Clinicopathological features of the rare form of Creutzfeldt-Jakob disease in R208H-V129V PRNP carrier

Genetic transmissible spongiform encephalopathy (TSE) diseases are always associated with one of the more than 50 disease-associated point or insert mutations of the PrP gene (PRNP) [12] and represent approximately 10 to 20% of all forms of TSE diseases [9]. Each mutation is often associated with specific clinic-pathological phenotype [12] that are generally represented by Creutzfeldt-Jakob disease (CJD) [3, 8], Gerstmann–Sträussler–Scheinker disease or inherited prion protein cerebral amyloidoses [5], and fatal familial insomnia [4]. The methionine/valine polymorphism at codon 129 of PRNP plays also a role in determining the disease phenotype, especially when co-segregates with the pathogenic mutation [3]. Most PRNP mutations responsible for the CJD phenotype, including the R208H, are extremely rare and often there is no evidence of CJD in other family members. In particular, the R208H mutation co-segregates either with methionine or valine at codon 129 and it has been fully described in only 12 patients carrying M129 and 4 patients with V129 [8]. Here, we report clinical and neuropathological details of the fourth worldwide case of CJD carrying the rare R208H-129 Val PRNP genotype with a suggestive positive family history for dementia

Sporadic Creutzfeldt-Jakob disease VM1: phenotypic and molecular characterization of a novel subtype of human prion disease

The methionine (M)-valine (V) polymorphic codon 129 of the prion protein gene (PRNP) plays a central role in both susceptibility and phenotypic expression of sporadic Creutzfeldt-Jakob diseases (sCJD). Experimental transmissions of sCJD in humanized transgenic mice led to the isolation of five prion strains, named M1, M2C, M2T, V2, and V1, based on two major conformations of the pathological prion protein (PrPSc, type 1 and type 2), and the codon 129 genotype determining susceptibility and propagation efficiency. While the most frequent sCJD strains have been described in codon 129 homozygosis (MM1, MM2C, VV2) and heterozygosis (MV1, MV2K, and MV2C), the V1 strain has only been found in patients carrying VV. We identified six sCJD cases, 4 in Catalonia and 2 in Italy, carrying MV at PRNP codon 129 in combination with PrPSc type 1 and a new clinical and neuropathological profile reminiscent of the VV1 sCJD subtype rather than typical MM1/MV1. All patients had a relatively long duration (mean of 20.5 vs. 3.5 months of MM1/MV1 patients) and lacked electroencephalographic periodic sharp-wave complexes at diagnosis. Distinctive histopathological features included the spongiform change with vacuoles of larger size than those seen in sCJD MM1/MV1, the lesion profile with prominent cortical and striatal involvement, and the pattern of PrPSc deposition characterized by a dissociation between florid spongiform change and mild synaptic deposits associated with coarse, patch-like deposits in the cerebellar molecular layer. Western blot analysis of brain homogenates revealed a PrPSc type 1 profile with physicochemical properties reminiscent of the type 1 protein linked to the VV1 sCJD subtype. In summary, we have identified a new subtype of sCJD with distinctive clinicopathological features significantly overlapping with those of the VV1 subtype, possibly representing the missing evidence of V1 sCJD strain propagation in the 129MV host genotype

Italian good practice recommendations on management of persons with Long-COVID

A significant number of people, following acute SARS-CoV-2 infection, report persistent symptoms or new symptoms that are sustained over time, often affecting different body systems. This condition, commonly referred to as Long-COVID, requires a complex clinical management. In Italy new health facilities specifically dedicated to the diagnosis and care of Long-COVID were implemented. However, the activity of these clinical centers is highly heterogeneous, with wide variation in the type of services provided, specialistic expertise and, ultimately, in the clinical care provided. Recommendations for a uniform management of Long-COVID were therefore needed. Professionals from different disciplines (including general practitioners, specialists in respiratory diseases, infectious diseases, internal medicine, geriatrics, cardiology, neurology, pediatrics, and odontostomatology) were invited to participate, together with a patient representative, in a multidisciplinary Panel appointed to draft Good Practices on clinical management of Long-COVID. The Panel, after extensive literature review, issued recommendations on 3 thematic areas: access to Long-COVID services, clinical evaluation, and organization of the services. The Panel highlighted the importance of providing integrated multidisciplinary care in the management of patients after SARS-CoV-2 infection, and agreed that a multidisciplinary service, one-stop clinic approach could avoid multiple referrals and reduce the number of appointments. In areas where multidisciplinary services are not available, services may be provided through integrated and coordinated primary, community, rehabilitation and mental health services. Management should be adapted according to the patient's needs and should promptly address possible life-threatening complications. The present recommendations could provide guidance and support in standardizing the care provided to Long-COVID patients

