Background. Increased recall of categorical autobiographical memories is a phenomenon unique to depression and post-traumatic stress disorder, and is associated with a poor prognosis for depression. Although the elevated recall of categorical memories does not change on remission from depression, recent findings suggest that overgeneral memory may be reduced by cognitive interventions and maintained by rumination. This study tested whether cognitive manipulations could influence the recall of categorical memories in dysphoric participants.

Surprisingly, far less is known of the manifestations of post-traumatic stress reactions in children than in adults. Research over the past I0 years has begun to describe phenomena very similar to PTSD in adults, particularly among young adolescents. This paper will briefly review recent research and will describe our own experiences working with children who survived the Herald of Free Enterprise ferry disaster. Attention will be paid not only to the reactions of the children, but also to the reactions of their families, and to problems experienced subsequent& at school. An account will be given of two therapeutic groups run in parallel for children and parents. It is concluded that children, at least in the 8-to 16-year age range, show problems very similar to adult PTSD, but that these will not be picked up by commonly used screening instruments.

No abstract

Cryopyrin-associated periodic syndrome (CAPS) is caused by gain-of-function NLRP3 mutations. Recently, somatic NLRP3 mosaicism has been reported in some CAPS patients who were previously classified as “mutation-negative.” We describe here the clinical and laboratory findings in eight British adult patients who presented with symptoms typical of CAPS other than an onset in mid-late adulthood. All patients underwent comprehensive clinical and laboratory investigations, including analysis of the NLRP3 gene using Sanger and amplicon-based deep sequencing (ADS) along with measurements of extracellular apoptosis-associated speck-like protein with CARD domain (ASC) aggregates. The clinical phenotype in all subjects was consistent with mid-spectrum CAPS, except a median age at disease onset of 50 years. Sanger sequencing of NLRP3 was non-diagnostic but ADS detected a somatic NLRP3 mutation in each case. In one patient, DNA isolated from blood demonstrated an increase in the mutant allele from 5 to 45% over 12 years. ASC aggregates in patients’ serum measured during active disease were significantly higher than healthy controls. This series represents 8% of CAPS patients diagnosed in a single center, suggesting that acquired NLRP3 mutations may not be an uncommon cause of the syndrome and should be sought in all patients with late-onset symptoms otherwise compatible with CAPS. Steadily worsening CAPS symptoms in one patient were associated with clonal expansion of the mutant allele predominantly affecting myeloid cells. Two patients developed AA amyloidosis, which previously has only been reported in CAPS in association with life-long germline NLRP3 mutations.

This series substantially increases the published experience of IL-1 blockade and reproduction including the first data on canakinumab and on paternal exposure to these agents. Data are generally reassuring, although the case of renal agenesis is the second reported in an anakinra-exposed pregnancy.

Although the introduction of PTSD (APA, 1980, 1987) has fueled much interest into the psychological sequelae of disaster, little attention has been paid to the more existential changes in outlook many survivors report. Preliminary work on the development of a measure to assess both positive and negative responses to disaster is presented.

Key Points We found no evidence of somatic NLRP3 mosaicism in the pathogenesis of Schnitzler syndrome. Pathogenic inflammasome activation is supported by increased ASC, IL-18, IL-6, and anakinra response.

Although the introduction of PTSD (APA, 1980(APA, , 1987 has fueled much interest into the psychological sequelae of disaster, little attention has been paid to the more existential changes in outlook many survivors report. Preliminary work on the development of a measure to assess both positive and negative responses to disaster is presented.