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Dementia, Amyotrophy, and Periodic Complexes on the Electroencephalogram
A Diagnostic Challenge
Juan C. Gomez Esteban, MD;
Begoña Atarés, MD;
Juan J. Zarranz, MD;
Fernando Velasco, MD;
Imanol Lambarri, MD
Arch Neurol. 2001;58:1669-1672.
Background The clinical diagnosis of neurodegenerative diseases is a challenge
to the neurologist. In many cases the diagnosis becomes neuropathological
only after the autopsy. Several consensus criteria have been defined for the
clinical diagnosis of different neurodegenerative diseases, among them the
various types of dementia as well as prion-induced diseases. When compared
with neuropathological findings, these criteria have proved to be reasonably
accurate for regular practice, research, and epidemiological studies. The
problem arises when a combination of complementary and clinical data are obtained
that do not easily match these diagnostic criteria.
Case Description We describe a patient with dementia and periodic complexes on an electroencephalogram,
suggesting a diagnosis of sporadic Creutzfeldt-Jakob disease.
Results When the condition progressed, signs and symptoms of a motoneuron disease
appeared. Thus, 2 different diagnoses were proposed: (1) an amyotrophic variant
of a prion-induced disease; or (2) an ELA dementia syndrome with periodic
complexes on the electroencephalogram, a finding that previously has not been
described.
From the Service of Neurology, Hospital of Cruces, Osakidetza-Basque
Health Service and Neurosciences Department, University of the Basque Country
(Drs Esteban, Zarranz, and Velasco), Vizcaya, Spain; the Service of Pathology,
Hospital of Txagorritxu, Osakidetza-Basque Health Service (Dr Atarés),
Vitoria, Spain; and the Service of Neurophysiology, Hospital of Cruces, Osakidetza-Basque
Health Service (Dr Lambarri), Vizcaya.
Corresponding author: Juan J. Zarranz, MD, Service of Neurology,
Osakidetza-Basque Health Service, Department of Neurosciences, University
of the Basque Country, Hospital of Cruces, 48903 Baracaldo, Vizcaya, Spain
(e-mail: secretaria.neuro{at}hcru.osakidetza.net).
THIS ARTICLE HAS BEEN CITED BY OTHER ARTICLES
Serial Positron Emission Tomographic Findings in an Atypical Presentation of Fatal Familial Insomnia
Bar et al.
Arch Neurol 2002;59:1815-1818.
ABSTRACT
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