We report a young woman with the clinical picture of Allgrove syndrome in whom neurological symptoms are prominent. It usually presents in the first decade of life with a deficiency of tears, recurrent vomiting and dysphagia due to achalasia, severe hypoglycemic seizures and shock due to adrenal insufficiency. Neurological symptoms such as hyperreflexia, dysarthria, hypernasal speech, ataxia, sensory impairment, muscle weakness, and mental retardation are extremely slow to develop and manifest at a later age. Diagnosis was based on clinical presentation and laboratory findings. She is the first patient from the Czech Republic with genetic confirmation of Allgrove syndrome. This patient is one of about 100 cases described in the literature and one of the few patients with all the main typical clinical features.

• MeSH
• achalázie jícnu diagnostické zobrazování   genetika   patofyziologie   MeSH
• adrenální insuficience diagnostické zobrazování   genetika   patofyziologie   MeSH
• dospělí MeSH
• elektromyografie MeSH
• kombinovaná terapie MeSH
• lidé MeSH
• magnetická rezonanční tomografie MeSH
• mozek diagnostické zobrazování   patologie   MeSH
• nemoci nervového systému diagnostické zobrazování   genetika   patofyziologie   MeSH
• neurologické vyšetření MeSH
• získané deformity nohy (od hlezna dolů) patologie   MeSH
• Check Tag
• dospělí MeSH
• lidé MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• kazuistiky MeSH

The parahippocampal cortex and hippocampus are brain structures known to be involved in memory. However, the unique contribution of the parahippocampal cortex remains unclear. The current study investigates memory for object identity and memory of the configuration of objects in patients with small thermo-coagulation lesions to the hippocampus or the parahippocampal cortex. Results showed that in contrast to control participants and patients with damage to the hippocampus leaving the parahippocampal cortex intact, patients with lesions that included the right parahippocampal cortex (RPH) were severely impaired on a task that required learning the spatial configuration of objects on a computer screen; these patients, however, were not impaired at learning the identity of objects. Conversely, we found that patients with lesions to the right hippocampus (RH) or left hippocampus (LH), sparing the parahippocampal cortex, performed just as well as the control participants. Furthermore, they were not impaired on the object identity task. In the functional Magnetic Resonance Imaging (fMRI) experiment, healthy young adults performed the same tasks. Consistent with the findings of the lesion study, the fMRI results showed significant activity in the RPH in the memory for the spatial configuration condition, but not memory for object identity. Furthermore, the pattern of fMRI activity measured in the baseline control conditions decreased specifically in the parahippocampal cortex as a result of the experimental task, providing evidence for task specific repetition suppression. In summary, while our previous studies demonstrated that the hippocampus is critical to the construction of a cognitive map, both the lesion and fMRI studies have shown an involvement of the RPH for learning spatial configurations of objects but not object identity, and that this takes place independent of the hippocampus.

• Klíčová slova
• hippocampus, human, location, parahippocampal gyrus, spatial,
• Publikační typ
• časopisecké články MeSH

Spatial disorientation and learning problems belong to the integral symptoms of Alzheimer's disease (AD). A circular arena for human subjects (2.9 m diameter, 3 m high) was equipped with a computerized tracking system, similar to that used in animals. We studied navigation in 11 subjects diagnosed with early stages of Alzheimer's disease (AD), 27 subjects with subjective problems with memory or concentration, and 10 controls. The task was to locate one or several unmarked goals using the arena geometry, starting position and/or cues on the arena wall. Navigation in a real version and a computer map view version of the tests yielded similar results. The AD group was severely impaired relative to controls in navigation to one hidden goal in eight rotated positions. The impairment was largest when only the cues on the wall could be used for orientation. Also, the AD group recalled worse than controls the order of six sequentially presented locations, though they recalled similarly to controls the positions of the locations. The group with subjective problems was not impaired in any of the tests. Our results document the spatial navigation and non-verbal episodic memory impairment in the AD. Similar results in real and map view computer tests support the use of computer tests in diagnosis of cognitive disturbances.

• MeSH
• Alzheimerova nemoc patofyziologie   MeSH
• lidé středního věku MeSH
• lidé MeSH
• orientace fyziologie   MeSH
• paměť fyziologie   MeSH
• pátrací chování fyziologie   MeSH
• počítačová grafika MeSH
• poruchy paměti patofyziologie   MeSH
• pozornost fyziologie   MeSH
• prostorové chování fyziologie   MeSH
• senioři MeSH
• seriálové učení fyziologie   MeSH
• stupeň závažnosti nemoci MeSH
• vnímání prostoru fyziologie   MeSH
• Check Tag
• lidé středního věku MeSH
• lidé MeSH
• mužské pohlaví MeSH
• senioři MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• klinické zkoušky MeSH
• práce podpořená grantem MeSH
• srovnávací studie MeSH