Multiple sclerosis: benign vs. malignant

Posted on
1.3.2024
Edited on
23.8.2024
Reading time:
6 minutes

Symptoms of multiple sclerosis can differ in their occurrence, frequency and severity. The EDSS scale is often used to assess impairment.

This article explains what the EDSS score is and what is meant by benign and malignant MS.

What is the EDSS scale?

EDSS stands for Expanded Disability Status Scale. It provides information about the degree of disability in people with multiple sclerosis and is used by healthcare professionals to assess and document the progression of multiple sclerosis. The scale ranges from zero to ten. The scale is not linear, i.e. a value of 6.0 is not twice as bad as 3.0. Instead, the degree of disability increases exponentially within the scale levels, meaning that there are increasingly greater limitations from level to level. The EDSS scale is based on examinations of various human functional systems.

These are the

  • Pyramidal tract functional system (influences e.g. paralysis)
  • cerebellar functional system (affects e.g. movement coordination disorders (ataxia), involuntary, rhythmic tremor)
  • Brainstem functional system (affects e.g. speech and/or swallowing disorders)
  • Sensory-functional system (affects e.g. reduction in the sense of touch)
  • Bladder and/or rectal functional system (affects e.g. incontinence)
  • Visual function system (affects e.g. restricted field of vision)
  • Cerebral functional system (affects e.g. memory difficulties, change in character)

However, the EDSS scale is only one instrument for determining the impairment of multiple sclerosis. Influencing factors such as fine motor skills, concentration disorders or fatigue are only marginally taken into account and are therefore assessed separately.

Benign MS

Benign MS is defined as a benevolent, benign course of the disease. It is defined as a form of progression in which an EDSS value of three or less is present more than 15 years after the onset of the disease. Grade 3 on the EDSS scale is assigned if

  • grade 2 (mild disability) of impairment is found in three to four functional systems or
  • grade 3 (moderate disability) of impairment is determined in one functional system and
  • all other functional systems are assessed as zero or one.

It is not possible to predict whether a patient will have a benign course.

However, Swedish scientists were able to show in a data set with 11,222 MS patients that 21% of this patient group had a benign course. Women were more likely to have a benign course than men (75.5% vs 70.1%). Multiple sclerosis was also diagnosed at a younger age than in patients with a non-benign course (28.3 vs. 32.9 years).The researchers also found that the probability of further benign progression decreased with increasing disease duration: after 20 years, the probability of further benign progression was 53%, after 25 years 28% and after 30 years 15%.

Maligned MS

Malignant MS, also known as the Marbug variant of multiple sclerosis, affects around 2% of MS patients. In contrast to benign MS, malignant MS describes a particularly aggressive, malignant course.

Malignant MS is a very rare form of multiple sclerosis that describes a rapid or fulminant course of MS. It particularly affects young people. The most common initial symptoms include fever, headaches, vomiting, confusion and neurological complaints. With malignant MS, life expectancy may be significantly shortend.

Multiple sclerosis can be associated with many different symptoms. Also Progression forms are individually. Two extreme forms of multiple sclerosis are benign and malignant MS.

Benign MS is a mild form in which symptoms remain minimal or disappear completely after the first episodes, leaving hardly any limitations behind. Malignant MS, on the other hand, is an aggressive form of MS that progresses rapidly and results in severe disability. Patients with malignant MS experience frequent and severe relapses that rapidly worsen neurological functions.

These forms represent the extremes of the course of MS, while most sufferers have a course in between.