A 37-year-old lady presented to us with subacute onset progressive ataxia, with tendency to sway toward the left, of 2 month duration. She had significant weight loss, anorexia, and recurrent fevers for 6 months. She was emaciated, had oral candidiasis, and hepatomegaly. Neurological examination revealed left gaze evoked nystagmus, incoordination involving the left extremities, and slurred speech. HIV serology done was positive with a CD4 count of 28/μL. Cerebrospinal fluid study revealed normal opening pressure, mild elevation of protein (65 mg%) with no cells and normal glucose.\nMRI of the brain multiplanar T1, T2, FLAIR, diffusion and postcontrast done on a 1.5 Tesla system (Avanto-SQ Engine, Siemens Medical Systems, Erlanger, Germany) revealed the classical HCB sign in pons. In addition, there were asymmetrical T2 hyperintense lesions involving the left middle and inferior cerebellar peducle extending into left cerebellar hemisphere with atrophy of the same structures []. The left cerebellar hemispheric hyperintensities were partially suppressible on FLAIR and not enhancing on contrast []. Magnetic resonance spectroscopy revealed reduction of N-Acetylaspartic acid peak with mild elevation of choline. Imaging features suggested a possible necrotic demyelinating process asymmetrically, involving the left cerebellar white matter and transverse pontine fibers consistent with PML.
An 18-year-old woman complained of swelling with morning stiffness in multiple small joints, including the metacarpophalangeal (MCP) and proximal interphalangeal of both hands, and the 1st metatarsophalangeal joint of the left foot, without pain, fever, or signs of Raynaud symptoms. In recent years, the swelling in the joints worsened and joint deformation occurred; in addition, an oval mass appeared on the lateral margin of the right foot. When she was 2 years old, she had multiple nodules on the elbow joint, popliteal fossa, and hip, without any inducements. After surgical resection, the lesion relapsed again symmetrically so that the patient underwent an open excisional biopsy bilaterally on the elbows, first web, heel, and popliteal fossa under general anesthesia in our hospital 3 years ago. After the retraction of the subcutaneous tissue, 4 cm × 4 cm × 4 cm (the largest), solid, soft, sharply defined, yellowish masses were detected. A biopsy specimen showed juvenile xanthogranuloma (Fig. ). Immunohistochemical staining revealed that CD68 (+), CK (−), Ki-67 (+), CD1a (−), S100 (−), and acid fast staining (−).\nHer routine laboratory examination showed that serum cholesterol and low-density lipoprotein cholesterol (LDLC) levels were above normal, and the levels of antistreptolysin O and anticyclic citrullinated peptide antibodies (Anti_ccp) were also higher than the normal range; but the levels of erythrocyte sedimentation rate, antiextractable nuclear antigen, human leukocyte antigen-B27, rheumatoid factor, antinuclear antibody, antineutrophil cytoplasmic antibodies, complement, total protein, C-reactive protein, and blood glucose were normal.\nComputed tomography of the right hand showed that there was only soft tissue swelling on multiple small joints, which revealed hypointensities on T1-weighted images and hyperintensities on T2-weighted images (Figs. and ). In additional, there was an interesting finding of an RA appearance, which was thickened, fusiform, and not uniformly enhanced (Fig. ). Synovial hyperplasia or inflammation of the small joints was also observed.\nRegarding our findings and the clinical progression, the patient underwent an open excisional biopsy and tenolysis of the 3rd MCP joint in her left hand. A biopsy specimen showed several foamy cells in fibrous and fat tumor-like hyperplasia (Fig. ). Further, immunohistochemical staining revealed CD68 (+), S100 (−), MDM-2 (+), HHF (focal+), LCA (+), and Ki-67 (5%+). Finally, it was diagnosed as xanthoma. After 3 months of follow-up, no recurrence or complications were detected regarding a full range of motion remaining of the affected joints.\nIn this case, the patient was an 18-year-old woman with positive Anti_ccp and morning stiffness, which was easily misdiagnosed for RA, especially in the early stage. According to early rheumatoid arthritis (ERA) diagnostic criteria[ (Table ), the patient could be confirmed as ERA. However, RA is defined as a systemic disease of unknown etiology, manifesting as chronic, symmetric, polyarthritis. When there is inflammation, there should be pain. In this case, no joint pain was detected, which did not conform to the typical performance of RA. Taking laboratory examination results into consideration, the levels of total cholesterol and triglycerides were also above normal; it should also be considered that this disease related to whole body metabolic abnormalities or abnormal lipid deposition. It is imperative to evaluate the lesion with the biopsy, because the treatment principle of xanthoma is essentially different from the RA. For the xanthoma, the basic treatment principle is a balanced diet and reducing blood fat, namely, to strengthen health education, adjust the diet structure, restrict calories in food intake, give lipid-decreasing drugs regularly, and detect hematic fat on a regular basis. Moreover, xanthomas on different parts of the body should be treated accordingly. For example, nodular xanthoma on the Achilles tendon can be resected; however, eyelid xanthoma can be treated in steps by using freeze or laser surgery, etc.\nThe patient was treated with 20 mg of atorvastatin daily to reduce blood fat during her hospitalization. Her cholesterol and LDLC levels fell from 16.12 and 13.30 mmol/L to 11.92 and 10.20 mmol/L, respectively. After being discharged, she was switched to 10 mg of rosuvastatin daily. However, during her regular 3-month follow-up, her lipid level effect was poorly controlled—the levels of cholesterol and LDLC were higher.