Sjogren's syndrome meets Meige's syndrome.

This report details a rare case where a patient simultaneously suffered from Sjogren's syndrome (SS) and Meige's syndrome (MS). SS, an autoimmune disorder, and MS, a rare neurological condition characterized by involuntary eyelid closure, presented in a 73-year-old male. The patient had been experiencing dry eye symptoms for the past 5 years, with the onset of eyelid spasms and tetanic eye closure occurring 3 years ago. Traditional treatments, including subthalamic nucleus deep brain stimulation, provided only temporary relief. Diagnostic evaluations, including blood tests and imaging, confirmed SS and MS coexistence. Treatment involved a combination of steroids, immunosuppressants, and immunoglobulin, leading to significant symptom relief. This case suggests a potential association between SS and the development of MS, highlighting the importance of immunomodulatory therapy in managing neurological symptoms. Further research is needed to explore the relationship between these two conditions and to develop more effective treatment strategies.

[Mental disorders in blepharospasm]. / Psikhicheskie rasstroistva pri blefarospazme.

OBJECTIVE: To analyze mental disorders in blepharospasm (BS) before and after botulinum therapy (BT). MATERIAL AND METHODS: We examined 25 patients with BS (9 men and 16 women), aged 50 to 85 years (mean 64.1±18.5), with BS (main study group). The control group consisted of 20 healthy individuals (7 men and 13 women, mean age 63.5±8.5). Patients were examined before and after BT (after 3 weeks) using a diagnostic structured interview Mini International Neuropsychiatric Interview, GAD-7, PHQ-9, fear of negative assessment (short version) and The Liebowitz Social Anxiety Scale (LSAS). RESULTS: Fifty-six percent of patients with BS, as assessed by the GAD-7, showed a high level of anxiety, while depression, measured by the PHQ-9 and found in 52% of patients, was mainly manifested by mild disorders. In the group of patients with BS, the mean scores were higher on the GAD-7, PHQ-9, fear of negative assessment (p<0.001) and LSAS (p<0.05) than in the control group. After treatment with BT, the levels of anxiety and depression in patients with BS decreased slightly and remained higher compared with the control group. Psychiatric examination in the majority (64%) of patients revealed mental disorders that could not be explained by the occurrence of BS. The remaining 36% of patients had adaptation disorders (nosogenic reactions) caused by BS. Affective mental pathology (recurrent depressive disorder and dysthymia) and anxiety disorders (social phobia and adjustment disorders) were more often observed in the main study group compared with the control group (24% versus 5% and 68% versus 10%, respectively). CONCLUSION: A significant proportion of patients with BS have anxiety and depressive disorders, the severity of which does not depend only on the severity of motor symptoms and does not significantly decrease after successful BT, but is caused by mental disorders that preceded the manifestation of BS. Identification of mental disorders to varying degrees associated with BS, not only on the basis of psychometric scales, but also consultation with a psychiatrist, will allow, in addition to the correction of motor symptoms of BS, to differentiate the therapeutic approach through psychotherapy and psychopharmacotherapy.

Profile of patients with essential blepharospasm and hemifacial spasm in the two largest ophthalmology reference centers in Brazil.

PURPOSE: Information is scarce regarding the comprehensive profile of patients with essential blepharospasm and hemifacial spasm in Brazil. The present study aimed to assess the clinical features of patients with these conditions, followed up in two reference centers in Brazil. METHODS: The study included patients with essential blepharospasm and hemifacial spasm, followed up at the Departments of Ophthalmology at Universidade Federal de São Paulo and Universidade de São Paulo. Apart from demographic and clinical features, past stressful events related to the first symptoms (triggering event), aggravating factors, sensory tricks, and other ameliorating factors for the eyelid spasms were assessed. RESULTS: A total of 102 patients were included in this study. Most patients were female (67.7%). Essential blepharospasm was the most frequent movement disorder [51/102 patients (50%)], followed by hemifacial spasm (45%) and Meige's syndrome (5%). In 63.5% of the patients, the onset of the disorder was associated with a past stressful event. Ameliorating factors were reported by 76.5% of patients; 47% of patients reported sensory tricks. In addition, 87% of the patients reported the presence of an aggravating factor for the spasms; stress (51%) was the most frequent. CONCLUSION: Our study provides information regarding the clinical features of patients treated in the two largest ophthalmology reference centers in Brazil.

Influence of unilateral eyelid spasms and botulinum toxin treatment on intraocular pressure measured by transpalpebral tonometer.

