1,721,242 research outputs found
Current and investigated alternatives to botulinum toxin for managing blepharospasm
No full textIntroduction: Benign essential blepharospasm (BEB) is an adult-onset focal dystonia characterized by involuntary spasms of the orbicularis oculi (OO) muscle. BEB typically develops in the fifth to sixth decade, has a variable female preponderance, and a tendency to spread to adjacent body regions. Idiopathic BEB is thought to be a multifactorial disorder resulting from the contribution of both environmental and genetic factors. To date, BEB treat- ment is largely based on botulinum A toxin (BTX).
Areas covered: This article summarizes current knowledge on the therapeutic approaches that can be proposed to BEB patients who are truly unresponsive to BTX. Current and investigated alternatives to BTX can be included in the following categories: i) alternative therapeutic weakening of the OO muscle; ii) treatments of ophtalmological complaints; and iii) therapeutic interven- tions on central nervous system mechanisms underlying BEB.
Expert opinion: Therapeutic strategies to manage BEB of patients who are truly unresponsive to BTX are very limited. Currently available treatments include myectomy, oral medication and deep brain stimulation. Experimental therapeutics include topical acetyl hexapeptide-8, a promising new drug for extending the duration of action of BTX, and transcranial magnetic stimula- tion. At present, the choice of the best treatment strategy, including medical, surgical and non-invasive treatments remains largely empirical and depend- ing on existing reports of toxicity rather than efficacy
Adult-onset focal dystonias: To lump or split
No full text: The adult-onset focal dystonias are a group of clinically heterogeneous disorders that affect different regions of the body. Although they affect different regions with different clinical manifestations, there is evidence that etiopathogenesis is shared at the anatomical, physiological, and genetic levels. However, there is also evidence that etiopathogenesis varies. This chapter summarizes the evidence for lumping or splitting these apparently different clinical phenotypes. It also includes some potential explanations to explain the similarities and differences
The epidemiology of primary dystonia: current evidence and perspectives
No full textThe number of existing cases of primary dystonia in the population is not precisely known, but the condition is probably much more frequent than reported. By minimum prevalence estimates, primary dystonia should be considered the third most frequent movement disorders after essential tremor and Parkinson's disease. The most likely etiologic scenario suggested by epidemiological data is that primary dystonias are products of a genetic background and an environmental insult. Current information on the causation of primary dystonia, late-onset dystonia in particular, is often unreliable because of methodological problems inherent to case-control investigation and to the heterogeneity of dystonia. To expand our knowledge on dystonia, we need to design population-based studies, to perform association studies taking into account the heterogeneity of dystonia, and to collect exhaustive clinical data in a standardized and reliable way
The natural history of treated PD in an incident, community-based cohort: does the future begin?
No full textEpidemiology of primary and secondary dystonia
No full textDuring the last decade, dystonia has been increasingly recognized. However, the body of work concerning the epidemiology of this condition is not extensive. In the past, barriers to epidemiologic study have included perceptions that dystonia is rare and associated with relatively low morbidity. In addition, the absence of validated diagnostic markers for dystonia has limited the careful classification of subjects with this hyperkinetic movement disorder. A further difficulty has arisen because dystonia is a heterogeneous condition that is classified according to the age of onset (early-and late-onset dystonia), body distribution (focal, segmental, multifocal, generalized, and hemidystonia), and etiology (primary and secondary dystonia) (1). Lastly, the evolution of numerous molecular and clinical subtyping systems has lead to some variation in neurologist attitudes in the recognition and treatment of dystonia (2)
Author Response: Teaching Video NeuroImage: Bilateral Hemifacial Spasm in Giant Cell Arteritis
No full textWe appreciate the comments on our case.(1) Although we agree that a bilateral hemifacial spasm is typically characterized by asynchronous muscle contractions on the left and right sides, contractions are also often asymmetric in severity, as observed in our case. The cheek movements on the right side of the face were likely the consequence of a stronger contraction of the palpebral part of the orbicularis oculi muscle, rather than the result of cheek muscle contractions as the orbicularis oris did not move during spasms. While a central origin cannot entirely be ruled out, the exclusive involvement of the upper facial muscles in our patient seems more consistent with a peripheral irritation of the superior branch of the facial nerves
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