Dostęp skalisty przedni – analiza techniki operacyjnej na podstawie symulacji na zwłokach

The aim of the study was to present consecutive stages of the anterior petrosal approach (APA).Eight simulations of APA were performed on non-fixed human cadavers without any known pathologies of the head and neck. The consecutive stages of the procedure were documented with photographs and schemes.The starting point for APA is a temporal craniotomy and extradural exposition of the base of the middle cranial fossa. Mobilisation of the trigeminal nerve allows for removal of the apex of the petrous bone. Approach to the upper part of the clivus is achieved by elevation of the temporal lobe and section of the tentorium and superior petrosal sinus with surrounding dura.Anterior petrosal approach is a reproducible technique, which provides surgical penetration of the upper clivus and related regions. This approach is particularly useful in the treatment of tumors of the mentioned above anatomical areas.     

Dostęp podskroniowy rozszerzony do dołu podskroniowego i jego otoczenia – analiza techniki operacyjnej na podstawie symulacji na zwłokach

The aim of the study was to present consecutive stages of the extended subtemporal approach (ESA).Seven simulations of ESA were performed on non-fixed human cadavers without any known pathologies in the head and neck. The consecutive stages of the procedure were documented with photographs and schemes.The starting point for ESA is osteotomy of the zygomatic arch and craniectomy including the greater wing of the sphenoid bone. Dislocation or removal of subtemporal fossa contents allows one to penetrate its inside and related structures. Additional widening of inspection allows osteotomy of the condyloid process of the mandible.ESA is a reproducible technique which provides surgical penetration of the subtemporal fossa and related structures. This approach is particularly useful in the surgical treatment of tumours expanding in the orbit, maxillary sinus, pterygopalatine fossa, nasopharynx, sphenoid sinus, cavernous sinus, parapharyngeal space, retromandibular fossa and surroundings of the petrosal part of the internal carotid artery.     

Choroba Wilsona – czynniki wpływające na obraz kliniczny

Wilson disease (WD) is a genetic disorder with copper metabolism disturbances leading to copper accumulation in many organs with their secondary damage. It is caused by mutation in the ATP7B gene on chromosome 13, which encodes ATP-ase 7B involved in copper transport. The age of neurologic symptom onset in WD is 20–30 years, but there is a wide spectrum of disease including: age at onset, clinical signs and treatment efficacy. More than 500 mutations of ATP7B have been described so far, but the WD genotype does not explain the disease variability. Several other factors are suspected to influence WD presentation, including polymorphisms in the genes encoding: apolipoprotein E, prion-related protein, methyltenetetrahydrofolate reductase, Murr1, antioxidant-1, X-linked inhibitor of apoptosis as well as iron metabolism disturbances, gender impact, inflammatory reactions and oxidative stress. The explanation of their significance can change the therapy of WD. The aim of our study was to review and assess the clinical significance of the factors affecting WD presentation.     

Proteinopatie TDP-43 – od zwyrodnienia czołowo-skroniowego do wtrętowego zapalenia mięśni

TDP-43, a newly described neurodegenerative protein, is of great interest to both neurologists and geneticists. At the beginning, its dysfunction was recognized in sporadic amyotrophic lateral sclerosis, frontotemporal lobar degeneration with ubiquitinated inclusions and in mixed forms. However, it was also proved that TDP-43 inclusions are in addition present in many other diseases, for example in inclusion body myositis. Furthermore, many genes and different loci may be involved in pathological TDP-43 accumulation in cells and tissues. Mutations in the TARDPB gene, progranulin gene (PGRN) and valosin-containing protein gene (VCP) as well as a gene on chromosome 9p were found. The present paper is a summary on possible involvement of TDP-43 in various neurodegenerative disorders.     

Zespół post-polio: Część II. Postępowanie terapeutyczne

The care of patients with post-polio syndrome ought to be carried out by a multidisciplinary team of specialists, including medical professionals, specialists of rehabilitation, psychologists and social workers. Many therapeutic strategies might be employed to reduce the late effects of polio. Today, the management of post-polio syndrome is based on non-pharmacological intervention, including lifestyle modification, decrease of physical activity, rest periods during the day and an individually tailored training program.