PURPOSE: Eyelid spasms might be associated with elevated intraocular pressure (IOP) in hemifacial spasm (HFS) patients. IOP assessment using a Goldmann applanation tonometer (GAT) is often compromised by eyelid spasms. This study aimed to assess the effect of HFS on IOP measurements using the transpalpebral tonometer Diaton® before and after treatment with botulinum toxin type A (BTX-A) and compared Diaton® and GAT measurements after treatment with BTX-A. METHODS: IOP measurements were obtained with Diaton® in 27 patients with moderate-to-severe HFS before and after treatment with BTX-A. After treatment, the IOP was also measured using GAT and the results were compared with the ones measured with a Diaton®. The patients underwent automated perimetry, OCT, and pachymetry for screening to glaucoma. RESULTS: Mean IOP with Diaton® was 11 ± 3.42 mmHg before treatment in the affected eye and 9 ± 2.98 mmHg in the contralateral eye. This difference was statistically significant (P = 0.012). However, after treatment with BTX-A, no interocular difference was found in IOP obtained with Diaton® (P = 0.204) or GAT (P = 0.971). Comparison between GAT and Diaton® measurements showed no significant differences after BTX-A treatment between the affected (P = 0.212) and contralateral eye (P = 0.971). CONCLUSIONS: A significant reduction in IOP measurements on the affected side of HFS patients was observed after treatment with BTX-A, demonstrating that eyelid spasms may increase the IOP. No significant difference was observed between Diaton® and GAT measurements after the application of BTX-A. No differences were found in automated perimetry, OCT, and CCT when comparing affected eyes with contralateral eyes.

Genetic Screening of a Hungarian Cohort with Focal Dystonia Identified Several Novel Putative Pathogenic Gene Variants.

Dystonia is a rare movement disorder which is characterized by sustained or intermittent muscle contractions causing abnormal and often repetitive movements, postures, or both. The two most common forms of adult-onset focal dystonia are cervical dystonia (CD) and benign essential blepharospasm (BSP). A total of 121 patients (CD, 74; BSP, 47) were included in the study. The average age of the patients was 64 years. For the next-generation sequencing (NGS) approach, 30 genes were selected on the basis of a thorough search of the scientific literature. Assessment of 30 CD- and BSP-associated genes from 121 patients revealed a total of 209 different heterozygous variants in 24 genes. Established clinical and genetic validity was determined for nine heterozygous variations (three likely pathogenic and six variants of uncertain significance). Detailed genetic examination is an important part of the work-up for focal dystonia forms. To our knowledge, our investigation is the first such study to be carried out in the Middle-European region.

What can epidemiological studies teach on the pathophysiology of adult-onset isolated dystonia?

Several demographic and environmental factors may play an important role in determining the risk of developing adult-onset isolated dystonia (AOID) and/or modifying its course. However, epidemiologic studies have provided to date only partial insight on the disease mechanisms that are actively influenced by these factors. The age-related increase in female predominance in both patients diagnosed with AOID and subjects carrying its putative mediational phenotype suggests sexual dimorphism that has been demonstrated for mechanisms related to blepharospasm and cervical dystonia. The opposite relationship that spread and spontaneous remission of AOID have with age suggests age-related decline of compensatory mechanisms that protect from the progression of AOID. Epidemiological studies focusing on environmental risk factors yielded associations only with specific forms of AOID, even for those factors that are not likely to predispose exclusively to specific focal forms (for example, only writing dystonia was found associated with head trauma, and only blepharospasm with coffee intake). Other factors show biological plausibility of their mechanistic role for specific forms, e.g., dry eye syndrome or sunlight exposure for blepharospasm, scoliosis for cervical dystonia, repetitive writing for writing dystonia. Overall, the relationship between environment and AOID remains complex and incompletely defined. Both hypothesis-driven preclinical studies and well-designed cross-sectional or prospective clinical studies are still necessary to decipher this intricate relationship.

Quantitative evaluation of blinking in blepharospasm using electrooculogram-integrated smart eyeglasses.

