What are the other Names for this Condition? (Also known as/Synonyms)

• CLIPPERS (Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids)

What is Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids? (Definition/Background Information)

• Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids (CLIPPERS) is an inflammatory disease of the central nervous system
• The main symptoms of CLIPPERS include double vision, nystagmus, uncoordinated movement (ataxia) and facial numbness or tingling
• This condition can be treated by suppressing the immune system with steroids

(Source: Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids; Genetic and Rare Disease Information Center (GARD) of National Center for Advancing Translational Science (NCATS), USA.)

Who gets Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids? (Age and Sex Distribution)

• Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids is a rare disorder. The presentation of symptoms typically occurs in adulthood
• Both males and females may be affected
• Worldwide, individuals of all racial and ethnic groups may be affected

What are the Risk Factors for Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids? (Predisposing Factors)

• Currently, no risk factors have been clearly identified for Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases one’s chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids? (Etiology)

The exact cause of formation of Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids is not known at the present time.

What are the Signs and Symptoms of Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids?

The signs and symptoms of Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids may include the following:

• Abnormal facial sensations
• Ringing in the ears
• Double vision
• Uncontrolled eye movements
• Uncoordinated walking
• Difficulty speaking
• Tremors
• Distortion of taste
• Nausea
• Partial paralysis of legs

(Source: Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids; Genetic and Rare Disease Information Center (GARD) of National Center for Advancing Translational Science (NCATS), USA.)

How is Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids Diagnosed?

Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids is diagnosed on the basis of the following information:

• Complete physical examination
• Thorough medical history evaluation
• Assessment of signs and symptoms
• Laboratory tests
• Imaging studies, specifically magnetic resonance imaging of the brain
• Brain biopsy (may be necessary)
• Confirmation by improvement of symptoms and MRI findings with use of steroid medication

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids?

The complications of Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids may include:

• Risk of falls and injury due to unsteady gait
• Inability to communicate due to speech difficulties
• Low self-esteem, depression

Complications may occur with or without treatment, and in some cases, due to treatment also.

How is Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids Treated?

The signs and symptoms of Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids typically improve after treatment with steroids.

• Initial treatment may involve a short course of high dose steroids given intravenously, and then oral steroids
• Many patients experience a relapse when steroids are tapered off, so it is usually necessary to continue treatment that suppresses the immune system
• Long term treatment may include a low dose of oral steroids and another type of immune suppressant, such as methotrexate or rituximab
• Because there have been very few patients with CLIPPERS reported in medical journals, the best course of treatment has not yet been determined

(Source: Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids; Genetic and Rare Disease Information Center (GARD) of National Center for Advancing Translational Science (NCATS), USA.)

How can Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids be Prevented?

• The exact cause or risk factors for the development of Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids is not known, and hence, no guidelines or methods are presently available for its prevention 
• Active research is currently being performed to explore the possibilities for treatment and prevention of disorders such as CLIPPERS
• Regular medical screening at periodic intervals with tests and physical examinations are recommended

What is the Prognosis of Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids? (Outcomes/Resolutions)

• The prognosis of Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids is dependent upon the severity of the signs and symptoms and associated complications, if any
• The condition is manageable with steroid treatment. However, depending on the severity and duration of symptoms before treatment began, some affected individuals may not recover completely
• In many cases, long-term treatment with steroids and other immune-suppresive medication may be necessary to prevent worsening or recurrence of symptoms 

Additional and Relevant Useful Information for Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids:

The following DoveMed website link is a useful resource for additional information:

http://www.dovemed.com/diseases-conditions/rare-disorders/