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Purpose of Review: Impaired eye movements are frequently seen in ophthalmic and neurologic clinical practice, especially in individuals with movement disorders. Identification of the abnormal movement can aid initial diagnosis and improve understanding of the underlying disease pathophysiology. The present article reviews the ocular motor manifestations and recent research on them in common movement disorders., Recent Findings: Ocular motor manifestations and their pathophysiologic correlates are being defined. In particular, study of eye movements can help clarify the changing clinicopathologic spectrum of atypical parkinsonian disorders. The pathophysiology and natural history of blepharospasm are being elucidated. Recent research focuses on high-resolution imaging and other technological advances to improve the sensitivity of the ocular motility exam. Eye movements are being studied as biomarkers for diagnosis and progression in clinical care and trials., Summary: The current review summarizes ocular motor manifestations in common movement disorders, and presents recent research investigating their cause and treatment.

Purpose of Review: To review new developments in the medical and surgical treatment options for benign essential blepharospasm (BEB)., Recent Findings: Botulinum toxin injections remain the mainstay treatment for BEB with several formulations currently commercially available. Reports in the medical literature support photochromatic modulation for the symptoms of photophobia, as well as oral medications and surgical myectomy for control of the motor signs of eyelid protractor spasm., Summary: Although there remains no cure for BEB, several treatment options are available to effectively manage the signs and symptoms of the condition.

The aim of the study was to evaluate the morphological changes of meibomian glands (MGs) in primary blepharospasm (PBS) by in vivo laser scanning confocal microscopy (LSCM) and to investigate the correlations between clinical data of PBS and LSCM parameters of MGs. This prospective and case-control study recruited 30 consecutive PBS patients and 30 age- and gender-matched healthy controls. After questionnaire assessments of ocular surface disease index (OSDI), Jankovic rating scale, and blepharospasm disability index, all subjects underwent blink rate evaluation, tear film break-up time (TBUT), corneal fluorescein staining (CFS), Schirmer test, MG expressibility, meibum quality, MG dropout, and LSCM examination of the MGs. The main LSCM outcomes included the mean MG acinar area and density, orifice diameter, meibum secretion reflectivity, acinar irregularity, and inhomogeneity of interstice and acinar wall. The PBS patients had significantly higher blink rate, higher OSDI and CFS scores, lower TBUT and Schirmer test value, and worse MG expressibility than the controls (All P < 0.05), whereas meibum quality showed no difference (P > 0.05). The PBS patients showed lower values of MG acinar area, orifice diameter and meibum secretion reflectivity, and higher scores of acinar irregularity and inhomogeneity of interstices than the controls (All P < 0.05). For the PBS patients, the severity of blepharospasm evaluated by JCR scale was strong correlated with MG acinar area (P < 0.001), orifice diameter (P = 0.002), meibum secretion reflectivity (P = 0.002), and MG acinar irregularity (P = 0.013). The MG expressibility was significantly correlated to MG acinar area (P = 0.039), orifice diameter (P < 0.001), and MG acinar irregularity (P = 0.014). The OSDI score was moderate correlated with MG acinar irregularity (P = 0.016), whereas the TBUT value was positively correlated with MG acinar area (P = 0.045) and negatively correlated to MG acinar irregularity (P = 0.016). The CFS score was negatively correlated to MG orifice diameter (P = 0.008). The LSCM provided a noninvasive tool for in vivo histopathologic studies of MGs in PBS patients. The excessive constriction of lid muscles closely related to MG morphological alterations of PBS, which offered a new research approach to interpret the interactional mechanism between dry eye and PBS.

Purpose: The somatosensory-evoked blink reflex (SBR) is one of the release phenomena of blink reflex, possibly resulting from increased excitability of brainstem reticular formation., Methods: The authors investigated trigeminal blink responses and SBR in 26 patients with postparalytic facial syndrome (PFS) with synkinesia, 18 patients with essential blepharospasm, and 36 healthy volunteers (control participants)., Results: Trigeminal blink reflex responses were elicited in all participants, whereas SBRs were elicited in 44.4% of control participants, 38.9% of patients with essential blepharospasm, and 65.4% of patients with PFS. The mean R2 amplitude and duration and the mean amplitude and duration of SBR were highest in patients with essential blepharospasm. The mean latency of SBR was shorter on the symptomatic side of patients with PFS when compared with the asymptomatic side. The mean R2 duration on the symptomatic side of the patients with PFS was longer than the control participants., Conclusions: These results showed that somatosensory stimulation could be used as an alternative method to demonstrate increased excitability in facial motor neuron in patients with PFS and essential blepharospasm. Disease states relating to different peripheral and/or suprasegmental structures could also influence blink reflex and change its basal excitability and manner in which the reflex responds to modulatory factors.

