Roles of preoperative anxiety and depression in the outcomes of microvascular decompression in hemifacial spasm for adolescent patients.

ABSTRACT: Hemifacial spasm (HFS) has been recognized as the frequently occurring disease of cranial nerve. At the same time, several articles indicate that, dystonia results in certain psychological disorders. Consequently, this study aimed to examine the association of preoperative depression and anxiety with HFS severity; meanwhile, the role in microvascular decompression (MVD) outcomes after surgery among adolescent patients was also examined.All cases had been classified as two groups based on MVD outcomes among HFS cases; in addition, the preoperative Hamilton anxiety rating scale (HARS) and the Hamilton depression rating scale (HDRS) scores were compared between patients not and still suffering from spasm. Moreover, the multiple logistic regression model was employed in assessing the relationship between preoperative HARS as well as HDRS scores and outcomes of adolescent cases undergoing MVD.The preoperative HARS and HDRS scores showed positive correlation with Cohen spasm grades in HFS patients. Meanwhile, compared with spasm-free group, patients of persistent spams group had apparently higher preoperative HARS and HDRS scores.Our results suggest that, preoperative anxiety and depression status show close association with HFS severity, and they could also impact the MVD outcomes for adolescent cases.

Personality traits associated with blepharospasm: A comparison with healthy subjects, patients with facial hemispasm and patients with hyperhidrosis.

The aim of this study is to explore the existence of specific personality traits related to patients with blepharospasm (BSP), treated with injections of botulinum neurotoxin (BTX). Sixteen patients with BSP, 22 with facial hemispasm (HFS), 20 with essential hyperhidrosis (EH) and 20 healthy controls (HCs) completed the Temperament and Character Inventory-Revised to explore personality traits based on Cloninger's Psychobiological Model. The results revealed that the four groups differed on the Harm Avoidance (HA) scale and fear of uncertainty subscale, as well as on Persistence (PS). On HA, BSP group did not differ from HCs, but had higher scores than HFS and EH groups. On PS scales, BSP and HFS patients did not differ between them but showed higher score than HCs and EH patients. Our findings suggested that a high level of Harm Avoidance and Persistence seem to be associated with BSP, when compared with any disorders treated with BTX. An evaluation of the personality traits might help the clinicians to early identify BSP patients at greater risk of developing psychopathological disturbances.

Strengthened thalamoparietal functional connectivity in patients with hemifacial spasm: a cross-sectional resting-state fMRI study.

OBJECTIVE: In spite of the well-known importance of thalamus in hemifacial spasm (HFS), the thalamic resting-state networks in HFS is still rarely mentioned. This study aimed to investigate resting-state functional connectivity (FC) of the thalamus in HFS patients and examine its association with clinical measures. METHODS: 25 HFS patients and 28 matched healthy controls underwent functional MRI at rest. Using the left and right thalamus as seed regions respectively, we compared the thalamic resting-state networks between patient and control groups using two independent sample t-test. RESULTS: Compared with controls, HFS patients exhibited strengthened bilateral thalamus-seeded FC with the parietal cortex. Enhanced FC between right thalamus and left somatosensory association cortex was linked to worse motor disturbance, and the increased right thalamus-right supramarginal gyrus connection were correlated with improvement of affective symptoms. CONCLUSION: Our findings indicate that the right thalamus-left somatosensory association cortex hyperconnectivity may represent the underlying neuroplasticity related to sensorimotor dysfunction. In addition, the upregulated FC between the right thalamus and right supramarginal gyrus in HFS, is part of the thalamo-default mode network pathway involved in emotional adaptation. ADVANCES IN KNOWLEDGE: This study provides new insights on the integrative role of thalamo-parietal connectivity, which participates in differential neural circuitry as a mechanism underlying motor and emotional functions in HFS patients.

The long-term effects of microvascular decompression on social phobia and health-related quality of life in patients with hemifacial spasm: a 3-year prospective study.

