neurological sign of PAN. This case highlights the importance of considering inflammatory systemic disorders in patients with acute diplopia particularly when they are young, lack vascular risk factors or cause, and complain of associated systemic symptoms.
Asunto(s)
Poliarteritis Nudosa/complicaciones , Enfermedades del Nervio Troclear/etiología , Diplopía/fisiopatología , Ojo/patología , Femenino , Humanos , Inmunosupresores/uso terapéutico , Recuento de Leucocitos , Persona de Mediana Edad , Parestesia , Poliarteritis Nudosa/diagnóstico , Poliarteritis Nudosa/patología , Punción Espinal , Nervio Sural/patología , Enfermedades por Picaduras de Garrapatas/fisiopatología , Tomografía Computarizada por Rayos X , Enfermedades del Nervio Troclear/diagnóstico , Enfermedades del Nervio Troclear/patologíaRESUMEN
BACKGROUND: Two professional painters experienced significant changes in their art as the main consequence of minor stroke located in the left occipital lobe or thalamus. METHODS: The features of this artistic conversion were analysed on the basis of extensive neurological, neuropsychological, and psychiatric evaluations. RESULTS: Both painters, initially unaware of the artistic changes, exhibited mild signs of executive dysfunction, but no general cognitive decline. The first painter, who showed mild visual-perceptive difficulties (dyschromatopsia and scotoma in his right upper visual field after left occipital stroke), together with increased anxiety and difficulty in emotional control, switched to a more stylised and symbolic art. The second painter, who also presented features of emotionalism related to his left latero-thalamic stroke, switched from an impressionist style to a more joyous and geometric, but more simplistic, abstract art. CONCLUSIONS: These findings show that mild cognitive and affective modifications due to focal posterior brain lesions can have significant repercussions on artistic expression.
Asunto(s)
Arte , Trastornos del Conocimiento/etiología , Trastornos del Humor/etiología , Arteria Cerebral Posterior/patología , Accidente Cerebrovascular/complicaciones , Accidente Cerebrovascular/patología , Anciano , Trastornos del Conocimiento/diagnóstico , Electrocardiografía , Humanos , Angiografía por Resonancia Magnética , Imagen por Resonancia Magnética , Masculino , Persona de Mediana Edad , Trastornos del Humor/diagnóstico , Pruebas Neuropsicológicas , Lóbulo Occipital/irrigación sanguínea , Lóbulo Occipital/patología , Índice de Severidad de la Enfermedad , Tálamo/irrigación sanguínea , Tálamo/patologíaRESUMEN
OBJECTIVE: To investigate the association between early depressive behavior after stroke onset and occurrence of poststroke depression (PSD) at 3- and 12-month follow-up evaluations. METHODS: The study prospectively included 273 patients with first-ever single uncomplicated ischemic stroke. In the stroke unit, nurses scored crying, overt sadness, and apathy daily using an observational method to include patients with comprehension deficits. The Barthel Index was used to assess disability. Follow-up evaluation at months 3 and 12 included psychiatric assessment based on the Diagnostic and Statistical Manual of Mental Disorders, 4th edition. RESULTS: Crying (19.8%), overt sadness (50.5%), and apathy (47.6%) were observed. Of the patients observed crying, 4 showed pathologic crying, 19 emotionalism, and 12 catastrophic reactions. Crying and overt sadness, but not apathy, were associated with a subjective experience of depression (p < 0.05). Thirty of 52 (58%) patients observed crying, 12 of 19 (63%) patients with emotionalism, and 5 of 12 (41%) patients with catastrophic reactions developed PSD within the first year. Multiple logistic regression analysis showed that only severe functional disability (odds ratio [OR], 4.31; 95% CI, 2.41 to 7.69), crying behaviors (OR, 2.66; 95% CI, 1.35 to 5.27), and an age <68 years (OR, 2.32; 95% CI, 1.30 to 4.13) were (p < 0.05) predictors of late PSD development (13% of the variance). CONCLUSIONS: In the stroke unit, crying and overt sadness are more reliable indicators of depressed mood than apathy. In patients with first-ever stroke, crying behaviors soon after stroke, a younger age, and severe disability are predictors of poststroke depression occurrence within the first year after stroke onset.
