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Background and purpose: In Parkinson's disease (PD), the course of the disorder is highly variable between patients. Well‐designed, prospective studies for identifying PD progression biomarkers are necessary. Our aim was to show the results of baseline evaluations of an ongoing global PD project, COPPADIS‐2015 (Cohort of Patients with PArkinson's DIsease in Spain, 2015). Methods: This was an observational, descriptive, nationwide study (Spain). The recruitment period ended in October 2017. Baseline evaluation included more than 15 validated scales and complementary studies in a subgroup of participants. Results: In total, 1174 subjects from 35 centres were considered valid for baseline analysis: 694 patients (62.6 ± 8.9 years old, 60.3% males), 273 caregivers (58.5 ± 11.9 years old, 31.8% males) and 207 controls (61 ± 8.3 years old, 49.5% males). The mean disease duration was 5.5 ± 4.4 years. Hoehn and Yahr stage was 1 or 2 in 90.7% of the patients whilst 33.9% and 18.1% of them presented motor fluctuations and dyskinesias, respectively. The mean Non‐Motor Symptoms Scale total score was 45.4 ± 38.1, and 30.4% of the patients presented cognitive impairment, 16.1% major depression, 12.7% impulse control disorder, 7.2% compulsive behaviour, 57.2% pain and 13.2% falls. Compared to the control group, PD patients presented a significantly higher burden of non‐motor symptoms and a worse quality of life. More than 300 subjects conducted complementary studies (serum biomarkers, genetic and neuroimaging). Conclusions: Parkinson's disease is a complex disorder and different non‐motor symptoms are frequently present and are more prevalent than in controls. In real clinical practice it is important to ask for them. [ABSTRACT FROM AUTHOR]