Objective: To design and validate a clinical diagnostic guideline for aiding physicians in confirming or refuting suspected blepharospasm., Methods: The guideline was developed and validated in a 3-step procedure: 1) identification of clinical items related to the phenomenology of blepharospasm, 2) assessment of the relevance of each item to the diagnosis of blepharospasm, and 3) evaluation of the reliability and diagnostic sensitivity/specificity of the selected clinical items., Results: Of 19 clinical items initially identified, 7 were admitted by content validity analysis to further assessment. Both neurologists and ophthalmologists achieved satisfactory interobserver agreement for all 7 items, including "involuntary eyelid narrowing/closure due to orbicularis oculi spasms," "bilateral spasms," "synchronous spasms," "stereotyped spasm pattern," "sensory trick," "inability to voluntarily suppress the spasms," and "blink count at rest." Each selected item yielded unsatisfactory accuracy in discriminating patients with blepharospasm from healthy subjects and patients with other eyelid disturbances. Combining the selected items, however, improved diagnostic sensitivity/specificity. The best combination, yielding 93% sensitivity and 90% specificity, was an algorithm starting with the item "stereotyped, bilateral, and synchronous orbicularis oculi spasms inducing eyelid narrowing/closure" and followed by recognition of "sensory trick" or, alternatively, "increased blinking.", Conclusion: This study provides an accurate and valid clinical guideline for diagnosing blepharospasm. Use of this guideline would make it easier for providers to recognize dystonia in clinical and research settings.

Objective: The aim of this study was to investigate whether increased blink rate (BR) is part of the clinical spectrum of primary blepharospasm (BSP)., Methods: We enrolled 40 patients (16 patients with an increased BR but without typical orbicularis oculi [OO] spasms, and 24 patients with typical involuntary OO spasms) and 18 healthy subjects. The BR, blink reflex recovery cycle, and somatosensory temporal discrimination threshold (STDT) were tested in patients and controls., Results: Patients who had typical OO spasms had an altered R2 recovery cycle whereas those who had an increased BR alone had a normal blink reflex recovery cycle. STDT values were higher in patients than in healthy subjects and no difference was found in the STDT abnormalities in the 2 groups of patients., Conclusions: Our study shows that, despite the similar STDT abnormalities, the different changes in the R2 recovery cycle in patients with BSP and those with increased BR alone suggest that these disorders arise from different pathologic mechanisms.

Background: Psychogenic blepharospasm is difficult to distinguish clinically from benign essential blepharospasm (BEB). The blink reflex recovery cycle measures the excitability of human brainstem interneurons and is abnormal in BEB. We wished to study the blink reflex recovery cycle in patients with atypical (presumed psychogenic) blepharospasm (AB)., Methods: This was a prospective data collection study investigating the R2 blink reflex recovery cycle at interstimulus intervals (ISI) of 200, 300, 500, 1,000, and 3,000 msec in 10 patients with BEB, 9 patients with AB, and 9 healthy controls. All patients had spasm of the orbicularis oculi muscles. To compare individual patients, an R2 recovery index was calculated as average of the recovery values at ISIs of 200, 300, and 500 msec, with the upper limit of normal defined as mean (control group) + 2 SD., Results: The R2 recovery cycle was significantly disinhibited in patients with BEB, whereas patients with AB did not differ from controls on a group level. The upper limit of normal for the R2 recovery index was 61%. The R2 index was abnormal in 9 out of 10 patients with BEB and in none of the patients with AB., Conclusions: A normal blink reflex recovery cycle indicates normal brainstem interneuron excitability. Assessment of the R2 recovery cycle may provide a useful diagnostic tool to distinguish patients with psychogenic blepharospasm from BEB and is worthy of further study.

