Primary-Progressive Multiple Sclerosis (PPMS)

Multiple Sclerosis (MS) is a disease of the central nervous system, which is made up of the brain, spinal cord and optic nerve. This disorder causes destruction of the coating (myelin) that surrounds and protects nerve fibers (axons). As a result, the damage disrupts the normal flow of messages (nerve impulses) from the central nervous system (CNS), causing a reduction or loss of body function. In many cases, the nerve fibers (axons) are also destroyed.

Primary-progressive multiple sclerosis (PPMS) is a form of the disease that is characterized from the beginning of the disease as a progressively worsening condition. This is in contrast to relapsing-remitting multiple sclerosis (RRMS) and secondary-progressive multiple sclerosis (SPMS), which are characterized by episodes of symptoms, brought on by inflammation, followed by periods of limited or no symptoms.

The progressive worsening of symptoms is caused by nerve damage or loss rather than the inflammation.

Symptoms

The symptoms of primary-progressive multiple sclerosis are similar to those of secondary-progressive multiple sclerosis. The main symptom of both forms of the condition is a gradual worsening of disability.

This may be experienced through increased:

  • Fatigue
  • Numbness or tingling
  • Vision problems, such as double vision
  • Spasticity or stiffness of the muscles
  • Difficulty controlling the bladder or bowels
  • Problems with cognition, such as learning and memory or information processing
  • Difficulty with walking and coordination
  • Trouble walking
  • Muscle weakness
  • Paralysis
  • Dizziness
  • Shakiness
  • Mood changes
  • Depression
  • Sexual dysfunction

Causes and Risk Factors

Ten percent of all multiple sclerosis patients are diagnosed with PPMS. The cause of PPMS is unknown.

Patients with PPMS tend to be diagnosed in their mid-to-late 30s. The condition affects men and women equally.

Diagnosis

Diagnosis of all forms of MS begins with a detailed medical history and neurological examination.

Because MS can have similar symptoms of other nervous system disorders, diagnostic tests help rule out other causes and confirm a diagnosis. These tests may include a magnetic resonance imaging (MRI) scan of the brain and spinal cord. The patient's medical team may order a lumbar puncture, also known as a spinal tap, and cerebrospinal fluid analysis, as well as nerve function tests. Blood tests may be performed to rule out other conditions that have similar symptoms.

The only definitive way to diagnose PPMS apart from other forms of multiple sclerosis is by observation.

In order to confirm a diagnosis of PPMS, the patient must:

  • Have a year of consistent progression of the condition, including worsening neurologic function
  • Meet two of the following criteria:
  • A type of lesion in the brain that is recognized by experts in as being typical of multiple sclerosis
  • Two or more lesions of a similar type in the spinal cord
  • Evidence in the spinal fluid of oligoclonal bands or an elevated IgG index, both of which are indicative of immune system activity in the central nervous system

Treatments

There is no cure for PPM, but it is not considered fatal.

Although the FDA has approved medications for relapsing-remitting forms of the disease, there are currently no approved medications for PPMS. Treatment of PPMS is focused on managing the symptoms and increasing daily functioning.

Symptoms of PPMS can affect a patient's daily life, so physical and occupational therapy may be used to manage symptoms and help patients adjust to living and working situations.

Certain medications may be used to address specific symptoms such as:

  • Depression
  • Muscle spasms
  • Need for frequent urination
  • Erectile dysfunction

Choosing the right medication requires careful consideration of the risks and benefits, along with close collaboration with an experienced neurologist with experience in treating the disease, such as those at the Multiple Sclerosis Center in Cedars-Sinai's Department of Neurology.