Nocturnal Paroxysmal Dystonia
Dystonia
Entopeduncular Nucleus
Dystonic Disorders
Dystonia Musculorum Deformans
Botulinum-A toxin in the treatment of painful post-stroke nocturnal paroxysmal dystonia triggered by periodic limb movements of sleep: case report. (1/1)
INTRODUCTION: Sleep disorders presenting involuntary movements may be very annoying to patients, apart from their negative influence on sleep. OBJECTIVE: To report the use of botulinum type-A toxin (BoNT-A) to manage the case of a patient whose sleep was severely disrupted by episodes of dystonic posturing of the right lower limb triggered by periodic limb movements of sleep (PLMS). METHOD: A 79-year-old woman with mild post-stroke right hemiparesis presented with recurrent painful episodes of dystonia of the right lower limb, which disrupted her sleep. The dystonic episodes could also be voluntarily triggered by extension of the right hallux. Polysomnography confirmed that the dystonic episodes were triggered by PLMS. Twenty units of BoNT-A (20U/500U vial) were injected into her right extensor hallucis longus. RESULTS: Shortly after BoNT-A was injected, the dystonic symptoms abated, and the patient achieved better sleep efficiency. CONCLUSION: The PLMS-related involuntary extension of the hallux was probably triggering the nocturnal post-stroke lower limb dystonic paroxysms. BoNT-A injection into the right extensor hallucis longus was effective in managing this condition and thus resolved the associated disruption of sleep. (+info)Nocturnal Paroxysmal Dystonia is actually an outdated term that was previously used to describe a rare neurological disorder characterized by recurrent episodes of dystonia (sustained muscle contractions that cause twisting and repetitive movements or abnormal postures) occurring predominantly during sleep. These episodes are often associated with other symptoms such as autonomic dysfunction (e.g., sweating, tachycardia), vegetative symptoms (e.g., flushing, pallor), and sometimes pain.
Currently, this disorder is more commonly referred to as "Paroxysmal Dyskinesias" or "Nocturnal Paroxysmal Kinesigenic Dyskinesia" if the episodes are triggered by sudden movements or "Nocturnal Paroxysmal Non-Kinesigenic Dyskinesia" if they occur spontaneously, without any apparent trigger. These disorders can be caused by various genetic mutations and may respond to different treatment approaches.
Dystonia is a neurological movement disorder characterized by involuntary muscle contractions, leading to repetitive or twisting movements. These movements can be painful and may affect one part of the body (focal dystonia) or multiple parts (generalized dystonia). The exact cause of dystonia varies, with some cases being inherited and others resulting from damage to the brain. Treatment options include medications, botulinum toxin injections, and deep brain stimulation surgery.
The entopeduncular nucleus (EP) is a small, compact collection of neurons located in the ventral region of the diencephalon, specifically within the posterior intralaminar complex of the thalamus. It is present in various mammals, including humans. The EP nucleus receives inputs from the basal ganglia and projects to the brainstem and other thalamic nuclei.
In rodents, the entopeduncular nucleus is also known as the globus pallidus internus (GPi). However, in primates, including humans, the GPi is a separate structure located near the EP nucleus. Both structures are part of the basal ganglia circuitry and play essential roles in motor control, procedural learning, and habit formation.
The entopeduncular nucleus has been implicated in several neurological conditions, such as Parkinson's disease, Huntington's disease, and dystonia. Deep brain stimulation (DBS) of the EP nucleus or GPi is an effective treatment for reducing motor symptoms associated with these disorders.
Dystonic disorders are a group of neurological conditions characterized by sustained or intermittent muscle contractions that result in involuntary, repetitive, and often twisting movements and abnormal postures. These movements can affect any part of the body, including the face, neck, limbs, and trunk. Dystonic disorders can be primary, meaning they are caused by genetic mutations or idiopathic causes, or secondary, resulting from brain injury, infection, or other underlying medical conditions.
The most common form of dystonia is cervical dystonia (spasmodic torticollis), which affects the muscles of the neck and results in abnormal head positioning. Other forms of dystonia include blepharospasm (involuntary eyelid spasms), oromandibular dystonia (affecting the muscles of the jaw, face, and tongue), and generalized dystonia (affecting multiple parts of the body).
Dystonic disorders can significantly impact a person's quality of life, causing pain, discomfort, and social isolation. Treatment options include oral medications, botulinum toxin injections, and deep brain stimulation surgery in severe cases.
'Dystonia Musculorum Deformans' is a medical term that refers to a rare inherited neurological disorder, which is now more commonly known as "Generalized Dystonia." This condition is characterized by sustained muscle contractions, leading to twisting and repetitive movements or abnormal postures.
The onset of symptoms typically occurs during childhood or adolescence, and they can progress over time, affecting various parts of the body. The exact cause of Generalized Dystonia is not fully understood, but it is believed to involve genetic mutations that affect the functioning of certain proteins in the brain. Treatment options may include medications, botulinum toxin injections, or even deep brain stimulation surgery in severe cases.