Is Primary Progressive MS the Worst Kind? 27 Symptoms & 5 Types

Each patient may have a unique experience with PPMS due to varied symptoms.

Primary progressive multiple sclerosis (PPMS) affects 10 to 15 percent of patients with multiple sclerosis (MS) and is characterized by a slow but constant increase in neurological impairment. PPMS is an uncommon kind of MS.

According to studies, people with PPMS have a worse prognosis than those with other kinds of MS. PPMS may have a poor prognosis due to uncertain pathophysiology and a progressive disease course, which make developing effective drugs or therapies challenging.

What is primary progressive MS?

Multiple sclerosis (MS) is a chronic autoimmune illness of the central nervous system that causes varying degrees of impairment. Primary progressive multiple sclerosis (PPMS) can give rise to persistent neurological impairment.

Independent of relapses, MS can develop from the start (primary progressive) or after a relapsing-remitting course (secondary progressive). In contrast to the aggressive inflammation observed in the relapsing-remitting phases of the illness, the slow deterioration of disability in PPMS is caused by complicated immunological processes and neurodegeneration.

Early signs of PPMS may start with difficulty walking and balancing. The speed and progression of PPMS might vary with patients.

27 possible symptoms of PPMS

Each patient may have a unique experience with PPMS due to varied symptoms.

Chronic pain in the legs, feet, and back
Muscle spasms
Electric-shock sensations that run down the back and limbs when the neck is bent (Lhermitte sign)
Paralysis, temporary or permanent
A difficulty with clear thought processes
Trouble walking
Vision problems
Muscle weakness
Longstanding fatigue or exhaustion
Trouble staying balanced
Paralysis
Numbness
Prickling feelings
Dizziness
Shakiness
Trouble thinking clearly
Mood changes
Sexual problems
Trouble with bowel and bladder control
Cognitive issues including poor memory
Poor hand-eye-body coordination
Sexual-related problems; decreased sex drive
Numbness and tingling sensation
Unable to stand or walk due to weakened and stiffened leg muscles
Depression and mood swings
Consistent headaches
Trouble speaking or swallowing

What are the causes of primary progressive MS?

The exact cause of multiple sclerosis (MS) and its types are unknown, but it is considered an autoimmune disorder.

MS is characterized by the body's immune system attacking the protective myelin sheath covering the nerve fibers, resulting in a variety of neurological symptoms.
The lesions in primary progressive multiple sclerosis (PPMS) prevent appropriate signaling across the nerve cells, resulting in the symptoms of multiple sclerosis.

8 possible risk factors of PPMS

Observed in middle-aged adults, most commonly in adults between 35 and 39 years of age (some studies indicate an age group of 40 to 50 years)
Family history of multiple sclerosis
People of Northern European descent
Presence of autoimmune diseases, such as type I diabetes mellitus and thyroid disease
Smoking
Studies have shown that a lack of vitamin D may lead to MS
Epstein-Barr virus infection
Individuals with certain HLA gene types have an increased risk for MS

With PPMS, there are no symptom flare-ups (relapses or attacks) and recuperation (remission). Patients' rates of progression of this condition may differ. There could be times when things are steady and brief (temporary) periods of small improvement. However, nervous system issues worsen over time. This kind of MS affects everyone equally.

How is primary-progressive MS diagnosed?

A doctor can use numerous approaches to assess whether a patient has primary progressive multiple sclerosis (PPMS). The diagnosis of the condition begins with a discussion of the symptoms encountered to establish a comprehensive picture of medical history and changes in the health of the patient. This will most likely be followed by a physical test to assess the present function of nerves and muscles.

4 common tests for PPMS

Magnetic resonance imaging of the brain and spinal cord
Visual evoked potential tests the functionality of the optic nerve
Optical coherence tomography measures the nerve fibers located within the retina
Lumbar puncture, also called a spinal tap, involves drawing out a sample of cerebrospinal fluid, so it can be tested in the laboratory

Patients must meet two of the following to get a confirmed diagnosis of PPMS:

One or more brain lesions in the periventricular, cortical, juxtacortical, or infratentorial portions of the brain
There is more than one lesion in the spinal cord
Presence of oligoclonal bands (these are proteins identified in cerebrospinal fluid examinations)

Unfortunately, unless there is already a significant advancement, most tests will not provide much information to identify PPMS because nerve damage begins and increases over time. This implies that even if a multiple sclerosis diagnosis is confirmed, it may take years to determine if the illness is primarily progressive multiple sclerosis.