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Introduction: In a degenerative disorder such as Parkinson's disease (PD), it is important to establish clinical stages that allow to know the course of the disease. Our aim was to analyze whether a scale combining Hoehn and Yahr's motor stage (H&Y) and the nonmotor symptoms burden (NMSB) (assessed by the nonmotor symptoms scale (NMSS)) provides information about the disability and the patient's quality of life (QoL) with regard to a defined clinical stage., Materials and Methods: Cross-sectional study in which 603 PD patients from the COPPADIS cohort were classified according to H&Y (1, stage I; 2, stage II; 3, stage III; 4, stage IV/V) and NMSB (A: NMSS = 0-20; B: NMSS = 21-40; C: NMSS = 41-70; D: NMSS ≥ 71) in 16 stages (HY.NMSB, from 1A to 4D). QoL was assessed with the PDQ-39SI, PQ-10, and EUROHIS-QOL8 and disability with the Schwab&England ADL (Activities of Daily Living) scale., Results: A worse QoL and greater disability were observed at a higher stage of H&Y and NMSB ( p < 0.0001). Combining both (HY.NMSB), patients in stages 1C and 1D and 2C and 2D had significantly worse QoL and/or less autonomy for ADL than those in stages 2A and 2B and 3A and 3B, respectively ( p < 0.005; e.g., PDQ-39SI in 1D [ n  = 15] vs 2A [ n  = 101]: 28.6 ± 17.1 vs 7.9 ± 5.8; p < 0.0001)., Conclusion: The HY.NMSB scale is simple and reflects the degree of patient involvement more accurately than the H&Y. Patients with a lower H&Y stage may be more affected if they have a greater NMS burden., Competing Interests: Santos García D has received honoraria for educational presentations and advice service by AbbVie, UCB Pharma, Lundbeck, KRKA, Zambon, Bial, Italfarmaco, and Teva. Paz González JM has received honoraria for educational presentations and/or advice service by UCB Pharma, Lundbeck, KRKA, and Zambon. Jesús S has received honoraria from AbbVie, Bial, Merz, UCB, and Zambon and holds the competitive contract “Juan Rodés” supported by the Instituto de Salud Carlos III. She has received grants from the Spanish Ministry of Economy and Competitiveness (PI18/01898) and the Consejería de Salud de la Junta de Andalucía (PI-0459-2018). Aguilar M obtained from UCB and Schwabe with assistance to a Congress and Nutricia with assistance to a Congress and payment of lecture. Planellas LL has received travel bursaries grant from AbbVie. García Caldentey J has received honoraria for educational presentations and advice service by Qualigen, Nutricia, AbbVie, Italfarmaco, UCB Pharma, Lundbeck, Zambon, Bial and Teva. Caballol N has received honoraria from Bial, Italfarmaco, Qualigen, Zambon, UCB, Teva, and KRKA and sponsorship from Zambon, TEVA, and AbbVie for attending medical conferences. Legarda I has received honoraria for educational presentations and advice service by AbbVie, UCB Pharma, Zambon, Bial, and Teva. Hernández Vara J has received travel bursaries and educational grants from AbbVie and has received honoraria for educational presentations from AbbVie, Teva, Bial, Zambon, Italfarmaco, and Sanofi-Genzyme. Cabo I has received honoraria for educational presentations and advice service by AbbVie, Zambon, and Bial. López Manzanares L compensated advisory services, consulting, research grant support, or speaker honoraria from AbbVie, Acorda, Bial, Intec Pharma, Italfarmaco, Pfizer, Roche, Teva, UCB, and Zambon. Ávila Rivera MA has received honoraria from Zambon, UCB Pharma, Qualigen, Bial, and Teva and ana sponsorship from Zambon and Teva for attending conferences. Puente V has served as consultant for AbbVie and Zambon and has received grant/research from AbbVie. García Moreno JM has received honoraria for educational presentations and advice service by AbbVie, Ital-Pharma, Lundbeck, Merz, KRKA, UCB, Pharma, Zambon, Bial, and Teva. Solano Vila B has received honoraria for educational presentations and advice service by UCB, Zambon, Teva, AbbVie, Bial. Álvarez Sauco M has received honoraria for educational presentations and advice service by AbbVie, UCB Pharma, Zambon, Bial, and Teva. Vela L has received honoraria for educational presentations and advice service by AbbVie, UCB Pharma, Lundbeck, KRKA, Zambon, Bial, and Teva. Escalante S has received honoraria for educational presentations and advice service by AbbVie, Zambon, and Bial. Cubo E has travel grants from AbbVie, Allergan, and Boston; lecturing honoraria from AbbVie and International Parkinson's Disease Movement Disorder Society. Carrillo Padilla F has received honoraria from Zambon (SEN Congress assistance). Martínez Castrillo JC has received research support from Lundbeck, Italfarmaco, Allergan, Zambon, Merz, and AbbVie and speaking honoraria from AbbVie, Bial, Italfarmaco, Lundbeck, Krka, TEVA, UCB, Zambon, Allergan, Ipsen, and Merz. Sánchez Alonso P has received honoraria for educational presentations and advice service by AbbVie, UCB Pharma, Lundbeck, KRKA, Zambon, Bial, and Teva. Alonso Losada MG has received honoraria for educational presentations and advice service by Zambon and Bial. López Ariztegui N has received honoraria for educational presentations and advice service by AbbVie, Italfarmaco, Zambon, and Bial. Gastón I has received research support from AbbVie and Zambon and has served as a consultant for AbbVie, Exelts, and Zambon. Kulisevsky J has obtained the following: (1) consulting fees: Roche and Zambon; (2) honoraria (e.g., lecture fees): Zambon, Teva, Bial, and UCB; (3) research funding: Roche, Zambon, Ciberned, Instituto de SaludCarlos III, and Fundació La Marató De TV3. Blázquez Estrada M has received honoraria for educational presentations and advice service by AbbVie, Abbott, UCB Pharma, Allergan, Zambon, Bial, and Qualigen. Seijo M has received honoraria for educational services from KRKA, UCB, Zambon, and Bial and travel grants from Daiichi and Roche. Ruiz Martínez J has received honoraria for educational presentations, attending medical conferences, and advice service by AbbVie, UCB Pharma, Zambon, Italfarmaco, Bial, and Teva. Valero C has received honoraria for educational services from Zambon AbbVie and UCB. Kurtis M has received honoraria from Bial, the Spanish Neurology Society, and the International and Movement Disorders Society. de Fábregues O has received honoraria for educational presentations and advice service by Bial, Zambon, AbbVie, KRKA, and Teva. González Ardura J has received honoraria for speaking from Italfarmaco, Krka, Genzyme, UCB, Esteve, Psyma iberica marketing research SL and Ferrer, course grant from Teva, and travel grant from Merck. López Díaz L has received honoraria from UCB, Lundbeck, and KRKA. Mir P has received honoraria from AbbVie, Abbott, Allergan, Bial, Merz, UCB, and Zambon and has received grants from the Spanish Ministry of Economy and Competitiveness (PI16/01575) cofounded by ISCIII (Subdirección General de Evaluación y Fomento de la Investigación) and by Fondo Europeo de Desarrollo Regional (FEDER), the Consejería de Economía, Innovación, Ciencia y Empleo de la Junta de Andalucía (CVI-02526, CTS-7685), the Consejería de Salud y Bienestar Social de la Junta de Andalucía (PI-0437-2012, PI-0471-2013), the Sociedad Andaluza de Neurología, the Jacques and Gloria Gossweiler Foundation, the Fundación Alicia Koplowitz, and the Fundación Mutua Madrileña. Martínez-Martin P has received honoraria from Editorial Viguera for lecturing in courses, International Parkinson and Movement Disorder Society (IPMDS) for management of the Program on Rating Scales, Air Liquide, AbbVie, and HM Hospitales de Madrid for advice in clinic-epidemiological studies; license fee payments for the King's Parkinson's Disease Pain scale; financial support by the IPMDS for attending the IPMDS International Congress 2018; and grant for research from IPMDS for development and validation of the MDS-NMS. Borrué C, Ordás C, Catalán MJ, Nogueira V, González Aramburu I, Cosgaya M, Pastor P, De Deus T, Cores Bartolomé C, Valdés Aymerich L, Muñoz Enríquez JG, and Suárez declare that they have no conflicts of interest., (Copyright © 2021 D. Santos García et al.)

