Limited chronic focal encephalitis: another variant of Rasmussen syndrome?
OBJECTIVE
To describe a more limited and less malignant form of Rasmussen encephalitis (RE).


METHODS
Three subjects (all women; 37, 31, and 32 years of age) developed childhood or late onset chronic focal encephalitis, with a relatively nonprogressive form of the disorder.


RESULTS
In our patients, clinical features were dominated by partial seizures without marked focal motor deficit and in two with choreo-dystonic movements. The diagnosis of RE was supported by histologic examination and anatomic and functional MRI.


CONCLUSIONS
These cases extend the phenotypic presentations of Rasmussen encephalitis and confirm Theodore Rasmussen's suggestion that there may be mild and nonprogressive forms of the disease.
Gambardella
A
A
Institute of Neurology, University Magna Graecia, Catanzaro Viale Europa, Località Germaneto, 88100 Catanzaro, Italy. a.gambardella@isn.cnr.it
Andermann
F
F
Shorvon
S
S
Le Piane
E
E
Aguglia
U
U
D000328:Adult; D000367:Age Factors; D017668:Age of Onset; D000927:Anticonvulsants / Q000627:therapeutic use; D001264:Athetosis / Q000188:drug therapy / Q000209:etiology / Q000503:physiopathology; D001284:Atrophy / Q000175:diagnosis / Q000209:etiology / Q000503:physiopathology; D001921:Brain / Q000473:pathology / Q000503:physiopathology; D002634:Chemotaxis, Leukocyte / Q000276:immunology; D002819:Chorea / Q000188:drug therapy / Q000209:etiology / Q000503:physiopathology; D002908:Chronic Disease; D018450:Disease Progression; D004660:Encephalitis / Q000150:complications* / Q000175:diagnosis / Q000503:physiopathology*; D004827:Epilepsy / Q000175:diagnosis / Q000209:etiology* / Q000503:physiopathology*; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D009069:Movement Disorders / Q000175:diagnosis / Q000209:etiology* / Q000503:physiopathology*; D012008:Recurrence
D000927:Anticonvulsants