Table of Contents
- 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)
- Less than 5% of patients with MS have this disease.
- Cause is unknown
- Genetic factors may play a role
- Affecting mostly children and adults
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)
- Bladder and bowel problems (e.g. Constipation)
- Visual difficulties (blurry vision, eye pain on movement)
- Focus and attention difficulties
- Mood swings including depression
- Walking difficulties
- The quick onset of symptoms in Marburg’s Variant;
- Inability to talk
- Inability to move
- Severe confusion
- 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)
- Cognitive deficits
- 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
Courtesy of Intermountain Medical Imaging, Boise, Idaho.
- Balo’s Concentric Sclerosis
- Acute Disseminated Encephalomyelitis (ADEM)
- Tumefactive MS
- 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.
- 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
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