[Magnetic resonance of brain involvement in progressive facial hemiatrophy (Romberg's disease). Reconsideration of a syndrome].
Progressive facial hemiatrophy (PFH) is a sporadic disease of unclear etiology, characterized by shrinking and deformation of one side of the face. Reports and interpretations of CNS involvement in PFH, as deduced from the occurrence of seizures in some patients and documented by pneumoencephalography and CT findings in small series of patients, are contradictory. We examined three female patients with PFH, one with partial epilepsy, with the view to gaining further insight into the pathogenesis of the disease.


METHODS
Routine MR examinations of the head and face were performed.


RESULTS
Only the patient with epilepsy showed pathological findings, confined to the cerebral hemisphere homolateral to the facial hemiatrophy, and including monoventricular enlargement, meningo-cortical dysmorphia and white matter changes.


CONCLUSIONS
The MR morphology, and corresponding neuroradiological and histopathological findings disclosed by a review of the literature, indicate that homolateral hemiatrophy is a typical finding for a subgroup of PFH patients, but do not support the model of a simple or nutritive atrophic process. We reconsider chronic localized meningo-encephalitis with vascular involvement as possible underlying cause of the occasional brain involvement in PFH.
Terstegge
K
K
Departament of Radiology, Universitätsklinikum Rudolf Virchow, Freie Universität Berlin, Deutschland.
Kunath
B
B
Felber
S
S
Speciali
J G
JG
Beckert
M
M
Henkes
H
H
Hosten
N
N
D000328:Adult; D001921:Brain / Q000473:pathology*; D005150:Facial Hemiatrophy / Q000150:complications / Q000473:pathology*; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D008875:Middle Aged; D013577:Syndrome
Ressonância magnética do envolvimento cerebral na hemiatrofia facial progressiva (doença de Romberg). Reconsideração de uma síndrome.