BACKGROUND: Although not a life-threatening condition, hemifacial spasm (HFS) frequently leads to social phobia because it causes significant facial disfigurement and consequently reduces health-related quality of life (HRQoL). The purpose of the current study was to examine the long-term effects of MVD on psychological aspects and HRQoL in HFS patients with social anxiety over a 36-month follow-up. METHODS: Thirty patients with HFS who underwent MVD from January 2015 to May 2015 were included in this prospective study. Clinical data, including standardized measures of general anxiety and depression (Hospital Anxiety Depression Scale (HADS)), social anxiety (Liebowitz Social Anxiety Scale (LSAS)), and the severity of HFS, were collected postoperatively, and 6 months and 36 months after MVD. Likewise, data on HRQoL were collected at baseline, and 6 months and 36 months after MVD using the Korean version of the Short Form 36 (SF-36). RESULTS: Twenty-two patients who completed the 36-month follow-up were classified into social phobia group and non-social phobia group based on the LSAS total scores of 60. Repeated measures analysis of variance demonstrated significant differences between the two groups over time for the total LSAS score (p < 0.001), anxiety subscale score of the HADS (p = 0.002), and the Mental Component Summary (MCS) (p = 0.046) of the SF-36. A comparison of these two groups in terms of differences observed in their scales at 6 months after MVD has shown that the improvements of the social phobia group in HADS anxiety subscale (p = 0.010), LSAS total score (p = 0.008), and MCS (p = 0.040) were significantly more improved than the those of non-social phobia group. And at 36 months after surgery, the improvement of the scales mentioned above was maintained, and additionally Vitality (p = 0.040) and Mental Health (p = 0.040) dimensions showed a statistically significant improvement. CONCLUSIONS: The improvements previously observed in psychological aspects and HRQoL over a short-term follow-up after MVD in HFS patients with social phobia were maintained for at least 36 months after MVD.

Evaluation of stigmatization in hemifacial spasm and quality of life before and after botulinum toxin treatment.

Hemifacial spasm (HFS) is a chronic disease that is characterized by involuntary tonic and clonic contractions of the muscles innervated by the ipsilateral facial nerve. Botulinum toxin (BTX) is the most effective medical treatment of this condition. The aim of our study was to evaluate stigma among patients diagnosed with HFS and to search quality of life and depression before and after BTX injection. Seven self-rating items (HFS-7) and seven questions related to stigmatization were administered to HFS patients. Participants also answered SF-36 health outcome measure and Beck depression inventory before and 4 weeks after the routine BTX injection. The severity of HFS was graded based on a five-point scale. Descriptive statistics and paired t test were applied. The level of significance was set at α = 0.05. Fourty HFS patients were prospectively included. Twenty-one (%52.5) were female and nineteen were male (47.5%) with a mean age of 57.1 (SD = 12.13; min-max = 27-78). 60% (n: 24) of patients were feeling themselves different from people without HFS. Beck depression inventory scores improved after BTX injection significantly (p < 0.05). All domains of SF-36 showed positive improvement after BTX injections. The improvement in general health perception, physical functioning, and vitality was statistically significant (p < 0.05). Although more than half of the patients felt themselves different from people without HFS, treatment of HFS with BTX significantly improved mental health and physical health, and depressive symptoms of the patients.

Improvement of Quality of Life in Patients with Hemifacial Spasm After Microvascular Decompression: A Prospective Study.

OBJECTIVE: Although not life threatening, hemifacial spasm (HFS) can be disabling and significantly affect quality of life (QOL). The aim of this study was to assess the major factors affecting the QOL and further to investigate the impact of microvascular decompression (MVD) on QOL in patients with HFS. METHODS: Patients with HFS who underwent MVD in our department between 2013 and 2014 were included in this study. The validated, disease-specific questionnaire (HFS-30) was used to evaluate the QOL in patients with HFS before surgery and 1 year after MVD. The clinical data of these patients were collected prospectively and statistically analyzed. RESULTS: A total of 116 consecutive patients were enrolled in this study, including 69 women and 47 men, with a mean age of 43.3 ± 7.8 years. The global QOL was significantly impaired in patients with HFS. There was a positive correlation of severity of HFS with patient's QOL both in physical and mental health domains (r = 0.34, P = 0.02; r = 0.46, P = 0.03). Patients with a higher educational level tended to have a worse QOL in the mental health domain (r = 0.43, P = 0.02). Seventy-nine patients (68.1%) were spasm free immediately postoperatively, 106 (91.4%) were spasm free at 1-year follow-up. There were significant improvements across all subscales of the HFS-30 questionnaire between preoperative and postoperative responses, and 84.5% of patients experienced significant improvement in QOL after MVD. CONCLUSIONS: HFS affects QOL both physically and mentally. Patients with severe HFS symptoms or a higher educational level are at higher risk of worse QOL. MVD not only provides high spasm-relief rate but also leads to significantly higher QOL after surgery.

