Sporadic Guam Parkinsonian Complex or the Co-incidence of Several Neurodegenerative Conditions?


Authors: K. Farníková;  P. Kaňovský;  I. Nestrašil;  P. Otruba
Authors‘ workplace: Neurologická klinika LF UP a FN Olomouc
Published in: Cesk Slov Neurol N 2008; 71/104(3): 342-351
Category: Case Report

Overview

Background:
The parkinsonian complex of Guam is an endemic neurodegenerative condition, which has been described only in the islands of 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, and dementia.

Patients and Methods:
4 consecutive patients of caucasian and Czech origin, presenting with the complex syndrome of slowly progressive parkinsonism, amyotrophic lateral sclerosis and dementia were completely examined clinically, including neuropsychological examination, and they were assessed using MRI, EMG and EP. The blood and CSF samples were also examined, and the levels of inflammatory and neurodegenerative markers (beta-amyloid, cystatin C and tau-proteins) were established.

Results:
The clinical phenotype in all four patiens was corresponding to that one described in the parkinsonian complex of Guam, including the presence of 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 patiens. 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 rersearch.

Key words:
parkinsonism – dementia – motor neuron disease


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Labels
Paediatric neurology Neurosurgery Neurology

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