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.
A 42-year-old man made a complaint of restriction of motion particularly in the 4th and 5th fingers of his left hand. He had never been examined before because the restriction of motion had only arisen in the past month. The painless masses were growing slowly and were causing dysfunction of the affected joints. Although all of the MCP joints were covered with these solid and painless masses (), only the 4th and 5th MCP joints exhibited extension loss (20 and 30 degrees, resp., in the left hand). The neurovascular examination was intact. The patient has bilateral arcus cornea, which may be an indication of hypercholesterolemia (). The patient's serum cholesterol level was above normal, and he has a positive family history of early death due to myocardial infarction. There were no abnormal findings observed in the plain X-rays; however, multiple solid masses that were engaged to the extensor tendons were detected in the superficial ultrasound (USG). The severity of engagement was remarkable at the level of the MCP joints of the 4th and 5th fingers. Regarding our findings and the clinical progression, the patient underwent an open excisional biopsy and tenolysis of the extensor tendon at the 4th and 5th MCP joints.\nUnder local anesthesia and tourniquet control, a “V-shaped” excision was used to explore the masses over the extensor side of the 4th and 5th MCP joints. After the retraction of the subcutaneous tissue, 2 × 2 cm and 3 × 2 cm, solid, yellowish masses were detected over the extensor side of the 4th and 5th MCP joints, respectively (). Peritendinous fibrosis were observed as these masses infiltrated the extensor tendons thus restricting the gliding of the tendons. All masses were meticulously dissected from the tendon with devoted attention to maintain the continuity of the tendon by preserving the paratenon. The full range of motion was achieved after excision of the masses and the wound was sutured. Immediate active and passive ranges of motion exercises were started to avoid stiffness. Although the literature mentions wound complications, no issue with wound healing was encountered [].\nA biopsy specimen showed xanthoma cells dispersed between the collagen fibers of the tendon (). After thirty months followup, no recurrence or complications were detected in regard to maintaining a full range of motion of the affected joints.