The importance of alternative host plants as reservoirs of the cotton leaf hopper, Amrasca devastans, and its natural enemies

Many agricultural pests can be harboured by alternative host plants but these can also harbour the pests’ natural enemies. We evaluated the capacity of non-cotton plant species (both naturally growing and cultivated) to function as alternative hosts for the cotton leaf hopper Amrasca devastans (Homoptera: Ciccadellidae) and its natural enemies. Forty-eight species harboured A. devastans. Twenty-four species were true breeding hosts, bearing both nymphal and adult A. devastans, the rest were incidental hosts. The crop Ricinus communis and the vegetables Abelmoschus esculentus and Solanum melongena had the highest potential for harbouring A. devastans and carrying it over into the seedling cotton crop. Natural enemies found on true alternative host plants were spiders, predatory insects (Chrysoperla carnea, Coccinellids, Orius spp. and Geocoris spp.) and two species of egg parasitoids (Arescon enocki and Anagrus sp.). Predators were found on 23 species of alternative host plants, especially R. communis. Parasitoids emerged from one crop species (R. communis) and three vegetable species; with 39 % of A. devastans parasitised. We conclude that the presence of alternative host plants provides both advantages and disadvantages to the cotton agro-ecosystem because they are a source of both natural enemy and pest species. To reduce damage by A. devastans, we recommend that weeds that harbour the pest should be removed, that cotton cultivation with R. communis, A. esculentus, and S. melongena should be avoided, that pesticides should be applied sparingly to cultivate alternative host plants and that cotton crops should be sown earlier

The PRIAMO study: age- and sex-related relationship between prodromal constipation and disease phenotype in early Parkinson's disease.

OBJECTIVES: To explore the impact of sex and age on relationship between prodromal constipation and disease phenotype in Parkinson's disease at early stages. METHODS: A total of 385 Parkinson's disease patients from the PRIAMO study were classified according to the presence of prodromal constipation and followed for 24 months. Multivariable mixed-effect models were applied. All analyses were performed separately for sex (64.1% men) and median age (different by sex: 67 years-old in men and 68 years-old in women). RESULTS: As for sex, prodromal constipation was associated with greater odds of attention/memory complaints and apathy symptoms in women only. As for age, prodromal constipation was associated with lower cognitive and higher apathy scores in older patients only. CONCLUSIONS: Prodromal constipation anticipates lower cognitive performances and more severe apathy since the earliest stages in women and older patients. Sex- and age-related heterogeneity of prodromal markers of Parkinson's disease may impact disease phenotype

Notes on the occurrence of Mortonagrion aborense Laidlaw, 1914 (Odonata: Coenagrionidae) from lower West Bengal, India

A new distribution record of an Odonata species from lower West Bengal. Mortonagrion aborense laidlaw, 1914 is recorded for the first time from Purba Medinipur district, lower West Bengal. Previously the species was recorded only from north-east India (Mizoram, West Bengal, Assam and Nagaland). Diagnostic characters with photographic details of male anal appendages are also given for easy identification of this rare damselfly species.</p

An updated list of butterfly (Lepidoptera, Rhopalocera) fauna of Tadoba National Park, Chandrapur, Maharashtra, Central India

The present study was carried out to reveal the checklist of butterfly species in the Tadoba National Park, Chandrapur area of 623 sq. km. Study was carried out from 2011 to 2021. A total of 134 species were recorded, with an addition of 27 new records for Tadoba National Park. Of the total, 60 species were very common, 34 species were common, 9 were frequent common, 19 were rare, and 12 were very rare. Most of the butterflies recorded belong to the Nymphalidae (43 species) with 4 new records. Of Lycaenidae, 41 species with 12 new records. In Pieridae 19 species with 3 new records were recorded. A total of 20 Hesperiidae species with 6 new records and 10 species were recorded from the Papilionidae with 2 new records and one species recorded from the family Riodinidae. About 12 species of the recorded ones come under the protection category of the Indian Wild Life protection Act 1972. The study provided an updated list of butterflies of Tadoba National Park