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The Neurologic Disorders in Film
Published in Eelco F. M. Wijdicks, Neurocinema—The Sequel, 2022
The film shows the enormous impact a tiny change in motor function can bring about—common knowledge for all neurologists first diagnosing a neurodegenerative disease. When professional musicians develop Parkinson’s disease, it is often the end of a career and, sadly, early in the process. Inability to make rapid fingering shifts results in loss of tempo; additionally, long passages of music requiring close harmonization with other musicians are quite challenging. While many other patients with Parkinson’s disease are successfully treated and lead valuable lives, the demands on a musician, playing complex musical parts and memorizing transitions, may become too challenging both physically as well as mentally if cognition becomes impaired in later stages. In these situations, successfully treating the movement disorder in tremor-dominant asymmetric Parkinson’s disease may not resolve the issue completely. It is also likely much different than the treatment of focal dystonia—a disorder commonly found in musicians.
Movement disorders
Published in Henry J. Woodford, Essential Geriatrics, 2022
Around 25% of people with parkinsonism will have a small response to levodopa. This medication should not be continued in the absence of a continued benefit. Benzodiazepines may help reduce myoclonus but with a risk of adverse effects. Botulinum toxin injections may be useful for focal dystonia.
Hyperkinetic Movement Disorders
Published in Philip B. Gorelick, Fernando D. Testai, Graeme J. Hankey, Joanna M. Wardlaw, Hankey's Clinical Neurology, 2020
Morales-Briceno Hugo, Victor S.C. Fung, Annu Aggarwal, Philip Thompson
Distribution: Focal: dystonia involves a single body part.Cranial (blepharospasm, oromandibular, laryngeal [spasmodic dysphonia], cervical [torticollis]).Upper limb, hand: writer's cramp, musician's, other occupational dystonias.Axial (back, trunk): causing scoliosis, lordosis, kyphosis.Segmental: dystonia involves two contiguous regions of the body.Multifocal: dystonia involves two noncontiguous regions of the body.Generalized: dystonia involves leg, trunk, and any other body region.Hemidystonia: dystonia restricted to one side of the body.
An overview of the pharmacotherapeutics for dystonia: advances over the past decade
Published in Expert Opinion on Pharmacotherapy, 2022
O. Abu-hadid, J. Jimenez-Shahed
Dystonia, as defined in the Movement Disorder Society consensus of 2013, is ‘a movement disorder characterized by sustained or intermittent muscle contractions causing abnormal, often repetitive, movements, postures, or both. Dystonic movements are typically patterned, twisting, and may be tremulous. Dystonia is often initiated or worsened by voluntary action and associated with overflow muscle activation.’ [1]. To help aid in organizing a framework for classifying dystonia, it is described based on two axes: (I) clinical characteristics, and (II) etiology [1]. One of the main clinical characteristics that dictate treatment is the distribution of dystonia, which can be generalized, focal, or segmental. Generalized dystonia involves multiple non-contiguous body parts and can result from structural and genetic etiologies or be idiopathic. Focal dystonia involves a specific body part and can be triggered by performing a specific task, referred to as task-specific dystonia. Examples of focal dystonia are cervical dystonia (CD), spasmodic dysphonia, and blepharospasm (BS), whereas task-specific dystonias include writer’s cramp and musician’s dystonia. Finally, segmental dystonia occurs in multiple contiguous segments of the body such as Meige syndrome. Dystonia can also be clinically characterized as isolated, where the only movement disorder is dystonia with or without tremor, or combined, when the dystonia is associated with other movement disorders. Based on etiology, dystonia can be considered inherited, acquired, or idiopathic.
New approaches to discovering drugs that treat dystonia
Published in Expert Opinion on Drug Discovery, 2019
Sarah Pirio Richardson, H. A. Jinnah
Dystonia is a chronic, typically, lifelong condition causing abnormal muscle contractions that result in twisting postures and abnormal movements [1]. Dystonia is characterized as isolated when it is the sole symptom and combined when dystonia is present but there are other neurologic signs and symptoms present [2]. The current consensus classification also includes Axis I, which focuses on clinical features (age at onset, body distribution, temporal pattern and associated features) as well as an Axis II, which covers etiology [3]. In adults, the most frequent presentation of dystonia is focal, with the most common sites being the neck (cervical dystonia or CD) and the eye region (blepharospasm) [4] (Figure 1). Dystonia can also begin in childhood and is typically associated with a more generalized distribution of body parts affected as well as associated with inherited syndromes – both with genes known and genes yet to be discovered [1]. Dystonia is associated with poor quality of life, pain and reduced ability to work as well as high rates of anxiety and depression [5,6]. Currently, first-line treatment for focal dystonia is botulinum toxin (BoNT) injections [7,8]. Oral medications are also used as ‘off-label’ therapies despite limited clinical trial support [9].
Blepharospasm in Japan: A Clinical Observational Study From a Large Referral Hospital in Tokyo
Published in Neuro-Ophthalmology, 2018
M. Wakakura, A. Yamagami, M. Iwasa
The most common symptoms reported by patients were difficulty opening the eyes due to persistent photophobia and/or ocular pain, suggesting that blepharospasm is associated with sensory-motor disintegration.15–17 Although it is difficult to conclude whether the motor system or sensory system is primarily involved in the mechanism of blepharospasm, our other study18 based on patients’ statement indicates that a number of patients with drug-induced blepharospasm primarily or predominantly realise sensory disturbance such as photophobia and ocular or periocular pain/irritation. This results are compatible to the hypothesis in focal dystonia that abnormal processing of the somatosensory input in the central nervous system induces insufficient sensorimotor integration.19 Several resting-state functional MRI studies support the notion that primary blepharospasm is associated with decreased connectivity within the sensory-motor network.15,17,20