Chronic Lymphocytic Inflammation With Pontine
Perivascular Enhancement Responsive to Steroids
Following Influenza Vaccination
Paul B. Hillesheim, DO; John R. Parker, MD; Joseph C. Parker Jr, MD; Edward Escott, MD; Joseph R. Berger, MD
Inflammatory processes within the central nervous
system are challenging for the clinician, radiologist, and
pathologist alike. They often can mimic other more well known
and defined disease processes. We present the case
of a patient with a newly described inflammatory process
that primarily involves the pons and adjacent structures,
which is called chronic lymphocytic inflammation with
pontine perivascular enhancement responsive to steroids
(CLIPPERS). An 80-year-old man presented with numbness
of his right hand that ultimately progressed to involve both
lower extremities and face and was associated with mild
dysarthria and ataxia. He had received the influenza
vaccination 2 weeks prior. The biopsy revealed primarily
reactive T-cell lymphocytic infiltrates with macrophages
and gliosis. Treatment required long-term immunosuppressive
therapy. CLIPPERS is a recently described central
nervous system inflammatory condition that should be
considered in the differential diagnosis when a prominent
lymphocytic inflammatory infiltrate is encountered in
brainstem, spinal cord, midbrain, or cerebellar biopsies.
(Arch Pathol Lab Med. 2012;136:681?685; doi: 10.5858/
arpa.2011-0428-CR)
[snip]
The neurologic complications of vaccination are fortunately
rare, but can include panencephalitis, acute disseminated
encephalomyelitis, Guillain-Barre? syndrome, cerebellar
ataxia, parkinsonism, transverse myelitis, Bell palsy, myasthenia
gravis, neuropathy, sensorineural hearing loss,
seizures, mental retardation, and autism.9?11 CLIPPERS is
another complication that should be added to this list. Many
of the diseases listed previously are autoimmune in nature,
further adding credibility to the possible underlying
autoimmune cause of CLIPPERS.
In summary, CLIPPERS is a newly described condition
that should be considered by pathologists when a
prominent lymphocytic inflammatory infiltrate is encountered
in specimens of the brainstem, spinal cord, midbrain,
or cerebellum. The histopathologic features must be
correlated with the clinical and radiologic findings to
arrive at an accurate diagnosis. Awareness of this new
entity helps to differentiate this disease process from other
brainstem disorders.
