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

Introducción: El examen de la sensibilidad al contraste permite determinar la calidad de la función visual en pacientes con esclerosis múltiple (EM). El objetivo de este estudio es analizar las modificaciones evolutivas de la función visual en pacientes con EM remitente-recurrente. Métodos: Estudio longitudinal de 61 pacientes clasificados en 3 grupos: a) pacientes libres de enfermedad (grupo control); b) pacientes con EM y sin antecedentes de neuritis óptica (NO), y c) pacientes con EM y antecedentes de NO unilateral. A todos los pacientes se les realizó una exploración oftalmológica que incluía agudeza visual y test de sensibilidad al contraste tipo Pelli-Robson mono y binocularmente, tanto al inicio como a los 6 años de seguimiento. Resultados: La sensibilidad al contraste monocular en pacientes con EM con y sin antecedentes de NO fue significativamente inferior al grupo control tanto al inicio (p=0,00 y p=0,01) como a los 6 años (p = 0,01 y p = 0,02), manteniéndose estable a lo largo del seguimiento excepto en el grupo de pacientes con NO en el cual existe una pérdida significativa de sensibilidad al contraste (p = 0,01). La agudeza visual y la sensibilidad al contraste binocular al inicio y a los 6 años de seguimiento fueron significativamente inferiores en el grupo de pacientes con antecedentes de NO que en el grupo control (p = 0,003 y p = 0,002; p = 0,006 y p = 0,005) y con EM sin NO (p = 0,04 y p = 0,038; p = 0,008 y p = 0,01); sin embargo, no encontramos diferencias significativas en el seguimiento (p = 0,1 y p = 0,5). Conclusiones: El test de Pelli-Robson monocular podría servir como marcador evolutivo del deterioro de la función visual en ojos con NO

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

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.

Localización del punto de fuga de líquido cefalorraquídeo en un caso de hipotensión de líquido cefalorraquídeo / Locating the site of cerebrospinal fluid leak in a patient with low cerebrospinal fluid pressure

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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 / Analysis of macular and nerve fiber layer thickness in multiple sclerosis patients according to severity level and optic neuritis episodes

Introducción: Evaluar cuantitativamente el grosor macular y de la capa de fibras nerviosas en pacientes con esclerosis múltiple en relación con la escala expandida del estado de discapacidad (EDSS) con o sin antecedentes previos de neuritis óptica. Métodos: Sesenta y dos pacientes diagnosticados de esclerosis múltiple (53 remitente recidivante y 9 secundariamente progresiva) y 12 libres de enfermedad fueron reclutados para el estudio. Se les realizó una exploración oftalmológica, incluyendo el análisis cuantitativo de la capa de fibras nerviosas retinianas y el grosor macular mediante tomografía óptica de coherencia. Los pacientes fueron clasificados según la escala EDSS en: A: inferior a 1,5; B: entre 1,5 y 3,5, y C: superior a 3,5. Resultados: El grosor medio ± desviación estándar de la capa de fibras nerviosas en los grupos control, A, B y C fue de 103,35 ± 12,62, 99,04 ± 14,35, 93,59 ± 15,41 y 87,36 ± 18,75 μm, respectivamente, con diferencias estadísticamente significativas (p < 0,05). En pacientes sin una historia previa de neuritis, o con un episodio de esta patología entre 3 y 6 meses de evolución o anterior a 6 meses, el grosor medio fue de 99,25 ± 13,71, 93,92 ± 13,30, 80,07 ± 15,91 μm, respectivamente, con diferencias significativas (p < 0,05). El grosor macular medio en el grupo control, A, B y C se situó en 220,01 ± 12,07, 217,78 ± 20,02, 217,68 ± 20,77 y 219,04 ± 24,26 μm, respectivamente. Las diferencias observadas entre grupos no fueron estadísticamente significativas. Conclusiones: El grosor medio de la capa de fibras nerviosas en pacientes con esclerosis múltiple se relaciona con el nivel en la escala EDSS. Los pacientes con historia previa de neuritis óptica cursan con una disminución del grosor de esta capa respecto a aquellos sin antecedentes de neuritis. El grosor macular no se relaciona con el grado de afectación en la EDSS

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 ± 18.75 μm 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 ± 15.91 μm 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 ± 24.26 μm 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 leve

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.

