We report a 44-year-old woman, with a family history of early-onset dementia, presenting with primary progressive aphasia. This clinically variable syndrome has multiple underlying pathologies, and correlations between clinical manifestations and postmortem neuropathologic findings are controversial. Our patient suffered worsening language impairment with major word-finding difficulties but preserved comprehension. She also developed episodic memory impairment. Her condition progressed to dementia with behavioral changes. Magnetic resonance imaging showed early left perisylvian and bitemporal atrophy. The patient died shortly afterward from colon cancer. Neuropathologic examination revealed advanced early-onset Alzheimer and Lewy body disease, plus a clinically nonrelevant metastasis of her colon cancer in her left parietal lobe. Genetic examination revealed a p.Glu184Asp mutation in the presenilin1 gene. Our findings confirm the importance of a thorough appreciation for the clinical and neuropathologic correlations in patients with atypical neurodegenerative dementias.

• MeSH
• Alzheimerova nemoc komplikace   diagnóza   genetika   patologie   MeSH
• demence s Lewyho tělísky komplikace   diagnóza   genetika   patologie   MeSH
• dospělí MeSH
• fatální výsledek MeSH
• lidé MeSH
• magnetická rezonanční tomografie MeSH
• mozek patologie   MeSH
• neurity patologie   MeSH
• neurofibrilární klubka patologie   MeSH
• neuropsychologické testy MeSH
• presenilin-1 genetika   MeSH
• primární progresivní afázie diagnóza   etiologie   patologie   MeSH
• Check Tag
• dospělí MeSH
• lidé MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• kazuistiky MeSH
• Názvy látek
• presenilin-1 MeSH
• PSEN1 protein, human MeSH Prohlížeč

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

OBJECTIVE: We aimed to assess personality characteristics in patients with Parkinson disease (PD) with and without impulse control disorders (ICD). METHODS: We tested patients and controls with the Minnesota Multiphasic Personality Inventory-2 (MMPI-2) scales that have expected high sensitivity to apparent addictive behavior. We recorded mean disease duration and mean levodopa dose in the PD groups. RESULTS: Of the 46 PD patients, 13 had ICD: hypersexuality, binge eating, or dopamine dysregulation. The PD patients with ICD had a longer duration of disease (11 vs. 5 y) and were taking higher doses of levodopa (900 vs. 500 mg/d). They scored above the pathologic threshold in 4 domains of the MMPI-2 Clinical Scales and in 8 Clinical Subscales and Content Scales. The most significant abnormality was Alienation-Self and Others. CONCLUSIONS: ICDs in the general population have similarities to disorders of substance addiction. In PD patients, some personality profiles could play a role in development of ICDs or dopamine dysregulation syndrome. The MMPI-2 may be a useful test for PD patients in general, and for detecting ICD in particular.

• MeSH
• časové faktory MeSH
• impulzivní poruchy komplikace   psychologie   MeSH
• levodopa terapeutické užití   MeSH
• lidé středního věku MeSH
• lidé MeSH
• MMPI statistika a číselné údaje   MeSH
• Parkinsonova nemoc komplikace   farmakoterapie   psychologie   MeSH
• senioři MeSH
• studie případů a kontrol MeSH
• Check Tag
• lidé středního věku MeSH
• lidé MeSH
• mužské pohlaví MeSH
• senioři MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• srovnávací studie MeSH
• Názvy látek
• levodopa MeSH

Research in neural plasticity of adult cortical representations brought hope of significant potential for further improvement in therapy after cerebrovascular stroke, but the same processes involved in plasticity also allow for maladaptive changes whether spontaneous or caused by inappropriate therapeutic manipulations. Within the extensive network of multiple and bilateral motor cortical and subcortical areas, this paper focuses on the primary motor cortex. We review selected data from humans and primates regarding its functional anatomy and the mechanisms of adaptive neuroplasticity in the presence of brain insults, and the impact of motor skill learning in normals and rehabilitation therapy in patients. The discussion centers on the potential impact of the mechanisms of motor cortex neuroplasticity, especially of the phenomenon of competition among primary motor cortical representations, on the rehabilitation of paretic hand and shoulder after stroke. Application of results from neurophysiology and functional brain imaging research into the clinical practice is in the initial stages and remains a challenge for the future. Nevertheless, even the available research provides an important message for clinical rehabilitation of stroke patients: the need to widen multimodal and interdisciplinary approaches to rehabilitation of the paretic hand.

• MeSH
• cévní mozková příhoda komplikace   patofyziologie   MeSH
• chronické poškození mozku etiologie   patofyziologie   rehabilitace   MeSH
• elektrostimulační terapie metody   MeSH
• ergoterapie metody   MeSH
• funkční lateralita MeSH
• lidé MeSH
• motorické dovednosti MeSH
• motorické korové centrum patofyziologie   MeSH
• nervové dráhy patofyziologie   MeSH
• neuroplasticita * MeSH
• obnova funkce MeSH
• paréza etiologie   patofyziologie   rehabilitace   MeSH
• rehabilitace po cévní mozkové příhodě * MeSH
• ruka patofyziologie   MeSH
• terapie cvičením metody   MeSH
• Check Tag
• lidé MeSH
• Publikační typ
• časopisecké články MeSH
• práce podpořená grantem MeSH
• přehledy MeSH