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6.ULUSLARARASI VE 17. AKADEMİK GERİATRİ KONGRESİ , Muğla, Turkey, 16 - 20 October 2024, pp.61, (Full Text)
Creutzfeldt-Jacob disease (CJD) is a rare, difficult-to-diagnose, neurodegenerative prion disease with a mortality rate of 100%, occurring in one in a million people per year(1).Here we present an 81-year-old male patient with known coronary artery disease, chronic obstructive pulmonary disease, diabetes and hypertension who presented to the geriatrics outpatient clinic with complaints of behavioural changes, amnesia, sleep disturbance, aggression and paranoia for the last 1 month. There was no infarction, haemorrhage or mass on brain MRI at an external centre and no response to quetiapine 200 mg, olanzapine 10 mg, mirtazapine 15 mg treatments initiated by psychiatry and neurology departments. On physical examination, he was disorientated and agitated. Haemogram, biochemistry, thyroid functions and acute phase reactant tests were normal. ECG revealed newly diagnosed atrial flatter. The patient was admitted to geriatrics ward for further investigation and treatment . Atrial flatter treatment was organised.Brain imaging and electroencephalography (EEG) were requested. Haloperidol drip was started for the patient whose agitation and aggression increased in the follow-up, but no response was obtained. Intamuscular haloperidol was administered for the safety of both the patient and healthcare workers. He was evaluated as delirium by psychiatry and antipsychotic and antidepressant treatments were discontinued. EEG revealed generalised epileptiform abnormalities on the basis of moderate to severe slowing of cerebral bioelectric activity. Antiepileptic treatment was started. The patient, whose consciousness tended to sleep, whose need for 02 developed and whose general condition deteriorated, was re-evaluated by neurology and transferred to the general intensive care unit with a prediagnosis of encephalitis. The patient who was intubated in intensive care unit had myoclonic seizures during follow-up. Cranial MRI showed nonspecific diffuse hyperintensities in the parietooccipital region and frontal cortex. The patient was consulted to neurology with the prediagnoses of autoimmune encephalitis and Creutzfeldt-Jakob disease and lumbar puncture was recommended. No cell was observed in CSF analysis and protein was normal. CSF culture was negative, HSV, Lyme, VDRL, CMV were negative in CSF. In CSF analysis, limbic panel was negative but 14.4.3 protein was positive. The patient was diagnosed as probable CreutzfeldtJakob Disease with clinical, EEG, imaging and CSF findings. The patient, whose myoclonic seizures continued, whose culture results showed growth and whose treatment was followed up by infectious diseases, developed cardiac arrest on the 64th day of intensive care unit hospitalisation and died. 80% to 95% of human prion diseases are sporadic CJD (2). After the first symptoms including psychiatric symptoms, cognitive functions deteriorate and a rapidly progressive dementia picture is observed.Myoclonus frequently accompanies in the later stages of the disease(3). The average age of onset of the disease is between 57 and 62 years; however, cases have also been described in people older than 80 years (4). Clinical presentation may be varied and vague, which makes the diagnosis difficult. In our 81-year-old patient with multiple comorbidities, we wanted to emphasise that this rare and fatal disease should be considered in the differential diagnosis of rapidly progressive cognitive impairment. Keywords: dementia, delirium, prion, CJD References 1. Weihl CC, Roos RP. Creutzfeldt-Jacob disease, new variant CreutzfeldtJacob disease and Bovine spongiform encephalopathy. Neurol Clin North Am. 1999;17:835–59. 2. Maddox RA, Person M, Minino A, et al. P.85: improving CreutzfeldtJakob disease incidence estimates by incorporating results of neuropathological analyses, United States, 2003–2011; Presented at Prion 2015 International Research Congress; Prion. Fort Collins. May 26–29, 2015; pp. S55–6. 3. Collins SJ, Sanchez-Juan P, Masters CL ve ark. (2006) Determinants of diagnostic investigation sensitivities across clinical spectrum of sporadic Creutzfeldt-Jakob disease. Brain 129:2278–87 4. Mortality from Creutzfeldt-Jakob disease and related disorders in Europe, Australia, and Canada. Ladogana A, Puopolo M, Croes EA, et al. Neurology. 2005;64:1586–1591.