A handful of peppers appear in the brain? What are the diseases of this imaging phenomenon?

Today we introduce a "pepper" in the head, what is the disease?in recent years, due to medical progress, found a lot of rare neurological diseases, as a neurologist also need to understand, although not often met, but does not mean not to meetsteroid hormone reactive chronic lymphocytic inflammation is associated with cerebral bridge vascular peripheral reinforcement (CLIPPERS), referred to as CLIPPERS syndromeIt is a relatively rare chronic inflammatory disease of the central nervous system, which is mainly lymphocytic immersion and effective in steroid hormone therapyCLIPPERS syndrome was first described in 2010 with an average age of 50 years (age range 13-86 years) and a ratio of 3:1 for men and womenMRI lesions appear in specific areas (mainly brain stem and cerebellum) of the patchy, radial abnormal signal shadow, enhanced after the appearance of characteristic imaging performance - "pepper-like" diffuse speckled, irregular line-like reinforcementbut some diseases have imaging manifestations similar to CLIPPERS syndrome, such as multiple sclerosis, MOG antibody-related diseases, primary central nervous system vasculitis (PACNS), primary central nervous system lymphoma (PCNSL), and central nervous system lymphoma-like granuloma (CNS-LYG)to this end, 2017 proposed a new CLIPPERS syndrome diagnostic criteria:the pathological manifestations ofCLIPPERS syndrome(A) brain bridge pathology biopsy showed significant inflammatory immersion(B) Lymphocytes around the blood vessels are immersed mainly and substance diffuse, with no obvious boundary(C) Scar-like tissue adjacent to the inflammatory area(D) T lymphocyte immersion(E) B lymphocyte immersion(F) CD8 plus cells are less than 50% of CD3 plus cells(G) macrophage leaching participation(H) Reduced shaft density in the inflammatory area and the axial ball sample change prompts the shaft rope damage(I) Myelin staining suggests demyelination(J) the arrows are shown and enlarged to see eosinophils(K) Lymphocytes and plasma cells are distributed around neurons(L) The pulp cells are soaked(M) Astro-collocytes hyperplasiaThe clinical manifestations ofCLIPPERS syndromegait co-op disorders, re-vision, facial sensory abnormalities, and concoction disorders are commonOther clinical manifestations include dizziness, nausea, tinnitus, hearing loss, cognitive impairment, taste disorders, false ball palsy, motor tremor, eye tremor, conticence, difficulty swallowing, etcCLIPPERS syndrome MRITypical MRI is characterized by brain bridge and cerebellum dot-enhanced lesions, half of patients with lesions over the brain bridge and the cerebellum, including the spinal cord, middle brain, ementomy, cerebral hemisphereUsually after hormone therapy, the fortification lesions subside completelycharacteristics: T2 abnormal signal range did not exceed T1 radon after strengthening lesions, hormone therapy after the strengthening lesions completely subsided, strengthen the lesions 3mmFigure 1 The brain bridge and the cerebellum are suffering
Figure 2 High neck and myelin the CLIPPERSs 1 MOG antibody-related diseases
patient male, 43 years old, due to gait co-factor, speech confusion for 8 months Nervous system: cerebellum disorders, eye tremors, mild sound impairment and right side-effect sensation MRI shows the high signal of the fusion T2FLAIR between the brain bridge and the medium brain, and the lesions are centered around the fourth ventricle and the middle brain water pipe Enhanced post-dot and line-like reinforcement There was no abnormality in the spinal MRI Cerebral spinal fluid protein, cell number is normal, pathogen test negative autoimmune antibodies include ANA, ENA, ANCA, antithyroid antibodies, ACE, anti-GQ1b, and secondary tumor antibodies negative There is no abnormality in the PEC CT scan Hormone shock therapy (1g/day) changed to 80 mg/day orally after 3 days and stopped within 3 months The patient's clinical symptoms soon fully recovered the clinical recurrence of patients after 4 months of hormone deactivation, the appearance of re-vision, gait cohort disorder severity MRI shows the re-emanation of the lesions in the brain bridge, accompanied by point-like or nodule-like reinforcement Serum anti-MOG antibody positive at review Restart the hormone combined with thiopental (150mg/day) orally, the patient's clinical symptoms recovered and followed up without re-issue for 5 years 2 Multiple sclerosis (MS) patient, 28 years old, was admitted to hospital with multiple relapse remission (2000 monolithal neurotis; 2001 paralysis, cerebellum disorder, urethra sphincter dysfunction, lower limb sensory abnormalities; 2002 Lhermitte) perfect pathogen test, vasculitis-related antibodies, antithyroid