Multiple Sclerosis (MS)

Primary Category

Multiple Sclerosis

P-Category

Secondary Category

S-Category

Authors:

Tuba Khan,
Junaid Kalia MD

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.

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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

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

Figure 2: MRI showing MS lesions

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

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

"SSA - POMS: DI 23022.620 - Malignant Multiple Sclerosis - 09/16/2020". secure.ssa.gov. Retrieved 2021-10-26.

Siddiqui, N., Bell, D. Marburg variant of multiple sclerosis. Reference article, Radiopaedia.org. (accessed on 20 Jan 2022) https://doi.org/10.53347/rID-12276

Rahmlow, M. R., & Kantarci, O. (2013). Fulminant demyelinating diseases. The Neurohospitalist, 3(2), 81–91. https://doi.org/10.1177/1941874412466873

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

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