Smart eyeglasses with an integrated electrooculogram (EOG) device (JINS MEME ES_R®, JINS Inc.) were evaluated as a quantitative diagnostic tool for blepharospasm. Participants without blepharospasm (n = 21) and patients with blepharospasm (n = 19) undertook two voluntary blinking tests (light and fast) while wearing the smart eyeglasses. Vertical (Vv) and horizontal (Vh) components were extracted from time-series voltage waveforms recorded during 30 s of the blinking tests. Two parameters, the ratio between the maximum and minimum values in the power spectrum (peak-bottom ratio, Fourier transform analysis) and the mean amplitude of the EOG waveform (peak amplitude analysis) were calculated. The mean amplitude of Vh from light and fast blinking was significantly higher in the blepharospasm group than in the control group (P < 0.05 and P < 0.05). Similarly, the peak-bottom ratio of Vv from light and fast blinking was significantly lower in the blepharospasm group than in the control group (P < 0.05 and P < 0.05). The mean amplitude of Vh and peak-bottom ratio of Vv correlated with the scores determined using the Jankovic rating scale (P < 0.05 and P < 0.01). Therefore, these parameters are sufficiently accurate for objective blepharospasm classification and diagnosis.

Relations between nonmotor manifestations and motor disorders in patients with benign essential blepharospasm.

PURPOSE: To evaluate the relations between nonmotor manifestations (dry eye, mood disorders, and sleep disturbance) and motor disorders in patients with benign essential blepharospasm (BEB), and to determine whether relieving motor disorders by botulinum neurotoxin can improve the nonmotor manifestations. METHODS: In this prospective case series study, 123 BEB patients were enrolled for evaluations. Among them, 28 patients underwent botulinum neurotoxin therapy and attended another two postoperative visits at 1 month and 3 months. Motor severity was measured with Jankovic Rating Scale (JRS) and Blepharospasm Disability Index (BSDI). We assessed dry eye using OSDI questionnaire, Schirmer test, tear break-up time (TBUT), tear meniscus height, lipid layer thickness (LLT) and corneal fluorescence staining. Zung's Self-rating Anxiety and Depression Scale (SAS, SDS) and Pittsburgh Sleep Quality Index (PSQI) were for mood status and sleep quality evaluations. RESULTS: Patients with dry eye or mood disorders had higher JRS scores (5.78 ± 1.13, 5.97 ± 1.30) than those without (5.12 ± 1.40, 5.50 ± 1.16; P = 0.039, 0.019, respectively). BSDI values of patients with sleep disturbance (14.61 ± 4.71) was higher than those without (11.89 ± 5.44, P = 0.006). Correlations were found between JRS, BSDI and SAS, SDS, PSQI, OSDI, TBUT. Botulinum neurotoxin effectively relieved JRS, BSDI and improved PSQI, OSDI, TBUT, LLT (8.11 ± 5.81, 21.77 ± 15.76, 5.04 ± 2.15 s, 79.61 ± 24.11 nm) at the 1-month visit compared to baseline (9.75 ± 5.60, 33.58 ± 13.27, 4.14 ± 2.21 s, 62.33 ± 22.01 nm; P = 0.006, < 0.001, = 0.027, < 0.001, respectively). CONCLUSIONS: The BEB patients with dry eye, mood disorders, or sleep disturbance had more severe motor disorders. Motor severity was associated with the severity of the nonmotor manifestations. Relieving motor disorders by botulinum neurotoxin was effective in improving dry eye and sleep disturbance.

Debunking the Puzzle of Eyelid Apraxia: The Muscle of Riolan Hypothesis.

