Nesim KUĞU, Orhan DOĞAN, Önder KAVAKÇI, İbrahim TERLEMEZ

Frontotemporal demans: Bir olgu sunumu

Fronto temporal demans (FTD), orta yaşlarda Alzheimer hastalığından sonra en sık görülen primer dejeneratif demans türüdür. