La asistencia telefónica de las enfermedades neurológicas: una revisión sistemática / Telephone assistance for neurological diseases: a systematic review

Introducción y objetivo: Es conocido que parte de la asistencia a los pacientes neurológicos se presta por teléfono, pero desconocemos las enfermedades que se atienden de esta manera y qué partes de la asistencia se proporcionan así. Pretendemos averiguarlo a través de esta revisión bibliográfica. Materiales y métodos: Se han revisado sistemáticamente las referencias sobre asistencia telefónica a enfermedades neurológicas accesibles a través de las plataformas PubMed, Embase y Cochrane, sin fecha de inicio y hasta el 3 de abril de 2022. Se encontraron 618 referencias, de las que 219 no pasaron los criterios de exclusión, por lo que se revisaron 399. Resultados: Hay un aumento de publicaciones en los últimos años y, aunque la demencia no es la enfermedad más prevalente, es el área de la neurología con más publicaciones sobre asistencia telefónica. Le siguen los ictus, el traumatismo craneoencefálico, la esclerosis múltiple, la enfermedad de Parkinson y trastornos del movimiento, la epilepsia, las enfermedades neuromusculares y otras. Discusión y conclusiones. Las demencias son las enfermedades con más referencias bibliográficas sobre su asistencia telefónica a pesar de no ser las más prevalentes. Con frecuencia, el teléfono se utiliza para administrar escalas diagnósticas o apoyar a los cuidadores, y es especialmente útil en enfermedades que dificultan la movilidad y acudir presencialmente.(AU)

Introduction and aim: While part of the care for neurological patients is done by telephone, it is not well known what neurological diseases and which part of that care is provided by telephone. Our goal is to find it out through a bibliographic review. Materials and methods: References on telephone care for neurological diseases accessible through the PubMed, Embase, and Cochrane platforms have been systematically reviewed, with an unspecified start date and up to March 2022. We found 618 references, and as 219 did not pass the exclusion criteria, 399 were finally included in the review. Results: Dementia is the area of neurology with more publications about its telephone assistance. It is followed by stroke, head trauma, multiple sclerosis, Parkinson’s disease and movement disorders, epilepsy, neuromuscular disorders, and others. Discussion and conclusions: Dementias are the diseases with more bibliographic references on their telephone assistance despite not being the most prevalent. The telephone is frequently used to administer diagnostic scales or support caregivers and is particularly useful in diseases that limit mobility and attending a medical practice.(AU)

[Telephone assistance for neurological diseases: a systematic review]. / La asistencia telefónica de las enfermedades neurológicas: una revisión sistemática.

INTRODUCTION AND OBJECTIVE: While part of the care for neurological patients is done by telephone, it is not well known what neurological diseases and which part of that care is provided by telephone. Our goal is to find it out through a bibliographic review. MATERIALS AND METHODS: References on telephone care for neurological diseases accessible through the PubMed, Embase, and Cochrane platforms have been systematically reviewed, with an unspecified start date and up to March 2022. We found 618 references, and as 219 did not pass the exclusion criteria, 399 were finally included in the review. RESULTS: Dementia is the area of neurology with more publications about its telephone assistance. It is followed by stroke, head trauma, multiple sclerosis, Parkinson's disease and movement disorders, epilepsy, neuromuscular disorders, and others. DISCUSSION AND CONCLUSIONS: Dementias are the diseases with more bibliographic references on their telephone assistance despite not being the most prevalent. The telephone is frequently used to administer diagnostic scales or support caregivers and is particularly useful in diseases that limit mobility and attending a medical practice.

TITLE: La asistencia telefónica de las enfermedades neurológicas: una revisión sistemática.Introducción y objetivo. Es conocido que parte de la asistencia a los pacientes neurológicos se presta por teléfono, pero desconocemos las enfermedades que se atienden de esta manera y qué partes de la asistencia se proporcionan así. Pretendemos averiguarlo a través de esta revisión bibliográfica. Materiales y métodos. Se han revisado sistemáticamente las referencias sobre asistencia telefónica a enfermedades neurológicas accesibles a través de las plataformas PubMed, Embase y Cochrane, sin fecha de inicio y hasta el 3 de abril de 2022. Se encontraron 618 referencias, de las que 219 no pasaron los criterios de exclusión, por lo que se revisaron 399. Resultados. Hay un aumento de publicaciones en los últimos años y, aunque la demencia no es la enfermedad más prevalente, es el área de la neurología con más publicaciones sobre asistencia telefónica. Le siguen los ictus, el traumatismo craneoencefálico, la esclerosis múltiple, la enfermedad de Parkinson y trastornos del movimiento, la epilepsia, las enfermedades neuromusculares y otras. Discusión y conclusiones. Las demencias son las enfermedades con más referencias bibliográficas sobre su asistencia telefónica a pesar de no ser las más prevalentes. Con frecuencia, el teléfono se utiliza para administrar escalas diagnósticas o apoyar a los cuidadores, y es especialmente útil en enfermedades que dificultan la movilidad y acudir presencialmente.

Profilaxis antiepiléptica en meningiomas: revisión sistemática y metaanálisis / Seizure prophylaxis in meningiomas: A systematic review and meta-analysis

Introducción: En la actualidad, no existe una indicación formal de profilaxis anticomicial en neurocirugía oncológica. Tampoco existen recomendaciones específicas sobre el uso de fármacos antiepilépticos (FAE) en pacientes portadores de meningiomas y libres de crisis que van a ser intervenidos. En general, se prescriben FAE de forma discrecional, teniendo en cuenta diversos factores de riesgo clínico-radiológicos. Presentamos una revisión sistemática y metaanálisis sobre la efectividad de la profilaxis anticomicial en meningiomas sin historia previa de crisis. Métodos: Se realizó una búsqueda sistemática en las bases de datos PubMed/MEDLINE, Cochrane Central Register of Controlled trials, Embase y clinicaltrials.gov. De los 4.368 estudios inicialmente identificados, finalmente se incluyeron 12 para la extracción de datos y análisis cualitativo. Los datos clínicos permitieron incluir únicamente 6 estudios en el metaanálisis. Se realizaron estudios de heterogeneidad, cálculo de OR combinada, evaluación del sesgo de publicación y análisis de sensibilidad. Resultados: La profilaxis con FAE en meningiomas sin crisis previas no redujo de forma significativa la incidencia de crisis postoperatorias respecto a los controles (OR combinada de Mantle-Haenszel, efectos aleatorios, de 1,26, IC del 95%, 0,60-2,78, sobre 2.041 pacientes). Sin embargo, la ausencia de estudios prospectivos, la presencia de sesgo de selección en los estudios, una probable infraestimación del número de crisis durante el seguimiento y la influencia marcada de un estudio sobre el efecto global impiden establecer una recomendación sólida en contra de la profilaxis anticomicial. Conclusiones: Dentro de las limitaciones de esta revisión, los resultados del metaanálisis no apoyan el uso rutinario de la profilaxis antiepiléptica en pacientes con meningiomas sin historia previa de crisis.(AU)

Introduction: No formal indication currently exists for seizure prophylaxis in neurosurgical oncology patients. Neither have specific recommendations been made on the use of antiepileptic drugs (AED) in seizure-free patients with meningiomas scheduled for surgery. AEDs are generally prescribed on a discretionary basis, taking into consideration a range of clinical and radiological risk factors. We present a systematic review and meta-analysis exploring the effectiveness of antiepileptic prophylaxis in patients with meningioma and no history of seizures. Methods: We performed a systematic review of the PubMed/MEDLINE, Cochrane Central Register of Controlled Trials, Embase, and clinicaltrials.gov databases. Of a total of 4368 studies initially identified, 12 were selected for extraction of data and qualitative analysis. Based on the clinical data presented, we were only able to include 6 studies in the meta-analysis. We performed heterogeneity studies, calculated a combined odds ratio, evaluated publication bias, and conducted a sensitivity analysis. Results: AED prophylaxis in patients with meningioma and no history of seizures did not significantly reduce the incidence of post-operative seizures in comparison to controls (Mantel-Haenszel combined odds ratio, random effects model: 1.26 [95% confidence interval, 0.60-2.78]; 2041 patients). However, we are unable to establish a robust recommendation against this treatment due to the lack of prospective studies, the presence of selection bias in the studies reviewed, the likelihood of underestimation of seizure frequency during follow-up, and the strong influence of one study on the overall effect. Conclusions: Despite the limitations of this review, the results of the meta-analysis do not support the routine use of seizure prophylaxis in patients with meningioma and no history of seizures.(AU)

Epidemiology of ataxia and hereditary spastic paraplegia in Spain: A cross-sectional study.

INTRODUCTION: Ataxia and hereditary spastic paraplegia are rare neurodegenerative syndromes. We aimed to determine the prevalence of these disorders in Spain in 2019. PATIENTS AND METHODS: We conducted a cross-sectional, multicentre, retrospective, descriptive study of patients with ataxia and hereditary spastic paraplegia in Spain between March 2018 and December 2019. RESULTS: We gathered data from a total of 1933 patients from 11 autonomous communities, provided by 47 neurologists or geneticists. Mean (SD) age in our sample was 53.64 (20.51) years; 938 patients were men (48.5%) and 995 were women (51.5%). The genetic defect was unidentified in 920 patients (47.6%). A total of 1371 patients (70.9%) had ataxia and 562 (29.1%) had hereditary spastic paraplegia. Prevalence rates for ataxia and hereditary spastic paraplegia were estimated at 5.48 and 2.24 cases per 100 000 population, respectively. The most frequent type of dominant ataxia in our sample was SCA3, and the most frequent recessive ataxia was Friedreich ataxia. The most frequent type of dominant hereditary spastic paraplegia in our sample was SPG4, and the most frequent recessive type was SPG7. CONCLUSIONS: In our sample, the estimated prevalence of ataxia and hereditary spastic paraplegia was 7.73 cases per 100 000 population. This rate is similar to those reported for other countries. Genetic diagnosis was not available in 47.6% of cases. Despite these limitations, our study provides useful data for estimating the necessary healthcare resources for these patients, raising awareness of these diseases, determining the most frequent causal mutations for local screening programmes, and promoting the development of clinical trials.

Seizure prophylaxis in meningiomas: a systematic review and meta-analysis.

INTRODUCTION: No formal indication currently exists for seizure prophylaxis in neurosurgical oncology patients. Neither have specific recommendations been made on the use of antiepileptic drugs (AED) in seizure-free patients with meningiomas scheduled for surgery. AEDs are generally prescribed on a discretionary basis, taking into consideration a range of clinical and radiological risk factors. We present a systematic review and meta-analysis exploring the effectiveness of antiepileptic prophylaxis in patients with meningioma and no history of seizures. METHODS: We performed a systematic review of the PubMed/MEDLINE, Cochrane Central Register of Controlled Trials, Embase, and clinicaltrials.gov databases. Of a total of 4368 studies initially identified, 12 were selected for extraction of data and qualitative analysis. Based on the clinical data presented, we were only able to include 6 studies in the meta-analysis. We performed heterogeneity studies, calculated a combined odds ratio, evaluated publication bias, and conducted a sensitivity analysis. RESULTS: AED prophylaxis in patients with meningioma and no history of seizures did not significantly reduce the incidence of post-operative seizures in comparison to controls (Mantel-Haenszel combined odds ratio, random effects model: 1.26 [95% confidence interval, 0.60-2.78]; 2041 patients). However, we are unable to establish a robust recommendation against this treatment due to the lack of prospective studies, the presence of selection bias in the studies reviewed, the likelihood of underestimation of seizure frequency during follow-up, and the strong influence of one study on the overall effect. CONCLUSIONS: Despite the limitations of this review, the results of the meta-analysis do not support the routine use of seizure prophylaxis in patients with meningioma and no history of seizures.

Phases in the decision to request euthanasia and brain structures involved. / Fases en la decisión de solicitar la eutanasia y estructuras cerebrales involucradas.

INTRODUCTION: Euthanasia laws do not mention as an obstacle brain diseases other than dementia that damage circuits involved in decision-making. DEVELOPMENT: Narrative review of the stages of the decision to request euthanasia and the brain areas involved. The amygdala, the cingulate and insular cortex, and different parts of the prefrontal lobes are activated during decisions with similarities to that of requesting euthanasia. CONCLUSIONS: When an injury or malfunction of any of the structures involved in making decisions is known, a specific evaluation should be made of the influence it may have on the competence of the patient to request euthanasia.

TITLE: Fases en la decisión de solicitar la eutanasia y estructuras cerebrales involucradas.Introducción. Las leyes de eutanasia no mencionan como obstáculo las enfermedades cerebrales diferentes de la demencia, pero que dañan los circuitos involucrados en la toma de decisiones. Desarrollo. Revisión narrativa de las etapas de la decisión de solicitar la eutanasia y las áreas cerebrales involucradas. La amígdala, la corteza cingulada, la ínsula y distintas partes de los lóbulos prefrontales se activan durante decisiones con similitudes a la de solicitar la eutanasia. Conclusiones. Cuando se conoce una lesión o mal funcionamiento de alguna de las estructuras involucradas en la toma de decisiones, se debe realizar una evaluación específica de la influencia que pueda tener en la competencia del paciente para solicitar la eutanasia.

Fases en la decisión de solicitar la eutanasia y estructuras cerebrales involucradas / Phases in the decision to request euthanasia and brain structures involved

Introducción: Las leyes de eutanasia no mencionan como obstáculo las enfermedades cerebrales diferentes de la demencia, pero que dañan los circuitos involucrados en la toma de decisiones. Desarrollo: Revisión narrativa de las etapas de la decisión de solicitar la eutanasia y las áreas cerebrales involucradas. La amígdala, la corteza cingulada, la ínsula y distintas partes de los lóbulos prefrontales se activan durante decisiones con similitudes a la de solicitar la eutanasia. Conclusiones: Cuando se conoce una lesión o mal funcionamiento de alguna de las estructuras involucradas en la toma de decisiones, se debe realizar una evaluación específica de la influencia que pueda tener en la competencia del paciente para solicitar la eutanasia.(AU)

Introduction: Euthanasia laws do not mention as an obstacle brain diseases other than dementia that damage circuits involved in decision-making. Development: Narrative review of the stages of the decision to request euthanasia and the brain areas involved. The amygdala, the cingulate and insular cortex, and different parts of the prefrontal lobes are activated during decisions with similarities to that of requesting euthanasia. Conclusions: When an injury or malfunction of any of the structures involved in making decisions is known, a specific evaluation should be made of the influence it may have on the competence of the patient to request euthanasia.(AU)

Epidemiology of ataxia and hereditary spastic paraplegia in Spain: a cross-sectional study. / Mapa epidemiológico transversal de las ataxias y paraparesias espásticas hereditarias en España.

INTRODUCTION: Ataxia and hereditary spastic paraplegia are rare neurodegenerative syndromes. We aimed to determine the prevalence of these disorders in Spain in 2019. PATIENTS AND METHODS: We conducted a cross-sectional, multicentre, retrospective, descriptive study of patients with ataxia and hereditary spastic paraplegia in Spain between March 2018 and December 2019. RESULTS: We gathered data from a total of 1.809 patients from 11 autonomous communities, provided by 47 neurologists or geneticists. Mean (SD) age in our sample was 53.64 (20.51) years; 920 patients were men (50.8%) and 889 were women (49.2%). The genetic defect was unidentified in 920 patients (47.6%). A total of 1371 patients (70.9%) had ataxia and 562 (29.1%) had hereditary spastic paraplegia. Prevalence rates for ataxia and hereditary spastic paraplegia were estimated at 5.48 and 2.24 cases per 100 000 population, respectively. The most frequent type of dominant ataxia in our sample was SCA3, and the most frequent recessive ataxia was Friedreich ataxia. The most frequent type of dominant hereditary spastic paraplegia in our sample was SPG4, and the most frequent recessive type was SPG7. CONCLUSIONS: In our sample, the estimated prevalence of ataxia and hereditary spastic paraplegia was 7.73 cases per 100 000 population. This rate is similar to those reported for other countries. Genetic diagnosis was not available in 47.6% of cases. Despite these limitations, our study provides useful data for estimating the necessary healthcare resources for these patients, raising awareness of these diseases, determining the most frequent causal mutations for local screening programmes, and promoting the development of clinical trials.