Asunto(s)
Isquemia Encefálica/psicología , Depresión/etiología , Anciano , Anciano de 80 o más Años , Comprensión , Depresión/epidemiología , Emociones , Femenino , Humanos , Incidencia , Masculino , Persona de Mediana Edad , Valor Predictivo de las Pruebas , Estudios Prospectivos , Factores de Riesgo , LágrimasRESUMEN
Foreign accent syndrome (FAS), a rare disorder characterized by the emergence of a new accent perceived as foreign by listeners, is usually reported with left brain damage. We here report the case of a 28-year-old native Brazilian who appeared, to the examiner, to show a North American accent during recovery from Broca's aphasia. The lesion was due to a frontal hematoma. Without referring specifically to speech, we asked 10 independent observers to comment on a videotape of the patient's interview. Seven reported that the patient had a foreign accent, while 3 simply noted a 'strange' accent. The observers did not agree on the origin of the accent, 5 identifying it as Spanish, 1 as German, and 1 as south Brazilian. These findings suggest that FAS is not due to the acquisition of a specific foreign accent, but to impairment of the suprasegmental linguistic abilities (tone, accent, pauses, rhythm, and vocal stress) that make it possible to distinguish native language.
Asunto(s)
Hemorragia Cerebral/complicaciones , Lenguaje , Trastornos del Habla/etiología , Conducta Verbal/fisiología , Adulto , Hemorragia Cerebral/patología , Femenino , Humanos , Imagen por Resonancia Magnética/métodos , Masculino , Persona de Mediana Edad , Trastornos del Habla/patologíaRESUMEN
Twelve patients with a catastrophic reaction (CR) (an outburst of frustration, depression, and anger when confronted with a task) were identified in a prospective cohort population (n = 326) with first-ever stroke admitted within 48 hours from onset. The authors' findings suggest that CR is a rare though not exceptional phenomenon in acute stroke and is associated with nonfluent aphasias and left opercular lesions. CR, poststroke depression, and emotionalism are distinct but related disorders.
Asunto(s)
Afasia/fisiopatología , Hemorragia Cerebral/fisiopatología , Infarto Cerebral/fisiopatología , Depresión/fisiopatología , Frustación , Hostilidad , Embolia Intracraneal/fisiopatología , Reflejo Anormal/fisiología , Adulto , Anciano , Anciano de 80 o más Años , Afasia/diagnóstico , Afasia/psicología , Mapeo Encefálico , Corteza Cerebral/fisiopatología , Hemorragia Cerebral/diagnóstico , Hemorragia Cerebral/psicología , Infarto Cerebral/diagnóstico , Infarto Cerebral/psicología , Estudios de Cohortes , Depresión/diagnóstico , Depresión/psicología , Femenino , Estudios de Seguimiento , Humanos , Infarto de la Arteria Cerebral Media/diagnóstico , Infarto de la Arteria Cerebral Media/fisiopatología , Infarto de la Arteria Cerebral Media/psicología , Embolia Intracraneal/diagnóstico , Embolia Intracraneal/psicología , Imagen por Resonancia Magnética , Masculino , Persona de Mediana Edad , Examen Neurológico , Estudios Prospectivos , Tálamo/fisiopatología , Tomografía Computarizada por Rayos XAsunto(s)
Infecciones por VIH/tratamiento farmacológico , Inhibidores de la Proteasa del VIH/uso terapéutico , VIH-1 , Nelfinavir/uso terapéutico , Inhibidores de la Transcriptasa Inversa/uso terapéutico , Didesoxinucleósidos/uso terapéutico , Quimioterapia Combinada , Humanos , Lamivudine/uso terapéutico , Nevirapina/uso terapéutico , Estudios Retrospectivos , Estavudina/uso terapéuticoRESUMEN
Idiopathic polymyositis (IPM) and HIV polymyositis (HIV-PM) are considered to be related autoimmune diseases whose target is skeletal muscle. They have been associated to a T cell-mediated and MHC-I-restricted cytotoxic phenomenon, but both etiology and physiopathology remain incompletely understood. Their histological hallmarks are mononuclear leukocyte infiltrates as well as necrosis, degeneration, and regeneration of muscle fibers. In the present study, we have investigated the immunohistochemical expression of cell adhesion molecules, cytokines, and leukocyte surface antigens in biopsies of HIV-PM and IPM patients. The aim was to better define factors involved in lymphocyte recruitment and in inflammatory changes seen in PM. Notable upregulation of ICAM-1 and TNF-alpha was detected on capillary and venular endothelia and on inflammatory cells, whereas no significant VCAM-1 and ELAM-1 expression was present. LFA-1, the main ICAM-1 counter-receptor, was found to be highly expressed on lymphocytes and monocytes, especially at the vicinity of damaged fibers. The majority of infiltrating cells were CD8+CD45 RO-T cells, which are thought to have memory capacities. These findings suggest that in IPM and HIV-PM, enhanced ICAM-1 and LFA-1 expression possibly induced by TNF-alpha, may regulate the homing process of selected lymphocyte clones in muscle tissue. Lymphocyte proliferation and differentiation into memory subsets may further potentiate tissue-restricted homing capabilities.