Background: Although depression is known to be frequent in Parkinson's disease (PD), it is unclear how mood can change and/or impact on patient's quality of life (QoL) over time. Our aim was to analyze the frequency of depression, mood related factors and the contribution of mood to a patient's QoL perception in regard to disease duration., Methods: PD patients recruited from the COPPADIS cohort from January 2016 to November 2017 were included in this cross-sectional study. Three groups were defined: <5 years (Group A); from 5 to <10 years (Group B); ≥10 years (Group C). Analysis with well-planned linear regression models was conducted to determine how different factors contribute to mood (Beck Depression Inventory-II [BDI-II] as dependent variable), to health-related QoL (39-item Parkinson's Disease Questionnaire [PDQ-39SI] as dependent variable) and to global QoL (European Health Interview Survey - Quality of Life Eight-Item Index [EUROHIS-QOL8] as dependent variable)., Results: Six hundred and sixty-three PD patients (62.6 ± 8.9 years old, 59.6% males) were included: Group A, 50.1% (n = 332); Group B, 33.3% (n = 221) and Group C, 16.6% (n = 110). There were no differences between the three groups in terms of the frequency of depressive symptoms nor the frequency of depression type (major vs. minor vs. subthreshold) (p = 0.729). However, the unique percent variance of PDQ-39SI and EUROHIS-QOL8 explained by BDI-II total score was 2 (23.7%) and threefold (26.9%), respectively, in Group C compared to the other two groups. EUROHIS-QOL8 total score provided the highest unique contribution to mood (16.8%)., Conclusions: Although depression-type frequency does not appear to change over time in PD; the contribution of mood on QoL perception is greater in patients with longer disease duration., (© 2020 John Wiley & Sons Ltd.)

Background: The role of subthreshold depression (subD) in Parkinson's Disease (PD) is not clear. The present study aimed to compare the quality of life (QoL) in PD patients with subD vs patients with no depressive disorder (nonD). Factors related to subD were identified., Material and Methods: PD patients and controls recruited from the COPPADIS cohort were included. SubD was defined as Judd criteria. The 39-item Parkinson's disease Questionnaire (PDQ-39) and the EUROHIS-QOL 8-item index (EUROHIS-QOL8) were used to assess QoL., Results: The frequency of depressive symptoms was higher in PD patients (n = 694) than in controls (n = 207) (p < 0.0001): major depression, 16.1% vs 7.8%; minor depression, 16.7% vs 7.3%; subD, 17.4% vs 5.8%. Both health-related QoL (PDQ-39; 18.1 ± 12.8 vs 11.6 ± 10; p < 0.0001) and global QoL (EUROHIS-QOL8; 3.7 ± 0.5 vs 4 ± 0.5; p < 0.0001) were significantly worse in subD (n = 120) than nonD (n = 348) PD patients. Non-motor Symptoms Scale (NMSS) total score was higher in subD patients (45.9 ± 32 vs 29.1 ± 25.8;p < 0.0001). Non-motor symptoms burden (NMSS;OR = 1.019;95%CI 1.011-1.028; p < 0.0001), neuropsychiatric symptoms (NPI; OR = 1.091; 95%CI 1.045-1.139; p < 0.0001), impulse control behaviors (QUIP-RS; OR = 1.035; 95%CI 1.007-1063; p = 0.013), quality of sleep (PDSS; OR = 0.991; 95%CI 0.983-0.999; p = 0.042), and fatigue (VAFS-physical; OR = 1.185; 95%CI 1.086-1.293; p < 0.0001; VAFS-mental; OR = 1.164; 95%CI 1.058-1.280; p = 0.0001) were related to subD after adjustment to age, disease duration, daily equivalent levodopa dose, motor status (UPDRS-III), and living alone., Conclusions: SubD is a frequent problem in patients with PD and is more prevalent in these patients than in controls. QoL is worse and non-motor symptoms burden is greater in subD PD patients., (Copyright © 2020 Elsevier B.V. All rights reserved.)