Background: Traditionally, benign essential blepharospasm (BEB) is considered a disorder caused by basal ganglia dysfunction. Electrophysiologic and brain imaging studies suggest pathologic changes in excitability in the primary motor cortex (MC), anterior cingulate (AC), and secondary motor areas, such as premotor (PMC) and supplementary motor cortices (SMA)., Methods: In this pilot study of 7 patients with BEB, we experimentally reduced cortical excitability of 4 areas: MC (first dorsal interosseus area), PMC, SMA, and AC, each with 3 noninvasive techniques: low-frequency repetitive transcranial magnetic stimulation (lfrTMS), continuous theta burst stimulation (cTBS), and cathodal transcranial direct current stimulation (tDCS). Primary outcome was the clinical effects on blepharospasm (blink rate observation by an investigator blinded to the intervention and subjective rating by the patient); secondary outcome was the blink reflex recovery curve (BRR)., Results: lfrTMS resulted in a significant improvement over all 4 brain areas for physician rating, patient rating, and BRR, whereas cTBS and tDCS showed only trends for improvement in physician rating, but no improvements for patient rating and BRR. lfrTMS had a significantly higher effect over AC than MC for physician rating, but no differences were seen for other pairwise comparisons of stimulated brain areas., Conclusions: Electrophysiologic and clinical improvements by functional inhibition of the medial frontal areas using low-frequency repetitive transcranial magnetic stimulation suggests that hypersensitivity of the anterior cingulate is directly or indirectly involved in the pathophysiology of benign essential blepharospasm. Inhibition of these areas using low-frequency repetitive transcranial magnetic stimulation could provide a therapeutic tool and is worthy of a larger study.

Background: Persistent, frequent, nonepileptic paroxysmal eyelid movements were observed in 19 children and adults with well-controlled generalized epilepsy., Methods: Patients were identified from five epilepsy centers., Results: Seventeen patients were female and two male. All had generalized photosensitive epilepsy requiring antiepileptic drugs (AEDs). In two children, paroxysmal eyelid movements began 2 to 4 years before their epilepsy was noted; in the remainder, it was noted when epilepsy was first diagnosed. Age at last follow-up was 8 to 38 years (average 21 years) with average follow-up of 9 years. All patients showed photosensitive generalized spike-wave discharges on EEG. Paroxysmal eyelid movements were a source of diagnostic confusion, but direct examination and video during EEG recording distinguished the attacks from absence seizures. In all cases, the epilepsy is completely or nearly completely controlled with AEDs, but the paroxysmal eyelid movements have not resolved with age. In 12 cases, there was a family history of the eyelid disorder without epilepsy. Videos of patients and an affected parent are available on the Neurology Web site., Conclusion: There is an association between paroxysmal eyelid movements and photosensitive generalized epilepsy, creating diagnostic confusion.

Objective: To investigate brain areas involved in the initiation and execution of eyelid spasm in patients with benign essential blepharospasm., Methods: The authors used fMRI and correlated the blood oxygenation level-dependent (BOLD) signal with epochs of frequent eyelid spasm in six patients and with epochs of voluntary eye blinks in four healthy subjects., Results: Spasm epochs were accompanied by activation in a subregion of the putamen in all patients, whereas voluntary blinking in healthy subjects was not. Other areas of activation common to patients and healthy subjects included frontal and parietal operculum, supplementary motor area, primary sensorimotor cortex, various visual areas, and the cerebellum., Conclusions: The striatum may be involved in the initiation or execution of eyelid spasm. Future studies, possibly including electromyography (EMG) during fMRI, are needed to detect the sequence and role of other concomitantly activated areas.

Benign essential blepharospasm is a common focal dystonia characterized by involuntary eyelid closure. Its etiology, supported by animal models, appears to be multifactorial, representing the influence of a genetic background and an environmental trigger. The genetic background could be responsible for the reduced brain inhibition, identified with physiologic studies that would set up a permissive condition for increased brain plasticity. Reduced D2 receptors identified with PET might be an indicator of this reduced inhibition. The trigger could be repetitive use or local ocular disease. Although symptomatic therapy is available, better approaches are needed and will likely become available as the genetics and pathophysiology become well understood.

