Director Neuroscience Critical Care Service Community Hospital Munster
Introduction: Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids or CLIPPERS is a rare inflammatory disease of the CNS. It mainly effects brainstem and in particular pons. The disease clinically present with signs and symptoms localizable to brainstem. Diagnosis is established on characteristic neuroimaging findings on MRI which show contrast enhancing lesion in pons and peri-pontine area, and responsiveness to glucocorticoid therapy (GCS) based immunosuppressive therapy. There is no serum or CSF biomarker currently available for diagnosis. There has been only 50 or so cases reported in literature.
Description: 60 years-old woman with presented with progressive dysarthria characterized by slurring of her speech, and a subjective feeling of tongue swelling. She also noted progressive gait instability. She was previously diagnosed with "sinusitis" and treated with oral antibiotics. Clinical examination showed subtle left hemiparesis, left nasolabial flattening and mild dysarthria. Ancillary testing showed normal complete blood count, comprehensive metabolic panel, thyroid function test and autoimmune panel. MRI showed classic pontine vasogenic edema with contrast enhancement. CSF analysis showed lymphocytic pleocytosis, mild elevation of CSF protein and elevated myelin basic protein. CSF cytology was negative for malignancy. She was diagnosed with CLIPPERS and treatment initiated with high dose intravenous methylprednisolone for five days followed by oral prednisone 10 mg daily as maintenance dose. She had remarkable improvement in her symptoms within days of initiating GCS therapy. Repeat MRI brain in 4 weeks showed significant improvement in pontine vasogenic edema and only faint contrast enhancement, and no new lesions were identified.
Discussion: Through this case report, we like to highlight the significance of early recognition of this potentially treatable disease is important as initiation of treatment can lead to good outcomes. Interruption of therapy or inability to start treatment may result in potential worsening relapse which may become severely disabling. Steroids sparing immunosuppressant medications may be used to maintain remission.