PURPOSE: Apraxia of eyelid opening (AEO) has been defined by the presence of an intermittent nonparalytic bilateral loss of the volitional ability to open the eyes or to maintain the eyelids in a sustained elevated position. It is not known whether the condition represents an apraxia, a dystonia, or a freezing phenomenon, and several different nomenclatorial terms have been suggested for this condition including the so-called AEO (scAEO), blepahrocolysis, focal eyelid dystonia, and so on. The primary goal of this review is to attempt to clarify the pathogenetic mechanisms underlying scAEO as a clinical phenomenon. This review also addresses the issue of whether scAEO is part of the spectrum of blepharospasm (BSP) which includes BSP, dystonic blinks and other dystonic eyelid conditions, or whether it is a separate phenomenologically heterogeneous disease with clinical features that merely overlap with BSP. METHODS: A literature review was conducted in PubMed, MEDLINE, PubMed Central (PMC), NCBI Bookshelf, and Embase for several related keywords including the terms "apraxia of eyelid opening," "pretarsal blepharospasm," "blepharocolysis," "eyelid freezing," "eyelid akinesia," "levator inhibition," "blepharospasm-plus," as well as "blepharospasm." The clinical findings in patients with scAEO who fulfilled the classic diagnostic criteria of the disease that were originally set by Lepore and Duvoisin were included, while patients with isolated blepharospasm or dystonic blinks (DB) were excluded. In addition, electromyographic (EMG) studies in patients with scAEO were reviewed in detail with special emphasis on studies that performed synchronous EMG recordings both from the levator muscle (LPS) and the pretarsal orbicularis oculi muscle (OO). RESULTS: The apraxia designation is clearly a misnomer. Although scAEO behaves clinically as a hypotonic freezing phenomenon, it also shares several cardinal features with focal dystonias. The authors broadly categorized the EMG data into 3 different patterns. The first pattern (n = 26/94 [27.6%]) was predominantly associated with involuntary discharges in the OO muscle and has been termed pretarsal blepharospasm (ptBSP). The commonest pattern was pattern no. 2 (n = 53/94 [56.38%]), which was characterized by involuntary discharges in the OO muscle, together with a disturbed reciprocal innervation of the antagonist levator muscle and is dubbed disturbed reciprocal innervation (DRI). This EMG pattern is difficult to discern from the first pattern. Pattern no. 3 (n = 15/94 [15.9%]) is characterized by an isolated levator palpebrae inhibition (ILPI). This levator silence was observed alone without EMG evidence of contractions in the pretarsal orbicularis or a disturbed reciprocal relation of both muscles. CONCLUSION: EMG evidence shows that the great majority (84%) of patients show a dystonic pattern, whereas ILPI (16%) does not fit the dystonic spectrum. The authors propose that a spasmodic contraction of the muscle of Riolan may be the etiological basis for levator inhibition in patients with ILPI. If this is true, all the 3 EMG patterns observed in scAEO patients (ptBSP, DRI, and ILPI) would represent an atypical form of BSP. The authors suggest coining the terms Riolan muscle BSP ( rmBSP ) for ILPI, and the term atypical focal eyelid dystonia ( AFED ) instead of the term scAEO, as both terms holistically encompass both the clinical and EMG data and concur with the authors' theorem.

Diagnosis and classification of blepharospasm: Recommendations based on empirical evidence.

BACKGROUND: Blepharospasm is one of the most common subtypes of dystonia, and often spreads to other body regions. Despite published guidelines, the approach to diagnosis and classification of affected body regions varies among clinicians. OBJECTIVE: To delineate the clinical features used by movement disorder specialists in the diagnosis and classification of blepharospasm according to body regions affected, and to develop recommendations for a more consistent approach. METHODS: Cross-sectional data for subjects diagnosed with all types of isolated dystonia were acquired from the Dystonia Coalition, an international, multicenter collaborative research network. Data were evaluated to determine how examinations recorded by movement disorder specialists were used to classify blepharospasm as focal, segmental, or multifocal. RESULTS: Among all 3222 participants with isolated dystonia, 210 (6.5%) had a diagnosis of focal blepharospasm. Among these 210 participants, 34 (16.2%) had dystonia outside of upper face region. Factors such as dystonia severity across different body regions and number of body regions affected influenced the classification of blepharospasm as focal, segmental, or multifocal. CONCLUSIONS: Although focal blepharospasm is the second most common type of dystonia, a high percentage of individuals given this diagnosis had dystonia outside of the eye/upper face region. These findings are not consistent with existing guidelines for the diagnosis and classification of focal blepharospasm, and point to the need for more specific guidelines for more consistent application of existing recommendations for diagnosis and classification.

[Dry eye and qualitative tear alteration in essential blepharospasm]. / Sécheresse oculaire et altération qualitative des larmes dans le blépharospasme essentiel.

PURPOSE: We aimed to analyze the tears of patients with Benin Essential Blepharospasm (BEB) to explore possible corneo-conjunctival mechanisms for photophobia, tear deficiency and ocular pain. METHODS: Using an observational cohort of 42 patients diagnosed with BEB, we performed Schirmer's testing, measurement of tear film pH, tear film protein electrophoresis and conjunctival impression cytology. RESULTS: Schirmer's testing showed dry eye (8.4±9.7mm) with 71.3% eyes having a Schirmer's score<10mm. Surprisingly, the pH was basic (8.4±0.4) and was improved in patients receiving the standard treatment of quarterly botulinum toxin injections (8.32±0.36 for treated patients vs. 8.74±0.53 for untreated patients; P=0.045). Together, tear protein electrophoresis and conjunctival impression cytology showed conjunctival inflammation associated with BEB. CONCLUSION: For the first time, this study emphasizes corneo-conjunctival mechanisms to explain the photophobia, dry eye and pain in Benign Essential Blepharospasm. While encouraging, complementary studies remain necessary to evaluate the effect of correcting tear film pH and inflammation on the quality of life of blepharospasm patients.

Tear osmolarity, dry eye syndrome, blepharospasm and botulinum neurotoxin.

PURPOSE: To assess the severity of dry eye disease in patients with blepharospasm (BEB) before (control) and after injection of botulinum neurotoxin A (BoNT/A). DESIGN: Cross-sectional study. METHODS: Patients with clinically diagnosed BEB and no known history of dry eye syndrome prior to the onset of the disease, before (controls) or after injection with one of the three available types of BoNT/A: onabotulinumtoxinA (Botox®; Allergan, Irvine, CA, USA), abobotulinumtoxinA (Dysport®; Beaufour Ipsen Pharma SAS, Paris, France) or incobotulinumtoxinA (Xeomin®; Merz Pharma GmbH, Frankfurt, Germany) every 3 months. Tear osmolarity and Schirmer's I-test were measured at the first visit for non-treated BEB patients as controls and 3 months after BoNT/A injection. RESULTS: The study consisted of 101 BEB patients (86 females, 15 males) with a mean age of 67 years (range 30-86 years). Seventeen patients were untreated, 26 treated with onabotulinumtoxinA, 23 treated with abobotulinumtoxinA and 35 treated with incobotulinumtoxinA. Post-injection mean tear osmolarity was not significantly higher among patients treated with onabotulinumtoxinA, abobotulinumtoxinA, or incobotulinumtoxinA (P=0.65, P=0.92, and P=0.15, respectively), compared to controls, remaining less than 308 mosm/mL. Mean Schirmer's I-test results remained under 5mm and did not vary between the four groups. CONCLUSIONS: The results clearly demonstrate that reduced tear secretion appears to be present in BEB patients even prior to treatment. This decreased lacrimal secretion was not correlated with hyperosmolarity. Clinicians should proactively treat dry eye syndrome in conjunction with management of the blepharospasm.

Sex related differences in nonmotor symptoms of patients with idiopathic blepharospasm.

Idiopathic blepharospasm shows a female predominance in prevalence, whether there are sex-related differences in distributions of nonmotor symptoms (NMSs) and predictors of quality of life are unknown. Four hundred and twenty-five patients with idiopathic blepharospasm were consecutively recruited, and underwent assessments including dystonia severity, mood disturbances, sleep disturbances, cognition, ocular symptoms, and quality of life. Frequencies and distributions of NMSs, and predictors of quality of life in female and male patients were investigated. NMSs existed in majority of male (94.0%) and female (95.8%) patients. The frequencies of depression, cognition dysfunction, and poor sleep quality were higher in female patients, while the frequency of excessive daytime sleepiness was higher in male patients. More female (79.5%) patients had multiple NMS domains affected than male (70.1%) patients (p = 0.040). Quality of life was associated with depression, anxiety and motor severity for female patients (adjusted R2 = 0.367, p < 0.001), while associated with depression, excessive daytime sleepiness and motor severity for male patients (adjusted R2 = 0.430, p < 0.001). The highly prevalent coexistence of multiple NMSs found in patients with blepharospasm support that blepharospasm is a network disorder. The sex-related differences in the pattern of NMSs and predictors of quality of life may aid the development of tailored management of blepharospasm.

Diagnostic criteria for blepharospasm: A multicenter international study.

BACKGROUND: There are no widely accepted criteria to aid the physician in diagnosing BSP. OBJECTIVE: To validate recently proposed diagnostic criteria for blepharospasm in a larger and geographically diverse population and to develop a screening system for blepharospasm. METHODS: Video-recordings from 211 blepharospasm patients and 166 healthy/disease controls were examined by 8 raters. Agreement for presence of orbicularis oculi spasms, sensory trick, and increased blinking was measured by k statistics. Inability to voluntarily suppress the spasms was asked by the examiner but not captured in the video. Patients/controls were also requested to fill a self-administered questionnaire addressing relevant blepharospasm clinical aspects. The diagnosis at each site was the gold standard for sensitivity/specificity. RESULTS: All the study items yielded satisfactory inter/intra-observer agreement. Combination of items rather than each item alone reached satisfactory sensitivity/specificity. The combined algorithm started with recognition of spasms followed by sensory trick. In the absence of a sensory trick, including "increased blinking" or "inability to voluntarily suppress the spasms" or both items yielded 88-92% sensitivity and 79-83% specificity. No single question of the questionnaire yielded high sensitivity/specificity. Serial application of the questionnaire to our blepharospasm and control subjects and subsequent clinical examination of subjects screening positive by the validated diagnostic algorithms yielded 78-81% sensitivity and 83-91% specificity. CONCLUSION: These results support the use of proposed diagnostic criteria in multi-ethnic, multi-center cohorts. We also propose a case-finding procedure to screen blepharospasm in a given population with less effort than would be required by examination of all subjects.

