Relación de los espacios de Virchow-Robin con la enfermedad de Alzheimer: a propósito de un caso / Relationship between Virchow-Robin spaces and Alzheimer disease: A case repor

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[Paraproteinemic neuropathies: foundations for a specific therapy related to patients' quality of life]. / Neuropatias paraproteinemicas: bases para un tratamiento especifico con relacion a la calidad de vida de los pacientes.

INTRODUCTION: Monoclonal gammopathy (MG) is reported in 1% of subjects above 50 years of age and in 3% of those above the age of 70. Of all patients with MG, 3% present polyneuropathy (PNP). The abbreviation MGUS (monoclonal gammopathy of undetermined significance) is used to describe a benign proliferation of M component (monoclonal) according to Kyle's criteria, with a potential to be malignant that is indicated by clinical, biochemical and haematological parameters. Aims. The aim of this study was to evaluate the effect of the clinical exacerbation of MGUS associated neuropathy, by referring to the existing literature and to our own experience in order to set out a number of proposals for treatment based on the deterioration of the patient's quality of life. CASE REPORTS: Case 1: a 53 year old female with predominantly sensory sensory motor demyelinating polyradiculoneuropathy, associated to IgG lambda MGUS. Case 2: a 65 year old female with demyelinating sensory motor polyradiculoneuropathy associated to IgM kappa MGUS. In both cases there was a rapidly progressing neurological deterioration and no other data exist to suggest malign lymphoproliferation. CONCLUSIONS: In addition to the treatments established for MG linked PNP, which are based on the modulation of the immune response, it has also been proved that when malign proliferation of plasmacytes exists specific treatment of the gammopathy can significantly stabilise or improve the neuropathic symptoms. We propose gammopathy specific haematological treatment in patients who still meet Kyle's criteria for MGUS with a torpid neurological course and scarce response to therapy with immunomodulators, prior to an important deterioration in the quality of life. We also suggest establishing this rapidly progressing clinical course as a criterion that indicates the process of becoming malign as put forward by Eurelings et al.

[Visual neglect secondary to left frontal haemorrhage]. / Negligencia visual secundaria a hemorragia frontal izquierda.

INTRODUCTION: The term visual neglect refers to a lack of attention to visual stimuli coming from the contralateral hemifield. The patient does not seek objects in the abnormal field and often only sees half of a paragraph when reading. Although the right inferior parietal lobe is the most frequently damaged region, the same symptomatology can be due to injury to the frontal lobe, the basal ganglia and the thalamus. CASE REPORT: Female aged 71 with a mitral prosthesis and atrial fibrillation in treatment with dicumarols, who presented a left frontal parenchymatous haemorrhage. The exploration revealed right visual hemineglect without ipsilateral homonymous hemianopsia, associated to a right hemiparesis. DISCUSSION: Even when visual neglect has been described in lesions affecting the parietal lobe, especially on the right hand side, the same symptomatology can be due to injury to the basal ganglia, the thalamus and the frontal lobe. In this last case, it seems that the basis for the pathogenesis lies in the damage done to the underlying white matter, which would disconnect the posterior parietal cortex from the prefrontal cortex.

Negligencia visual secundaria a hemorragia frontal izquierda / Visual neglect secondary to left frontal haemorrhage

Introducción. El término negligencia visual hace referencia a una falta de atención a los estímulos visuales procedentes del hemicampo contralateral. El paciente no busca objetos en el campo anormal, y a menudo sólo lee la mitad del párrafo. Si bien el lóbulo parietal inferior derecho es la región más frecuentemente lesionada, la misma sintomatología puede obedecer a lesiones del lóbulo frontal, ganglios de la base y tálamo. Caso clínico. Mujer de 71 años de edad portadora de prótesis mitral con fibrilación auricular y en tratamiento con dicumarínicos, que presenta una hemorragia parenquimatosa frontal izquierda. En la exploración se objetivó una heminegligencia visual derecha sin hemianopsia homónima ispilateral, asociada a una hemiparesia derecha. Discusión. Aun cuando la negligencia visual se ha descrito en lesiones que afectan al lóbulo parietal, sobre todo el derecho, la misma sintomatología puede obedecer a lesiones de los ganglios de la base, tálamo y lóbulo frontal. En este último supuesto, parece ser que la base patogénica radica en la afectación de la sustancia blanca subyacente, que desconectaría el córtex parietal posterior del córtex prefrontal (AU)