Introduction: The dopamine psychosis that appears in Parkinson's disease (PDP) is a complication that is often related with frequent intake of antiparkinsonian agents, especially levodopa and dopamine agonists. Morbidity and the risk of being institutionalised are increased in such patients and therapeutic management is difficult. Its treatment is based on reducing the intake of antiparkinsonian drugs or the use of atypical antipsychotics, due to the fact that they have scarce or no motor effects (at least in theory). In this work, we examine the role that ziprasidone (ZPS) can play in PDP., Development: We reviewed the studies in which ZPS was used to treat PDP that have appeared in the literature. To date, very few studies have been conducted and only a small number of patients were included in each case. Nevertheless, it seems that, at doses below those used in schizophrenia (20-80 mg/day), ZPS is an effective antipsychotic. In general, the extrapyramidal side effects that do appear are infrequent and mild. Pathological laughter was detected in some patients. The drug was well tolerated and safe., Conclusions: ZPS seems to be an effective antipsychotic for PDP, with scarce or no extrapyramidal side effects; it is also safe and well tolerated. In view of the scarcity of studies and patients, however, further research is needed (if possible, by means of double-blind randomised trials) to be able to assess the true role of ZPS in PDP.

Sneeze is an ubiquitous phenomenon that happens to everyone. In spite of this, little attention has been paid to it, among medical literature in general, and even less in neurologic texts. A curious entity, called photic sneeze reflex, solar sneeze reflex, light sneeze reflex or autosomal dominant compelling helio-ophthalmic outburst syndrome, known perhaps since ancient Greek, has been scarcely described in the scientific literature, mainly as clinical notes and letters to the editor, but in a detailed way, we can find just a few reports. This reflex appears when subjects are exposed suddenly to intense sunlight and it consists of long incoercible sneeze bursts. It is usually ignored by its sufferers, who report it as a curiosity or a minor complaint, and its importance has been neglected in spite of its hereditary nature and its apparently high prevalence. We review the history, epidemiology, genetics, neuroanatomy, neurophysiology and physiopathology of this reflex hereditary response.

Introduction: Sneeze is an ubiquitous phenomenon that happens to everyone. In spite of this, little attention has been paid to it, among medical literature in general, and even less in neurologic texts. A curious entity, called autosomal dominant compelling helio-ophthalmic outburst syndrome, has been scarcely described in the scientific literature. This reflex appears when subjects are exposed suddenly to intense sunlight and it consists of long incoercible sneeze bursts. There are no publications on this subject among the spanish literature.and intensity increase with time., Objective: To study the clinical and physiological features of the reflex in Spanish families., Patients and Method: Affected subjects were identified by personal interview and given a questionnaire drawn up for this study. Besides, they were instructed to give the questionnaire to their relatives.and intensity increase with time., Results: All the six families showed a high-penetrance, autosomal dominant inheritance. The reflex had a high consistency, a latency about 3 seconds, an intersneeze interval of 2 seconds and a frequency of 2-3 sneezes/ burst. Refractory period was long.and intensity increase with time., Discussion: Our study suggest a higher consistency, shorter latency and lower age of onset of the reflex in our patients than general population, and that frequency and intensity increase with time.

Introduction: Much has been written in the literature about the use of intrathecal baclofen in an infusion pump in spasticity, but it has only recently been applied to cases of dystonia., Aims: The purpose of this study was to determine whether intrathecal baclofen is a therapeutic option in patients with a long history of generalised dystonias who have failed to respond to conventional treatment., Case Reports: We conducted a prospective study involving five patients from whom the following data were collected: age, sex, time since the onset of dystonia and the type of dystonia. They were also chosen because they had failed to respond to any kind of treatment, including oral baclofen, but had experienced a clinical improvement following the administration of baclofen by means of a spinal tap in progressive 25, 50, 75 and 100 mg boluses. The Burke-Fahn-Marsden scale was used before and after implanting the pump to evaluate functional capacity. Of the five patients, one improved remarkably to the point of being able to lead a normal life; three others showed marked improvements, although they are still to some extent limited when it comes to carrying out certain basic activities; and in the case of the third female patient the pump had to be withdrawn due to the absence of any kind of response., Conclusions: Intrathecal baclofen is a therapy that has only recently been introduced but these results allow us to state that it does bring about an improvement in the symptoms of patients with a long history of dystonia in whom conventional treatment has failed.

Introduction: The availability of the interferon beta in its three forms at the moment available in our country and of glatiramer acetate has marked a point of flexion in the natural history of multiple sclerosis (MS), but the high cost of these treatments cause that its use is questioned. In this work we have studied the effectiveness and efficiency of the processing with interferon beta, and the cost-utility of these treatments in MS in Spain has been also analyzed., Patients and Methods: For this work we studied 102 patients affected of RR MS, treated with the three interferons beta which we have available in our country. We used as control 330 patients who had participated in the pivotal clinical interferon trials with both interferon beta 1a. In these patients in addition to effectiveness data, we have studied the disability measured as area below curve and the quality of life (AVACs). We also calculated the economical costs, considering the relation cost-utility in our country., Results: Besides to confirm the data of effectiveness of three interferons, in this study a saving of 23 days/year is demonstrated what corresponds to 0.063 AVACs. The additional cost of interferons is greater than the avoided cost until the fifth year of treatment in which the tendency is reversed in favor of the group of treated patients, if we assume that the same effectiveness that we found in the first years is maintained in the long term., Conclusion: The use of the treatment with interferon beta is justified by its effectiveness, efficacy and efficiency. The additional cost of the treatment will be compensated in the long term if the effectiveness of the interferon beta is maintained.

