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  Adult-onset Neuronal Ceroid Lipofuscinosis Presenting with Early Onset Dementia [Turk J Neurol]
Turk J Neurol. 2006; 12(3): 215-219

Adult-onset Neuronal Ceroid Lipofuscinosis Presenting with Early Onset Dementia

Neşe Tuncer1, Kadriye Ağan1, Aynur Mollahasanoğlu1, Yasin Bez2, Serap Şirvancı3, Nalan Turan1, Tülin Tanrıdağ1
1Marmara Üniversitesi Tıp Fakültesi Nöroloji, Anabilim Dalı, İSTANBUL.
2Marmara Üniversitesi Tıp Fakültesi Psikiyatri, Anabilim Dalı, İSTANBUL
3Marmara Üniversitesi Tıp Fakültesi Histoloji, Anabilim Dalı, İSTANBUL

Scientific background: The adult onset form of neuronal ceroid lipofuscinosis (Kufs Disease) is a rare and poorly characterized entity of metabolic neurodegenerative disease.Objectives: We report a patient with psychiatric symptoms and progressive cognitive decline. We were able to show granular osmiophilic deposits in myelin sheath and fingerprint profile in Schwann celi cytoplasm indicating the presence of adult neuronal ceroid lipofuscinosis.Discussion: Kufs disease should be considered in the differential diagnosis of early onset, progressive cognitive decline with psychiatric symptoms, seizures and motor disturbances especially in patients with positive family history.

Keywords: neuronal ceroid lipofuscinoses, Kufs disease, dementia, psychiatric symptoms, autosomal dominant

Neşe Tuncer, Kadriye Ağan, Aynur Mollahasanoğlu, Yasin Bez, Serap Şirvancı, Nalan Turan, Tülin Tanrıdağ. Adult-onset Neuronal Ceroid Lipofuscinosis Presenting with Early Onset Dementia. Turk J Neurol. 2006; 12(3): 215-219

Corresponding Author: Neşe Tuncer

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