Hyperkinetic Movement Disorders
Philip B. Gorelick, Fernando D. Testai, Graeme J. Hankey, Joanna M. Wardlaw in Hankey's Clinical Neurology, 2020
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.
Neurology
Paul Bentley, Ben Lovell in Memorizing Medicine, 2019
Types: Idiopathic focal dystonia – this is the commonest form of dystonia: Spasmodic torticollisHemifacial or blepharospasmOromandibular dystonia or spasmodic dysphoniaWriter’s, musician’s or sportsman’s crampSecondary focal or generalized
Neurology
Shibley Rahman, Avinash Sharma in A Complete MRCP(UK) Parts 1 and 2 Written Examination Revision Guide, 2018
Other drugs used in the management of multiple sclerosis include: Glatiramer acetate: immunomodulating drug.Natalizumab: a recombinant monoclonal antibody that antagonises alpha 4 betal integrin found on the surface of leucocytes, thus inhibiting migration of leucocytes across the endothelium into parenchymal tissue.Symptom control.> Spasticity: baclofen and gabapentin are first-line. Other options include diazepam, dantrolene and tizanidine.Botulinum toxin is the treatment of choice for focal dystonia (such as torticollis and hemifacial spasm) and focal dystonia. The primary action of the toxin is to block acetylcholine release at the neuromuscular junction and so to produce muscle weakness. Occasionally systemic absorption of the toxin can affect distal muscles causing symptoms such as diplopia and dysphagia.
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].
Medical treatment of blepharospasm
Published in Expert Review of Ophthalmology, 2018
Dhanya Vijayakumar, Joseph Jankovic
The prevalence of idiopathic blepharospasm is estimated to range between 20 and 133 cases per million, depending on the geographic area [12]. The prevalence is about three times higher in women compared to men [13]. Among patients with adult onset focal dystonia, the risk of spread to other adjacent muscle groups is highest for blepharospasm and typically occurs within the first five years of symptom onset [14]. Symptoms often spread to other cranial and cervical muscles (cranial–cervical dystonia).
Related Knowledge Centers
- Dystonia
- Writer'S Cramp
- Yips
- Neuron
- Motor Cortex
- Afferent Nerve Fiber
- Transcranial Magnetic Stimulation
- Interneuron
- Blepharospasm
- Oromandibular Dystonia