Kufs disease is the rare, adult-onset form of the neuronal ceroid-lipofuscinoses, characterized by early-onset dementia, progressive myoclonic epilepsy, gait disturbances, cerebellar and extrapyramidal symptoms, due to intraneuronal/extraneuronal accumulation of lipopigments (ceroid and lipofuscin-like). We describe the clinical evolution of an atypical case clinically characterized by slow progressive dementia and occipital recurrent status epilepticus.
Adult neuronal ceroid lipofuscinoses (Kufs disease): An atypical case with recurrent occipital status epilepticus | [Ceroido-lipofuscinosi dell'adulto (malattia di Kufs): Un caso atipico con stati di male occipitali ricorrenti] / Pugnaghi, Matteo; A., Zini; Monti, Giulia; A., Ariatti; A., Todeschini; Nichelli, Paolo Frigio; G., Cenacchi; Meletti, Stefano. - In: BOLLETTINO-LEGA ITALIANA CONTRO L'EPILESSIA. - ISSN 0394-560X. - STAMPA. - 136-137:136-137(2008), pp. 47-49.
Adult neuronal ceroid lipofuscinoses (Kufs disease): An atypical case with recurrent occipital status epilepticus | [Ceroido-lipofuscinosi dell'adulto (malattia di Kufs): Un caso atipico con stati di male occipitali ricorrenti]
PUGNAGHI, Matteo;MONTI, Giulia;NICHELLI, Paolo Frigio;MELETTI, Stefano
2008
Abstract
Kufs disease is the rare, adult-onset form of the neuronal ceroid-lipofuscinoses, characterized by early-onset dementia, progressive myoclonic epilepsy, gait disturbances, cerebellar and extrapyramidal symptoms, due to intraneuronal/extraneuronal accumulation of lipopigments (ceroid and lipofuscin-like). We describe the clinical evolution of an atypical case clinically characterized by slow progressive dementia and occipital recurrent status epilepticus.
Pubblicazioni consigliate
I metadati presenti in IRIS UNIMORE sono rilasciati con licenza Creative Commons CC0 1.0 Universal, mentre i file delle pubblicazioni sono rilasciati con licenza Attribuzione 4.0 Internazionale (CC BY 4.0), salvo diversa indicazione.
In caso di violazione di copyright, contattare Supporto Iris
