[A retrospective study of Creutzfeldt-Jakob disease in North of Portugal 1993-2002: demographic, clinical and neuropathological features].
OBJECTIVE
Description of the demographic, clinical and neuropathological features of 11 cases of Creutzfeldt-Jakob disease (CJD).


METHODS
Review of the clinical and neuropathological features of patients with CJD diagnosed in hospitals in the North of Portugal between 1993 and 2002.


RESULTS
Eleven patients were identified, 4 females: mean age of onset of symptoms--64 years, mean duration of disease--8 months. All presented with a syndrome of progressive dementia with myoclonus, with four patients presenting with cerebellar signs. Neuropathological examination of brain at autopsy showed spongiosis and reactive gliosis associated with neuronal loss. In eight cases immunocytochemistry for prion protein (PrP) was carried out and was positive.


CONCLUSIONS
The group of patients described represents the heterogeneity of the clinical phenotypes possible in CJD. Neuropathological examination is still indispensable to make the definitive diagnosis of the disease.
Silva
Ana Martins
AM
Serviço de Neurologia, Hospital Geral de Santo António, Porto, Portugal. anadmsilva@yahoo.com
Pires
Manuel Melo
MM
Bastos Leite
Antonio J
AJ
Honavar
Mrinalini
M
Mendes
Alexandre
A
Correia
Manuel
M
Nora
Manuel
M
Silva
Mário Rui
MR
Costa
Manuela
M
Guimarães
Antonio
A
Monteiro
Luís
L
D017668:Age of Onset; D000368:Aged; D001284:Atrophy; D001921:Brain / Q000473:pathology*; D007562:Creutzfeldt-Jakob Syndrome / Q000235:genetics / Q000473:pathology*; D003937:Diagnosis, Differential; D004569:Electroencephalography; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D011174:Portugal; D011328:Prions / Q000032:analysis; D012189:Retrospective Studies; D014057:Tomography, X-Ray Computed
D011328:Prions