Opinion Article - Neurophysiology Research (2023) Volume 5, Issue 2
Myelin Disorders: Understanding the Pathology and Therapeutic StrategiesJonas Mary*
Department of Anatomy and Cell Biology, University of Wuerzburg, Koellikerstr, Wuerzburg, Germany
- *Corresponding Author:
- Jonas Mary
Department of Anatomy and Cell Biology
University of Wuerzburg
Koellikerstr, Wuerzburg, Germany
Received: 31-Mar-2023, Manuscript No. AANR-23-96097; Editor assigned: 03-Apr-2023, PreQC No. AANR-23-96097(PQ); Reviewed: 17-Apr-2023, QC No. AANR-23-96097; Revised: 21-Apr-2023, Manuscript No. AANR-23-96097(R); Published: 26-Apr-2023, DOI: 10.35841/aanr-5.2.144
Citation: Mary J. Myelin disorders: understanding the pathology and therapeutic strategies. Neurophysiol Res. 2023;5(2):144
Myelin disorders are a group of neurological conditions that involve damage or dysfunction of myelin, the protective sheath that wraps around nerve fibres in the central nervous system (CNS) and peripheral nervous system (PNS). These disorders can have a significant impact on the normal functioning of the nervous system, leading to a wide range of symptoms and disabilities. In this article, we will explore the pathology of myelin disorders, including their causes, types, clinical manifestations and current therapeutic strategies.
Myelin disorders, Clinical manifestations, Nerve fibres, Peripheral nervous system, Central nervous system.
The pathology of myelin disorders involves disruptions in the structure and function of myelin, which is essential for efficient nerve conduction. Myelin is composed of multiple layers of lipid-rich membranes that are produced by specialized cells called oligodendrocytes in the CNS and Schwann cells in the PNS. Myelin acts as an insulating layer around nerve fibers, allowing for rapid and efficient transmission of nerve signals along the axons .
Various factors can lead to myelin disorders, including genetic mutations, autoimmune responses, infections, metabolic abnormalities, and environmental factors. Genetic mutations are known to be a major cause of myelin disorders, with several genes identified that are associated with different types of myelin diseases, such as multiple sclerosis (MS), leukodystrophies, and Charcot-Marie-Tooth disease. Autoimmune responses, where the immune system mistakenly attacks the myelin as if it were a foreign invader, can result in demyelinating diseases such as MS, neuromyelitis optica, and acute disseminated encephalomyelitis. Infections, such as viral or bacterial infections, can trigger an immune response that targets myelin, leading to myelin damage. Metabolic abnormalities, such as those seen in metabolic leukodystrophies, can disrupt the production or maintenance of myelin, resulting in myelin disorders. Environmental factors, such as exposure to toxins or radiation, can also contribute to myelin damage .
Myelin disorders can be categorized into two main types: demyelinating disorders and demyelinating disorders. Demyelinating disorders involve the destruction or loss of myelin, which disrupts nerve conduction and can lead to a wide range of neurological symptoms. Examples of demyelinating disorders include MS, neuromyelitis optica and Guillain- Barre syndrome. Demyelinating disorders, on the other hand, involve abnormal or defective myelin formation, resulting in structurally abnormal myelin sheaths. Dysmyelinating disorders are typically caused by genetic mutations and can result in a wide range of clinical manifestations, depending on the specific gene affected. Examples of dysmyelinating disorders include leukodystrophies, Pelizaeus-Merzbacher disease and Charcot-Marie-Tooth disease [3, 4].
The clinical manifestations of myelin disorders can vary widely depending on the location and extent of myelin damage. Common symptoms of myelin disorders include weakness, numbness or tingling in the limbs, difficulty with coordination, impaired vision, speech difficulties, cognitive impairment, and fatigue. These symptoms may occur acutely, in the case of demyelinating disorders with acute inflammatory attacks, or may progress gradually over time in chronic demyelinating disorders. The severity and progression of symptoms can vary greatly among individuals and among different types of myelin disorders .
Diagnosis of myelin disorders typically involves a thorough medical history, physical examination, and various imaging studies, such as magnetic resonance imaging (MRI) and nerve conduction studies. Laboratory tests, including blood tests and cerebrospinal fluid analysis, may also be performed to rule out other possible causes of symptoms and to aid in the diagnosis. Genetic testing may be indicated in suspected dysmyelinating disorders with a suspected genetic basis.
- AYDINLI Fİ, Çelik E, Vatandaşlar BK, et al. Myelin disorders and stem cells: as therapies and models. Turk J Biol. 2016;40(5):1068-80.
- Boda E. Myelin and oligodendrocyte lineage cell dysfunctions: New players in the etiology and treatment of depression and stress‐related disorders. Eur J Neurosci.2021;53(1):281-97.
- Duncan ID, Radcliff AB. Inherited and acquired disorders of myelin: the underlying myelin pathology. Exp Neurol. 2016;283:452-75.
- Jensen BK, Monnerie H, Mannell MV, et al. Altered oligodendrocyte maturation and myelin maintenance: the role of antiretrovirals in HIV-associated neurocognitive disorders. J Neuropathol Exp Neurol. 2015;74(11):1093-118.
- Volpi VG, Touvier T, D'Antonio M. Endoplasmic reticulum protein quality control failure in myelin disorders. Front Mol Neurosci.2017;9:162.