[Klüver-Bucy syndrome. Seven year follow-up of one patient].
OBJECTIVE
To present a left-handed patient who had an acute encephalopathy, possibly of viral etiology, followed by remote and recent memory loss, several types of apraxia and emotional disturbance, without any motor abnormalities. He had cerebral lesions that involved both temporal lobes and other brain regions. All features corresponded to the Klüver-Bucy syndrome. After a seven year follow-up, no improvement of the neurological and neuroradiological, mainly by magnetic resonance imaging (MRI) features was observed.


METHODS
A 14 year-old left-handed boy suffered sudden onset of fever (40.4 degrees C), headache, vomiting, focal and generalized seizures and coma. After the acute illness, the patient had severe neurological sequels consisting in total loss of memory without any capacity to recognize persons (including family members) and remote events and he was not capability of remember hardly anything that he was taught after his disease, these alterations continued almost completely during the seven years (from 14 to 21 years) that we followed him up. The only abilities that he conserved in similar conditions to those prior to his acute disease were his capacity to swim (including the style of jumping into the water), bike riding, playing football, dominoes, cards, etc., which he had learned during his childhood, to say the numbers and the alphabet letters rapidly by heart (without knowing them) and to avoid cars on the street. MRI showed post-inflammatory lesions in the temporal and the parieto-temporo-occipital regions bilaterally (cortical and subcortical regions) and in the left occipital region. He presented almost all types of apraxia.


CONCLUSIONS
Klüver-Bucy syndrome, which can be secondary to more than 50 different causes, not only presents remote memory loss but also recent memory loss as in our patient, who appeared to be isolated from the surrounding world. Motor function and automated activities learned before the acute brain illness were not affected and could be recovered. Etiology, location and extent of the anatomic brain lesion appear to be the most important prognostic conditions.
Pascual-Castroviejo
I
I
Servicio de Neurología Pediátrica, Hospital Universitario La Paz, Madrid. pascas@ines.es
Pascual-Pascual
S-I
SI
Viaño
J
J
D000293:Adolescent; D000328:Adult; D001072:Apraxias / Q000209:etiology / Q000503:physiopathology; D005500:Follow-Up Studies; D006801:Humans; D020232:Kluver-Bucy Syndrome / Q000175:diagnosis* / Q000473:pathology / Q000503:physiopathology*; D008297:Male; D008569:Memory Disorders / Q000209:etiology / Q000503:physiopathology; D009483:Neuropsychological Tests; D011379:Prognosis