Facial Onset Sensory and Motor Neuronopathy: New Cases, Cognitive Changes, and Pathophysiology.

Purpose Of Review: To improve our clinical understanding of facial onset sensory and motor neuronopathy (FOSMN).

Recent Findings: We identified 29 new cases and 71 literature cases, resulting in a cohort of 100 patients with FOSMN. During follow-up, cognitive and behavioral changes became apparent in 8 patients, suggesting that changes within the spectrum of frontotemporal dementia (FTD) are a part of the natural history of FOSMN.

Neuropsychological outcome of a case of Susac syndrome: A two-year follow-up study.

Susac syndrome is a rare condition characterised by the clinical triad of encephalopathy, branch retinal artery occlusion, and sensorineural hearing loss. Of the few published cases, there is variability with regard to cognitive outcome. We describe the clinical course and neuropsychological performance of a 21-year-old male patient presenting with severe encephalopathy and later developing the full triad fulfilling the diagnosis of Susac syndrome.

Late-onset Tay-Sachs disease.

We discuss the assessment and differential diagnoses of a young adult Hungarian man with a 1-year history of a progressive and symmetric amyotrophic lateral sclerosis-like syndrome, along with irregular action tremor and stimulus-sensitive myoclonus of the arms. MR scan of the brain showed isolated cerebellar atrophy and formal neuropsychometric testing identified significant subclinical deficits in attention, processing speed and memory. We suspected a form of GM gangliosidosis, and white cell enzyme analysis showed markedly reduced enzymatic activity of β-hexosaminidase A.

Novel photodynamic therapy does not prevent new skin cancers--randomized controlled trial.

Objectives: To determine whether field photodynamic therapy (PDT) of actinic keratoses using a novel preparation of 5-aminolevulonic acid (novel ALA) results in fewer subsequent invasive skin cancers developing on the face of individuals with previous facial cutaneous malignancy in a prospective randomized controlled trial.

Methods And Materials: Intervention patients received two treatments of novel ALA 2 weeks apart. Controls were observed.

Prolonged adverse events following photodynamic therapy: regulatory implications.

Objective: To determine whether field photodynamic therapy (PDT) of actinic keratoses (AKs) using a novel preparation of 5-aminolevulonic acid (ALA) would result in fewer subsequent invasive skin cancers developing on the face.

Design: A prospective multi-center randomized controlled trial. The protocol was approved by the Bond University Human Research Ethics Committee in accord with the TGA's Clinical Trial Notification Scheme.

Post procedural pain with photodynamic therapy is more severe than skin surgery.

Objective: To compare prospective data on pain experienced by patients undergoing large facial skin cancer surgery with pain experienced with novel face photodynamic therapy (PDT).

Design: A comparison of pain data sets from two prospective trials in the same centre.

Setting: Referral skin cancer centre in Australia.