Long-term effects of cognitive rehabilitation on brain, functional outcome and cognition in Parkinson's disease.

BACKGROUND AND PURPOSE: Cognitive rehabilitation has demonstrated efficacy in producing short-term cognitive and brain changes in patients with Parkinson's disease (PD). To date, no study has assessed the long-term effects of cognitive rehabilitation using neuroimaging techniques in PD. The aim was to assess the longitudinal effects of a 3-month cognitive rehabilitation programme evaluating the cognitive, behavioural and neuroimaging changes after 18 months. METHODS: Fifteen patients with PD underwent a cognitive, behavioural and neuroimaging assessment at pre-treatment (T0 ), post-treatment (T1 ) and after 18 months (T2 ). This study examined the long-term effects (from T0 to T2 ) and the maintenance of the changes (from T1 to T2 ). T1-weighted, diffusion-weighted, functional magnetic resonance imaging during both a resting-state and a memory paradigm were acquired. Voxel-based morphometry and tract-based spatial statistics were used for grey and white matter analyses. A region-of-interest-to-region-of-interest approach was used for resting-state functional connectivity (FC) and a model-based approach was used for brain activation during the memory paradigm. RESULTS: Patients with PD showed increased cognitive performance, decreased functional disability, increased brain FC and activation at T2 compared with T0 (P < 0.05, FDR). Moreover, patients showed maintenance of the improvements in cognition and functionality, and maintenance of the increased brain FC and activation at T2 compared with T1 . However, significant grey matter reduction and alterations of white matter integrity were found at T2 (P < 0.05, FWE). CONCLUSIONS: Findings suggest that the improved cognitive performance and increased brain FC and activation after cognitive rehabilitation were significantly maintained after 18 months in patients with PD, despite the structural brain changes, consistent with a progression of neurodegenerative processes.

[Reversible alterations in the dentate nuclei and rapid-onset cerebral atrophy due to neurotoxicity caused by lithium]. / Alteraciones reversibles en los nucleos dentados y atrofia cerebral de rapida instauracion debido a neurotoxicidad por litio.

INTRODUCTION: Treatment with lithium can cause several neurological side effects, even at therapeutic levels. CASE REPORT: We report the case of a 49-year-old woman, with bipolar disorder and depression, undergoing treatment with lithium, antidepressants and antipsychotics, who was admitted to hospital due to a clinical picture of visual hallucinations with an elevated lithaemia of 2.1 mEq/L (therapeutic range: 0.6-1.2 mEq/L). The patient developed a severe encephalopathy that required the use of assisted ventilation in the intensive care unit. Initial magnetic resonance imaging showed a reversible bilateral symmetrical hyperintensity in the dentate nuclei in T2 and T2-FLAIR sequences. Over the following months she gradually developed a pancerebellar syndrome with evidence of a marked loss of bilateral volume in the cerebellum, above all at the expense of the vermis, which was accompanied by a permanent and disabling cerebellar syndrome. CONCLUSIONS: Although treatment with lithium can cause a variety of neurological side effects, they are usually reversible. However, they occasionally give rise to permanent and disabling sequelae, as in the case of the patient reported here, with a marked and progressive cerebellar atrophy, accompanied by permanent sequelae in the form of a disabling cerebellar syndrome. The cerebellar neurotoxicity of lithium must be taken into account in the broad differential diagnosis of cerebellar ataxia in adults.

TITLE: Alteraciones reversibles en los nucleos dentados y atrofia cerebral de rapida instauracion debido a neurotoxicidad por litio.Introduccion. El tratamiento con litio puede ocasionar diversos efectos adversos neurologicos, incluso con niveles terapeuticos. Caso clinico. Mujer de 49 años, con trastorno bipolar y depresion, en tratamiento con litio, antidepresivos y antipsicoticos, que ingreso por un cuadro de alucinaciones visuales con una litemia elevada de 2,1 mEq/L (rango terapeutico: 0,6-1,2 mEq/L). Progreso a una encefalopatia grave que requirio asistencia respiratoria en la unidad de cuidados intensivos. La resonancia magnetica cerebral inicial mostro una hiperintensidad simetrica bilateral reversible en los nucleos dentados en las secuencias T2 y T2-FLAIR. A lo largo de los meses posteriores desarrollo de forma progresiva un sindrome pancerebeloso con evidencia de una marcada perdida de volumen bilateral en el cerebelo, sobre todo a expensas del vermis, que se acompaño clinicamente de un sindrome cerebeloso permanente e invalidante. Conclusiones. Aunque el tratamiento con litio ocasiona efectos adversos neurologicos variados, estos suelen ser reversibles. Puede dar lugar a secuelas permanentes e incapacitantes, como la paciente descrita, con una atrofia cerebelosa marcada y progresiva, acompañada de secuelas permanentes en forma de sindrome cerebeloso invalidante. La neurotoxicidad cerebelosa del litio debe considerarse en el amplio diagnostico diferencial que representa la ataxia cerebelosa del adulto.