Introduction: Multiple sclerosis (MS) is the neurological non-traumatic disease that produces permanent incapacity in the young people with the greatest frequency. An almost total consensus exists on the implication of environmental and genetic factors in the pathogenesis of the disease. In a considerable percentage of patients, antecedent relatives of other cases of MS exist, who are separated by other healthy relatives sometimes., Aims: We try to study the familial antecedents of the MS patients, to locate to the healthy members of the family including in the forced line of the possible genetic transmission of the disease and ruling out subclinical involvement by the use of magnetic resonance imaging (MRI)., Patients and Methods: We reviewed the familial antecedents of the MS patients followed by the MS Unit of the Service of Neurology of the Hospital Virgen Macarena of Seville. After the accomplishment of their genealogical trees, we identified the cases of familial MS. We locate and practice MRI on the healthy subjects of the family, who are in the genetic line of communication of the disease (obligate carriers)., Results and Conclusions: We were able to identify 14 obligate carriers of the gene in 12 of the families. In the MRIs that were done, we found MS-compatible lesions in 10 subjects. These findings confirm the existence of silent forms of the disease, that make difficult the knowledge of the genetic implications in the pathogenesis of the disease.

Introduction: Lyme disease is a complex infectious disease affecting multiple organic systems. The most frequent complications are of a dermatological, rheumatological, ophthalmological, cardiac and neurological nature. Among the neurological complications, pseudotumour cerebri is considered to be a rare manifestation of the disease. Hypothyroidism, however, is known to be only infrequently associated to pseudotumour cerebri. Paparone recently reported a case in which Lyme disease and primary hypothyroidism were concurrent, and Becker and Trock described the case of three patients with concurrent thyrotoxicosis and Lyme disease., Case Report: We present the case of a 19 year old female with pseudotumour cerebri and hypothyroidism secondary to autoimmune thyroiditis, which improved after treating her for concomitant Lyme., Conclusions: We suggest there is a common aetiopathogenic connection between the three processes through autoimmune thyroiditis triggered by Borrelia burgdorferi. Thus, in this patient, the pseudotumour cerebri could be due to either a direct mechanism, triggered by B. burgdorferi, or indirectly, through hypothyroidism secondary to thyroiditis induced by B. burgdorferi, or perhaps to both mechanisms. It might be wise to consider infection by B. burgdorferi in patients with pseudotumour cerebri or thyroiditis coming from areas in which Lyme disease is endemic

Aims: The aims of this paper is to demonstrate the existence of neuropsychological disorders in amyotrophic lateral sclerosis (ALS) and to perform an in depth study of the cognitive functioning of the prefrontal lobes., Patients and Methods: A neuropsychological study of 14 patients with ALS was conducted using an extensive battery of tests and were compared with a group of 14 healthy controls. Both groups were homogeneous as regards age, sex, education and manual dominance. In this clinical and research study, as well as the neuropsychological variables (subtest of the Barcelona PIEN Test neuropsychological battery), we also took the evolution of the disease, the age and neurological clinical features of the patients suffering from ALS into account., Results: We found neuropsychological disorders in the ALS patients that were centred, either directly or indirectly, on functions of the prefrontal lobe, and in particular of the dorsolateral and premotor cortices, which had already been observed in other research work. No memory disorders were found, something which is usually mentioned in studies about neuropsychological disorders in this type of patients., Conclusions: Apart from the primary motor zones affected in ALS, there appears to be a degenerative process in most of the frontal lobe, and there is a need for longitudinal studies of the possible progressive disorders of the frontal lobe in these patients. This is difficult, since these patients end up with serious neurological alterations which prevent a correct neuropsychological exploration from being carried out cognitively, and hence no conclusions can be drawn either

Aims: Although it is commonly recognised that diseases of the thyroids can simulate extrapyramidal disorders, a review of the causes of Parkinsonism in the neurology literature shows that they are not usually mentioned or, if so, only very briefly. The development of hypothyroidism in a patient with Parkinson s disease can go undetected, since the course of both diseases can involve similar clinical features. Generally speaking there is always an insistence on the need to conduct a thyroidal hormone study in any patient with symptoms of Parkinson, but no emphasis is put on the need to continue to rule out dysthyroidism throughout the natural course of the disease, in spite of the fact that the concurrence of both pathological conditions can be high and that, in the same way hypothyroidism can simulate Parkinson s disease, the latter can also conceal hypothyroidism., Case Report: We report the case of a female patient who had been suffering from Parkinson s disease for 17 years and started to present on off fluctuations that did not respond to therapy. Hypothyroidism was observed and the hormone replacement therapy used to resolve the problem allowed the Parkinsonian fluctuations to be controlled., Conclusions: We believe that it is very wise to suspect hypothyroidism in patients known to be suffering from Parkinson s disease, and especially so in cases where the clinical condition worsens and symptoms no longer respond properly to antiparkinsonian treatment. These observations stress the possible role played by thyroid hormones in dopaminergic metabolism and vice versa.

Besides Babinski's, Lhermitte's sign is likely the eponym mostly used in the neurological literature. We review here the history of this eponym as well as recent advances on its pathophysiology and treatment. Lhermitte's phenomenon is, on one hand, a symptom as it is spontaneously explained by patients, and, on the other hand, a sign as it may be triggered by flexion of the nape. Initially described after head and cervical spine trauma, firstly by Marie and then by Babinski, it was Jean Lhermitte who recognized on it an etiological specificity, namely, a demyelinating sign of cervical spinal posterior cords. He also made a pathophysiological interpretation of the phenomenon, namely, a stretching of posterior cords during flexion of the neck. All authors agree that this phenomenon is more common in multiple sclerosis, although it has been descibed in many other conditions. The history of how this sign was hatched, whose pathophysiology remains a mystery, is fascinating. And it is fascinating the fact that patients compare the phenomenon with a current, especially if we bear in mind that few people may have suffered an electrocution, mainly in that time when just a few ones could actually enjoy domestic electricity.

Objective: Neuropsychological investigations over the past 20 years have shown the high prevalence of cognitive dysfunction in multiple sclerosis, although there are still large gaps. In this article we review current concepts regarding the different types of neuropsychiatric disorders, their physiopathology, diagnosis and treatment., Development: We present a classification of the most important types of cognitive and psychiatric alterations in multiple sclerosis, and their physiopathology. We review the methods used in diagnosis, follow up and assessment of these patients. Finally, we analyze the current treatment available for each of these disorders., Conclusions: Although considerable advances have been made in understanding the neuropsychological disorders occurring in multiple sclerosis, there are still many questions to be answered. There is a need for a unified, agreed, validated battery of neuropsychological tests for the study of specific cognitive deficits in multiple sclerosis so that results may be compared. There is also a lack of biological markers which would help in diagnosis and prognosis. Finally the design and carrying out of clinical trials in the search for specific, effective drugs for the treatment of neuroconductual disorders of multiple sclerosis is necessary.

Primary tumors of the heart are usually benign, with the malignant forms being rare. In this article we report the case of an angiosarcoma with the only manifestation being a cardiac tamponade. When the patient being admitted to the hospital, an important differential diagnosis was pulmonary tumor. A mass in the right atrium was later detected in the echocardiogram. Further image studies, such as MRI, showed spectacular images of this mass suggestive of neoplasm. An intra-operatory biopsy diagnosed the mass as an angiosarcoma. Since there was no evidence of primary neoplasm in other studies carried out, the neoformation was diagnosed as a primary angiosarcoma. Surgery was carried out with a survival of only 13 months after the procedure. We also use this case to make a brief review of the primary tumors of the heart, especially angiosarcomas.

Objectives: We show the effectiveness of treatment with continuous intrathecal baclofen infusion in a case of hereditary generalized dystonia refractory to anticholinergics, tetrabenazine, pimozide, L-dopa, benzodiazepines and thalamotomy., Clinical Case: A 26 years old female patient, when she was 11 years old began with torsion dystonia in her left feet, that progressively worsened to involve her entire body. She had painful spasms. She had four brothers, three of them with dystonia and one healthy. Her uncle grandfather had similar symptoms. Complementary explorations to reject secondary origin was negatives. She was treated with high and progressive dosages of anticholinergics, pimozide, tetrabenazine, benzodiazepines, L-dopa and thalamotomy without improvement. Underwent intrathecal baclofen test dosing, we used 25, 50, 100 micrograms/day, the last one with improvement during 10 hours. A pump was inserted with an initial dose of 220 micrograms/day. After pump insertion, baclofen dosage was gradually increased to 450 micrograms/day. She had a great improvement in right part of her body and less in her left body. Painful spasms had disappeared., Conclusion: We propose continuous baclofen intrathecal infusion pump for patients with severe torsion dystonia that not response to ordinal treatment.

Introduction: Multiple sclerosis (MS) is a demyelinating and inflammatory disease of the central nervous system (CNS) with a clear proved genetic susceptibility. The real frequency and clinical features of familial MS is although not well established in Spain., Objective: We studied the clinical and CSF features of familial MS (FMS) patients in comparison with the rest of our patients., Patients and Methods: We reviewed 308 definite MS patients looking for patients with other familial members with MS. We analyzed clinical characteristics (age, age at onset, sex, evolution time, evolution course, symptoms at onset, disability measured by EDSS scale) and IgG synthesis measured by Tibbling-Link index., Results: We found 23 patients (10 men and 13 women) in 18 independent families with at least other family member diagnosed of definite MS (7.47% of our total MS population). Age and age at onset were no different from non FMS cases. The clinical course was relapsing-remitting in 21 out of 23 FMS cases and secondary progressive in two. No primary progressive cases were found among FMS. At onset the symptom most frequently found in FMS was optic neuritis. Mean EDSS score was lower in FMS cases in comparison with the rest of cases. Link index was increase in 93.7% of patients with FMS., Conclusion: In comparison with non familiar forms FMS patients in Spain, present more often remitting courses, are less disabled, optical neuritis is more frequently seen as onset symptom and IgG synthesis is more often increased.

Introduction: Parkinson's disease of juvenile onset is known to be rarely seen in clinical neurology. Occasionally it may be secondary to other pathological processes which have to be excluded by investigation of such cases, since the clinical, therapeutic and prognostic significance varies considerably depending on whether the condition is idiopathic or secondary., Clinical Case: We present the case of a patient whose illness started when she was 16 years old. She complained of rigidity, tremor and akinesia. Her condition was studied to find whether the syndrome was secondary. Thus, on four occasions, at annual visits during a period of five years, immunological changes suggestive of systemic lupus erythematous were observed. Neuroimaging, magnetic resonance and computerized axial tomography studies were completely normal on all four occasions over the five years during which the patient had annual follow-up reviews., Conclusion: We considered whether the rigidity-akinesia-tremor syndrome might have been secondary to systemic lupus erythematous, due to a probable pathological focus of cerebral vasculitis, in this clinical case which we report.

We present the case of an 60-year-old male patient that after a acute myocardial infarction inferior diagnostic was subjected to a fibrinolysis with streptokinase. The evolution from the cardiologic point of view was favourable, but at fourth day postacute myocardial infartion he starts with peripherics vasculars symptoms, pain and livedo reticularis in lower part of the body. A symptomatic treatment was made requiring amputation of his fifth right toe. The evolution was towards healing without any organic afectation at another level.

Background: The aim of the present study was to determine whether the adverse effects (AE) of interferon beta 1b (IFNb-1b) are related to patient weight, height and body surface area. Moreover, whether the basal incapacity and/or some of these AE may be related to the evolutive prognosis were also studied., Methods: Twenty-nine females and 14 males with remittent-recurrent multiple sclerosis treated with IFNb-1b were studied. The clinical data and the AE, were compared with the number of outbreaks and the progression of incapacity over the first year of treatment in patients with high weight, height and body surface area with respect to those under the mean., Results: Although the presence of fever was similar in the two groups during the first month (8/19 in the high weight patients and 11/24 in the low weight group), in the following months, overall, the low weight patients continued to present fever. Fever during the first month was associated with a lower number of outbreaks at one year of evolution (0.04 +/- 0.1, n = 19 vs 0.30 +/- 0.5, n = 24; p = 0.04). Furthermore, the patients with lesser incapacity presented a better evolution than those with more incapacity, particularly at 2 years (0.21 +/- 1.3 vs 1.36 +/- 0.9; p = 0.05)., Conclusions: During the first month of treatment fever is very frequent in all the patients regardless of weight and fever was correlated with a lower number of outbreaks at one year of evolution. From the third month fever was less frequent in the lower weight patients group.

Introduction: We report that interferon beta decreases CD8 T cells percentage and increases CD4/CD8 cell's rate in vivo in Multiple Sclerosis (MS) patients., Patients and Methods: We studied 40 patients (22 women and 18 men) with clinically definite active MS who received IFN beta. Twenty-six were treated with nIFN (9 MU/week) and 14 with rIFN (28 MU/week). All patients except two with secondary progressive forms presented relapsing remitting courses. Mean age and mean age at onset were 36.5 +/- 9 and 27.8 +/- 7 years respectively. Mean EDSS score was 2.96 +/- 1.8. Patients were reviewed at four weeks and every eight weeks and periodical studies of immunity were performed. T cell subpopulations (CD3, CD4, CD8 and NK) were studied byflow cytometry., Results: The evolution of CD8 T cell percentage showed a statistically significant decrease in all blood samples after 20 weeks of treatment with rIFN (24.3 +/- 8 vs 34.7 +/- 5 in the control group) and after 36 weeks for nIFN beta group (25.7 +/- 6 vs 33.0 +/- 4 in the control group). No changes were detected in CD4 T cell subset. The evolution of CD4/CD8 T cell rate showed an increase over the cut-off (2.200) in all blood samples after 20 weeks of treatment with rIFN (2.302 +/- 1.12, 2.332 +/- 0.99 and 2.488 +/- 1.61 for 20, 28 and 36 weeks respectively) and after 52 weeks for nIFN beta group (2.128 +/- 1.07, 2.346 +/- 1.09 and 3.168 +/- 3.87 for 52, 60 and 68 weeks respectively)., Conclusions: Both nIFN and rIFN beta are able in vivo to decrease CD8 percentage of T cells and increase CD4/CD8+ T cell rate. The increase in the rate is produced earlier in the rIFN treated group.

