ABSTRACT Chronic neurological disorders generate disabilities affecting multiple aspects of life, including sexuality. Objective To describe the presence of sexual dysfunction and comorbidities in a population with chronic neurological disorders. To analyze the relationship between disability and sexual dysfunction. Methods A cross-sectional case-control study was carried out. Patients with amyotrophic lateral sclerosis (ALS), multiple sclerosis (MS), Parkinson’s disease (PD), and stroke of at least one year since the onset of symptoms were included, and compared with controls with no neurological disease, matched by age and sex. Results We included 71 participants: 29 controls, with a mean age of 49.4 years, and 42 patients with a mean age of 53.8 years. Sexual dysfunction was present in 22.5% of the controls and 77.5% of the patients. A statistically significant relationship between sexual dysfunction and disability was found in the logistic regression analysis (OR = 20.38, 95%CI: 2.5 –165.86). Conclusions Disability proved to be the main variable related to the presence of sexual dysfunction. Patients with ALS had the worst rates of sexual dysfunction. Patients with MS were similar to the control group. As for the PD group, no patient had normal sexuality. Finally, in stroke patients, the presence of comorbidities and their treatment may have negatively influenced sexuality. These findings showed that patients with chronic neurological diseases have sexual dysfunction and underscore the need for neurologists to know and address this problem.

RESUMO Las enfermedades neurológicas crónicas generan discapacidad afectando múltiples aspectos de la vida, incluida la sexual. Objetivo Describir en una población con enfermedades neurológicas crónicas la presencia de disfunción sexual y posibles comorbilidades acompañantes. Analizar la relación entre discapacidad y disfunción sexual. Métodos Se realizó un estudio transversal de tipo casos y controles. Se incluyeron pacientes con Esclerosis Lateral Amiotrófica (ELA), Esclerosis Múltiple (EM), enfermedad de Parkinson (EP) y secuelados por enfermedad cerebrovascular (sACV) de al menos un año de evolución, controlando con sujetos sin enfermedad neurológica pareados por edad y sexo. Resultados Se incluyeron 71 sujetos: 29 controles, con una edad media 49,4 años y 42 casos con una edad media de 53,8 años. Presentaron disfunción sexual el 22,5% de los controles y el 77,5% de los casos. En el análisis por regresión logística se encontró una relación estadísticamente significativamente entre disfunción sexual y discapacidad. (OR = 20.38, IC95%: 2.5–165.86). Conclusiones La discapacidad demostró ser la principal variable relacionada con la presencia de disfunción sexual. Los enfermos con ELA fueron los que peores índices de disfunción sexual presentaron. Los pacientes con EM se comportaron de forma similar al grupo control. En cuanto al grupo de EP todos los pacientes tuvieron algún trastorno en su sexualidad. Por último, en sACV la presencia de comorbilidades y su tratamiento podrían influir negativamente en la sexualidad. Estos hallazgos evidencian que la disfunción sexual está presente en los pacientes con enfermedades neurológicas crónicas y confirma la necesidad de conocer este problema por parte de los neurólogos.

Espasmos tônicos têm sido mais frequentemente associados com esclerose múltipla. Foram publicados até agora poucos relatos de série de pacientes com neuromielite óptica e espasmos tônicos.Métodos:Foram analisadas as características e a frequência de espasmos tônicos em 19 indivíduos com neuromielite óptica. Os dados foram coletados por meio de um questionário semiestruturado para espasmos tônicos, mediante a avaliação retrospectiva dos prontuários e a análise dos dados clínicosResultados:Todos os pacientes com neuromielite óptica exceto um apresentaram espasmos tônicos. Os principais fatores desencadeantes foram movimentos bruscos e fatores emocionais. Espasmos foram frequentemente associados a perturbações sensoriais e se agravaram durante a fase aguda da doença. A carbamazepina foi utilizada frequentemente para tratar os sintomas, com boa resposta.Conclusões:Os espasmos tônicos são manifestações clínicas frequentes em pacientes com neuromielite óptica.

Tonic spasms have been most commonly associated with multiple sclerosis. To date, few reports of series of patients with neuromyelitis optica and tonic spasms have been published.Methods:We analyzed the characteristics and frequency of tonic spasms in 19 subjects with neuromyelitis optica. Data was collected using a semi-structured questionnaire for tonic spasms, by both retrospectively reviewing medical records and performing clinical assessment.Results:All patients except one developed this symptom. The main triggering factors were sudden movements and emotional factors. Spasms were commonly associated to sensory disturbances and worsened during the acute phases of the disease. Carbamazepine was most commonly used to treat the symptom and patients showed good response to the drug.Conclusions:Tonic spasms are a common clinical manifestation in patients with neuromyelitis optica.

