| Coexistence of parkinsonism, dementia and upper motor neuron syndrome in four Czech patients. | |
| | |
MedLine Citation:
|
PMID: 20619856 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
|
BACKGROUND: The parkinsonian complex of Guam is an endemic neurodegenerative condition, which has been described only in the islands of the Guam archipelago and at the Kii peninsula of Japan. Up to now, only one "sporadic" case has been described (including the autopsy) in Japan. STUDY OBJECTIVE: To describe the clinical, laboratory and neurophysiological characteristics of the neurodegenerative disorder presenting in 4 patients with the complex syndrome of parkinsonism, amyotrophic lateral sclerosis (ALS), and dementia. PATIENTS AND METHODS: Four consecutive patients of Caucasian and Czech origin, presenting with the complex syndrome of slowly progressive parkinsonism, amyotrophic lateral sclerosis and dementia were examined clinically, including neuropsychological examination, and they were assessed using magnetic resonance imaging, electromyography and evoked potentials. The blood and CSF samples were also examined, and the levels of inflammatory and neurodegenerative markers (beta-amyloid, cystatin C and tau-proteins) were assessed. RESULTS: The clinical phenotype in all four patients corresponded to the one described in the parkinsonian complex of Guam, including the presence of a cognitive deficit at the level of mild to severe dementia. The findings of EMG examination in all cases were those typically seen in ALS, and they met the El Escorial criteria. CSF levels of neurodegenerative markers (tau-protein) were elevated in all four patients. CSF levels of inflammatory markers were normal. CONCLUSION: The unique appearance of the syndrome typical for the endemic Guam complex in patients of Caucasian origin in Europe raises a question of endemicity and heredity of the Guam complex and deserves further research. |
| | |
Authors:
|
Katerina Farníková; Petr Kanovský; Igor Nestrasil; Pavel Otruba |
Related Documents
:
|
1613716 - A patient with werner's syndrome and osteosarcoma presenting as scleroderma. 21687566 - Corneal microstructural analysis in weill-marchesani syndrome by in vivo confocal micro... 19698836 - Impact of del22q11, trisomy 21, and other genetic syndromes on surgical outcome of cono... 20339736 - Low frequency of bipolar disorder, dopamine dysregulation syndrome, and punding in braz... 11309216 - The exploding head syndrome. 189266 - Oral-facial manifestations of klippel-trenaunay-weber syndrome. report of two cases. |
Publication Detail:
|
Type: Case Reports; Journal Article |
Journal Detail:
|
Title: Journal of the neurological sciences Volume: 296 ISSN: 1878-5883 ISO Abbreviation: J. Neurol. Sci. Publication Date: 2010 Sep |
Date Detail:
|
Created Date: 2010-08-04 Completed Date: 2010-11-22 Revised Date: - |
Medline Journal Info:
|
Nlm Unique ID: 0375403 Medline TA: J Neurol Sci Country: Netherlands |
Other Details:
|
Languages: eng Pagination: 47-54 Citation Subset: IM |
Copyright Information:
|
2010 Elsevier B.V. All rights reserved. |
Affiliation:
|
Department of Neurology, Palacky University Medical School, University Hospital, Olomouc, Czech Republic. |
Export Citation:
|
APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
|
Adult Aged Czech Republic Dementia / cerebrospinal fluid, complications* Disease Progression Electroencephalography Electromyography Female Humans Magnetic Resonance Imaging Male Middle Aged Motor Neuron Disease / cerebrospinal fluid, complications* Nerve Degeneration / complications, pathology Parkinson Disease / cerebrospinal fluid, complications* |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
Previous Document: Where are all the PNPs? Pediatric Nurse Practitioner Practice Opportunities and Challenges.
Next Document: Intracranial arterial dissections in ischemic stroke assessed by 3D rotational angiography.