antibodies, anti-AQP4 antibodies test negative Meet McDonald MS diagnostic criteria for beta-interferon treatment In 2011, patients suffered from sudden vertigo, nausea, gait disorders, hormone shock therapy (1g/day, 5 days) brain MRI (adjacent to the right quadripartite nerve), the ventricle around, the spinal cord T2 high signal Enhanced the back brain bridge dot and line-like reinforcement lesions, similar to CLIPPERS After 6 months, the MRI was reviewed without the foregoing lesions 3 Primary central nervous system lymphoma (PCNSL) patient, 58, was admitted to hospital for two months for co-op disorder and re-vision Nervous system: cerebellum co-stress, eye tremor, left peripheral facial palsy cerebrospinal fluid (CSF): protein 99mg/dl, lymphocyte 19/mm3, no oligoclonbelts and heterotypes The cranial MRI T1 enhances the visible dot and linear fortification lesions, which affect the brain bridge, the middle part of the cerebellum, the cerebellum, and the right inner sac (A, B) There was no abnormality in the spinal MRI Hormone shock therapy (1g/day, 5 days) clinical and imaging remission (C, D), after which it was changed to oral hormones 18 months after the patient re-emerged and free-range disorder, gradually aggravated to bedridden, review the brain MRI to show brain bridge lesions fusion, left thalamus, the body pressure and the knee of the new plaque-like lesions (E, F) A pathological biopsy of the left frontal lobe was confirmed as lymphoma of the central nervous system 4 Primary central nervous system vasculitis (PACNS) PACNS is a rare severe immunoitis disease that is mainly confined to small and medium blood vessels in the cerebral essence, spinal cord and soft meninges It is often spread over many blood vessels in different regions and is distributed in segments, so it is often a multiple lesions PACNS lesions range is wide, to the scene of the most lesions, brain stem, cerebellum can also be affected, occasionally see spinal cord lesions Acute-phase enhanced scans have enhanced performance, most patients are patchy fortification, followed by cerebral resust or clumps, the rarest of ring-like reinforcement About 5% to 15% of PACNS is shown as lump-like lesions, which are difficult to identify with brain tumors 30%-50% of PACNS patients experience cerebrovascular events (A) DSA osteopathic cerebral artery stenosis (B) DWI indicates limited dispersion in different blood vessel blood supply areas (C) Black blood imaging can be seen on the left side of the M1 section of vascular wall strengthening (Another patient) (D) two-sided MCA multiple segment stenosis (E) Two-sided substrate dWI dispersion is limited (F) A substantial bleeding in the left ventricle 5 Central nervous system lymphoma-like granuloma (CNS-LYG) lymphoma-like granulomatosis (lymphomatoid granulomatosis, LYG) is a rare blood vessel-centered, vandalised lymphocyte hyperplasia disease associated with EB virus infection CNS-LYG, similar to CLIPPERS, is rare THE IMAGING PERFORMANCE OF CNS-LYG CAN BE DIVIDED INTO TWO CATEGORIES OF DIFFUSE LEACHING LESIONS OR CLUMPY LESIONS, MRI EXAMINATION CAN BE SEEN IN THE BRAIN'S PHYSICAL SINGLE OR MULTIPLE LESIONS, T2WI HIGH SIGNAL, LESIONS ARE POINT-LIKE OR LINEAR REINFORCEMENT, CAN BE ACCOMPANIED BY IRREGULAR STRENGTHENING OF THE MENINGES AND BRAIN NERVES Clump-shaped lesions are many reinforced unevenly, there is a clear edema zone around them, and can be further evolved into a central no-strengthening and surrounding ring-like fortification of the lesions Tobin and other comparative analysis of 23 cases of CLIPPERS and 12 cases of CLIPPERS mimics MRI characteristics thus, reiterated the MRI characteristics of CLIPPERS syndrome: mainly distributed in the brain bridge and the small brain uniform reinforced reinforced nodules, no ring reinforcement and occupancy effect, lesions diameter of 3mm steroid hormone therapy has significantly improved for the foregoing lesions uniform T2 abnormal signal, the range of which did not significantly exceed the size of the fortified lesions above 3 MRI characteristics are of great significance in excluding the above-mentioned CLIPPERS mimics, with the sensitivity and specificity of the new diagnostic criteria being 93% and 69%, respectively summary Clinically encountered pepper-like reinforcement lesions do not easily be diagnosed under CLIPPERS syndrome, some diseases similar to CLIPPERS, such as MS, MOG antibody-related diseases, PACNS, PCNSL, CNS-LYG Repeated comparisons of MRI performance, review during follow-up, and effective use of imaging techniques for etiological screening are required The introduction of the new CLIPPERS syndrome diagnostic criteria is conducive to further narrowing the scope of clinical diagnosis author: Shen Yaoyao Source: