Kufs' disease presenting as late-onset epilepsia partialis continua.
A 64-year-old woman, who had no personal or family history of neurologic diseases, had an 18-month history of epilepsia partialis continua (EPC) associated with a moderate intellectual deterioration and subtle extrapyramidal rigidity. There was no photosensitive response. A thorough laboratory investigation was unremarkable. A biopsy of the rectal mucosa revealed abundant fingerprint profiles diagnostic of Kufs' disease (KD). Our case expands the clinical picture of KD and suggests that such a diagnosis should be considered in adult-onset EPC.
Institute of Neurology, School of Medicine Catanzaro, Italy.
A
A
Gambardella
G
G
Pasquinelli
R
R
Cittadella
F
F
Bono
R L
RL
Oliveri
P
P
Valentino
M
M
Zappia
A
A
Quattrone
U
U
Aguglia
D017668:Age of Onset; D003937:Diagnosis, Differential; D017036:Epilepsia Partialis Continua / Q000175:diagnosis*; D005260:Female; D006801:Humans; D008875:Middle Aged; D009130:Muscle, Smooth / Q000473:pathology; D009472:Neuronal Ceroid-Lipofuscinoses / Q000175:diagnosis* / Q000473:pathology