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Demyelinating Disease

Demyelinating Nervous System Disease or Syndrome

Demyelinating diseases consists of a group of disorders characterized by the loss of myelin with relative axonal sparing. Primary demyelinating disorders may be attributed to autoimmune mechanisms. Demyelination is often secondary to infectious, metabolic, ischemic, or hereditary disorders or exposure to a toxin.


Presentation

Symptoms of demyelinating disorders are variable and depend on the neurologic deficits in the affected portion of the nervous system. Primary demyelinating disorders should be considered in patients presenting with diffuse or multifocal neurologic deficits of acute onset that wax and wane; in particular those that onset shortly after a vaccination or infection. The most common presenting symptom of demyelinating diseases are sensory disturbances of the limbs, vision loss, and subacute motor symptoms (e.g, weakness, spasticity, hyperreflexia, gait and balance disturbance) [1].

Common demyelinating disorders and presenting symptoms include:

Sleep Apnea
  • All participants completed questionnaires: Pittsburgh, Epworth, and Stanford scales, indirect symptoms of RLS and Obstructive Sleep Apnea, Fatigue Severity Scale, and Multiple Sclerosis Quality of Life-54.[ncbi.nlm.nih.gov]
  • A further 10 questions designed to identify the presence of obstructive sleep apnea syndrome (OSA) were also used.[jcsm.aasm.org]
Spastic Gait
  • Abstract We describe herein the case of a 57 year old man who, over the last five years, has presented ataxic and spastic gait on the right side, a reduction in fine motor movement of the fingers mainly on the right side, superficial right side brachiocrural[ncbi.nlm.nih.gov]
  • On first evaluation she had an atrophic optic disc on the left side, mild spastic gait and increased muscle stretch reflexes.[scielo.br]

Workup

The diagnosis of demyelinating disease consists of a history of present illness, physical exam, laboratory, neuromuscular and imaging tests. A history of any recent infections and/or vaccinations should be noted.

Laboratory tests that should be done include complete blood count, syphilis test (e.g., RPR), antinuclear antibodies, fluorescent treponemal antibody test, lyme titer, sedimentation rate, and angiotensin converting enzyme level [7].

A cerebrospinal fluid (CSF) exam may demonstrate pleocytosis (slight elevation of mononuclear white blood cells), oligoclonal immunoglobulin G (IgG) bands, and an elevated globulin to albumin ratio [8]. Patients with myelin damage will have elevated CSF myelin protein levels, which is indicative of myelin damage.

A magnetic resonance imaging (MRI) scan may show subclinical demyelinated lesions and/or enhancement of nerve roots with the use of gadolinium [9].

The "hot bath test" works by elevating the body's temperature, which leads to exacerbation of symptoms and worsened deficits in persons with demyelinating disease.

Neuromuscular testing including an electromyography (EMG) can be used to help determine presence of peripheral neuropathy and demyelination [10].

A sural nerve biopsy can be performed to add to the evidence for the diagnosis of a demyelinating disease.

Treatment

  • Carroll WM 1 , Saida T , Kim HJ , Kira J , Kermode AG , Tsai CP , Fujihara K , Kusunoki S , Tanaka M , Kim KK , Bates D ; Pam-Asian Committee on Treatment and Research of Multiple Sclerosis .[ncbi.nlm.nih.gov]
  • TIDD appears to be very similar to tumors such as gliomas on imaging, which may lead to incorrect or delayed diagnosis and treatment.[ncbi.nlm.nih.gov]
  • The aim of this study was to evaluate the safety and efficacy of this treatment in pediatric patients.[ncbi.nlm.nih.gov]
  • The patient initially showed a good response to steroid treatment, with full clinical recovery. However, after relapse of the same lesion, recovery was incomplete.[ncbi.nlm.nih.gov]
  • Although his symptoms and radiological findings improved following steroid pulse treatment, they worsened dramatically almost 1 year after the first surgery and even after an additional steroid pulse treatment.[ncbi.nlm.nih.gov]

Prognosis

  • Prognosis of Demyelinating Diseases Prognosis of Demyelinating Diseases depends on the condition.[epainassist.com]
  • Demyelinating Disease Prognosis The outcome of treatment depends on what type of demyelinating disorder is present in the patient.[hxbenefit.com]
  • Clinicopathologic findings and prognosis of chronic inflammatory demyelinating polyneuropathy. Neurology. 1999;52:498-503. Lewis RA. Multifocal motor neuropathy and Lewis Sumner syndrome: two distinct entities. Muscle Nerve. 1999;22:1738-39. Nevo Y.[rarediseases.org]

Etiology

  • Abstract Immune mediated demyelinating disease (IMDD) after allogeneic hemopoietic stem cell transplant (HSCT) is rare and its etiology unclear.[ncbi.nlm.nih.gov]
  • BACKGROUND: Gastroparesis has a number of etiologies. The main ones are secondary to a complication from diabetes mellitus, related to post vagotomy or post gastric surgical resections, or idiopathic when the etiology is unclear.[ncbi.nlm.nih.gov]
  • We suggest that the diagnosis of an unexplained acute neurologic or psychiatric syndrome should raise the question of a primary EBV etiology.[ncbi.nlm.nih.gov]
  • Recognizing the distinct clinical features of this rare illness, referring specifically from a spinal cord or ophthalmological etiology, is essential for its rapid diagnosis, and hence for initiation of appropriate therapy.[ncbi.nlm.nih.gov]
  • The close follow-on of the clinical signs seems to exclude a simple coincidence of two separate diseases and it may constitute further evidence for a possible etiological link between central and peripheral myelin damage.[ncbi.nlm.nih.gov]

