Ersin Kasım ULUSOY, Şule BİLEN, Turgay ALBAYRAK, MEHMET İLKER YÖN, İrfan ŞENCAN

Nöroleptik malign sendrom tablosunda acil servise getirilen Creuzfeldt-Jakob hastalığı olgusu

Creutzfeldt-Jakob Hastalığı hızlı ilerleyen demans, miyoklonus, piramidal ve ekstrapiramidal bulgularla karakterize nörodejeneratif bir prion hastalığıdır. Kranial MRG'de bilateral caudat nukleus ve putamen T2 sekansında hiperintens izlenirken difüzyon sekansında kortikal kurdela belirtisi görülür. EEG bulguları ve BOS'da 14-3-3 proteinin pozitifliği tanıyı desteklemektedir. Burada, hızlı gelişen demans ve davranış bozukluğu tablosu nedeni ile tedavisine 10 mg/gün olanzapin eklenen ve olanzapine bağlı geliştiği düşünülen nöroleptik malign sendrom nedeni ile acil servise getirilen, takiplerinde Creutzfeldt-Jakop hastalığı tanısı almış olan bir olgu sunul- maktadır.

A case of a Creuzfeldt-Jakob disease presented with neuroleptic malignant syndrome

Creutzfeldt-Jakob Disease is a neurodegenerative prion disease characterized by rapidly progressive dementia, myoclonus, pyramidal and extrapyramidal symptoms. Brain MRI reveals hyperintense bilateral caudate nucleus and putamen on T2 sequences and diffusion weighted brain MRI shows cortical riboning. Also characteristic EEG findings and detection of 14-3-3 protein in CSF support the diagnosis. In this article a case of 70 years old female patient accepted to the emergency department with neuroleptic malignant synd- rome was reported. The symptoms were high fever, myoclonus and rapidly progressive dementia under the treatment of olanzapine 10 mg/day. During hospitalization, diagnosis of Creutzfeldt-Jakob disease was considered according to the laboratory and MRI findings.

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Collins SJ, Lawson VA, Masters CL. Transmissible spongioform encephalopathies. Lancet 2004; 363: 51-61.

Collinge J. Variant Creutzfeldt-Jakob disease. Lancet 1999;354;317- 22.

Hope J. Prions and neurodegenerative diseases. Curr Opin Genet Dev 2000;10:568-74.

Brown P, Cathala F, Castaigne P, Gajdusek DC. Creutzfeldt-Jakob disease: clinical analysis of a consecutive series of 230 neuropatho- logically verified cases. Ann Neurol 1986;20:597-602.

Ersoy G, Karayel F, Toprak S, Creutzfeldt-Jakob hastalığı; otopsi bulguları ve genel bilgilerin değerlendirilmesi. Adli Tıp Derg 2003; 17:8-13.

Paterson RW, Torres-Chae CC, Kuo AL, et al. Differential diag- nosis of jakob-creutzfeldt disease. Arch Neurol 2012;69:1578-82.

Trollor JN, Chen X, Sachdev PS. Neuroleptic malignant synd- rome associated with atypical antipsychotic drugs. CNS Drugs 2009;23:477-92.

Fekadu A, Bisson JI. Neuroleptic malignant syndrome: diagnostic and therapeutic dilemmas. Behav Neurol 2005;16:9-13.

Ananth J, Parameswaran S, Gunatilake S, Burgoyne K, Sidhom T. Neuroleptic malignant syndrome and atypical antipsychotic dru- gs. J Clin Psychiatry 2004;65:464-70.

Viejo LF, Morales V, Punal P, Perez JL, Sancho RA. Risk factors in neuroleptic malignant syndrome. A case-control study. Acta Psyc- hiatr Scand 2003;107:45-9.

Finsterer J, Gelpi E. Creatine kinase elevation in creutzfeldt-jakob disease. Gerontology 2006;52:264-6.

Wile D, Dhaliwal H, Sarna JR, et al. Diaschisis as the presenting feature in sporadic Creutzfeldt-Jakob disease. JAMA Neurol 2013;70:408-9.

Verma R, Junewar V, Sahu R. Creutzfeldt-Jakob disease presenting with visual symptoms: a case of the 'Heidenhain variant'. BMJ Case Rep 2013;2013.

Poser S, Zerr I, Schroeter A, et al. Clinical and differential diag- nosis of Creutzfeldt-Jakob disease. Arch Virol Suppl 2000:153-9.