Síndrome de Miller Fisher como presentación de una encefalopatía de Wernicke / Miller fisher syndrome as the presenting symptom of wernicke’s encephalopathy

La encefalopatía de Wernicke es un síndrome neuropsiquiátrico agudo secundario a un déficit de tiamina, definido por la tríada característica de confusión, oftalmoparesia y ataxia, aunque se han descrito presentaciones raras que retrasan el diagnóstico. El síndrome de Miller Fisher se caracteriza por la tríada de oftalmoparesia, ataxia y arreflexia,y se considera una variante del síndrome de Guillain-Barré; su diagnóstico diferencial incluye la encefalopatía de Wernicke. Caso clínico. Mujer de 75 años con trastorno digestivo crónico, que desarrolla un cuadro agudo de oftalmoplejía internuclearbilateral, ataxia y arreflexia, con disociación proteinocitológica en el líquido cefalorraquídeo, por lo que se propuso el diagnóstico inicial de síndrome de Miller Fisher. Los estudios neurofisiológicos fueron normales, los anticuerpos anti-GQ1b,negativos, y la resonancia magnética cerebral sugirió una encefalopatía de Wernicke; la respuesta a la tiamina fue espectacular. Conclusiones. Las similitudes en la distribución lesional de ambas entidades, en la semiología y los resultados analíticos, así como la influencia de ciertos factores de confusión (hiponatremia, edad avanzada), conformaron un síndrome típico que favoreció un diagnóstico de sospecha etiológico erróneo, que pudo modificarse precozmente a la luz de ciertas pruebas diagnósticasy después de la respuesta terapéutica. Además de tratarse de una presentación atípica para una encefalopatía de Wernicke, este caso recuerda que la conjunción del diagnóstico sindrómico con el etiológico pasa por realizar un correcto diagnóstico diferencial apoyándonos en detalles de la anamnesis, en las pruebas complementarias necesarias y en el seguimientoevolutivo de los pacientes, incluso cuando nos encontramos ante síndromes típicos habitualmente relacionados con una etiopatogenia predominante

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 areflexiaand 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), wentto 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 anatypical 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 thepatient’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.

Hemiparkinsonismo secundario a hemorragia mesencefálica / Hemiparkinsonism secondary to mesencephalic bleeding

El parkinsonismo de origen vascular es la segunda causa de parkinson secundario, pudiendo originar una sintomatología variada y compleja. A pesar de ello es raro encontrar una lesión vascular aislada a nivel mesencefálico, siendo más extraño aún el hecho de que origine clínica parkinsoniana. Presentamos el caso de un paciente joven que tras sufrir una hemorragia mesencefálica derecha desarrolló un cuadro de hemiparkinsonismo izquierdo con una evolución clínica fluctuante y una respuesta al tratamiento imprevisible. Con las técnicas de neuroimagen estructural se evidenció la lesión a nivel de mesencéfalo y con la neuroimagen funcional se puso de manifiesto ausencia de captación a nivel del estriado derecho (AU)

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 (AU)

Meningiomas múltiples / Multiple meningiomas

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Falsa imagen aneurismática / False image of an aneurysm

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[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.

Esclerosis múltiple hemipléjica"seudoictal", una forma excepcionalde debut / "Pseudo-stroke" hemiplegic type multiple sclerosis, a rare form of debut

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Epilepsia del lóbulo temporal secundaria a aneurisma gigante intracerebral / Temporal lobe epilepsy, secondary to intracranial giant aneurysm

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