Multiple Sclerosis (MS)

Multiple Sclerosis (MS) is an immune-mediated demyelinating inflammatory disease of the central nervous system that can cause loss of function and debilitating effects in different neurological systems.

Primary Category
Multiple Sclerosis
P-Category
Secondary Category
S-Category
Authors:

Introduction

  • Multiple Sclerosis (MS) is an immune-mediated demyelinating inflammatory disease of the central nervous system that can cause loss of function and debilitating effects in different neurological systems.
Acute Fulminating MS is a malignant/aggressive type of MS that is also known as Marburg Multiple Sclerosis
  • It has a rapidly progressive course over a short period of time.
  • Other variants include Balo Concentric Sclerosis, Schilder’s disease, and Neuromyelitis Optica.
  • Relapsing-remitting course.
Other Types of Multiple Sclerosis:
  • Relapsing-Remitting MS (RRMS)
  • Secondary-Progressive MS (SPMS)
  • Primary-Progressive MS (PPMS)

Epidemiology

  • Less than 5% of patients with MS have this disease.

Predisposing Factors

  • Cause is unknown
  • Genetic factors may play a role
  • Affecting mostly children and adults

Clinical Presentation

History

💡
Onset between the ages 15 and 50 years
  • Relapses and remissions of symptoms (stated below)
  • Symptoms may be preceded by fevers
  • Patients may present with severe respiratory/cardiac compromise
  • Patients may complain of the following symptoms:
    • Numbness or Tingling
    • MS Hug (Squeezing sensation around torso)
    • Vertigo
    • Dizziness
    • Stiffness
    • Weakness
    • Bladder and bowel problems (e.g. Constipation)
    • Weakness
    • Visual difficulties (blurry vision, eye pain on movement)
    • Focus and attention difficulties
    • Mood swings including depression
    • Walking difficulties
    • Fatigue
  • The quick onset of symptoms in Marburg’s Variant;
    • Seizures
    • Inability to talk
    • Inability to move
    • Severe confusion

Neurological Examination

  • Optic neuritis
    • Vision loss
    • Eye Pain
    • Afferent pupillary defect
    • Visual field defect
  • Lhermitte sign
    • Electric shock-like sensation when the neck is flexed/moved
  • Internuclear ophthalmoplegia
    • Ocular movement disorder is characterized by impaired adduction of the ipsilateral eye along with nystagmus of the abducting eye.
  • Heat sensitivity (Uhthoff phenomenon)
  • Spasticity
  • Cognitive deficits

Diagnosis

  • MRI shows demyelination, mass effect, and incomplete/irregular multiple ring enhancements. Rarely small numerous lesions are seen throughout.
  • MRI (brain and spine) and blood tests can help rule out other diseases since fulminating MS has similar symptoms to remitting relapsing MS.
  • Spinal Tap to examine CSF. It may show an increase in WBC and antibody formation along with oligoclonal bands.
  • Biopsy to determine the level of demyelination is done. Axon and myelin destruction with tissue necrosis and presence of macrophages.
  • Nerve function test
 

Figure 1: MRI of a patient with fulminant MS

notion image
 
Case courtesy of Dr. Mohammad A. ElBeialy, Radiopaedia.org. From the case

Figure 2: MRI showing MS lesions

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Courtesy of Intermountain Medical Imaging, Boise, Idaho.

Differential Diagnosis

  • Balo’s Concentric Sclerosis
  • Acute Disseminated Encephalomyelitis (ADEM)
  • Tumefactive MS

Treatment

  • There is no treatment for fulminant MS
  • Corticosteroids can shorten and reduce the symptoms of remission
  • Beta-Interferons can reduce relapsing-remitting episodes of MS.
    • Available in the form of powder in vials for mixing, prefilled injections, or pen.
    • Interferon-beta 1a is injected once weekly 30 mcg intramuscular.
    • Interferon-beta 1b is injected 0.25 mg subcutaneous every other day.
  • Plasma exchange in conjunction with high dose glucocorticoids (Approximately IV-MP 1000 mg/day)
  • Immunosuppression (monthly mitoxantrone or cyclophosphamide).
    • High-dose cyclophosphamide treatment consists of four consecutive days with 50 mg/kg/day, a cumulative absolute dose of 14 g, and ocrelizumab as maintenance therapy.
  • Chemotherapy
  • Immunoglobulin

Prognosis

  • Brain stem involvement or herniation usually lead to death within 1-2 years
  • Many of the patients die within weeks or even months
  • Rarely remyelination occurs
  • Severe disability in those who survive

Further Reading

  • Gobbin, F., Marangi, A., Orlandi, R., Richelli, S., Turatti, M., Calabrese, M., ... & Gajofatto, A. (2017). A case of acute fulminant multiple sclerosis treated with alemtuzumab. Multiple sclerosis and related disorders, 17, 9-11.
  • Johnson, M. D., Lavin, P., & Whetsell, W. O., Jr (1990). Fulminant monophasic multiple sclerosis, Marburg's type. Journal of neurology, neurosurgery, and psychiatry, 53(10), 918–921. https://doi.org/10.1136/jnnp.53.10.918

Bibliography

  • Kimiskidis, V., Sakellari, I., Tsimourtou, V., Kapina, V., Papagiannopoulos, S., Kazis, D., Vlaikidis, N., Anagnostopoulos, A., & Fassas, A. & Fassas, A. (2008). Autologous stem-cell transplantation in malignant multiple sclerosis: a case with a favorable long-term outcome. Multiple Sclerosis (Houndmills, Basingstoke, England)14(2), 278–283. https://doi.org/10.1177/1352458507082604
  • Koska V, Förster M, Brouzou K, Hatami M, Arat E, Aytulun A, Albrecht P, Aktas O, Küry P, Meuth SG, and Kremer D (2021) Case Report: Successful Stabilization of Marburg Variant Multiple Sclerosis With Ocrelizumab Following High-Dose Cyclophosphamide Rescue. Front. Neurol. 12:696807. DOI: 10.3389/fneur.2021.696807
  • Fontaine, B. (2001). "Les formes frontières de sclérose en plaques" [Borderline forms of multiple sclerosis]. Revue Neurologique (in French). 157 (8–9 Pt 2): 929–34. PMID 11787357.
  • Case Report: Successful Stabilization of Marburg Variant Multiple Sclerosis With Ocrelizumab Following High-Dose Cyclophosphamide Rescue. (2021). Frontiers in Neurology. https://doi.org/10.3389/fneur.2021.696807
 
 
Tuba Khan

Written by

Tuba Khan

Neurodevelopmental Disabilities Specialist who specializes in Autism and Rare Neurological Disorders

    Junaid Kalia MD

    Written by

    Junaid Kalia MD

    Founder NeuroCare.AI, Practicing Neurologist, sub-specialized in the field of Neurocritical Care, Stroke & Epilepsy

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