Facial Weakness, Otalgia, and Hemifacial Spasm: A Novel Neurological Syndrome in a Case-Series of 3 Patients With Rheumatic Disease.

Bell palsy occurs in different rheumatic diseases, causes hemifacial weakness, and targets the motor branch of the 7th cranial nerve. Severe, persistent, and refractory otalgia having features of neuropathic pain (ie, burning and allodynic) does not characteristically occur with Bell palsy. Whereas aberrant regeneration of the 7th cranial nerve occurring after a Bell palsy may lead to a variety of clinical findings, hemifacial spasm only rarely occurs. We identified in 3 rheumatic disease patients (2 with Sjögren syndrome, 1 with rheumatoid arthritis) a previously unreported neurological syndrome of facial weakness, otalgia with neuropathic pain features, and hemifacial spasm. We characterized symptoms, examination findings, and response to therapy. All 3 patients experienced vertigo, as well as severe otalgia which persisted after mild facial weakness had completely resolved within 1 to 4 weeks. The allodynic nature of otalgia was striking. Two patients were rendered homebound, as even the barest graze of outdoor breezes caused intolerable ear pain. Patients developed hemifacial spasm either at the time of or within 3 months of facial weakness. Two patients had a polyphasic course, with recurrent episodes of facial weakness and increased otalgia. In all cases, otalgia and hemifacial spasm were unresponsive to neuropathic pain regimens, but responded in 1 case to intravenous immunoglobulin therapy. No patients had vesicles or varicella zoster virus in spinal-fluid studies. We have defined a novel neurological syndrome in 3 rheumatic disease patients, characterized by facial weakness, otalgia, and hemifacial spasm. As described in infectious disorders, the combination of otalgia, facial weakness, and 8th cranial nerve deficits suggests damage to the geniculate ganglia (ie, the sensory ganglia of the 7th cranial nerve), with contiguous involvement of other cranial nerves causing facial weakness and vertigo. However, the relapsing nature and association with hemifacial spasm constitute a unique part of this neurological syndrome.

Residual hemifacial spasm after microvascular decompression: prognostic factors with emphasis on preoperative psychological state.

Residual hemifacial spasm (HFS) after microvascular decompression (MVD) is common, and the factors associated with residual HFS are still controversial. In the present study, we analyzed the outcome of 212 patients with hemifacial spasm after a single microvascular decompression and evaluated the prognostic factors involved in residual hemifacial spasm. Based on our study, possible prognostic factors included indentation of the root exit zone (REZ), preoperative illness duration, and preoperative psychological state. We suggest that MVD should be performed as early as possible for it may decrease the rate of residual HFS. Preoperative assessment of psychological state in HFS patients is a timely intervention that should be implemented to minimize the residual HFS.

Structural changes in the CNS of patients with hemifacial spasm.

Hemifacial spasm (HFS) is a peripheral nerve disorder which impacts the living quality of patients both psychologically and physically. Whether HFS has structural changes under these specific stressors including psychological and physiological conditions in the CNS remains largely unknown. In the current study, voxel-based morphometry (VBM) was used to evaluate changes in gray matter (GM) by using T1-weighted imaging in 25 HFS patients and 25 demographically similar healthy volunteers. The severity of the spasm was assessed using a Cohen evaluation scale. Hamilton anxiety (HAMA) and Hamilton depression (HAMD) rating scales were used to evaluate the affective conditions of subjects. 3D-FIESTA and 3D-TOF sequences were applied to evaluate the neurovascular compression (NVC) rating in each subject. In our results, we found that HFS patients had higher NVC rating scores than those of healthy volunteers, and the spasm severity rating was positively correlated with the NVC rating (r=0.736, p<0.001). HFS patients had higher scores on the HAMA and HAMD compared with healthy volunteers. For the GM comparison, reductions were found in the thalamus, putamen, pallidum, dorsolateral prefrontal cortex, amygdala and parahippocampal gyrus in patients with HFS compared with healthy volunteers. Additionally, the GM volume changes in the amygdala did not exhibit any significant between-group differences with HAMA and HAMD scores as covariates. Our results suggested that HFS probably led to GM volume abnormalities of the CNS. We indicated that the GM volume changes of the amygdala may be highly related to emotional factors.

