Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) with simultaneo
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LETTER TO THE EDITOR
Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) with simultaneous presentation of Hashimoto thyroiditis Konstantina G. Yiannopoulou1 · Panagiotis Papanagiotou2 · Aikaterini I. Anastasiou3 · Dimitra A. Papadimitriou1 Received: 16 October 2019 / Accepted: 12 May 2020 © Belgian Neurological Society 2020
Keywords CLIPPERS · Hashimoto thyroiditis · Metabolic syndrome · T-cell-mediated autoimmunity
Introduction CLIPPERS is a recently described rare inflammatory central nervous system (CNS) disease, which is considered as a T-cell-mediated cause of inflammatory lesions affecting predominantly the brainstem and often the basal ganglia, cerebellum and spinal cord, presenting as punctate patchy contrast enhancement on MRI images. The usual clinical features of CLIPPERS manifest in a subacute manner and include diplopia, dysarthria, facial paresthesias and gait ataxia. Its dramatic response and dependence on corticosteroids are also remarkable, as a hallmark of the disease [1]. The histology of CLIPPERS is characterized by a meningeal, gray and white matter inflammation and a non-specific T-cell lymphocytic perivascular infiltration. The exact pathogenesis and prevalence of CLIPPERS are largely undetermined [1]. On the other hand, Hashimoto’s thyroiditis (HT) is the most prevalent chronic autoimmune thyroid disease. It is associated with thyroid hypofunction due to thyroid autoantibodies production (TPO-Ab and Tg-Ab) [2]. HT, just like CLIPPERS, is regarded as a disorder of T-cell-mediated immunity [3]. The coexistence of HT with other non-endocrine autoimmune diseases, such as pernicious anemia,
* Aikaterini I. Anastasiou [email protected] 1
Department of Neurology, Henry Dunant Hospital Center, Athens, Greece
2
Department of Interventional Neuroradiology, Henry Dunant Hospital Center, Athens, Greece
3
Medical School of Athens, National and Kapodistrian University of Athens, Tetrapoleos 18, 11527 Athens, Greece
rheumatological and cutaneous conditions, is well recognized [2]. We describe a patient in whom the two T-cell-mediated autoimmune diseases (CLIPPERS and HT) were simultaneously detected and we suggest that this may not be a simple coincidence.
Case presentation A 69-year-old Greek female patient with previous history of metabolic syndrome presented with ataxic gait, mild paresis of the left upper limb and diplopia of insidious onset and gradual deterioration over two months before her presentation. Initial magnetic resonance imaging (MRI) showed pontine and cerebellar hyperintense lesions on T1-weighted with gadolinium-enhancement (Fig. 1) and T2 images (Fig. 2). Digital subtraction angiography (DSA) was unremarkable. CSF analyses was also unremarkable (1 cell/μl, with normal protein and glucose levels) with no oligoclonal bands and negative polymerase chain reaction (PCR) for tuberculosis. Extensive serologic testing for neuronal antibodies, vasculitis screening, antibodies to gangliosides, aquaporin 4 and myelin o
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