What Are the Typical Symptoms of Primary Progressive Multiple Sclerosis?

The vast majority of patients who end up diagnosed with primary progressive multiple sclerosis (PPMS) begin noticing that they are having problems walking, which gradually gets worse.

However, the first symptoms of some people with PPMS are slowly worsening tremor and problems with balance.

Let’s take a look at these two “presentations” of PPMS:

Worsening Ability to Walk (Spinal Cord Syndrome)

This is by far the most common presentation of symptoms for PPMS, with as many as 80 to 85 percent of patients experiencing these symptoms. Also called progressive myelopathy, these symptoms consist of:

• An increasingly spastic gait (spastic paraparesis), with the legs stiffening up to cause a limp and/or rhythmic jerkiness
• Spastic hemiparesis, where there is weakness or inability to move on one side of the body (arms or legs) and/or an inability to hold things on that side
• Clumsiness, stiffness, dragging legs
• Exercise-related fatigue, meaning people are not able to walk far without resting
• Stumbling and falling

In patients that have these types of symptoms, the MRI scans of their brains show few (if any) lesions and few gadolinium-enhancing lesions. However, an MRI scan of their spines will often show a atrophy, which is a result of axon and oligodenrocyte cell loss and injury.

Tremor and Imbalance (Progressive Cerebellar Syndrome)

However, a small minority (up to 10%) of people with PPMS have these types of symptoms, which are characterized by:

• Severe intention tremor, the inability to perform small movements accurately due to shaking or trembling hands
• Hypotonia, a loss of muscle tone
• Problems with balance
• Incoordination
• Dysmetria, the lack of ability to coordinate movements, which is exhibited by the person “overshooting” (hypermetria) or “undershooting” (hypometria) the intended position of the hand, arm or leg
• Dysdiachokinesia, which is the inability to perform rapid, alternating movements
• Gait ataxia, a staggering way of walking

People with these symptoms are more likely to have abnormal brain MRI scans than people with cerebellar syndrome. These MRIs resemble those of people with SPMS or people that have had RRMS for a long time. PPMS in these people probably is more inflammatory (like SPMS and RRMS), than the PPMS of the people with spinal cord syndrome, which may be more degenerative in nature.

Other Possible Symptoms

While rare, the following symptoms can also be signs of PPMS:

Brainstem Syndrome: 1% of people with PPMS initially experience difficulties with swallowing, hoarseness, dizziness, nausea and vomiting, rapid involuntary movements of the eyes (nystagmus).

Vision Impairment or Loss: 1% of people with PPMS experience progressively worsening vision.

Cognitive Problems: 1% of people with PPMS have initial cognitive dysfunction symptoms, which includes problems with attention span, verbal memory, verbal fluency and spatial reasoning.

_Sources:

Ebers, George C. Natural history of primary progressive multiple sclerosis. Multiple Sclerosis. 2004; 10: S8-S15.

Thompson, Alan. Overview of primary progressive multiple sclerosis (PPMS): similarities and differences from other forms of MS, diagnostic criteria, pros and cons of progressive diagnosis. Multiple Sclerosis. 2004; 10: S2-S7.

Miller DH, Leary SM. Primary-progressive multiple sclerosis. Lancet Neurol. 2007 Oct;6(10):903-12.

Courtney, Susan Wells. Primary-Progressive Multiple Sclerosis: The Less Talked-about Form of MS. The Motivator. (Published by the Multiple Sclerosis Association of America) Winter/Spring 2007; 44-47, 63.