Psychiatric disorders in idiopathic-isolated focal dystonia.

Idiopathic-isolated focal dystonia (IIFD) is a movement disorder characterised by involuntary, sustained muscle contractions, leading to abnormal postures. Psychopathology is frequent in patients with IIFD, and while traditionally this was thought to be a secondary phenomenon, there is emerging evidence for shared neurobiological mechanisms. We conducted a single-centre cross-sectional study of 103 consecutive patients with IIFD and two comparison groups: 78 consecutive patients with hemifacial spasm (HFS) and 93 healthy control subjects. Assessments with regard to psychiatric disturbances were performed using self-report questionnaires, including the self-report version of the Yale-Brown Obsessive Compulsive Scale (Y-BOCS-SR), the Beck Anxiety Inventory (BAI), and the Beck Depression Inventory (BDI). Compared to healthy control subjects and patients with HFS, the IIFD group had higher OCS, anxiety, and depression scores as measured by the Y-BOCS-SR, BAI, and BDI, respectively. The Y-BOCS-SR, BAI, and BDI were highly correlated across all the subjects. Logistic regression analysis showed that the main driver of high obsessive-compulsive symptom scores, irrespective of neurological diagnosis, was the BDI, whereas it was BAI (and not BDI), that drives the association between the psychiatric rating scale scores and the neurological diagnosis. Our findings suggest that while clinically significant obsessive-compulsive symptoms are over-represented in IIFD patients relative to controls, the BAI may have better discriminatory power to distinguish between the psychiatric symptoms in IIFD patients.

Surgical results of microvascular decompression procedures and patient's postoperative quality of life: review of 139 cases.

Microvascular decompression (MVD) is effective for the relief of symptoms, but little is known about the impact of the MVD procedure on patient's quality of life (QoL) or which QoL factors are important. The surgical results of MVD and the impact of this procedure were evaluated on patient's QoL in 139 patients, 74 with hemifacial spasm (HFS) and 65 with trigeminal neuralgia (TN), who underwent MVD between 2004 and 2011 using the 36-Item Short Form Health Survey questionnaire. Symptoms had resolved in approximately 95% of patients after MVD. The QoL questionnaire was completed by 54 HFS patients and 38 TN patients. Although long-term QoL scores for both groups were comparable to the average national value, scores related to physical role, emotional role, and social function were significantly lower for patients within 12 months of receiving MVD for HFS, compared with the reference scores. Symptomatic improvements and complications were correlated with the QoL scores related to the social function domain for patients with HFS. No other significant relationships were observed between any of the factors or scores in any of the respective domains or periods. Subjective symptoms were the main self-reported causes of delayed recovery of QoL domains. Some QoL domains take a long time to recover and postoperative subjective symptoms might be major causes in addition to delayed relief of symptoms.

Factors affecting the quality of life in hemifacial spasm patients.

BACKGROUND AND PURPOSE: Hemifacial spasm (HFS), a movement disorder manifested by unilateral spasms of the muscles innervated by the facial nerve, interferes with social life in about 90% of patients, causing social isolation and depression and having a significant impact on the quality of life. The aim of the study was to assess factors affecting the quality of life in patients with HFS in respect of influence of the severity of depression symptoms and botulinum toxin type A (BTX-A) therapy. MATERIAL AND METHODS: Eighty-five out of 129 patients included in the HFS database of the Movement Disorders Outpatient Clinic, Department of Neurology, University Hospital, Cracow who fulfilled the inclusion criteria and had no exclusion criteria (suffering from concomitant movement disorders, other severe chronic diseases or cognitive impairment) were studied. Demographic and clinical data (age at onset, disease duration and accompanying symptoms) were collected. Severity of HFS was assessed by the five-point clinical scale and seven-point Clinical Global Impression scale. Quality of life was assessed with the HFS-36 questionnaire and severity of depressive symptoms was evaluated with the Beck Depression Inventory. HFS-36 was performed twice, before BTX-A injection and two weeks later. RESULTS: The mean global score of HFS-36 was 47 ± 31 (maximum: 140 pts). Decreased HFS-36 score resulted from divergent deterioration in all subscales included in the questionnaire. Independent risk factors of deterioration in HFS-36 were increased severity of HFS and depressive symptoms as well as accompanying trismus. The HFS-36 score depended on the number and type of accompanying symptoms as well. Botulinum toxin type A therapy led to a significant improvement of HFS-36, particularly high in patients with multiple (> 4) HFS-related symptoms. CONCLUSIONS: The HFS-36 score depends mostly on severity of HFS, depressive symptoms and occurrence of accompanying trismus. It improves after BTX-A treatment.