AIM: To investigate whether the cerebellum could participate in social cognition (SC). METHOD: General neuropsychological tests, executive tests (EF), social cognition tests, which assess the ability to infer other peoples’ mental states, and the Beck Depression Inventory were given to 10 non-demented patients with isolated cerebellar degenerative disease, and to 10 healthy controls matched for sex, age, and years of education. ANOVA and correlation coefficients were employed for the statistical analysis. RESULTS: Patients within the cerebellar group were significantly impaired (p?0.05) in EF test [Wisconsin Card Sorting Test (WSCT)] and belief questions (BQ) from Theory of Mind (ToM) tests. Performance in control questions (CQ) from ToM tests was similar for both groups. Lower scores in BQ correlated with a lower conceptual ability, the severity of apathy (NPI) and static ataxia. CQ correlated with measures of attention and free recall. CONCLUSION: The cerebellum may contribute in the control of social behavior through the processing of multimodal data, motor, cognitive and emotional.

OBJETIVO: Investigar la hipótesis según la cual, el cerebelo podría participar en las alteraciones de la cognición social (CS). MÉTODO: Se administraron tests neuropsicológicos generales, de función ejecutiva (FE), de CS que evaluaban la capacidad de inferir el estado mental de otras personas y la escala de Depresión de Beck a 10 pacientes adultos con enfermedad cerebelosa adquirida aislada (GEC) y a 10 controles (GCO) apareados por edad, sexo y educación. Se analizaron los datos mediante ANOVA y correlación. RESULTADOS: El GEC obtuvo puntajes significativamente menores (p?0,05) que el grupo GCO en tareas de FE (Test de Wisconsin) y preguntas de creencia (PCr) de Teoría de la Mente (ToM). El rendimiento en las preguntas de control (PCo) de ToM fue similar en ambos grupos. El menor rendimiento en las PCr correlacionó significativamente con una menor habilidad conceptual, la severidad de la apatía (NPI) y de la ataxia estática. Las PCo correlacionaron con medidas de atención y de recuerdo diferido libre. CONCLUSÍON: El cerebelo contribuiría en el monitoreo de la conducta mediante el control de datos multimodales motores, cognitivos y emocionales.

OBJECTIVE: To compare executive functions (EF) in non-demented mild to moderate Parkinson's disease (PD) (Hoehn and Yahr <3) and pure degenerative cerebellar disease (CD) in order to evaluate the relative contribution and differential role of basal ganglia and cerebellum in those functions. METHOD: 14 patients with PD and 14 patients with CD matched by sex, education, disease's duration and MMSE were selected. A standardized neuropsychological battery and the Wisconsin Card Sorting Test (WCST) were administered. Z scores were compared for both groups through t-test for independent samples were used. RESULTS: The cerebellar group showed significant lower performance in measures of attention and EF, with a significant increase in both perseverative and non perseverative errors during the WCST. On the other hand the PD group showed a selective increase of non perseverative errors, without reaching significant between group difference. CONCLUSION: The CD group appears to have greater deficits in EF with a pattern of prefrontal dysfunction.

OBJETIVO: Comparar el rendimiento en tareas de función ejecutiva (FE) en sujetos con enfermedad de Parkinson (EP) leve a moderada (Hoehn y Yahr <3) no dementes y en sujetos con enfermedad degenerativa cerebelosa pura (EC), a fin de evaluar el eventual rol diferencial de los ganglios basales y el cerebelo en esa función. MÉTODO: Se evaluaron 14 pacientes con EP y 14 con EC apareados por sexo, educación, duración de enfermedad y MMSE, mediante tests neuropsicológicos estándar y el test de Wisconsin [WCST]). Se compararon puntajes Z promedio de cada grupo mediante prueba de "t" para muestras independientes. RESULTADOS: El grupo EC rindió significativamente menos en pruebas de atención y FE presentando errores perseverativos y no perseverativos durante el WCST. El grupo EP mostró aumento significativo de errores no perseverativos en relación al estándar esperado, sin alcanzar diferencias significativas con el grupo EC. CONCLUSIÓN: el grupo EC mostró defectos significativamente mayores en tareas de FE exhibiendo un patrón de disfunción prefrontal.

Schwannomatosis is a rare disorder, still not quite well defined, seldom described in the literature. In this paper we report the case of male. Patient, 52 years old, who in the last 30 years developed five subcutaneous tumors within his limbs peripheral nerves, which histologically proved to be schwannomas. A brain computed tomography showed a partially calcified tumor in the left temporal lobe which most likely was a meningioma. A thorough clinical examination was unable to find signs of type I or type II neurofibromatosis. The present condition, probably a form of phacomatosis, has to be distinguished from neurofibromatosis and is considered as an independent clinical entity whose origin still awaits further detailed investigations.

Se describe un paciente de 52 años de edad que presentó 5 tumoraciones subcutaneas adheridas a diferentes nervios periféricos. La anatomía patológica de todas ellas, indicó que se trataba de schwannomas. Una TAC de cerebro demostró una tumoración en lóbulo temporal izquierdo con calcificaciones en su interior, compatible a meningioma. La ausencia de criterios diagnósticos para neurofibromatosis I y II y la característica particular del cuadro, schwannomas múltiples asociados a tumoración endocraneana, sugieren el diagnós tico de schwannomatosis.