Executive cognitive deficits in primary dystonia

Objectives: To investigate the impact of dystonia symptoms upon cognitive functioning, by comparing cognitive performance in focal and generalised dystonia subtypes, and examining the differential contribution of severity of symptoms and mood disorder.Background: Studies investigating the non-motor syndrome in isolated dystonia have

BackgroundThe nature and frequency of nonmotor symptoms in primary adult‐onset cervical dystonia (CD) and blepharospasm (BSP) patients in Chinese populations remain unknown.MethodsHamilton's Depression Scale (HAMD), Hamilton's Anxiety Scale (HAMA), Addenbrooke's Cognitive Examination Revised (ACE‐R), Pittsburgh Sleep Quality Index and Epworth Sleepiness Scale were used to evaluate NMS in 120 patients with primary focal adult‐onset dystonia (60 with BSP and 60 with CD) and 60 age‐, sex‐, and education level‐ matched healthy controls (HCs). Motor symptoms of BSP and CD patients were evaluated by Jankovic rating scale and Toronto Western Spasmodic Torticollis Rating Scale‐severity scale separately.ResultsTwenty patients had depression, and 29 patients had anxiety. The mean HAMD and HAMA scores were significantly higher in patient groups. Thirty‐six patients had cognitive decline based on the cut‐off score of 75. The total score and scores of each domain of ACE‐R were significantly lower in patient groups than that in HCs. Quality of sleep was impaired in patient groups, and patients with CD had worse quality of sleep than patients with BSP. Thirty‐three BSP patients and 43 CD patients suffered from sleep disorder separately. The frequency of excessive daytime sleepiness did not differ between patients and HCs. No significant correlation was found between NMS and motor severity in the two forms of dystonia.ConclusionsCurrent study suggests that NMS are prevalent in Chinese CD and BSP patients, and the motor severity of dystonia did not contribute to the severity of nonmotor symptoms. Assessment of nonmotor symptoms should be considered in clinical management of focal dystonia

-Background: Non-motor symptoms have been described in primary dystonia, but the results on cognitive impairment in this condition are discordant. Blepharospasm (BM) is a type of primary focal dystonia characterized by recurrent and involuntary eye blinking. Hemifacial spasm (HS), a condition with different pathophysiology, constitutes an adequate control group when investigating non-motor disturbances in BM. Objective: To compare the performance of patients with BM and HS in the Frontal Assessment Battery (FAB). Method: Twenty-two patients with BM and 29 patients with HS were submitted to the FAB and the MiniMental State Examination (MMSE). FAB scores were compared between the two groups. Correlations between FAB and sociodemographic and clinical variables were calculated. Results: BM group was not different from HS in relation to gender, age, length of symptoms, educational level and performance in the MMSE. FAB scores were also similar in both groups. FAB scores correlated negatively with age and positively with educational level and MMSE scores. Conclusion: Executive functioning as assessed by FAB is not altered in BM in comparison with HS.KEy wordS: dystonia, blefarospam, hemifacial spasm, executive function. funções executivas em pacientes com blefaroespasmo em comparação a pacientes com espasmo hemifacialResumo -Introdução: Alterações não-motoras são descritas na distonia primária, embora sejam conflitantes os resultados sobre prejuízo cognitivo nessa condição. Blefaroespasmo (BE) é um tipo de distonia primária focal caracterizada por contrações recorrentes e involuntárias das pálpebras. Espasmo hemifacial (EH), cuja fisiopatologia é distinta do BE, constitui bom grupo controle quando se investiga alterações não-motoras no BE. Objetivo: Comparar o desempenho de pacientes com BE e com EH na Bateria de Avaliação Frontal (BAF). Método: BAF e Mini-Exame do Estado Mental (MEEM) foram administrados a 22 pacientes com BE e 29 com EH. os escores da BAF foram comparados entre os dois grupos. Correlações entre resultados na BAF e variáveis sócio-demográficas e clínicas foram calculadas. Resultados: os grupos BE e EH foram semelhantes quanto a gênero, idade, duração dos sintomas, escolaridade e desempenho no MEEM. os escores dos dois grupos na BAF foram também similares, correlacionando-se negativamente com idade e positivamente com escolaridade e desempenho no MEEM. Conclusão: As funções executivas avaliadas pela BAF não estão alteradas no BE em relação ao EH.PAlAvrAS-CHAvE: distonia, blefaroespasmo, espasmo hemifacial, função executiva.