Obsessive-compulsive symptoms among patients with blepharospasm and hemifacial spasm.

OBJECTIVE: The aim of this study was to compare the prevalence and the severity of different obsessive-compulsive disorder (OCD) symptoms reported by patients with blepharospasm (BSP) with those reported by patients with hemifacial spasm (HFS). We hypothesized that, since patients with BSP present a dysfunctional striato-thalamo-cortical circuitry, they would exhibit higher prevalence and/or greater severity of OCD symptoms than patients with HFS, a condition that results from peripheral irritation of the facial nerve. METHODS: Twenty-two patients with BSP and 31 patients with HFS were systematically evaluated by means of a sociodemographic and clinical questionnaire, the Mini International Neuropsychiatric Interview, the Obsessive-Compulsive Inventory-Revised, the Beck Depression Inventory (BDI), the Beck Anxiety Inventory (BAI) and the Mini Mental State Examination (MMSE). Diagnostic groups were compared using the Mann-Whitney U test for continuous variables and the Pearson's goodness-of-fit χ(2) test for categorical ones; Fisher's Exact Test was employed when indicated. Correlations between continuous variables were evaluated by means of Spearman coefficients. RESULTS: Patients with BSP and HFS were not significantly different in terms of sociodemographic characteristics and most neuropsychiatric features. Nevertheless, while checking was associated with shorter duration of BSP (Spearman's rho=-0.54; P=.01), hoarding correlated with a longer duration of HFS (Spearman's rho=0.40; P=.04). Length of abnormal movements did not correlate with the BDI, BAI and MMSE scores. CONCLUSIONS: The finding that the severity of different OCD symptoms did not differ between the BSP and HFS groups suggests that BSP may not interfere significantly with behavioral components of the striato-thalamo-cortical circuitry. However, the fact that OCD symptoms were found to follow different courses in distinct diagnostic groups deserves further study.

Factors associated with quality of life in hemifacial spasm and blepharospasm during long-term treatment with botulinum toxin.

PURPOSE: This study was undertaken to assess the impact of hemifacial spasm (HFS) and blepharospasm (BS) on quality of life (QOL) during long-term treatment with botulinum toxin injections. METHODS: Fifty-three patients with HFS and 32 patients with BS as well as two healthy age- and sex-matched control groups were included in the study. All participants independently completed the disease-specific questionnaire for QOL, the HFS-30, and the Thai Depression Inventory, and also provided a peak improvement score assessment. RESULTS: The disease severity in both patient groups revealed mild functional impairment. The scores of HFS-30 in HFS patients (four of 8 subscales) and BS patients (3 of 8 subscales) were significantly higher than the control groups. In both HFS and BS, depression scores were positively correlated with the HFS-30 scores, while peak improvement and educational level were inversely correlated with the HFS-30 scores. Patients with BS were more affected in Mobility and Activities of Daily Living than HFS patients. CONCLUSIONS: Both HFS and BS affect QOL both physically and mentally despite clinical improvement with botulinum toxin. Depression and peak improvement after injection were associated with the level of QOL.

Surgical outcome and improvement in quality of life after microvascular decompression for hemifacial spasms: a case series assessment using a validated disease-specific scale.

