Longitudinal study of visual function in patients with relapsing-remitting multiple sclerosis with and without a history of optic neuritis. / Estudio longitudinal de la función visual en pacientes con esclerosis múltiple remitente-recurrente con y sin antecedentes de neuritis óptica.

INTRODUCTION: The contrast sensitivity test determines the quality of visual function in patients with multiple sclerosis (MS). The purpose of this study is to analyse changes in visual function in patients with relapsing-remitting MS with and without a history of optic neuritis (ON). METHODS: We conducted a longitudinal study including 61 patients classified into 3 groups as follows: a) disease-free patients (control group); b) patients with MS and no history of ON; and c) patients with MS and a history of unilateral ON. All patients underwent baseline and 6-year follow-up ophthalmologic examinations, which included visual acuity and monocular and binocular Pelli-Robson contrast sensitivity tests. RESULTS: Monocular contrast sensitivity was significantly lower in MS patients with and without a history of ON than in controls both at baseline (P=.00 and P=.01, respectively) and at 6 years (P=.01 and P=.02). Patients with MS and no history of ON remained stable throughout follow-up whereas those with a history of ON displayed a significant loss of contrast sensitivity (P=.01). Visual acuity and binocular contrast sensitivity at baseline and at 6 years was significantly lower in the group of patients with a history of ON than in the control group (P=.003 and P=.002 vs P=.006 and P=.005) and the group with no history of ON (P=.04 and P=.038 vs P=.008 and P=.01). However, no significant differences were found in follow-up results (P=.1 and P=.5). CONCLUSIONS: Monocular Pelli-Robson contrast sensitivity test may be used to detect changes in visual function in patients with ON.

[Analysis of the quantitative differences in the thickness of the retinal nerve fibre layer between time-domain and spectral-domain optical coherence tomography in patients with relapsing-remitting multiple sclerosis]. / Analisis de las diferencias cuantitativas en el grosor de la capa de fibras nerviosas retiniana entre la tomografia de coherencia optica de dominio-tiempo y de dominio-espectral en pacientes con esclerosis multiple remitente recurrente.

INTRODUCTION: Optical coherence tomography (OCT) is a tool that is increasingly more commonly used in the study of neuro-degenerative diseases. AIMS: To analyse and correlate the thickness of the retinal nerve fibre layer (RNFL) by means of time-domain and spectral-domain OCT in patients with relapsing-remitting multiple sclerosis (MS), with and without a history of optic neuritis (ON). SUBJECTS AND METHODS: We conducted a cross-sectional study of the thickness (mean and by quadrants) of the RNFL of 15 disease-free subjects, 28 with MS with no prior history of ON and 18 with a history of ON. The full ophthalmologic examination included measurement of the RNFL by means of time-domain and spectral-domain tomography. RESULTS: Statistically significant differences are found between the two tomography scans on comparing the mean thickness of the RNFL of the control group (p = 0.000), the group with a history of ON (p = 0.000) and the group without ON (p = 0.000). We obtained a strong, statistically significant and directly proportional correlation between the mean thickness of the RNFL measured with the two types of tomography in the control group (rho = 0.842; p = 0.000), and the groups of eyes without ON (rho = 0.91; p = 0.000) and with ON (rho = 0.902; p = 0.000). CONCLUSIONS: There is a strong correlation between the two tomography scans in the measurement of the thickness of the RNFL in patients with MS, with and without a history of ON. Time-domain OCT quantifies greater thicknesses, and therefore both types of tomography have proven to be effective in the study of MS, although the results cannot be interchanged or extrapolated.

TITLE: Analisis de las diferencias cuantitativas en el grosor de la capa de fibras nerviosas retiniana entre la tomografia de coherencia optica de dominio-tiempo y de dominio-espectral en pacientes con esclerosis multiple remitente recurrente.Introduccion. La tomografia de coherencia optica (OCT) es una herramienta cada vez mas extendida en el estudio de las enfermedades neurodegenerativas. Objetivos. Analizar y correlacionar el grosor de la capa de fibras nerviosas retiniana (CFNR) mediante OCT de dominio-tiempo y dominio-espectral en pacientes con esclerosis multiple (EM) remitente recurrente, con y sin antecedente de neuritis optica (NO). Sujetos y metodos. Estudio transversal del grosor medio y por cuadrantes de la CFNR en 15 sujetos libres de enfermedad, 28 con EM sin historia previa de NO y 18 con antecedentes de NO. La exploracion oftalmologica completa incluia la medicion de la CFNR mediante tomografos de dominio-tiempo y dominio-espectral. Resultados. Existen diferencias estadisticamente significativas entre ambos tomografos al comparar el grosor medio de la CFNR en el grupo control (p = 0,000), el grupo con antecedentes de NO (p = 0,000) y el grupo sin NO (p = 0,000). Hemos obtenido una fuerte correlacion, estadisticamente significativa y directamente proporcional entre el grosor medio de la CFNR medido con ambos tomografos en el grupo control (rho = 0,842; p = 0,000), y los grupos de ojos sin NO (rho = 0,91; p = 0,000) y con NO (rho = 0,902; p = 0,000). Conclusiones. Existe una fuerte correlacion en la medicion del grosor de la CFNR entre ambos tomografos en pacientes con EM, con y sin antecedente de NO. La OCT de dominio-tiempo cuantifica grosores mayores, por lo que ambos tomografos se demuestran eficaces en el estudio de la EM, aunque los resultados no son intercambiables ni extrapolables.

