Sporadický "guamský parkinsonský komplex" nebo koincidence více neurodegenerativních onemocnění?

Translated title of the contribution: Sporadic Guam parkinsonian complex or the co-incidence of several neurodegenerative conditions?

K. Farníková, Petr Kaňovský, I. Nestrašil, P. Otruba

Research output: Contribution to journalArticlepeer-review

1 Scopus citations

Abstract

Background: The parkinsonian complex of Guam, known as Lytico-Bodig disease, is a neurodegenerative condition endemic to the island of Guam. It usually affects patients in early middle age and the progression of symptoms resembles that found in Parkinson's disease, Amyotrophic lateral sclerosis, and dementia. Patients and Methods: Cognitive impairment and at the same time (or a little later) symptoms typical for Amyotrophic lateral sclerosis were observed in 4 patients in the Czech Republic undergoing treatment for Parkinson's disease for a period of over 5 years. Results: In all patients, a hyper-intense band on both sides of the putamen was detected by MRI scans of the brain. The L-DOPA or apomorphine test was positive for all patients. The results of routine CSF tests were normal for all patients. Detailed CSF analysis revealed noticeably higher levels of neurodegenerative markers for all patients, while levels of markers for inflammation were normal. Cognitive impairment (of different levels) was diagnosed in all patients. EMG findings were typical for Amyotrophic lateral sclerosis, meeting the El Escorial criteria. Conclusion: The coincidence of neurodegenerative diseases is not frequent but has been adequately documented in global literature. Nevertheless, the coincidence of neurodegenerative illnesses whose effect correspond to the Guam complex have never yet been recorded. Even though it is as yet unclear whether the origin of this illness is familial or endemic, it seems that this specific disorder of the nervous system cannot exist in an isolated form.

Translated title of the contributionSporadic Guam parkinsonian complex or the co-incidence of several neurodegenerative conditions?
Original languageCzech
Pages (from-to)342-351
Number of pages10
JournalCeska a Slovenska Neurologie a Neurochirurgie
Volume71
Issue number3
StatePublished - Jul 31 2008

Keywords

  • Dementia
  • Motor neuron disease
  • Parkinsonism

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