Latest Neurology News

Trending on MedicineNet

Are Your MS Drugs
Working?

Learn More on

5 types of primary progressive multiple sclerosis (PPMS)

Active PPMS
- May experience relapse (sudden increases in the severity of symptoms)
- May develop new multiple sclerosis lesions on brain scans over time
Steady PPMS
- PPMS is further characterized as having or not having progression
- When the disease progresses, their symptoms worsen
- When the disease does not progress, they have little or no new symptoms. This is referred to as steady PPMS.
Active PPMS with progression
- In the first few years after a person is diagnosed, they may have new lesions that develop on brain scans. During this same period, their symptoms could slowly get worse. This would be active PPMS with progression.
Not active and without progression
- If over the next few years, symptoms do not get worse and brain scans remain the same, PPMS could be called not active and without progression
Not active but with progression
- Several years later, the same person could have worsening symptoms but no new brain scan findings. At this point, their PPMS would be called not active but with progression.

All these groups of PPMS can happen to the same person. They can change over time.

QUESTION

What kind of disease is multiple sclerosis?
See Answer

How is primary progressive MS treated and managed?

There is no cure for primary progressive multiple sclerosis (PPMS). Not all multiple sclerosis (MS) drugs can effectively treat PPMS. Research is currently underway to find more treatments for PPMS.

Disease-modifying drugs

Ocrelizumab is the only medicine that has shown a fair amount of effectiveness in PPMS patients. It suppresses the immune system by attaching to and destroying types of white blood cells that cause demyelination.
The U.S. Food and Drug Administration (FDA) has authorized ocrelizumab with the condition that the manufacturer does further post-marketing clinical research to clarify its safety profile.

Rehabilitation strategies

Physical and occupational therapy could be helpful. Therapists may teach you exercise strategies to manage new symptoms that develop.
By regulating the immune system, physical activity can help reduce inflammation.
Regular exercise can enhance the muscle strength required to manage balance and coordination challenges, improve heart health, reduce tiredness and spasticity, improve emotional conditions, and regulate mood.
A multimodal approach to therapy has been found to reduce the burden of MS symptoms by boosting self-performance and independence.
It comprises physiotherapy, stretching and positioning procedures, and gait and balance training.

Nutritional support

Some evidence suggests that food and nutritional practices may help promote therapy and minimize the signs and symptoms of MS.

Apart from its involvement in calcium metabolism and bone production, vitamin D has been demonstrated to influence the immune system and control cell development, reducing MS disease activity.
Biotin, a type of B vitamin, was tested in people with main or secondary progressive MS. Biotin at large dosages was able to ameliorate impairment in some people without causing major side effects.
People with MS may benefit from foods high in omega-3 and omega-6 fatty acids, as well as a low-fat diet.
They should have fresh and dried fruits, vegetables, and plenty of water.

Stem cells (a promising approach to managing PPMS)

Stem cells have been the subject of significant research in recent decades in the context of illness therapy.
Using mesenchymal stromal cells (MSCs) produced from bone marrow has potentially advantageous features that can repair and protect cells of the central nervous system and thus, can influence the course of PPMS.

Other approaches

If the body’s temperature rises, the symptoms may worsen for a brief period. So, avoid doing anything that might cause overheating. Spending lengthy amounts of time in the sun or overexerting oneself are examples of this.
MS management could be challenging, frustrating, and terrifying. If a person is experiencing anxiety or despair, they should get assistance from their physician. Individual therapy and support groups for patients and families can help individuals deal with problems and changes that people with MS and their caregivers encounter.
Currently, FDA-approved monoclonal antibody medicines are available to decrease immune system activity in the treatment of PPMS.
Acute attacks of the disorder are treated with corticosteroids and plasma exchange therapy (plasmapheresis).
Pain medications could be administered when required.
Use of other drugs, such as baclofen (to reduce muscle spasm) or benzodiazepine.
Cholinergic drugs can be used to decrease urinary-related signs and symptoms.
Antidepressants could be used for mood-or behavior-associated conditions.
Medications for sexual dysfunction (including erectile dysfunction).
Healthy living, including a well-balanced diet and exercising, including adequate sleep and rest.