BACKGROUND: Hemifacial spasm (HFS) is a movement disorder characterized by intermittent, involuntary clonic or tonic-clonic contractions of muscles innervated by the ipsilateral facial nerve. Recent studies have documented change in quality of life after HFS management with botulinum toxin injection. However, we failed to locate any study that documented change in quality of life after surgical management with retrosigmoid microvascular decompression (MVD). METHODS: Our study objectives were 3-fold. Firstly, to use a disease-specific, validated quality of life assessment scale to document any change in quality of life after MVD for HFS. Secondly, to determine the time period in which the majority of patients undergoing MVD could be expected to benefit from surgery. Finally, to determine factors affecting the postoperative quality of life following MVD. A retrospective analysis of HFS patients treated with MVD at a single institution by a single surgeon (K.J.B.) between January 2000 and December 2007 was undertaken. A modification of a previously developed validated disease-specific quality of life assessment scale that included the addition of a parameter for difficulty in sleep was used to assess quality of life before and after surgery. RESULTS: A total of 21 patients (14 female and 7 male) underwent treatment as specified. Eighty-five percent (17/20) of the patients reported prolonged remission of symptoms (mean follow-up period = 4.15 years). Five percent (1/20) reported occasional recurrence of twitches. The overall mean quality of life score improved from 11.1 preoperatively to 2.2 postoperatively. CONCLUSIONS: MVD offers significant and prolonged improvement in quality of life for the HFS patients we studied, as measured using a disease-specific, validated quality of life assessment scale. Postoperative quality of life, however, was strongly influenced by both the success of surgery in resolving the symptoms and the absence of any permanent complications of surgery.

Validation of a Chinese version of disease specific quality of life scale (HFS-36) for hemifacial spasm in Taiwan.

BACKGROUND AND OBJECT: There was no Chinese questionnaire to evaluate the health-related quality of life (HRQoL) in patients with hemifacial spasm (HFS). In this study, we aimed to validate a new disease-specific HRQoL scale for HFS (HFS-36) in Chinese version, and compared it to SF-36, a generic HRQoL scale. PATIENTS AND METHODS: The HFS-36 Chinese version was modified from English version of HFS-30, including subscales of mobility, activities of daily living (ADL), emotional well-being, stigma, social support, cognition, bodily discomfort, and communication. All the items were scored on the 5-point scales, ranging from 0(never) to 4(always). Patients with HFS were asked to answer HFS-36 and SF-36 questionnaires on the same day before and 6-8 weeks after Botulinum toxin (BTX) injections, respectively. The reliability and validity of HFS-36 scale were evaluated statistically. RESULTS: Totally, 103 patients (68 females; 35 males) were recruited in this study, with a mean age of 57.6 +/- 11.5 years and a mean duration of HFS for 7.6 +/- 5.8 years. The intra-class correlation (ICC) and Cronbach's alpha were over 0.7 in the majority of items. HFS-36 showed a good correlation to HFS severity before BTX treatment and a significant improvement of subscale scoring after BTX treatment. HFS-36 also had a significant correlation to the mental health of SF-36. CONCLUSIONS: The Chinese version of HFS-36 demonstrated a good reliability and validity in subscales of motility, ADL, emotion well-being, stigma and bodily discomfort. The HRQoL was significantly improved after BTX treatment assessed by HFS-36 or SF-36. Compared to SF-36, HFS-36 scale was more sensitive and specific to evaluate the HRQoL in HFS.

Executive functioning in patients with blepharospasm in comparison with patients with hemifacial spasm.

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 Mini-Mental 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.

Executive functioning in patients with blepharospasm in comparison with patients with hemifacial spasm / Funções executivas em pacientes com blefaroespasmo em comparação a pacientes com espasmo hemifacial

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 Mini-Mental 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.

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.

Treatment outcome correlates with knowledge of disease in hemifacial spasm.

OBJECTIVES: Hemifacial spasm (HFS), a potentially disabling facial condition affects quality of life (QOL) and botulinum toxin is an effective treatment. No studies have examined whether a better level of knowledge of the disease would lead to an improved quality of life and treatment response in HFS. We examined the relationship between knowledge of disease with improvement in QOL following botulinum toxin treatment in HFS patients. PATIENTS AND METHODS: A total of 106 HFS patients (mean age of 56.8+/-9.9 years) were prospectively included. A baseline knowledge questionnaire and a validated disease-specific quality of life scale (HFS-7) were administered before and after botulinum toxin treatment. RESULTS: A better educational level was an independent predictor of high knowledge of HFS (p=0.02). Multivariate analysis using improvement in HFS-7 (total and subscore) as outcomes, and adjusting for age, gender, education, severity and duration of HFS, showed that high knowledge was predictive of a bigger improvement in HFS-7 total (p=0.03) and HFS-7 subscore (p=0.03). CONCLUSIONS: HFS patients with high knowledge of disease reported better improvement in QOL following botulinum toxin treatment. Better educational efforts will augment current medical and surgical treatments in improving QOL in HFS. Our findings could potentially be extended to many other medical conditions.