[Paroxysmal dystonia and multiple sclerosis].
H El Otmani1, Y Benmansour1, A Araqi-Houssaini1
1Service de neurologie, CHU Ibn Rochd, 1, rue des Hôpitaux, 20360 Casablanca, Maroc.
Revue Neurologique
|November 26, 2013
Summary
Paroxysmal dystonia is a rare but under-recognized movement disorder in multiple sclerosis. Steroid treatment often resolves these symptoms, suggesting inflammation plays a role.
Area of Science:
- Neurology
- Neuroscience
- Movement Disorders
Background:
- Multiple sclerosis (MS) rarely presents with movement disorders beyond tremor.
- Paroxysmal dystonia, or tonic spasm, is an uncommon manifestation of MS.
Observation:
- Two cases of MS with paroxysmal dystonia are presented.
- Patients experienced brief, recurrent episodes of hemibody dystonia.
- Brain MRI revealed demyelination, particularly in the posterior limb of the internal capsule.
Findings:
- Dystonia in MS may stem from ephaptic transmission in demyelinated neurons.
- Impairment of the posterior limb of the internal capsule is a common site.
- Corticosteroid treatment led to rapid resolution of dystonic episodes in both cases.
Implications:
- Paroxysmal dystonia is an under-recognized symptom of MS.
- Inflammation likely contributes to MS-related dystonia, as indicated by steroid responsiveness.
- Understanding the pathophysiology of these episodes can improve MS patient care.
Related Concept Videos
01:12Alterations in Muscle Tone ll
31
Alterations in muscle tone are common manifestations of neurological disorders and reflect dysfunction within different nervous system regions. Spasticity, paratonia, and dystonia represent distinct forms of hypertonia, each with unique mechanisms, clinical features, and diagnostic importance.CharacteristicsSpasticity happens from upper motor neuron lesions and is characterized by velocity-dependent resistance to passive movement. Clinical features include:Exaggerated deep tendon reflexesClonus...
31
01:19Multiple Sclerosis l: Introduction
31
Multiple sclerosis is a chronic autoimmune disease of the central nervous system (CNS) that affects the brain, spinal cord, and optic nerves. It is an inflammatory demyelinating disorder and a leading cause of neurological disability in young adults.EpidemiologyMS commonly begins between 20 and 40 years of age and is twice as common in women. Its exact cause remains unclear, but genetic susceptibility contributes, with higher risk in first-degree relatives and identical twins. A greater...
31
01:11Alterations in Muscle Tone lll
36
Rigidity and myotonia are distinct abnormalities of muscle tone that affect resistance and relaxation during movement. Although both involve altered muscle contraction, they arise from different neurological and muscular mechanisms.CharacteristicsRigidity is characterized by uniform resistance to passive movement across the entire range, independent of speed, affecting flexors and extensors equally. It may appear as lead-pipe rigidity (smooth, constant resistance) or cogwheel rigidity...
36
01:26Directly Acting Muscle Relaxants: Dantrolene and Botulinum Toxin
2.2K
Directly acting muscle relaxants like dantrolene and botulinum toxin (BoNT) have distinct mechanisms and applications. Dantrolene, a hydantoin derivative, acts on the ryanodine receptor (RYR1) in skeletal muscle cells. RYR1 are calcium channels present at the sarcoplasmic reticulum membrane. In response to excitation, they release calcium ions from the sarcoplasmic reticulum to the cytosol. Calcium promotes actin-myosin-mediated contraction of muscles.
The binding of dantrolene to the RYR1...
The binding of dantrolene to the RYR1...
2.2K
01:15Parkinson's Disease: Overview
2.2K
Neurodegenerative disorders are progressive diseases that cause irreversible damage and loss to neurons in specific brain areas. Examples of these disorders include Parkinson's disease, Alzheimer's disease, Multiple Sclerosis (MS), and Amyotrophic Lateral Sclerosis (ALS). These disorders share characteristics such as proteinopathies, selective neuronal vulnerability, and a complex interplay between genetic and environmental factors. The primary therapeutic goal for these conditions is...
2.2K
01:28Classification of Skeletal Muscle Relaxants
3.6K
Skeletal muscle relaxants are a group of drugs that can reduce muscle stiffness and induce temporary paralysis to relieve pain. These agents can act centrally to reduce muscle tone or spasms in painful conditions such as multiple sclerosis (MS), amyotrophic lateral sclerosis (ALS), or spinal injuries; they are called antispasmodics or spasmolytics.
Peripherally acting skeletal muscle relaxants interfere with the neurotransmission at the neuromuscular end plate to induce paralysis during...
Peripherally acting skeletal muscle relaxants interfere with the neurotransmission at the neuromuscular end plate to induce paralysis during...
3.6K