We reviewed 1233 cases of familial amyloidotic polyneuropathy (FAP) from 489 Portuguese families registered at the Centro de Estudos de Paramiloidose, Porto, Portugal. It was found that in 159 cases, neither parent had shown symptoms of this hereditary dominant form of peripheral neuropathy. These cases appear to form a distinct group, with a later age at onset (mean 45-1 years, SD 12-0) than the group of patients with one affected parent (mean 31-2 years, SD 6-9) and a geographical origin not quite in the areas where the disease is most prevalent.

Introduction: the perception of Quality of Life (QoL) has been used to evaluate the treatment and evolution of several pathologies. QoL evaluation allows a better understanding of the patient and his adaptation to the disease. An observational, community-based and descriptive correlational study was carried out to analyze stroke survivors’ perception of QoL. Methods: consecutive case-series of stroke survivors (≥3 months) followed in a single public primary health center (“Tavira Primary Health Centre”) from Algarve, southern Portugal. The Portuguese version of the World Health Organization Quality of life instrument was administered in 102 stroke survivors. Results: Perception of QoL was associated (p < 0.05) with specific sociodemographic (age, sex, marital status, academic training), economic (monthly family income) and clinical factors (number of vascular risk factors, type of stroke, evolution, chronic mRankin score). On multivariate analysis, chronic mRankin score on physical (R2 = 0.406; F = 8.757; p < 0.001), psychological (R2 = 0.286; F = 5.536; p < 0.001) and general domain (R2 = 0.357; F = 7.287; p < 0.001); and family income (R2 = 0.160; F = 3.156; p < 0.005) on environmental domain, emerged as predictors of QoL. Conclusion: Different socio-demographic, economic and health factors are associated with post-stroke QoL. Description of QoL contributes to the overall evaluation of the impact of stroke on health and should be a priority for health professionals.

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reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. carbon dioxide 30 [27][28][29][30][31][32][33][34][35] mmHg and median temperature 37.1 [36.8-37.3]°C. After removal of artefacts, the mean monitoring time was 22 h08 (8 h54). All patients had impaired cerebral autoregulation during their monitoring time. The mean IAR index was 17 (9.5) %. During H 0 H 6 and H 18 H 24 , the majority of our patients; respectively 53 and 71 % had an IAR index > 10 %. Conclusion According to our data, patients with septic shock had impaired cerebral autoregulation within the first 24 hours of their admission in the ICU. In our patients, we described a variability of distribution of impaired autoregulation according to time. ReferencesSchramm P, Klein KU, Falkenberg L, et al. Impaired cerebrovascular autoregulation in patients with severe sepsis and sepsis-associated delirium. Crit Care 2012; 16: R181. Aries MJH, Czosnyka M, Budohoski KP, et al. Continuous determination of optimal cerebral perfusion pressure in traumatic brain injury. Crit. Care Med. 2012.

DESCRIPTIONA previously healthy man aged 57 years presented with a 4-week duration of predominant decubitus daily holocranial headache after minor head injury. Apart from unintentional weight loss of 8 pounds, there were no associated constitutional or systemic manifestations. The general and neurological examination was unremarkable. The funduscopy showed bilateral grade 2 papilloedema. Brain magnetic resonance (MRI) findings are shown in figure 1A-E. The brain MRI showed the presence of diffuse dural thickening (figure 1B-E) hypointense on unenhanced T1 imaging ( figure 1B-F), with dural enhancement on T1-weighed imaging ( figure 1B-F). The opening cerebrospinal fluid (CSF) pressure was 380 mm Hg. With the exception of serum-elevated IgG4 immunoglobulin (IgG4=302 mg/dL) and elevated CSF protein level (68 mg/dL), the wide and extensive complementary study including body positron emission tomography scan, laboratory blood/CSF work-up was negative, excluding neoplastic, infectious, autoimmune and collagen vascular disorders. Meningeal biopsy showed fibrous thickening with moderate and predominant B and T cells inflammatory infiltrate. The patient improved with spinal taps evacuation and methylprednisolone treatment.Intracranial hypertrophic pachymeningitis is a severe and rare manifestation in the spectrum of IgG4-related diseases.

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