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.
Gambardella
A
A
Institute of Neurology, School of Medicine Catanzaro, Italy.
Pasquinelli
G
G
Cittadella
R
R
Bono
F
F
Oliveri
R L
RL
Valentino
P
P
Zappia
M
M
Quattrone
A
A
Aguglia
U
U
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