Seizure prophylaxis in meningiomas: A systematic review and meta-analysis. / Profilaxis antiepiléptica en meningiomas: revisión sistemática y metaanálisis.

INTRODUCTION: No formal indication currently exists for seizure prophylaxis in neurosurgical oncology patients. Neither have specific recommendations been made on the use of antiepileptic drugs (AED) in seizure-free patients with meningiomas scheduled for surgery. AEDs are generally prescribed on a discretionary basis, taking into consideration a range of clinical and radiological risk factors. We present a systematic review and meta-analysis exploring the effectiveness of antiepileptic prophylaxis in patients with meningioma and no history of seizures. METHODS: We performed a systematic review of the PubMed/MEDLINE, Cochrane Central Register of Controlled Trials, Embase, and clinicaltrials.gov databases. Of a total of 4368 studies initially identified, 12 were selected for extraction of data and qualitative analysis. Based on the clinical data presented, we were only able to include 6 studies in the meta-analysis. We performed heterogeneity studies, calculated a combined odds ratio, evaluated publication bias, and conducted a sensitivity analysis. RESULTS: AED prophylaxis in patients with meningioma and no history of seizures did not significantly reduce the incidence of post-operative seizures in comparison to controls (Mantel-Haenszel combined odds ratio, random effects model: 1.26 [95% confidence interval, 0.60-2.78]; 2041 patients). However, we are unable to establish a robust recommendation against this treatment due to the lack of prospective studies, the presence of selection bias in the studies reviewed, the likelihood of underestimation of seizure frequency during follow-up, and the strong influence of one study on the overall effect. CONCLUSIONS: Despite the limitations of this review, the results of the meta-analysis do not support the routine use of seizure prophylaxis in patients with meningioma and no history of seizures.

Neuroimágenes sin evidencia de déficit dopaminérgico (SWEDD) / SWEDDs: scans without evidence of dopaminergic deficit

Introducción. Aproximadamente un 10% de los pacientes diagnosticados inicialmente de Enfermedad de Parkinson (E.P.), no presentan alteraciones en la vía dopaminérgica nigroestriatal en su vertiente presináptica; se engloban bajo el acrónimo SWEDDs (Scans without evidence of dopaminergic deficit) [1]. Objetivo. Revisar aquellos aspectos clínicos que pueden ayudar al diagnóstico diferencial de los SWEDDs, así como las bases del tratamiento. Desarrollo. Las técnicas complementarias empleadas en el diagnóstico de la E.P. como el DAT-SCAN, han favorecido que se establezca un diagnóstico diferencial entre estas dos entidades: E.P. y SWEDDs, cuya evolución, pronóstico y tratamiento será diferente. Conclusiones. El término SWEDDs incluye pacientes con temblor de reposo asimétrico y ausencia de disfunción de la vía dopaminérgica nigroestriatal. En casos dudosos la prueba complementaria a realizar es el DAT-SCAN. La hipótesis de que el temblor en SWEDDs pueda ser una forma de distonía primaria debe ser contrastada en futuros estudios [1]. El tratamiento de los pacientes con SWEDDs se basa en fármacos anticolinérgicos (AU)

Introduction. Approximately 10% of all patients initially diagnosed with Parkinson's disease (PD) do not present any alterations in the presynaptic nigrostriatal dopaminergic pathway; they are classified under the acronym SWEDDs (Scans without evidence of dopaminergic deficit). Aims. Our aim is to review those clinical aspects that can be of use in the differential diagnosis of the SWEDDs, as well as the bases of treatment. Development. The complementary techniques employed in the diagnosis of PD, like DAT-SCAN, have made a valuable contribution to establishing a differential diagnosis between these two conditions, i.e. PD and SWEDDs, whose development, prognosis and treatment will be different. Conclusions. The term SWEDDs includes patients with asymmetrical tremor at rest and absence of dysfunction of the nigrostriatal dopaminergic pathway. In doubtful cases, the complementary test to be conducted is DAT-SCAN. The hypothesis that claims that the tremor in SWEDDs may be a form of primary dystonia must be tested in future studies. The treatment of patients with SWEDDs is based on anticholinergic drugs (AU)

[Digestive disorders in Parkinson's disease: dysphagia and sialorrhea]. / Trastornos digestivos en la enfermedad de Parkinson: disfagia y sialorrea.

