Clinical Summary

A 74-year-old Caucasian woman was admitted for a 2-week history of a rapidly evolving cerebellar syndrome including ataxia, vertigo, nausea, vomiting and generalized fatigue. Her past medical history was significant for mild COPD and hypothyroidism. She had no history of recent travel. Besides horizontal nystagmus and slight right upper limb dysmetria, the physical and routine bloodwork were unremarkable. The initial lumbar puncture showed a slight pleocytosis, mildly elevated cerebrospinal fluid (CSF) proteins, and mildly reduced CSF glucose. The numerous cultures (bacteria, mycosis, tuberculosis), PCR (multiplex, tuberculosis, toxoplasmosis, JCV, echinococcus, Whipple), and other tests (cryptococcus antigen, adenosine deaminase activity, flow cytometry, and neoplastic cell) were negative on the (CSF). Initial imaging showed non-specific cystic lesions in the right cerebellar hemisphere and vermis. A thoracoabdominal-pelvic CT scan with contrast showed a non-specific 14 cm enlarged spleen and small parenchymal opacities in the right superior pulmonary lobe, probably secondary to a bronchiolar infection. A first brain biopsy was performed. The patient was treated on quadri-therapy for tuberculosis and dexamethasone. After an initial improvement two month later the patient presented with fatigue and control imaging revealed a mixed evolution of the lesions with an increase in the size of vermis enhancement foci and regression of bilateral cerebellar hemisphere enhancement foci. Unfortunately, the patient was readmitted one week later for newly appearing aphasia, dysphagia and right brachiofacial paresis. A cerebral MRI confirmed the appearance in a few days of multiple masses affecting all lobes as well as the brainstem. A second biopsy was performed.

Discussion points

1. What is the diagnosis?