Introduction. The term visual neglect refers to a lack of attention to visual stimuli coming from the contralateral hemifield. The patient does not seek objects in the abnormal field and often only sees half of a paragraph when reading. Although the right inferior parietal lobe is the most frequently damaged region, the same symptomatology can be due to injury to the frontal lobe, the basal ganglia and the thalamus. Case report. Female aged 71 with a mitral prosthesis and atrial fibrillation in treatment with dicumarols, who presented a left frontal parenchymatous haemorrhage. The exploration revealed right visual hemineglect without ipsilateral homonymous hemianopsia, associated to a right hemiparesis. Discussion. Even when visual neglect has been described in lesions affecting the parietal lobe, especially on the right-hand side, the same symptomatology can be due to injury to the basal ganglia, the thalamus and the frontal lobe. In this last case, it seems that the basis for the pathogenesis lies in the damage done to the underlying white matter, which would disconnect the posterior parietal cortex from the prefrontal cortex (AU)

[Pharmacological treatment of Korsakoff's psychosis: a review of the literature and experience in two cases]. / Tratamiento farmacológico de la psicosis de Korsakoff: revisión de la bibliografía y experiencia en dos casos.

INTRODUCTION: Korsakoff s psychosis (KP) is a relatively frequent pathological condition in our community that has been infradiagnosed. The most common cause is chronic alcohol consumption, although it can be brought about by other aetiologies accompanied by vitamin deficiencies. The lack of thiamine entails an alteration in the synthesis of neurotransmitters, which provides the neurochemical foundation for the specific cognitive impairment that defines the syndrome. AIMS: To evaluate the application of pharmacological treatments, in accordance with the neurochemical disorders described in the literature, and report our experience in two cases treated with anticholinesterases. CASE REPORTS: 1) Female aged 47, with a history of addiction to alcohol. Following Wernicke s encephalopathy, which improved with parenteral thiamine, she presented a memory disorder compatible with KP. After two months treatment with donepezil, a cognitive improvement was observed in the neuropsychological tests. 2) Male aged 77, who presented KP a month after being diagnosed and treated for a post encephalitic vasculitis caused be varicella zoster virus. His cognitive and functional condition improved after 3 months treatment with donepezil. DISCUSSION AND CONCLUSIONS: There are not enough studies in the literature with representative samples that consider the effects of thiamine or of other forms of treatment on cognitive impairment in KP. Noradrenaline, serotonin, glutamate and acetylcholine have been proposed in the pathogeny of the syndrome. Based on experiences gained in cholinergic disorders, two cases responded to treatment with donepezil. Effective treatment must be based on a combination of aetiological and pharmacological treatment, and cognitive rehabilitation.

Tratamiento farmacológico de la psicosis de Korsakoff:revisión de la bibliografía y experiencia en dos casos / Pharmacological treatment of korsakoff's psychosis: a review of the literature and experience in two cases

Introducción. La psicosis de Korsakoff (PK) constituye una patología relativamente frecuente en nuestro medio y se ha infradiagnosticado. La causa más habitual es el consumo crónico de alcohol; pueden producirla otras etiologías que cursen con déficit vitamínicos. La carencia de tiamina conlleva una alteración en la síntesis de neurotransmisores, que constituye la base neuroquímica de los específicos déficit cognitivos que definen el síndrome. Objetivo. Evaluar la aplicación de tratamientos farmacológicos, según los trastornos neuroquímicos descritos en la literatura, y exponer nuestra experiencia en dos casos tratados con anticolinesterásicos. Casos clínicos. 1) Mujer de 47 años, con antecedentes de adicción al alcohol. Tras cuadro de encefalopatía de Wernicke, que mejora con tiamina parenteral, presenta trastorno de memoria compatible con PK. A los dos meses de tratamiento con donepecilo se observa mejoría cognitiva en los tests neuropsicológicos. 2) Varón de 77 años, que presenta PK al mes de haberse diagnosticado y tratado de una vasculitis postencefalitis por virus varicela zoster. Mejora su estado cognitivo y funcional a los tres meses de tratamiento con donepecilo. Discusión y conclusiones. No existen estudios suficientes en la literatura con muestras representativas de pacientes que valoren los efectos de la tiamina ni de otros tratamientos sobre los defectos cognitivos de la PK. Noradrenalina, serotonina, glutamato y acetilcolina se han postulado en la patogenia del síndrome. Sobre la base de los trastornos colinérgicos, dos casos han experimentado respuesta al tratamiento con donepecilo. La terapéutica se sustenta en la combinación de tratamiento etiológico, farmacológico y rehabilitación cognitiva (AU)

