NADİR GÖRÜLEN NÖROPSİKİYATRİK BOZUKLUK: FAHR HASTALIĞI

Giriş: Fahr Hastalığı; bazal gangliyon, talamus, hipotalamus, serebral korteks, serebellar subkortikal beyaz cevher ve dentat nukleusta simetrik kalsifikasyonlar ile karakterize nörolojik bir oluşumdur. Fahr Hastalığı genellikle asemptomatik olmasına rağmen çeşitli nörolojik ve psikiyatrik bulgularla ortaya çıkabilir. Hastalığın klinik özellikleri değişkendir ancak psikoz, demans, Parkinsonizm, nöbetler, başağrısı, dizartri, spastisite ve spastik paralizi, göz hareket bozuklukları ve atetoz ortaya çıkış bulgusu olarak görülebilir. Hastalığa özel tedavi seçeneği yoktur. Fahr hastalığı ekstrapiramidal bulgular olmadan, kognitif bozukluk ve davranışsal değişikliklerle de ortaya çıkabilir. Tedavi eşlik eden şikayet ve bulgulara göre düzenlenmelidir. Olgu: 76 yaşında kadın hasta bellek kaybı, saldırganlık, yönelim bozukluğu ve sinirlilik atakları ile başvurdu. Klinik veriler, laboratuvar test sonuçları ve nörogörüntüleme bulguları temelinde hastaya Fahr hastalığı tanısı koyuldu. Sonuç: Klinik bulgulardaki çeşitlilik nedneiyle Fahr hastalığı tanısı koymak zor olabilir. Nöropsikiyatrik ve serebellar bulguları olan, intraserebral yaygın kalsifikasyon saptanan hastalarda ayırıcı tanıda Fahr hastalığı akla gelmelidir

A RARE NEUROPHSYCHIATRIC DISORDER: FAHR’S DISEASE

Introduction: Fahr’s disease is a rare neurological entity characterized by symmetric calcifications at basalganglia, thalamus, hippocampus, cerebral cortex, cerebellar subcortical white matter and dentate nucleus.Patients with Fahr’s disease which is often asymptomatic, can be presented with diverse neurological andpsychiatric findings. Psychosis, dementia, parkinsonism, seizures, headache, dysarthria, spasticity and spasticparalysis, eye movement impairments and athetosis may be seen as presenting features. Still there is no specifictherapeuthicy option for the disease. Fahr’s disease may be presented with cognitive impairment and behavioralchanges without extrapyramidal signs. Treatment should be organized according to accompanying symptoms andfindings.Case: A 76 year old female patient who presented with episodes of memory loss, aggressivity, disorientation andirritability. On the basis of clinical data, laboratory test results and neuroimaging findings, the patient wasdiagnosed with Fahr’s disease.Conclusion: Due to the polymorphic clinical manifestations diagnosis of Fahr’s disease could be challenging.Fahr's disease should be considered in the differential diagnosis of patients with neuropsychiatric and cerebellarfindings and intracerebral extensive calcification.

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