Unlabelled: OBJECTIVE, PATIENTS AND METHOD: We present a genealogical study of a person with familial cerebral cavernomatosis (CCF) discovered following study of a man who presented with status epilepticus at the age of 35 years. This had also affected another three members of the family consisting of a total of 43 persons, studied over six generations., Results: In the family members studied we did not find cavernomas at other sites but there were dermatological lesions whose relation to cerebral cavernomas (CC) is discussed. CCs are the second commonest type of cerebral vascular malformations. Their major clinical features are epilepsy and cerebral haemorrhage. There are two forms of CC: a sporadic form with single lesions and a familial form with multiple lesions. Although the condition has been recognized since 1854, very little is known about it. Recently a gene causing the familial form has been found on chromasome 7., Conclusions: We discuss the clinical findings in this family with relation to descriptions in the literature. We emphasize the importance of MR in diagnosis of the condition and for detection of asumptomatic carriers. We consider a possible relationship between these findings and genes which have been mapped, and in view of recent genetic data, the hypothesis of a possible Spanish ancestor with the condition.

Introduction: Many different neurological conditions may be seen in the later stages of Lyme's Disease, such as blindness, epileptic crises, CVA, extrapyramidal disorders, amyotrophic lateral sclerosis, and dementia may be yet another form of presentation of chronic infection due to Borrelia burgdorferi (Bb). Progressive Supranuclear Paralysis (PSP), a disorder of unknown aetiology, considered to be the commonest cause of Parkinsonism-plus, one of the symptoms of which is dementia, has never been mentioned in this type of differential diagnosis., Clinical Case: We present the case of a 78 year old man with sub-acute mental deterioration, Bb positive serology in both plasma and CSF, and with clinical and epidemiological features compatible with Lyme's Disease. Complementary tests were negative. The syndrome corresponded to Lyme's Disease and improved after treatment with ceftriaxona., Conclusions: We consider aspects of the aetiology of PSP which are still not clear. In our patient, the aetiology seemed to be Bb infection, according to the criteria of the original description of the disease and in view of the neuropathological findings which have shown Bb in the substancia nigra of the mid-brain and the existence of an animal model in which Bb shows a particular tendency to colonize infratentorial structures.

Introduction: Epidural arterio-venous fistulae are a little known clinical condition. They are probably commoner than is thought, since diagnosis and angiographic demonstration are difficult., Clinical Case: We present the case of a 49 year old man with the clinical features of chronic myelo-radiculopathy with episodes of intermittent medullary claudication. This shows the use of myelography, which in certain cases allows clear detection of vascular structures which might otherwise pass unnoticed., Conclusions: One should think of this condition when compatible features are found and there is no other diagnosis, since if confirmed by myelography and/or magnetic resonance prior to angiographic study, it may benefit from embolization, which is an effective treatment for this.

The coexistence of organ-specific and nonorgan-specific autoimmune diseases is an interesting phenomenon. A 52-year-old woman was admitted with fever, general discomfort, polyarthritis, and Raynaud's phenomenon. Physical examination revealed a goiter of stony consistency, hardening, paleness, and atrophy of the skin on the face and upper limbs, and blood hypertension (180/110 mmHg). The biological data included leukopenia, moderate anemia, and a very high sedimentation rate. The latex test was positive (+++); LE cells positive (+); hypergammaglobulinemia (3.5 g); antinuclear antibodies, 1/1280 with an immunofluorescence granular pattern; antithyroid antibodies, 1/160. There was pulmonary, renal, and gastrointestinal involvement compatible with scleroderma, which was confirmed by skin biopsy. A thyroidectomy revealed the existence of a papillary carcinoma with thyroiditis. Responde to treatment with immunosuppressive agents, hypotensive drugs, and thyroid substitution therapy was initially good. The patient was readmitted 8 months later with general discomfort and a severe hyperproteinemia (10 g/100 ml), including 65 percent gammaglobulin and requiring various sessions of plasmapheresis. The patient was discharged, but died suddenly 4 months later. The association of lupus and scleroderma in this patient is discussed and the possibility of its being a mixed connective tissue disease is discarded. The association of this condition with Hashimoto's thyroiditis, and the latter with papillary carcinoma of the thyroid are analyzed. The peculiar features of this case are pointed out. The authors postulate that the cause of the sudden death was a vascular cerebral complication induced by the extreme hyperproteinemia.