Funkční porucha hybnosti Dotaz Zobrazit nápovědu
UNLABELLED: Presentation of two case reports about our experiences with diagnostics of paranasal tumours in patients with diplopia or ocular motility disorders which were the first symptoms of these tumours. Furthermore, the following diagnostic and therapeutic procedure which has been conducted in cooperation with other hospital departments is presented. The first case report is an example of primary paranasal tumour, the second case report represents secondary infiltration of maxillary sinus and orbital invasion. METHODS: A case reports.Key words: diplopia, paranasal tumours, orbital invasion, spinocellulary carcinoma.
- MeSH
- diplopie diagnóza MeSH
- eviscerace oka MeSH
- invazivní růst nádoru MeSH
- lidé středního věku MeSH
- lidé MeSH
- magnetická rezonanční tomografie MeSH
- nádory orbity diagnóza MeSH
- nádory vedlejších dutin nosních diagnóza MeSH
- počítačová rentgenová tomografie MeSH
- poruchy hybnosti oka diagnóza MeSH
- senioři MeSH
- spinocelulární karcinom diagnóza MeSH
- Check Tag
- lidé středního věku MeSH
- lidé MeSH
- mužské pohlaví MeSH
- senioři MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
- kazuistiky MeSH
BACKGROUNDS: This study aims to characterize eye movement abnormalities in Wilson disease and examine their association with the degree of brainstem atrophy. METHODS: Twenty patients (10 males, mean age 46.8, SD 8.9 years) with genetically confirmed neurological WD on stable anti-copper treatment and 20 age- and sex-matched healthy subjects were examined. Eye movements, including prosaccade and antisaccade tasks, were evaluated using infrared videooculography. MRI was performed using 1.5 T system, and T2-weighted images were used for the measurement of midbrain and pontine area on mid-sagittal slices. Clinical severity was assessed using the Unified Wilson's Disease Rating Scale (UWDRS). RESULTS: Compared to healthy controls, WD patients showed prolonged latencies of horizontal prosaccades and hypometry of both horizontal (p = 0.04) and vertical (p = 0.0046) prosaccades. In the antisaccade task, WD patients showed prolonged latency of both horizontal (p = 0.04) and vertical antisaccades (p = 0.047) and increased error rate of vertical antisaccades (p = 0.04). There is a significant association between midbrain area and horizontal latencies (r = -0.53; p = 0.02) and vertical maximum speed in prosaccades (r = 0.47; p = 0.04). The pons area inversely correlated with horizontal prosaccade and antisaccade latencies (p = 0.007). CONCLUSIONS: We showed impairments of ocular saccades such as prolonged latencies, hypometry, and increased error rate in antisaccades. The strong association between prolonged latencies of prosaccades and the brainstem atrophy suggests that VOG might serve as a sensitive electrophysiological marker of brainstem dysfunction in WD.
- Klíčová slova
- Brainstem, Eye movement, Wilson disease,
- MeSH
- atrofie MeSH
- dospělí MeSH
- hepatolentikulární degenerace komplikace patologie patofyziologie MeSH
- lidé středního věku MeSH
- lidé MeSH
- magnetická rezonanční tomografie MeSH
- měření pohybů očí MeSH
- mozkový kmen diagnostické zobrazování patologie patofyziologie MeSH
- poruchy hybnosti oka komplikace patologie patofyziologie MeSH
- sakadické oční pohyby fyziologie MeSH
- Check Tag
- dospělí MeSH
- lidé středního věku MeSH
- lidé MeSH
- mužské pohlaví MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
We describe a patient with corticobasal syndrome in whom neuropathological examination on autopsy revealed Pick and Alzheimer diseases in comorbidity. Corticobasal degeneration is a tauopathy usually associated with asymmetric parkinsonism, parietal lobe involvement, and cognitive impairment. Corticobasal syndrome is the clinical presentation of corticobasal degeneration without neuropathological confirmation. A 66-year-old right-handed man slowly developed speech difficulties, right-hand clumsiness, and forgetfulness. His speech apraxia progressed to mutism with preserved comprehension, and his clumsiness progressed to severe apraxia involving both hands. He developed behavioral changes and severe amnesia. All of these features were consistent with corticobasal syndrome. His loss of episodic, verbal, and visuospatial memory suggested Alzheimer disease; however, beyond his frontotemporal neuropsychological profile, he had few symptoms characteristic of frontal lobe dementia. Magnetic resonance imaging scans showed worsening temporal, frontal, and parietal atrophy, predominant in the left hemisphere. Neuropathological examination at autopsy revealed abundant neuritic plaques and neurofibrillary tangles consistent with fully developed Alzheimer disease, as well as numerous intraneuronal Pick bodies in the frontotemporal lobes. Our findings confirm the importance of clinical and neuropathological correlations in patients with atypical neurodegenerative dementias.
- MeSH
- Alzheimerova nemoc komplikace diagnóza patologie MeSH
- apraxie etiologie MeSH
- atrofie diagnóza MeSH
- bazální ganglia patologie MeSH
- demence patologie MeSH
- kognitivní poruchy patologie MeSH
- komorbidita MeSH
- lidé MeSH
- magnetická rezonanční tomografie MeSH
- mozková kůra patologie MeSH
- mutismus etiologie MeSH
- nemoci bazálních ganglií patologie MeSH
- Pickova nemoc mozku komplikace diagnóza patologie MeSH
- pitva MeSH
- poruchy paměti patologie MeSH
- senioři MeSH
- syndrom MeSH
- Check Tag
- lidé MeSH
- mužské pohlaví MeSH
- senioři MeSH
- Publikační typ
- časopisecké články MeSH
- kazuistiky MeSH
- práce podpořená grantem MeSH