Long-Term Survival after Progressive Multifocal Leukoencephalopathy in a Patient with Primary Immune Deficiency and NFKB1 Mutation.

Purpose: To describe the development of progressive multifocal leukoencephalopathy (PML) in a patient with primary immune deficiency (PID) due to a NFKB1 (nuclear factor kB subunit 1) mutation, who was treated successfully with a combination of mirtazapine and mefloquine.

Methods: We've based the treatment of our patient on literature research and provide a review of PML in CVID patients.

Results: Only a few reports have been published on the occurrence of PML in PID.

Grammar disruption in a patient with Neuro-Sweet syndrome.

This paper for the first time reports detailed neurolinguistic findings in a patient with Neuro-Sweet syndrome. In this patient the presenting symptoms of central nervous system (CNS) involvement primarily consisted of a selective grammar deficit restricted to spontaneous speech. On MRI a left prefrontal ischemic stroke (superior part BA 6) and two small subcortical left parietal infarctions were found.

Posterior fossa syndrome in adults: a new case and comprehensive survey of the literature.

Although the posterior fossa syndrome (PFS) can be considered as an aetiologically heterogeneous condition affecting children and adults, it most often occurs in paediatric patients after cerebellar tumour surgery. In patients with a tumoural aetiology, the syndrome is typically characterised by a short symptom-free postoperative period followed by mutism of variable duration and behavioural and affective changes. More than 200 paediatric cases have been described but reports of adult patients are extremely rare.