INTRODUCTION: The non-motor symptoms of Parkinson's disease are a frequent and often under-diagnosed disorder. Two of the most significant non-motor symptoms are perhaps dysphagia and sialorrhea (which are relatively common in advanced stages of the disease) owing to their important functional repercussions and to the associated comorbidity. DEVELOPMENT AND CONCLUSIONS: In recent years, different evaluation scales have been developed for clinical use and in screening the aforementioned symptoms. Of the different therapeutic options available, botulinum toxin represents the preferred treatment for sialorrhea. In contrast, speech therapy and an optimisation of the antiparkinsonian therapy are generally useful measures to treat dysphagia, percutaneous endoscopic gastrostomy being reserved for patients suffering from Parkinson who have severe dysphagia.

[Locomotion and gait disorders]. / Locomoción y trastornos de la marcha.

INTRODUCTION: Human beings are characterised by the specialisation of certain functions, such as language or the ability to walk. We have achieved this capacity thanks to the development of multiple connections among different areas of the central and peripheral nervous system, together with adaptation of the musculoskeletal system. These are all essential to be able to walk correctly and to keep our balance. DEVELOPMENT: Gait disorders are currently receiving a great deal of attention in neurology departments, and this fact is directly related to the phenomenon of the ageing of the population, since it is a pathology that is particularly prevalent among the elderly. One of the fundamental mainstays in the study of these disorders is being able to distinguish between the different clinical gait patterns and their classification according to the neural system that has been damaged. Observation, the use of different manoeuvres in the examination and the search for other associated clinical signs all enable us reach a good diagnostic approximation, which will later be confirmed with more specific complementary techniques. CONCLUSIONS: From the therapeutic point of view, an early multidisciplinary intervention by the neurologist, primary care, specialists in rehabilitation and physiotherapists improves patients' quality of life and lowers the rate of associated comorbidity and mortality, which also results in a reduction in spending on community health resources.

[A pilot study on a specific measure for sleep disorders in Parkinson's disease: SCOPA-Sleep]. / Estudio piloto sobre una medida especifica para los trastornos del sueno de la enfermedad de Parkinson: SCOPA-sueño.

INTRODUCTION: There is a high prevalence of sleep disorders in Parkinson's disease (PD). AIMS. To assess some basic metric attributes of the SCOPA-Sleep scale, a measure for PD patients; secondary objective: to check the impact caused by the sleep disorder on the health-related quality of life (HRQoL) of patients and their caregivers. SUBJECTS AND METHODS: 68 PD patients and their main caregivers; measures: Hoehn and Yahr staging, SCOPA-Motor, Clinical Impression of Severity Index (CISI-PD), PDSS, Hospital Anxiety and Depression Scale, SCOPA-Psychosocial, and EuroQoL. Carers filled in a PDSS questionnaire about patient sleep and HRQoL measures (SF-36, EuroQoL). SCOPA-Sleep acceptability, scaling assumptions, internal consistency, construct validity and precision were determined. RESULTS: SCOPA-Sleep acceptability and scaling assumptions resulted satisfactory, although the nocturnal sleep subescale (SC-Ns) showed a mild ceiling effect (22.1%) and a defective convergent validity was found for daytime sleepiness (SC-Ds) item 6. Internal consistency also was satisfactory for both scales (alpha = 0.84 and 0.75, respectively). The correlation between SC-Ns and PDSS was high (rs = -0.70), as it was between SC-Ns and PDSS questionnaire by caregiver (rs = -0.53). The corresponding coefficients with the SC-Ds gained lower values (rs = -0.41 y -0.50). Standard error of measurement was 1.45 for the SC-Ns and 1.76 for the SC-Ds. Both, patient and caregiver HRQoL showed a loose association with the sleep measures. CONCLUSION: SCOPA-Sleep is a feasible, consistent, and useful scale for assessment of sleep disorder in PD patients. A weak association between sleep disorder and HRQoL was found.

Estudio piloto sobre una medida específica para los trastornos del sueño de la enfermedad de Parkinson: SCOPA-sueño / A pilot study on a specific measure for sleep disorders in parkinson's disease: scopa-sleep

Introducción. En la enfermedad de Parkinson (EP) existe una alta prevalencia de trastornos del sueño. Objetivos. Comprobar los atributos métricos básicos de la escala SCOPA-sueño para pacientes con EP; objetivo secundario: analizar el impacto del trastorno del sueño en la calidad de vida relacionada con la salud(CVRS) del paciente y de su cuidador principal. Sujetos y métodos. 68 pacientes con EP y sus cuidadores principales. Se aplicaron: Hoehn y Yahr, SCOPA-motor, impresión clínica de gravedad (CISIPD),escala PDSS, Hospital Anxiety and Depression Scale, SCOPA-psicosocial y EuroQoL. El cuidador cumplimentó un cuestionario PDSS sobre el sueño del paciente y las medidas de la CVRS(SF-36, EuroQoL). Se analizaron la aceptabilidad, las asunciones escalares, la consistencia interna, la validez de constructo y la precisión de la SCOPA-sueño. Resultados. La SCOPA-sueño mostró aceptabilidad satisfactoria y asunciones escalares. La subescala sueño nocturno (SC-Sn) presentó leve efecto techo (22,1%), y la subescala somnolencia diurna (SC-Sd), defectuosa validez convergente del ítem 6; la consistencia interna de ambas resultó satisfactoria(alfa = 0,84 y 0,75, respectivamente). SC-Sn correlacionó significativamente con la PDSS (rS= –0,70) y con el cuestionario PDSS cumplimentado por el cuidador (rS= –0,53), y fueron menores los valores respectivos para la SC-Sd (rS= –0,41 y –0,50). Error estándar de la medida: SC-Sn, 1,45; SC-Sd, 1,76. La CVRS del pacientey la del cuidador mostraron una escasa correlación con las medidas de sueño. Conclusiones. La escala SCOPA-sueño es viable, consistente y útil para evaluar el trastorno del sueño en pacientescon EP. La relación entre la CVRS y la alteración del sueño fue débil

Introduction. There is a high prevalence of sleep disorders in Parkinson’s disease (PD). Aims. To assess some basicmetric attributes of the SCOPA-Sleep scale, a measure for PD patients; secondary objective: to check the impact caused by thesleep disorder on the health-related quality of life (HRQoL) of patients and their caregivers. Subjects and methods. 68 PDpatients and their main caregivers; measures: Hoehn and Yahr staging, SCOPA-Motor, Clinical Impression of Severity Index(CISI-PD), PDSS, Hospital Anxiety and Depression Scale, SCOPA-Psychosocial, and EuroQoL. Carers filled in a PDSSquestionnaire about patient sleep and HRQoL measures (SF-36, EuroQoL). SCOPA-Sleep acceptability, scaling assumptions,internal consistency, construct validity and precision were determined. Results. SCOPA-Sleep acceptability and scalingassumptions resulted satisfactory, although the nocturnal sleep subescale (SC-Ns) showed a mild ceiling effect (22.1%) and adefective convergent validity was found for daytime sleepiness (SC-Ds) item 6. Internal consistency also was satisfactory forboth scales (alpha = 0.84 and 0.75, respectively). The correlation between SC-Ns and PDSS was high (rS = –0.70), as it wasbetween SC-Ns and PDSS questionnaire by caregiver (rS = –0.53). The corresponding coefficients with the SC-Ds gainedlower values (rS = –0.41 y –0.50). Standard error of measurement was 1.45 for the SC-Ns and 1.76 for the SC-Ds. Both,patient and caregiver HRQoL showed a loose association with the sleep measures. Conclusion. SCOPA-Sleep is a feasible,consistent, and useful scale for assessment of sleep disorder in PD patients. A weak association between sleep disorder andHRQoL was found

[Huntington's disease mimicking Tourette syndrome]. / Enfermedad de Huntington que simula síndrome de Tourette.

