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 43-year-old African-American male presented with a 35-year history of multiple, slowly enlarging, subcutaneous nodules, which restrict his joint motion and are frequently painful. On examination, multiple ivory colored, firm, but mobile nodules overlie the dorsal aspects of the proximal and distal interphalangeal joints and multiple metacarpal phalangeal joints in the hands (fig. ). Nodules are also noted over the extensor surfaces of the knees (fig. ), Achilles tendons, right elbow (fig. ), sole of the feet, and the helix, scapha, and anti-helix of the ears (fig. ). He has had surgical removal of the nodules in the past, but they recur within a few years. Laboratory tests were remarkable for a total cholesterol of 518 mg/dl (13.4 mmol/l).