A new case of chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids.

Sir, We read with great interest the recent article published in Brain by Pittock and colleagues (2010) describing eight patients with a previously unrecognized distinct brainstem encephalitis named chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS). Here, we report on what we believe to be another patient with CLIPPERS, lending further support to the concept that CLIPPERS is a novel, definable, inflammatory CNS disease. The patient provided written informed consent for presentation of her case as a report. A 69-year-old female noticed general weakness, dizziness and abnormal fatigue with subacute onset 16 weeks prior to evaluation at the Department of Neurology, Charité, Universitätsmedizin, Berlin. Eleven weeks prior, the patient developed horizontal diplopia and walking difficulties that gradually worsened so that she became unable to walk without assistance. Additionally, she noticed dysarthria and dysphagia as well as facial tingling and paraesthesia in her fingertips. About 4 weeks prior to admission, she developed hyperacusis and a labile affect with involuntary crying. Her past medical history was unremarkable except for arterial hypertension, which was treated with bisoprolol. Her family history was negative. Neurological examination on admission revealed a cerebellar syndrome with limb, gait and stance ataxia, as well as intention tremor on the left, more than on the right-hand side. She had marked cerebellar dysarthria. Her gait was wide based and unsteady and she could not walk more than few steps with bilateral support. On horizontal eye movement testing she could only abduct her left eye indicating a one and a half syndrome. The remainder of the neurological examination was normal. MRI of the brain revealed multiple, small, disseminated, T2 hyperintense lesions in the pons, medulla oblongata, cerebellar peduncles and cerebellar hemispheres with prominent punctate or curvilinear gadolinium enhancement (Fig. 1). Spinal cord MRI demonstrated no extension of the lesions below the upper cervical cord (Fig. 1). CSF analysis was performed 7 and 16 weeks after symptom onset. The first examination showed a normal cell count (2 cells/ml; normal 55/ml), normal protein (42 mg/dl; normal range 15–45 mg/dl), and CSF-specific oligoclonal bands. The second CSF examination demonstrated a very mild pleocytosis (6 cells/ml), normal protein (34 mg/dl), but no oligoclonal bands in CSF or serum. Manual CSF cytology did not reveal any malignant cells. DNA polymerase chain reactions in CSF for Herpes simplex virus type 1 and 2, varicella zoster virus, JC virus, as well as mycobacterium tuberculosis complex and Tropheryma whipplei were negative. Further laboratory work-up revealed a moderate CD4 (420/ml; normal range 500–1200/ml) and CD8 (100/ml; normal range 300–800/ml) lymphopaenia, while the remaining blood count (including total leucocyte count) was unremarkable. Renal and liver function tests were normal. Serological testing excluded borreliosis, syphilis and HIV-1 infection. Angiotensin converting enzyme in serum was normal (63 U/l; normal range 15–80 U/l). Serum antibodies against the aquaporin-4 water channel, thyroid peroxidase, thyreoglobulin, glutamic acid decarboxylase, GQ1b and onconeural antibodies (anti-amphiphysin, anti-Ri, anti-Yo, anti-Hu, anti-CV2/ CRMP5, anti-Ma2/Ta), as well as anti-cardiolipin immunoglobulin M and immunoglobulin G (IgG) were all negative. Anti-nuclear doi:10.1093/brain/awr035 Brain 2011: 134; 1–3 | e185