Incidence an prevalence:

From 15 to 85 years  , Both genders

Clinical presentation

Unstable gait, Dysarthria, Diplopia, Dizziness,, Nausea, Tinnitus, Tremor, Nystagmus, Paraparesis,  Cognitive impairment 

Subacute pontocerebellar dysfunction, with or without CNS symptoms, cognitive dysfunction or myelopathy

Neurological symptoms that respond to corticosteroid therapy

There ir absence of peripheral nervous system disease, and lack of a better alternative explanation for the clinical presentation

Tobin et al 2017 propose the Diagnostic criteria for the differentiation of CLIPPERS from its mimics

CLIPPERS defined: Patient that meets all clinical, radiological, neuropathological criteria.

Likely CLIPPERS: patients who meet all clinical and radiological criteria without available neuropathology 

With the use of steroids there is a symptomatic and radiological improvement of the findings as pathognomonic data of the disease. The pathological findings present perivascular inflammation of T cells in the gray and white matter, as well as inflammation of the meninges. If they are unilateral lesions, infitlrative processes or granulomatous disease should be considered.

The extent of the T2 lesion is similar to that found in T1 images with contrast.  

Etiologies associated with infarctions, vessel abnormalities have been proposed, however when recovering with the use of steroids they have shown improvement, multiple sclerosis should also be considered in the diagnosis

Imaging patterns

Diagnostic criteria, previously established by Simon et al, 2012 in their article Expanding the clinical, radiological and neuropathological phenotype of chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) published in Neuromuscular Disorders

· Punctate and curvilinear enhancement at the pons, brachium pontis may extend to medulla and midbrain with or without spread in the cerebellar white matter in variable degrees.

· Coalescence of small hiperintense nodules in T2, FLAIR

· Blurry nodules on FLAIR an DWI, SWI with prominent veins and punctate pattern

· GD contrast enhancement

· Swelling pattern of the middle cerebellar peduncle and the pons without vasogenic edema or mass effect

· Not evident in Cerebral angiography or CT .

· Minor hypermetabolism on  PET-CT Spectroscopy increase in the Coline peak (Cho), normal of N-acetyl acetate (NAA), (suggest demyelination or necrosis)  

· Homogenous nodular images less than 3 mm without mass effect or annular enhancement

There is an association of CLIPPERS with T-cell lymphoma, type B hepatitis and oligodendrocytic glycoprotein bodies, granulomatosis, primary cerebral angitis, multiple sclerosis.

Susceptibility weighted imaging can reveal prominent veins and punctate regions of signal loss. 

The steroids treatment is highly associated with the complete resolution of post-contrast enhancement in T1 sequences, however there is residual enhancement in patients treated with steroids, minimal increase in signal in DWI-diffusion sequences is documented.

Immunohistochemistry; It is recommended to make myelin proteolipid proteins (PLP), fibrillar acid protein (GFAP), CD6, CD3, CD4, CD8, CD20 and CD138.

Autoimmune serum evaluation

Indirect immunoflorescence with amphiphysin, anti-neuronal nuclear (ANNA) -1 (anti-Hu), ANNA-2 (anti-Ri), ANNA-3, collapsin response-mediator protein (CRMP) -5, anti-glial antibodies / nuclear neuronal (AGNA) -1, Purkinje-cell cytoplasmic (PCA-1) (anti Yo), PCA-2, and PCA-Tr, glial fibrillary acidic protein (GFAP), N-methyl-D-aspartate (NMDA) , Aquaporin (AQP4)

Differential diagnosis

Tumoral; L lymphoma, Glioma, and Brainstem glioma

Inflamatory and infectious; Neurosarcoidosis, paraneoplastic disease, CNS angiitis

Tuberculosis, neurosyphilis,and parasitic infection

Whipple’s disease, Behcet and Colagenopathies

Bickerstaff brainstem encephalitis

Multiple Sclerosis, Optic Neuromyelities, ADEM os Acute Disseminated Encephalomyelitis 

Treatment and prognosis

 Steroids treatment based on prednisone 5 mg