The neuronal ceroid lipofuscinoses (NCLs or Batten disease) are a group of inherited, fatal neurodegenerative disorders of childhood. In these disorders, glial (microglial and astrocyte) activation typically occurs early in disease progression and predicts where neuron loss subsequently occurs. We have found that in the most common juvenile form of NCL (CLN3 disease or JNCL) this glial response is less pronounced in both mouse models and human autopsy material, with the morphological transformation of both astrocytes and microglia severely attenuated or delayed. To investigate their properties, we isolated glia and neurons from Cln3-deficient mice and studied their basic biology in culture. Upon stimulation, both Cln3-deficient astrocytes and microglia also showed an attenuated ability to transform morphologically, and an altered protein secretion profile. These defects were more pronounced in astrocytes, including the reduced secretion of a range of neuroprotective factors, mitogens, chemokines and cytokines, in addition to impaired calcium signalling and glutamate clearance. Cln3-deficient neurons also displayed an abnormal organization of their neurites. Most importantly, using a co-culture system, Cln3-deficient astrocytes and microglia had a negative impact on the survival and morphology of both Cln3-deficient and wildtype neurons, but these effects were largely reversed by growing mutant neurons with healthy glia. These data provide evidence that CLN3 disease astrocytes are functionally compromised. Together with microglia, they may play an active role in neuron loss in this disorder and can be considered as potential targets for therapeutic interventions.Electronic supplementary materialThe online version of this article (10.1186/s40478-017-0476-y) contains supplementary material, which is available to authorized users.

SummaryTwo case histories of pregnant women with Guillain Barre  syndrome (acute demyelinating polyradiculoneuritis) are reported. The ®rst required anaesthesia during the second trimester for a minor surgical procedure. The second woman was admitted to the Intensive Care Unit in the ®rst trimester and was ventilated for 18 weeks. Both babies were carried to term and delivered by Caesarean section. A review of the management of Guillain Barre  syndrome in pregnancy discusses anaesthetic management, intensive care and the use of plasmapheresis and g-globulins. The care of pregnant women recovered from Guillain Barre  syndrome is also discussed.

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This study is a review of our experience of using the cell saver in obstetrics. The main aim was to determine its role in decreasing the need for homologous blood transfusion. A retrospective study of patients identified as being at high risk of massive obstetric haemorrhage at the time of caesarean section during the period between July 2005 and August 2008, was undertaken. Cases were identified from our electronic database. A of 147 cases were identified. Cell saver was used in 77 (52%) of these cases. A total of 13 units of salvaged blood were transfused successfully, saving approximately GB£1,800. Cell salvage in our practice has not been very effective due to non-availability of trained staff in emergencies and unfamiliarity of techniques, leading to poor salvage via suction. Most of our blood loss is swabbed not suctioned and lack of salvaging blood from swabs is a contributory factor.

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