Extra-pontine lesions of CLIPPERS (chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids) (P4.074)

OBJECTIVE: To study the extra-pontine expansion of chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS). BACKGROUND: CLIPPERS was first described in 2010 by Pittock, et al. All reported cases suffer from the symptoms related to brainstem lesions and show characteristic magnetic resonance imaging (MRI) findings such as a peppering gadolinium enhancement of the pons. Brain biopsy shows the perivascular T cell-dominant lymphocytic infiltration. Some reports showed other lesions of central nervous system, such as cerebrum or spinal cord, however, the existence of generalized lymphadenopathy or lung lesions has never been described. DESIGN/METHODS: We performed a retrospective chart review of all adult cases admitted to Kobe University Hospital from 2001 to 2013 and picked up cases showing the characteristic brainstem MRI lesions of CLIPPERS. RESULTS: We identified three male cases showing characteristic features of CLIPPERS, among which two cases were diagnosed as lymphomatoid granulomatosis without atypical lymphocytes and leukoencephalopathy in 2001 and 2010, respectively (before the first report of CLIPPERS). The brain biopsy revealed perivascular lymphocytic infiltration in all cases. The lesion distributions were as follows: all cases had lesions in deep white matter of the cerebrum and cerebellum as well as pons. One case also showed lesions in spinal cord and thalamus. Surprisingly, systemic computed tomography (CT) scanning of this case revealed multiple nodules and consolidation in bilateral lung lobes and swollen left inguinal lymph node. The lymph node biopsy revealed perivascular lymphocytic infiltration as seen in brain biopsy. Another case showed diffuse high T2 intensity signals in white matter. CONCLUSIONS: The lesions of CLIPPERS vary and are not restricted to the brainstem. As our patient demonstrates, not only the extra-pontine but also extra-neuronal system manifestations of CLIPPERS can be seen, suggesting that CLIPPERS may be a systemic disease of T cell infiltrations. Study Supported by: None Disclosure: Dr. Sekiya has nothing to disclose. Dr. Sekiguchi has nothing to disclose. Dr. Kowa has nothing to disclose. Dr. Morinaga has nothing to disclose. Dr. Itoh has nothing to disclose. Dr. Kanda has nothing to disclose. Dr. Toda has nothing to disclose.