[How to explore a blepharospasm]. / Comment j'explore un blépharospasme.

Blepharospasm is a particularly distressing form of focal dystonia, specifically affecting the eyelids and causing involuntary closing of the eyes or an increased blinking. Despite its frequency, the diagnosis of this condition is often delayed. Diagnostic criteria are mainly clinical, but sometimes further investigations may be necessary as blepharospasm may be included in a complex clinical picture.

Le blépharospasme est une forme de dystonie focale particulièrement invalidante, touchant spécifiquement les paupières et engendrant une fermeture involontaire des yeux ou un clignement palpébral excessif. Malgré sa fréquence, cette affection souffre d'un délai de diagnostic souvent trop long. Celui-ci est avant tout clinique, mais il ne faut pas méconnaître des formes s'intégrant au sein de tableaux neurologiques complexes et nécessitant une mise au point plus complète.

[Secondary blepharospasm, analysis and pathophysiology of blepharospasm. French translation of the article]. / Blépharospasmes secondaires, analyse et réflexion sur la physio-pathogénie du blépharospasme.

PURPOSE: To localize the brain structures involved in blepharospasm. MATERIALS AND METHODS: This is a retrospective consecutive series of brain MRI's of patients with secondary blepharospasm whose immediate past medical history included cerebrovascular accident or head trauma. RESULTS: Six patients, including 4 with CVA with ischemic or hemorrhagic lesions of the thalamus and caudate nuclei and 2 with head trauma with contusive sequellae to the tectal plate and frontal cortical and cerebellar atrophy. CONCLUSION: According to the literature, brain lesions associated with blepharospasm involve mainly the thalamus, head of the caudate nucleus, corpus striatum, globus pallidus, internal capsule, cerebral cortex and cerebellum. This study demonstrates that blepharospasm is associated with a lesion of a complex neural network - cortex-thalamus-globus pallidus-cortex - and does not correspond to a single, unique lesion. This network is connected with ascending and descending sensory-motor pathways and motor nuclei.

Secondary blepharospasm, analysis and pathophysiology of blepharospasm.

PURPOSE: To localize the brain structures involved in blepharospasm. MATERIALS AND METHODS: This is a retrospective consecutive series of brain MRI's of patients with secondary blepharospasm whose immediate past medical history included cerebrovascular accident or head trauma. RESULTS: Six patients, including 4 with CVA with ischemic or hemorrhagic lesions of the thalamus and caudate nuclei and 2 with head trauma with contusive sequellae to the tectal plate and frontal cortical and cerebellar atrophy. CONCLUSION: According to the literature, brain lesions associated with blepharospasm involve mainly the thalamus, head of the caudate nucleus, corpus striatum, globus pallidus, internal capsule, cerebral cortex and cerebellum. This study demonstrates that blepharospasm is associated with a lesion of a complex neural network - cortex-thalamus-globus pallidus-cortex - and does not correspond to a single, unique lesion. This network is connected with ascending and descending sensory-motor pathways and motor nuclei.

A novel task-specific dystonia type: Hemifacial spasm in a photographer.

A 67-year-old male photographer who used traditional cameras that necessitated monocular focusing developed intermittent blepharospasms, evident only during and shortly after the voluntary contraction of the left eyelids while using the camera, a form of a task-specific blepharospasm. The spasms gradually progressed to involve the entire hemiface resulting in a task-specific hemifacial spasm that eventually evolved into a persistent hemifacial spasm. Our case report highlights the fact that focal dystonia may also develop in the facial muscles following chronic and repetitive muscle contractions, such as those performed by an older photographer who used traditional cameras that necessitated monocular focusing. To our knowledge, hemifacial spasm has not yet been recognized as a form of focal, task-specific dystonia. Moreover, occupational, focal dystonia has not been described in photographers.

A propósito de tres casos: ptosis y cefalea en diferentes escenarios / Ptosis and headaches in different scenaries: a presentation of 3 cases

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