Epidemiology

  • The first (UCID-SS) is an epidemiological surveillance study that already received ethical approvals, and started on the 1st September 2009.[ncbi.nlm.nih.gov]
  • , SNP Genetics, Inc., Seoul, Republic of Korea. 5 Department of Life Science, Sogang University, Seoul, Republic of Korea; Department of Genetic Epidemiology, SNP Genetics, Inc., Seoul, Republic of Korea.[ncbi.nlm.nih.gov]
  • EVIDENCE ACQUISITION: A detailed review of the published literature ranging from in vivo immune studies to human clinical studies of epidemiology, physiology, immunology, clinical, and radiological markers was undertaken.[ncbi.nlm.nih.gov]
  • Epidemiologic evidence supports a role for environmental pathogens, such as viruses, as potentially key contributors to MS induction.[ncbi.nlm.nih.gov]
  • Although disease-specific genotypes or strains were not identified, this study provides useful insights into the molecular epidemiology of EBV infection in Australia. Copyright 2012 S. Karger AG, Basel.[ncbi.nlm.nih.gov]
Sex distribution
Age distribution

Pathophysiology

  • This review discusses clinical, pathophysiological, radiological and therapeutic concepts of demyelinating disease of the CNS in SLE, focussing on its differentiation from MS and its relation with other CNS demyelinating processes, such as transverse[ncbi.nlm.nih.gov]
  • PATHOPHYSIOLOGY MS is a result of both humoral and cellular immune response to a pathogen which remains unknown There is breakdown of blood brain barrier, causing the humoraland the cellular factor to interact and ultimately destroy the myelin (and in[slideshare.net]
  • Various autoimmune diseases, including the following, can damage the myelin sheath: Devic’s Disease Schilder’s Disease Fibromyalgia Guillain-Barre Syndrome Demyelinating Disease Pathophysiology Doctors still do not know the exact mechanism of nerve demyelination[hxbenefit.com]
  • Topics include: types of MS, pathophysiology, symptomatology, pain, MS alternative treatments and more. Instructional Objectives Recall the pathophysiology of the demyelinating disorders.[nursece.com]

Prevention

  • However, it cannot be prevented in individuals with genetic factors causing nerve demyelination. Researches are being carried out to find out whether it is possible to prevent nerve demyelination in such people.[hxbenefit.com]
  • Cells/immunology Dendritic Cells/metabolism Dendritic Cells/pathology Disease Models, Animal Encephalomyelitis, Autoimmune, Experimental/immunology* Encephalomyelitis, Autoimmune, Experimental/microbiology Encephalomyelitis, Autoimmune, Experimental/prevention[ncbi.nlm.nih.gov]
  • Prevention Prevention requires abstinence from alcohol. Persons who consume small or moderate amounts of alcohol might theoretically help prevent nutritional complications of alcohol use with dietary supplements including B vitamins.[the-medical-dictionary.com]
  • Treatment of multiple sclerosis can be discussed in terms of the management of acute relapses, the prevention of relapses as modification of the disease process, and the management of symptoms and fixed neurologic deficits.[streetdirectory.com]

References

Article

  1. Olek, 2005 - adapted from Paty et al., 1994 and Studney D et al., 1993, MS COSTAR: A computerized patient record adapted for clinical research purposes. J Neurol Rehab; 7:145.
  2. McAlpine D, Lamsden CE, Acheson ED. Multiple Sclerosis - A Reappraisal, ed. 2, Edinburgh, Churchill and Livingstone, 1972.
  3. Gupte G, Stonehouse M, Wassmer E, et al. Acute disseminated encephalomyelitis: a review of 18 cases in childhood. J Paediatr Child Health. 2003;39:336–42
  4. Leake JA, Albani S, Kao AS, et al. Acute disseminated encephalomyelitis in childhood: epidemiologic, clinical and laboratory features. Pediatr Infect Dis J. 2004;23:756–64.
  5. Sugimoto C, Ito D, Tanaka K, et al. Amplification of JC virus regulatory DNA sequences from cerebrospinal fluid: diagnostic value for progressive multifocal leukoencephalopathy. Arch Virol 1998;143:249–62.
  6. Hammarin AL, Bogdanovic G, Svedhem V, et al. Analysis of PCR as a tool for detection of JC virus DNA in cerebrospinal fluid for diagnosis of progressive multifocal leukoencephalopathy. J Clin Microbiol 1996;34:2929–32.
  7. Jones CT. Childhood autoimmune neurologic diseases of the central nervous system. Neurol Clin. 2003;21:745–64.
  8. Park SJ, Jeong IH, Kong BS, et al. Disease Type- and Status-Specific Alteration of CSF Metabolome Coordinated with Clinical Parameters in Inflammatory Demyelinating Diseases of CNS. PLoS One. 2016;11:e0166277.
  9. Balashov K. Imaging of Central Nervous System Demyelinating Disorders. Continuum (Minneap Minn). 2016 Oct;22(5, Neuroimaging):1613-35.
  10. Hanewinckel R, Ikram MA, Van Doorn PA. Peripheral neuropathies. Handb Clin Neurol. 2016;138:263-82.

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Last updated: 2018-06-22 01:28