[Personality factors, degree of disability and therapeutic management of patients with migraine visiting a neurology unit for the first time (Psicomig study)]. / Factores de personalidad, grado de discapacidad y abordaje terapeutico de los pacientes con migrana atendidos en primera consulta en neurologia (estudio Psicomig).

INTRODUCTION: Migraine has recently been associated to certain personality profiles and styles of coping. AIM: To explore the association between personality factors, disability and the therapeutic management of migraine. PATIENTS AND METHODS: We conducted an epidemiological, cross-sectional, multi-centre study with patients with migraine visiting a neurology unit for the first time. Socio-demographic and clinical data were collected about the patients. The NEO-FFI (Neuroticism-Extraversion-Openness Five-Factor Inventory) was used to evaluate personality factors; the degree of disability was evaluated using the Headache Impact Test (HIT-6) and the number of lost workday equivalents (LWDE) was measured. Bivariate logistic regression analyses were also performed. RESULTS: A total of 736 patients were recruited, of whom 700 were suitable for inclusion in the analysis (75.6% females; mean age: 35.5 ± 11.5 years). In all, 68.9% presented migraine without aura, 1-4 seizures/month (66.7%) and of moderate intensity (58.1%). A total of 76.1% of patients had severe disability according to the HIT-6. Of the 554 active patients, the mean number of lost workday equivalents in the previous three months was 6.8 ± 8.2. Patients showed greater emotional instability than the general population and they scored lower on extraversion, openness, agreeableness and conscientiousness. All the patients were being treated for their migraine: 47.3% by means of stepped treatment between seizures; 39.9% intra-seizures, and stratified in only 12.9%. CONCLUSIONS: This study confirms the impact of migraine in terms of disability and in terms of loss of labour output, together with its association with personality factors.

Progression from amnesic mild cognitive impairment to Alzheimer's disease: ESR1 and ESR2 polymorphisms and APOE gene.

BACKGROUND: Many genes have been studied to determine how they might be involved in Alzheimer's disease (AD). Estrogens have a protective effect in the central nervous system. The mechanisms of action of estrogens are mediated by two estrogen receptors (ERs), ERα and ERß. Thus, these genes could also play a role in the progression of amnesic mild cognitive impairment (MCIa) to AD. The aim of this study was to examine the role of ER single nucleotide polymorphisms (SNPs) as a risk factor for MCIa, as well as the interaction with apolipoprotein E (APOE) ε4 in the progression to AD. METHODS: 79 MCIa patients and 138 healthy controls were analyzed. SNPs were genotyped via restriction fragment length polymorphisms and real-time PCR, RT-PCR or RT-PCR (TaqMan) assays. RESULTS: There is a lack of association between MCIa patients who converted to AD and ER SNPs. APOE ε4 allele is an independent risk factor of MCIa (OR=1.86; 95% CI=1.02-3.38, p=0.042) with a high prevalence in converted subjects. APOE ε4 is able to predict the progression from MCIa patients to AD (OR=2.55; 95% CI=1.20-5.42, p=0.015). CONCLUSIONS: The presence of the APOE ε4 allele, and not the alleles of ER SNPs, is a risk factor for MCIa. Furthermore, APOE genotype seems to predict the conversion from MCIa to AD.

Impact of preventive therapy with nadolol and topiramate on the quality of life of migraine patients.