Asunto(s)
Enfermedades Autoinmunes/inmunología , Moléculas de Adhesión Celular/sangre , Seropositividad para VIH/inmunología , Antígenos HLA/sangre , Polimiositis/inmunología , Adulto , Enfermedades Autoinmunes/sangre , Biopsia , Comunicación Celular/fisiología , Femenino , Humanos , Inmunohistoquímica , Masculino , Músculo Esquelético/patología , Polimiositis/sangre , Receptores Mensajeros de Linfocitos/sangreRESUMEN
Creutzfeldt-Jakob disease (CJD), a subacute spongiform encephalopathy, is generally included among the group of human and animal diseases which is transmissible by a non-conventional agent, the prion, whose expression is conditioned by the host's genome. The process leading to neuropathological changes is still unknown. We report the neuropathological findings in 2 cases of the "panencephalopathic" variant of CJD, which is relatively common in Japan, but extremely rare in Europe and North America. When compared with the classical form this variant is characterized by a relatively long clinical course with persistent vegetative state and primary involvement of the white matter presenting in the form of demyelination and gemistocytic gliosis. The selective involvement of certain thalamic nuclei is a particular pathological feature in both our cases. There was practically complete neuronal loss with diffuse gliosis of the anteroventral (AV) and dorsomedial (DM) nuclei, while the neuronal loss in the pulvinar remained moderate: the other nuclei were apparently spared. A similar involvement of the thalamus has been reported in fatal familial insomnia, a recently described prion disease in which these lesions are predominant. A comparable distribution has also been observed in other degenerative neurological diseases such as Steele-Richardson-Olszewski disease, Alzheimer disease, and thalamic dementia (selective thalamic atrophy or with multisystemic degeneration). The AV and DM nuclei, commonly referred to as "limbic thalamus" represent phylogenetically the most recent thalamic structures and would appear to play an important role in the superior functions in man as memory, attention and awareness. In our cases thalamic lesions are selective, bilateral, and symmetric, not explained by Wallerian degeneration. These lesions may be due to the primary pathogenetic properties of the infectious agent. The rapid clinical evolution in a persistent vegetative state could be consequential to precocious and severe disfunction of the limbic thalamus.
Asunto(s)
Síndrome de Creutzfeldt-Jakob/patología , Núcleos Talámicos/patología , Síndrome de Creutzfeldt-Jakob/metabolismo , Encefalomalacia/metabolismo , Encefalomalacia/patología , Femenino , Humanos , Inmunohistoquímica , Persona de Mediana Edad , Enfermedades Talámicas/metabolismo , Enfermedades Talámicas/patología , Núcleos Talámicos/químicaRESUMEN
Pathogenic mechanisms in human cerebral malaria remain unclear. We reevaluate the role of cell-mediated immune mechanisms in the pathogenesis of this disease based on autopsy findings in a 34-year-old Caucasian male. Histologic examination of brain tissue showed typical features of severe malaria infection (sequestration of Plasmodium falciparum-infected erythrocytes in vessels, cerebral oedema, petechial lesions and Dürck granulomas). In addition to these classical changes, we found that leukocytes that stained positively in immunohistochemistry for CD68 and tumor necrosis factor-alpha (TNF) coexisted with infected erythrocytes in capillaries, whereas in venules the monocyte population outnumbered the erythrocytes. Notable expression of ICAM-1 on endothelial cell surface was detected by immunohistochemistry in vessels with sequestered cells but not in unaffected vessels. These changes are identical to those of the murine model of the disease, in which cell-mediated immune mechanisms and TNF have been implicated. In vitro, ICAM-1 has been shown to be a potential ligand for P. falciparum-infected erythrocytes. In malaria patients, high serum TNF levels, which have been detected in close correlation with disease severity, may thus favor adhesion to endothelial cells of either red or white blood cells via enhanced ICAM-1 expression. The present observations are further evidence for a role of cell-mediated immunity in the pathogenesis of human cerebral malaria.