Amygdalae calcifications associated with disease duration in lipoid proteinosis.
OBJECTIVE
To describe imaging findings of central nervous system involvement in patients with lipoid proteinosis.


METHODS
Patients with lipoid proteinosis followed in the dermatology and neurology departments of the State University of Campinas between 2002 and 2004 were evaluated using high-resolution MRI and computerized tomography (CT).


RESULTS
We reviewed the charts of three patients (two women) with lipoid proteinosis with, ages ranging from 5 to 44 years. Symptomatic disease duration ranged from 2 to 39 years. MRI scans showed hypointense signal in FLAIR and T2 images in the amygdalae in two of three patients. Brain CT scans from two patients showed that MRI findings were calcifications. The calcification was more evident in patients with longer disease duration. Epilepsy was identified in one patient with longer disease duration. No other epileptogenic lesion was identified on MRI in this patient.


CONCLUSIONS
Lipoid proteinosis is associated with bilateral amygdalae calcification. These findings were more evident in patients with longer disease duration. Epilepsy, when present, may be related to these calcifications. Patients with lipoid proteinosis should be followed with MRI/CT in order to identify these abnormalities.
Appenzeller
Simone
S
Neuroimaging Lab, Cidade Universitaria Zeferino Vaz CEP, 13083970 Campinas-SP, Brazil.
Chaloult
Erik
E
Velho
Paulo
P
de Souza
Elemir Macedo
EM
Araújo
Verônica Zanardi
VZ
Cendes
Fernando
F
Li
Li Min
LM
D000328:Adult; D000679:Amygdala / Q000473:pathology*; D002114:Calcinosis / Q000000981:diagnostic imaging / Q000473:pathology*; D002675:Child, Preschool; D005260:Female; D006801:Humans; D008065:Lipoid Proteinosis of Urbach and Wiethe / Q000000981:diagnostic imaging / Q000473:pathology*; D008279:Magnetic Resonance Imaging; D008297:Male; D013997:Time Factors; D014057:Tomography, X-Ray Computed