Preventive therapy is aimed at reducing migraine frequency, but should also improve the much deteriorated quality of life of the migraneur. We aimed to evaluate the impact of preventive therapy with two widely employed drugs (topiramate and nadolol) on the quality of life of migraine patients. A population of consecutive migraineurs aged > or = 16 years, with frequent migraines, was selected prospectively for evaluation at baseline and after 16 weeks of therapy with nadolol or topiramate (40 mg and 100 mg daily, respectively) by generic and specific quality of life questionnaires (SF-36 and MSQOL) and by an anxiety and depression scale (HADS). Preventive therapy resulted in a statistically significant improvement in physical domains of the SF-36, whereas mental domains remained almost unchanged. Despite this improvement, all domains remained below the population norms. The HADS revealed a moderate depressive state at baseline that did not change with therapy. The MSQOL global score also revealed statistically significant improvement. Both drugs were similarly effective, although topiramate was superior on the role physical domain compared with nadolol. Preventive therapy with nadolol and topiramate significantly improves the quality of life of migraineurs, although additional efforts are needed to place them in a nearer-to-normal situation compared with the general population.

Empiema de fosa posterior como complicación de una tromboflebitis del seno cavernoso por Enterococcus faecium / Posterior fossa empyema as a complication deriving from thrombophlebitis of the cavernous sinus caused by Enterococcus faecium

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Mecanismos cerebrales implicados en la toma de decisiones / The brain mechanisms involved in making decisions

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[Cannabis and cerebrovascular disease]. / Cannabis y patología vascular cerebral.

INTRODUCTION: Drug use is a well-kown risk factor for cerebrovascular disease in young people. Cannabis is the most widely consumed among the illicit drugs worldwide, but it has only exceptionally been associated to cerebrovascular disease. CLINICAL CASE: We here describe 2 young patients (26 and 29 years, respectively) who suffered from ischemic stroke in temporal relation with cannabis consumption. CONCLUSIONS: The review of the literature on this topic reveals another 18 patients with stroke in association to cannabis use. They all were young people with ischemic stroke. Although a causal relationship is difficult to establish due to the widespread use of cannabis, this drug may play an etiologic role in ischemic stroke.

Cannabis y patología vascular cerebral / Cannabis and cerebrovascular disease

Introducción. El consumo de drogas es un conocido factor de riesgo cerebrovascular en personas jóvenes. El cannabis es la droga ilegal más extendida en el mundo, pero sólo de forma excepcional se ha asociado su consumo con la patología vascular cerebral. Casos clínicos. Describimos dos pacientes varones de 26 y 29 años sin factores de riesgo vascular y que tienen un ictus isquémico cerebral en estrecha relación temporal con el consumo de cannabis. Por otra parte, revisamos los casos clínicos de enfermos con ictus cerebral tras consumo de cannabis descritos previamente. Conclusiones. La revisión de la literatura arroja otros 18 pacientes con ictus asociado al cannabis. Todos son varones jóvenes, con patología vascular cerebral isquémica y en general consumidores habituales de cannabis, pero no de otras drogas. Aunque la relación causal no es fácil de establecer debido a su extenso consumo, el cannabis puede ser un factor de riesgo y estar implicado en la etiología del ictus isquémico en varones jóvenes

Introduction: Drug use is a well-kown risk factor for cerebrovascular disease in young people. Cannabis is the most widely consumed among the illicit drugs worldwide, but it has only exceptionally been associated to cerebrovascular disease. Clinical case: We here describe 2 young patients (26 and 29 years, respectively) who suffered from ischemic stroke in temporal relation with cannabis consumption. Conclusions: The review of the literature on this topic reveals another 18 patients with stroke in association to cannabis use. They all were young people with ischemic stroke. Although a causal relationship is difficult to establish due to the widespread use of cannabis, this drug may play an etiologic role in ischemic stroke

Recurrent stroke associated with cannabis use.

Drug misuse represents a risk factor for cerebrovascular disease, especially among young people. Despite the fact that cannabis is the most widely used illicit drug, there are only a few reports associating its use with cerebrovascular disease. We describe a patient who suffered three ischaemic strokes immediately after cannabis consumption. Other stroke aetiologies were ruled out, and neuroimaging revealed infarcts in different arterial areas as well as evidence of non-atherosclerotic arterial disease, which suggests an underlying vasculopathy of uncertain (toxic or inflammatory) origin. Cannabis use may be associated with ischaemic stroke in young patients, but its mechanism is unclear.

