Numerous in vitro and in vivo models of Parkinson’s disease (PD) demonstrate that pituitary adenylate cyclase-activating polypeptide (PACAP) conveys its strong neuroprotective actions mainly via its specific PAC1 receptor (PAC1R) in models of PD. We recently described the decrease in PAC1R protein content in the basal ganglia of macaques in the 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP) model of PD that was partially reversed by levodopa therapy. In this work, we tested whether these observations occur also in the rotenone model of PD in the rat. The rotarod test revealed motor skill deterioration upon rotenone administration, which was reversed by benserazide/levodopa (B/L) treatment. The sucrose preference test suggested increased depression level while the open field test showed increased anxiety in rats rendered parkinsonian, regardless of the received B/L therapy. Reduced dopaminergic cell count in the substantia nigra pars compacta (SNpc) diminished the dopaminergic fiber density in the caudate-putamen (CPu) and decreased the peptidergic cell count in the centrally projecting Edinger–Westphal nucleus (EWcp), supporting the efficacy of rotenone treatment. RNAscope in situ hybridization revealed decreased PACAP mRNA (Adcyap1) and PAC1R mRNA (Adcyap1r1) expression in the CPu, globus pallidus, dopaminergic SNpc and peptidergic EWcp of rotenone-treated rats, but no remarkable downregulation occurred in the insular cortex. In the entopeduncular nucleus, only the Adcyap1r1 mRNA was downregulated in parkinsonian animals. B/L therapy attenuated the downregulation of Adcyap1 in the CPu only. Our current results further support the evolutionarily conserved role of the PACAP/PAC1R system in neuroprotection and its recruitment in the development/progression of neurodegenerative states such as PD.
Ulnar dimelia, also known as “mirror hand disorder”, is a rare developmental disorder affecting the upper limb. Primarily, it involves the duplication of fingers, carpal bones, metacarpals, or ulna along the sagittal axis, and is often accompanied by the absence of the radius or thumb. The anomaly presents challenges in both bone and soft tissue development, impacting limb functionality and affecting a child’s quality of life. We present the case of a one-year-old girl with unilateral ulnar dimelia. Surgical intervention was considered to address functional and aesthetic concerns. The surgery involved creating an opposable thumb from preaxial fingers through a carefully tailored approach. Post surgical therapy included physiotherapy and psychotherapy to ensure both physical functionality and psychological adjustment. The surgical procedure successfully provided an adequate grip pattern, and the patient demonstrated age-appropriate use of the modified hand at the six-month follow-up. Comparison with similar cases highlights the diversity in ulnar dimelia presentations and the need for customised surgical solutions. The timing of surgery is typically recommended between one and two years, considering both anatomical readiness and the advantages of cerebral plasticity in young patients.