What is Clipper neurology?
Abstract. Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is a recently defined inflammatory central nervous system (CNS) disorder, prominently involving the brainstem and in particular the pons.
What is CLIPPERS disease?
Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids (CLIPPERS) is a rare chronic inflammatory neurological syndrome affecting multiple regions of the brain, including brainstem, cerebellum, and spinal cord, that has become increasingly recognized since its introduction in 2010 …
What is perivascular enhancement?
Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is a rare CNS inflammatory disorder involving the pons and other parts of the brainstem.
What are the symptoms of CLIPPERS?
Symptoms of CLIPPERS include subacute onset of brainstem symptoms–most prominently, ataxia, diplopia, dysarthria, and altered facial sensation. The core radiological features are punctate or curvilinear perivascular gadolinium enhancement, typically in the pons and adjacent cerebellum.
How many people have CLIPPERS disease?
CLIPPERS can be effectively treated with steroid hormones. It was first reported by Pittock in 2010, and to date, only about 100 cases have been reported.
How many people have clippers disease?
What is perivascular lymphocytic inflammation?
Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is a central nervous system inflammatory syndrome predominantly affecting the brainstem, cerebellum, and spinal cord.
How do you treat CLIPPERS?
CLIPPERS presents with distinctive clinical and MRI-findings and may be diagnosed after excluding other differential diagnoses. Patients are treated with corticosteroids with good clinical results.
How do you treat perivascular lymphocytic infiltrate?
A diagnosis of Jessner lymphocytic infiltration of the skin was made. Treatment with intralesional corticosteroids every 4 weeks and oral hydroxychloroquine resulted in resolution after 4 months of therapy, thus far with no recurrence.
What is perivascular lymphocytic?
Perivascular lymphocytic infiltration therefore has been identified in a variety of systemic vasculitides affecting small, medium, and large vessels and also is a characteristic feature of both acute and chronic forms of dermatitis.
What does mild lymphocytic infiltrate mean?
Lymphocytic infiltrate of Jessner is a rare skin condition that may be characterized by non-cancerous (benign) buildup of white blood cells, which present as lesions or lumps on the skin.
What do Clippers look like on MRI?
The appearance of CLIPPERS on MRI is fairly unique, characterized by multiple punctate, patchy and linear regions of contrast enhancement relatively confined to the pons 1. Similar changes may also be visible caudal and rostral to the pons, in the cerebellar peduncles, cerebellar hemispheres, and involving the cervical spinal cord 1,6 .
Can a brain biopsy be used to rule out Clippers?
Pathology findings are, however, not specific for CLIPPERS and should be used to rule out other causes. Even though brain biopsy in regions typically affected by CLIPPERS is complicated (eloquent areas: brainstem, cerebellum, and spinal cord), it should be considered in patients with atypical findings.
What are the neuropathological findings of Clippers?
The neuropathological findings in CLIPPERS are generally correlated to the inflammatory pattern seen on MRI but are also found to a lesser extent in regions with normal MRI appearance ( 04 ). Sections from brainstem of an autopsy case of CLIPPERS with lymphocytic infiltration on hematoxylin and eosin stain (left).
How is Clippers syndrome diagnosed?
Diagnosis of CLIPPERS is based on clinical, radiological, laboratory and CSF investigations and, if necessary, brain biopsy. Extensive investigations are mandatory to exclude alternative conditions that may mimic CLIPPERS syndrome. To date, validated diagnostic criteria for CLIPPERS are not available.