Analysis of macular and nerve fiber layer thickness in multiple sclerosis patients according to severity level and optic neuritis episodes. / Análisis del grosor macular y de capa de fibras nerviosas en pacientes con esclerosis múltiple en relación con su nivel de gravedad y antecedentes previos de neuritis óptica.

INTRODUCTION: Quantitative assessment of macular and nerve fibre layer thickness in multiple sclerosis patients with regard to expanded disability status scale (EDSS) and presence or absence of previous optic neuritis episodes. METHODS: We recruited 62 patients with multiple sclerosis (53 relapsing-remitting and 9 secondary progressive) and 12 disease-free controls. All patients underwent an ophthalmological examination, including quantitative analysis of the nerve fibre layer and macular thickness using optical coherence tomography. Patients were classified according to EDSS as A (lower than 1.5), B (between 1.5 and 3.5), and C (above 3.5). RESULTS: Mean nerve fibre layer thickness in control, A, B, and C groups was 103.35±12.62, 99.04±14.35, 93.59±15.41, and 87.36±18.75µm respectively, with statistically significant differences (P<.05). In patients with no history of optic neuritis, history of episodes in the last 3 to 6 months, or history longer than 6 months, mean nerve fibre layer thickness was 99.25±13.71, 93.92±13.30 and 80.07±15.91µm respectively; differences were significant (P<.05). Mean macular thickness in control, A, B, and C groups was 220.01±12.07, 217.78±20.02, 217.68±20.77, and 219.04±24.26µm respectively. Differences were not statistically significant. CONCLUSIONS: The mean retinal nerve fibre layer thickness in multiple sclerosis patients is related to the EDSS level. Patients with previous optic neuritis episodes have a thinner retinal nerve fibre layer than patients with no history of these episodes. Mean macular thickness is not correlated to EDSS level.

[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.

[Hemiparkinsonism secondary to mesencephalic bleeding]. / Hemiparkinsonismo secundario a hemorragia mesencefálica.

Vascular parkinsonism is the second cause of secondary parkinsonism, and can cause a complex clinical syndrome. In spite of this, it is not common to find an isolated vascular injury in the mesencephalic region, and even rarer for it to give rise to clinical parkinsonism. We present the case of a young patient who developed left hemiparkinsonism with a fluctuating clinical evolution and unpredictable response to the treatment after suffering right mesencephalic bleeding. Structural and functional neuroimaging techniques showed injury on the mesencephalic level and no uptake in the right striatal region, respectively.

[Moya-moya syndrome and arterial dysplasia associated to Down syndrome]. / Síndrome de Moyamoya y displasia arterial asociados a síndrome de Down.

INTRODUCTION: Ischemic vascular pathologies are predominant among the neurological processes associated to Down syndrome. Thrombosis of the venous sinuses, Moya-moya syndrome and arterial dysplasia, among others, stand out for their association with this syndrome. CASE REPORT: We describe the case of a 19-year-old female patient suffering from Down syndrome who visited because of deficiency symptoms of an ischemic origin; diagnosis was based on angiographic disorders compatible with Moya-moya syndrome in the left carotid tree and arterial dysplasia in the right carotid tree. Data concerning the medical history, physical examination, complementary tests, treatment applied and evaluation of the patient's clinical progress are reported. At the same time, the patient was also diagnosed as suffering from Graves-Basedow disease, which is contemplated in the aetiological study carried out on these and other vascular anomalies and which have often been described in relation to Down syndrome. CONCLUSIONS: The particular interest of our case lies in the fact that the two pathological processes occur in the same patient, although only one of them was symptomatic. Both of these vascular anomalies have been described in association with Down syndrome separately, but the simultaneous presence of both of them in the same patient is infrequent. We suggest the possibility of a common aetiological link between the two processes, which in turn accounts for their association with Down syndrome.