Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids

Alternate names

CLIPPERS

Definition

Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is a treatable inflammatory disease of the central nervous system. Specifically, it is a type of encephalomyelitis, which is a general term describing inflammation of the brain and spinal cord.

Cause

The underlying cause of CLIPPERS currently is unclear.

Signs and symptoms

• CLIPPERS predominantly affects the cerebellum, spinal cord, and brainstem - the part of the brain that directly connects to the spinal cord.
• Symptoms usually develop over weeks or months, and only rarely over days.
• The most common symptoms of CLIPPERS include uncoordinated walking (gait ataxia), double vision (diplopia), difficulty speaking (dysarthria), cognitive impairment, and abnormal facial sensations (such as numbness or tingling).
• Other symptoms may include pseudobulbar affect, ringing in the ears (tinnitis), shaking muscles (tremor), uncontrolled eye movements (nystagmus), distortion of taste, nausea, or paraparesis (partial loss of function of the legs).

Diagnosis

• There is no one laboratory or imaging test that confirms the diagnosis, but various laboratory and imaging tests may be needed to support the diagnosis, and to confirm or rule out many other conditions that cause overlapping symptoms.
• A brain MRI with contrast typically has characteristic findings.
• An important part of the ultimate diagnosis, and a classic feature of CLIPPERS, is that symptoms and MRI findings markedly improve with corticosteroid therapy.

Treatment

• Corticosteroids may first be taken intravenously (by IV) for several days, and then orally.
• Most people with CLIPPERS recover significantly within weeks of starting corticosteroids.
• However, depending on symptom severity and how long the disease was present before treatment, some people will not recover completely.
• Prolonged treatment with corticosteroids or other immunosuppressant medications (for at least one year) usually is necessary to prevent symptoms from recurring or worsening again.

NIH genetic and rare disease info

• NIH info on Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids

Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids is a rare disease.

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