AIM: To report a patient with Huntington's disease, confirmed by a molecular genetic study, presenting with clinical features suggesting Tourette's syndrome. CASE REPORT: A thirty-years male with personal antecedents of perinatal hypoxia, but normal development; and family history (paternal grandfather) of 'abnormal repetitive movements of the upper limbs' of adult onset. He had multiple motor tics since teenage, and associated vocal tics, bruxism, and compulsions with auto-and heteroaggresivity. Neurological examination showed motor tics in the orolingual area and occasional vocal tics. Neuropsychological examination showed a mild impairment of short-term memory and language. Analytical studies including blood smears to see acanthocytes and copper metabolism studies were normal, as it was a MRI imaging. The molecular genetic study of the patient showed 46 repeats of CAG triplets in the exon 1 of the IT-15 genes, while his father (who was asymptomatic) showed 40 repeats. It was not possible to study the paternal grandfather. CONCLUSIONS: The clinical onset of Huntington's disease mimicking Tourette's syndrome is infrequent. This diagnosis should be taken in mind in patients with possible Tourette's syndrome and atypical neuropsychiatric features.

Acute cerebellopathy as a probable toxic effect of flucytosine.

Cryptococcosis is the commonest fungal infection of the CNS and it is an important cause of morbidity and mortality in immunodeficient patients [1]. It has been occasionally described in immunocompetent patients [2]. We report a patient with no predisposing factors who was treated with flucytosine and amphotericin B for cryptococcal meningitis. Following treatment, she developed a reversible acute cerebellar syndrome that was probably secondary to the administration of flucytosine, an adverse effect that has not previously been described [3, 4]. An 87-year old women with no relevant personal or family history was admitted to the hospital for headache, fever, and confusion over the past week. The vital signs, general and neurological examination were normal. In laboratory tests, the urine, urea nitrogen, glucose, bilirubin, electrolytes, aspartate aminotransferase, creatine kinase, alkaline phosphatase, haematocrit, white-cell count, and platelet were also normal. A lumbar puncture was performed which showed: 60 typical lymphocytes per ml, adenosine deaminase (ADA) activity 6 U.l-1 (normal under 4 U.l-1), proteins 75.7 mg.dl-1, and glucose 13 mg.dl-1 with a glycaemia of 120 mg.dl-1. The microbiology study showed staining and a positive culture for Cryptococcus neoformans, and an antigen titre of 1/2080. The serology for HIV infection was negative, and other predisposing factors for this fungal infection, such as immunological defects, a lymphoreticular malignancy and sarcoidosis were excluded. A CT scan of the cranial-thoracic-abdominal regions was normal and tumour markers were absent.

[Cerebral cavernoma. A retrospective analysis of 24 cases]. / Cavernomas cerebrales. Análisis retrospectivo de una serie de 24 casos.

We retrospectively analyzed 24 patients with cerebral cavernomas diagnosed according to histological and/or radiological criteria; epidemiological data, complementary techniques, therapy and evolution are reviewed and the cases are compared to those of previously reported series. Diagnosis was based on histological data in 18 patients and on magnetic resonance imaging (MRI) in the remaining 6. Five patients had compatible clinical histories symptoms compatible with the diagnosis. Mean age of the patients was 37.7 years, the most frequent location was the parietal lobe (27%), and seizures were the most common clinical symptoms of presentation (62%). Surgery was performed on 75%, sequelae were reported in 37.5% and exitus in 11%; 54% were asymptomatic. Six patients did not undergo surgery: 4 had multiple cavernomata, 1 received drug treatment that controlled the seizures and in 1 the cavernoma was located in the protuberance. We recommend the use of cerebral MRI for initial diagnosis, along with follow-up and investigation into similar profiles among family members. The treatment of choice is surgery in patients with acute, progressive or recurring deficits, and when lesions are superficial. At present there is no consensus about the treatment to follow when cavernomas are located in the brain stem.