[Tuberculous meningioma en plaque without extracerebral tuberculosis. A case report]. / Tuberculoma meníngeo en placa sin tuberculosis extracerebral. A propósito de un caso.

INTRODUCTION: Tuberculosis continues to be an intensly extended disease in under developed countries in immunodepressed and immunocompetent persons. In the central nervous system tuberculous lesions are the most frequent cause of space occupying lesions. CLINICAL CASE: We report the case of a young, immunocompetent woman from Guinea, currently living in Spain. Following a seizure, neuro imaging showed there to be a right frontal space occupying lesion. The characteristics of the MR images obtained were fundamental to the determination of the meningeal site of the lesion en plaque and to suggest the aetiology as being an infectious inflammatory process, although a meningioma could not be ruled out. The absence of signs of extracerebral disease, the localization and distribution of the lesion and lack of short term response to medical treatment, which covered the different diagnostic possibilities, made it necessary to do a diagnostic meningeal biopsy. CONCLUSIONS: MR imaging is a great help in the diagnosis of tuberculous lesions of the central nervous system. The differential diagnosis between tuberculous pachymeningitis and meningioma, both in the form of plaques, is extremely difficult without obtaining a biopsy specimen. The finding of epithelioid granulomas with caseous necrosis and/or acid alcohol fast bacilli in the biopsy specimen is diagnostic of tuberculosis. A single meningeal lesion situated in the cortex but with no associated extracerebral disease causes considerable difficulty in diagnosis. Tuberculosis must always be remembered in view of the increasing number of immigrants from under developed countries.

Tuberculoma meníngeo en placa sin tuberculosis extracerebral.A propósito de un caso / Tuberculous meningioma en plaque without extracerebral tuberculosis. A case report

Introducción. La tuberculosis continúa siendo una enfermedad muy extendida en los países en vías de desarrollo en sujetos inmunodeprimidos e inmunocompetentes. En el ámbito de sistema nervioso central, las lesiones tuberculosas constituyen la causa más frecuente de lesión ocupante de espacio. Caso clínico. Presentamos el caso de una paciente joven, inmunocompetente, procedente de Guinea y residente actual de nuestro país, a la que, tras una crisis convulsiva, se le detectó, mediante neuroimagen, una lesión ocupante de espacio frontal derecho. Las características de las imágenes por RM fueron básicas para determinar la localización meníngea `en placa' de la lesión y para obtener una aproximación etiológica como proceso infeccioso-inflamatorio, aunque no descartaba meningioma. La ausencia de signos de enfermedad extracerebral, la localización y distribución de la lesión y la ausencia de respuesta a corto plazo a tratamiento médico, que cubría diferentes posibilidades diagnósticas, determinaron la práctica de una biopsia meníngea, que fue diagnóstica. Conclusiones. Las imágenes por RM constituyen un pilar de apoyo diagnóstico clave en las lesiones tuberculosas del sistema nervioso central. El diagnóstico diferencial entre paquimeningitis tuberculosa y meningioma, ambos en placa, es sumamente difícil sin una biopsia. El hallazgo de granulomas epitelioides con necrosis caseosa o bacilos ácido-alcohol resistentes en material de biopsia es diagnóstico de tuberculosis. Una lesión meníngea única, de localización cortical y sin enfermedad extracerebral, plantea importantes dudas diagnósticas. La posibilidad de tuberculosis debe estar presente, dado el creciente número de inmigrantes procedentes de países subdesarrollados (AU)

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