Background: Apraxia of lid opening (ALO) is a nonparalytic inability to open the eyes at will in the absence of visible contraction of the orbicularis oculi (OOc) muscle., Objective: To test the hypothesis that sustained activity of the OOc is a major factor in the difficulty in opening the eyes in this condition., Methods: Lid movement detected in an electromagnetic field and electromyogram activity of the septal and pretarsal portions of the OOc were recorded in 12 healthy control subjects and 12 patients with a clinical diagnosis of ALO. The latencies to onset and to complete eye opening and the time during which eye opening was sustained were measured and analyzed in relation to OOc activity., Results: The lid opening latencies and the lid movement duration were significantly increased in patients compared with control values. An abnormal persistence of OOc activity was present in 10 of the 11 patients with a delay in complete lid opening. The complete lid opening delay showed a strong positive correlation with the time it took to inhibit the OOc activity. This relationship fit the same linear regression in both groups., Conclusions: Patients with ALO show significant delays in eye opening. An abnormal persistence of OOc activity, detectable electromyographically but not clinically, could be the main factor contributing to the delay in lid opening in these patients.

Objectives: To analyze the clinical data and test results collected in a group of patients exhibiting eyelid-closure blepharoclonus (BLC) on clinical neurologic examination., Materials and Methods: Thirty-five patients were referred for neurologic evaluation for reasons other than BLC. Clinical electrophysiologic evaluations, including cranial nerve testing and electromyograms, were done according to standards. All patients had neuroimaging studies, including brain magnetic resonance imaging and head computerized tomography, or both, and many had electroencephalograms. Additional tests were done based on the patient's symptoms or reasons for referral., Results: Eight patients had reflex BLC. Two cases were precipitated by vertical gaze; one of these patients had hereditary palmoplantar keratoderma and cataplexy, and the other patient had Ehlers-Danlos syndrome and familial BLC. Other precipitants included speech in four cases, postural changes in two cases, and light stimulation in one case. Two patients had generalized myoclonus independent of their BLC, two patients had a history of sleep myoclonus, and several patients had BLC-associated facial myoclonus. One patient had BLC-associated myoclonus of the right shoulder. Synkinetic cranial movements were detected in 11 patients (four oculofacial, three oculopterygoid, one oculolingual, two dual cases, and one case of imitation synkinesis.) Three patients had familial BLC, seven patients had congenital developmental disorders, six patients had synkinetic tremors, and six patients had restless feet. Some indication of peripheral neuropathy was evident in eight patients., Conclusions: Eyelid-closure BLC is an underrecognized, sporadic or familial, mostly benign, chronic eyelid-movement disorder that may be associated with tremors, myoclonus, cranial synkinesis, and restless feet. Reflex mechanisms may be identified in some patients. Gaze-induced BLC seems to have the greatest clinical relevance. In the current series, there were no examples of posttraumatic BLC, multiple sclerosis, hydrocephalus, or blepharospasm conditions previously reported to be associated with BLC. No electroencephalographic abnormalities were recorded during BLC, ruling out eyelid-closure epilepsy.

Single-volume proton magnetic resonance spectroscopy, localized to basal ganglia, was carried out in 10 patients with primary blepharospasm (PB) to assess the levels of N-acetyl aspartate (NAA), creatine-phosphocreatine, and choline-containing compounds. NAA was reduced significantly in patients compared with control subjects. This result suggests a striatal neuronal loss in PB.

The variable clinical features and the relatively good response of blepharospasm to botulinum-toxin type A are now well established. The etiology and pathophysiology of blepharospasm and related facial movement disorders are still poorly understood. Genetic and histopathologic studies over the last year have contributed to our understanding of this disease. The most significant progress has been made in the electromyographic studies of the the levator palpebrae and orbicularis oculi muscles. Subclassification based on the electromyographic abnormalities of these two muscles have begun to improve our understanding of the variable responses to botulinum-toxin type A. Further electromyographic studies may help identify the best sites of injection for optimal response and differentiate patients requiring limited or complete myectomy. The development of the limited myectomy has provided excellent functional and cosmetic results with quick recovery times in selected patients.

Orofacial dyskinesia (Meige's syndrome) is a rare clinical entity. This disorder was first described in 1910 by Henry Meige as "spasm facial median," a disabling spasm of the facial musculature which had frequently been misdiagnosed and inappropriately treated. This report concerns a patient who presented with tonic hemifacial spasm twenty-two years after contracting Bell's palsy. The condition was initially thought to be secondary to faulty regeneration of the facial nerve until Meige's syndrome became fully manifested over a period of two years. The diagnostic characteristics as well as the forms of treatment for this unusual disorder are presented.