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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.


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:

Developmental Delay
  • Symptoms include developmental delay, followed by ataxia and spasticity manifesting at later stages. A man had a clinical picture suggestive of an acquired demyelinating disease.[ncbi.nlm.nih.gov]
Pelvic Pain
  • Pelvic pain as the presenting symptom of demyelinating disease is rare. We report on a 49-year-old female patient that initially had symptoms of pain and anesthesia in the perineum.[ncbi.nlm.nih.gov]
Spastic Gait
  • 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 hypoesthesia[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]
Spastic Gait
  • 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 hypoesthesia[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]


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.


  • 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]


  • 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]


  • Abstract Immune mediated demyelinating disease (IMDD) after allogeneic hemopoietic stem cell transplant (HSCT) is rare and its etiology unclear.[ncbi.nlm.nih.gov]


  • 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]
Sex distribution
Age distribution


  • 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]
  • 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]
  • 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]
  • Topics include: types of MS, pathophysiology, symptomatology, pain, MS alternative treatments and more. Instructional Objectives Recall the pathophysiology of the demyelinating disorders.[nursece.com]


  • 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]
  • 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]
  • 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]



  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: 2019-07-11 20:56