Brain FDG-PET correlates of saccadic disorders in early PSP.

BACKGROUND: New diagnostic criteria of Progressive Supranuclear Palsy (PSP) have highlighted the interest of Eye Movement Records (EMR) at the early stage of the disease. OBJECTIVES: To investigate the metabolic brain correlates of ocular motor dysfunction using [18F] Fluorodeoxyglucose Positron Emission Tomography (FDG-PET) in early PSP. METHODS: Retrospective observational descriptive study on longitudinal data with patients who underwent EMR and FDG-PET at the stage of suggestive and possible PSP according to Movement Disorders Society criteria. Longitudinal follow-up enables to confirm diagnosis of probable PSP. Using the Statistical Parametric Mapping software, we performed whole-brain voxel-based correlations between oculomotor variables and FDG-PET metabolism. RESULTS: Thirty-seven patients with early PSP who fulfilled criteria of probable PSP during the follow-up were included. Decrease in the gain of vertical saccades correlated with reduced metabolism in Superior Colliculi (SC). We also found a positive correlation between mean velocity of horizontal saccades and SC metabolism as well as dorsal nuclei in the pons. Finally, increase in horizontal saccades latencies correlated with decrease of posterior parietal metabolism. CONCLUSIONS: These findings suggest the early involvement of SC in saccadic dysfunction in the course of PSP.

[Spontaneous periodic hypothermia syndrome: A systematic review of the literature]. / Syndrome d'hypothermie périodique spontanée : une revue systématique de la littérature.

INTRODUCTION: Periodic spontaneous hypothermia syndrome (PSHS), also known as hypersudation-hypothermia syndrome, is a rare condition characterised by the occurrence of paroxysmal attacks of spontaneous hypothermia occurring without age limit. Few studies have attempted to synthesise the available data on PSHS. The objective of this study was to summarize the literature on this condition. METHODS: A literature review was conducted using the Pubmed and ScienceDirect databases. Cases from a registry of PSHS initiated at the CHU of Saint-Étienne were added to the analysis. For each clinical case, clinical, biological and morphological data as well as therapeutic management and evolution were collected. RESULTS: Seventy-one cases of PSHS were collected. The median age at symptom onset was 21.5 years. The clinical sign most frequently associated with hypothermia was the presence of hyperhidrosis. The triad of hypothermia-hypersudation-agenesis of the corpus callosum was present in 35.7% of cases. CONCLUSION: PSHS remains a poorly known cause of hypothermia, without consensual therapeutic options. The need to better describe cases and obtain more long-term data will allow a better definition and knowledge of this syndrome.