[Acute confusional state secondary to transient headache and neurological deficits with cerebrospinal fluid lymphocytosis]. / Estado confusional agudo secundario a síndrome de cefalea y déficit neurológicos transitorios con linfocitosis en líquido cefalorraquídeo.

Pseudomigraine with pleocytosis (now referred to as syndrome of transient headache and neurological deficits with cerebrospinal fluid lymphocytosis, IHS ICHD-II code 7.8) is only infrequently accompanied by a confusional state and no cases have been described so far in association with severe agitation. We report on a patient who suffered from two episodes of headache with transient focal neurological deficits that were consistent with pseudomigraine with pleocytosis but that were followed by an intense confusional, agitated state that required admission in the intensive care unit. This possibility should be included in the differential diagnosis of confusional, agitated states.

[Stroke and transient ischemic attack-mimicking conditions: a prospective analysis of risk factors and clinical profiles at a general hospital]. / Condiciones que simulan un ictus y un ataque isquémico transitorio: análisis prospectivo de los factores de riesgo y perfil clínico en un hospital general.

BACKGROUND: Nonvascular structural entities can simulate stroke and transient ischemic attacks (TIA) in a variable percentage, but this issue has not been properly addressed in our environment. Their differentiation is important, since the management of patients with a true cerebrovascular accident differs considerably from that of mimicking conditions. OBJECTIVES: To analyze prospectively those structural disorders that mimic stroke and TIA in the department of neurology of a general hospital and to determine if there are any distinctive risk factors and/or clinical presentations that would allow for their separation from true stroke and TIA. PATIENTS AND METHOD: All patients admitted to our Department of Neurology with stroke or TIA were evaluated prospectively for a 4-year period. Those in whom a mimicking condition was disclosed were the subjects of this study. Their clinical features and vascular risk factors were compared with a sample of randomly selected patients with true stroke or TIA admitted during the same period. RESULTS: A total of 28 patients comprised the stroke-TIA mimicking group, 21 of them had a stroke-like syndrome and 7 a TIA-like syndrome. The mimicking group represented a 1.9% (2% of strokes and 1.6% of TIA) of all the patients with true cerebrovascular disorders admitted during the same period. Cancer, either primary or metastatic, was the single most frequent mimicking condition, accounting for two-thirds of the cases. Risk factors for stroke and TIA were significantly more frequent in the true stroke-TIA group without differences in age or sex, while the opposite is true in terms of prior or concurrent cancer. No single clinical profile allowed for a distinction between the two groups, although vertebrobasilar dysfunction suggested true ischemia, and vomiting, isolated dysarthria and dysarthria-clumsy hand occurred only in the true stroke-TIA group. CONCLUSIONS: Nonvascular conditions can cause signs and symptoms indistinguishable from true stroke and TIA, representing about 2% of the patients admitted to a department of neurology with these diagnoses. Although there are some differences in clinical presentation and risk factors between both groups, neuroimaging studies will be required in the individual patient to rule out mimicking conditions.

[Postoperative bilateral brachial plexopathy mimicking the "man-in-the-barrel" syndrome]. / Plexopatía braquial bilateral simulando el síndrome del "hombre en el barril".

We describe a patient with a postoperative bilateral upper limb palsy due to involvement of the upper trunk of the brachial plexus. The weakness distribution (bilateral upper limb paresis without leg involvement) reminded of the classic "man-in-the-barrel" syndrome, which is usually due to bilateral watershed infarcts of the brain in relation with severe hypotension. Bilateral postoperative brachial plexopathies are very uncommon and should be distinguished from the "man-in-the-barrel" syndrome of "central" origin, since the former bears a much better prognosis and management is different. The mechanisms of plexus damage during surgery, as well as the different etiologies of the "man-in-the-barrel" syndrome, are discussed.

Condiciones que simulan un ictus y un ataque isquémico transitorio: análisis prospectivo de los factores de riesgo y perfil clínico en un hospital general / Stroke and transient ischemic attackmimicking conditions: a prospective analysis of risk factors and clinical profiles at a general hospital

FUNDAMENTO: Diversas condiciones estructurales de origen no vascular pueden simular un ictus o un ataque isquémico transitorio (AIT), aunque este problema nunca ha sido evaluado de forma prospectiva en nuestro entorno. Su diferenciación es fundamental, pues el diagnóstico y el tratamiento difieren considerablemente. OBJETIVOS: Analizar de modo prospectivo las entidades estructurales que simulan un ictus o un AIT en un servicio de neurología de un hospital general y determinar si existe algún perfil de presentación clínica o de factores de riesgo que pudiera proporcionar la clave para un diagnóstico correcto. PACIENTES Y MÉTODO: Se evaluaron de forma prospectiva todos los pacientes ingresados en nuestro servicio con un ictus o un AIT durante un período de 4 años. Aquellos en los que se detectó una 47.883 condición imitadora constituyeron el grupo de estudio, y sus características clínicas y perfil de riesgo vascular se compararon con un grupo de pacientes con ictus o AIT auténticos que ingresaron durante el mismo período y seleccionados al azar. RESULTADOS: Se encontraron 28 pacientes con condiciones estructurales que simulaban un ictus (21 pacientes) o un AIT (7 pacientes). Este grupo de 28 enfermos representaba un 1,9 per cent de todos los pacientes ingresados en nuestro servicio con accidente cerebrovascular real (2 per cent de los ictus y 1,6 per cent de los AIT). La causa imitadora más frecuente (dos tercios de los casos) fue el cáncer, tanto primario como metastásico. Los factores de riesgo vascular eran significativamente más frecuentes en el grupo de ictus y AIT verdaderos, sin diferencias en cuanto a edad o sexo, y lo contrario ocurrió con el antecedente de cáncer o su presencia actual. El patrón de presentación clínica no permitía la diferenciación de las condiciones imitadoras, aunque la disfunción vertebrobasilar sugería isquemia auténtica y la disartria aislada, los vómitos y el síndrome disartria-mano torpe sólo se presentaron en el grupo con enfermedad vascular verdadera. CONCLUSIONES: Aproximadamente el 2 per cent de los pacientes ingresados en un servicio de neurología general con el diagnóstico de ictus o AIT tienen una causa no vascular que justifica su cuadro clínico. Aunque existen algunas diferencias clínicas y de factores de riesgo vascular entre ambos grupos, los estudios de neuroimagen son necesarios en el paciente individual para establecer el diagnóstico correcto. (AU)

Plexopatía braquial bilateral simulando el síndrome del "hombre en el barril" / Postoperative bilateral brachial plexopathy mimicking the "man-in-the-barrel" syndrome

Describimos un paciente con una parálisis bilateral de los miembros superiores secundaria a una lesión posquirúrgica del plexo braquial superior. La distribución de la debilidad (parálisis de los miembros superiores con respecto de los inferiores) recordaba el síndrome del "hombre en el barril" clásico, que habitualmente es debido a infartos cerebrales en territorios arteriales limítrofes provocados por una hipotensión grave. La plexopatía braquial bilateral posquirúrgica es muy infrecuente y se debe diferenciar del síndrome del "hombre en el barril" por lesión del sistema nervioso central, pues el pronóstico y manejo de ambas entidades es muy diferente. Se plantean los diferentes mecanismos de lesión del plexo braquial durante la cirugía, así como las diferentes etiologías del síndrome del "hombre en el barril". (AU)

[Ischemic stroke in a patient with factor XII (Hageman) deficiency].

Unlike most other coagulation factor deficiencies, usually associated with abnormal bleeding, lack of factor XII (Hageman) can result in thromboembolic events as a result of a deficient fibrinolytic system. We report a patient with an ischemic stroke and factor XII deficiency, a rare hereditary disorder. The optimal therapy for these uncommon disorders is not well established, but they probably require long term anticoagulation to prevent subsequent thrombotic events.

Ischemic stroke in a patient with factor XII (Hageman) deficiency

A diferencia de la mayoría de los déficit de factores de la coagulación, que habitualmente cursan con hemorragia, la ausencia de factor XII (Hageman) puede dar lugar a fenómenos tromboembólicos como resultado de un sistema fibrinolítico deficitario. Se describe a un paciente con un infarto cerebral asociado a un déficit de factor XII, un trastorno hereditario infrecuente. El tratamiento idóneo de estas enfermedades tan poco comunes no está bien establecido, aunque pueden requerir anticoagulación a largo plazo para prevenir posteriores episodios tromboembólicos. (AU)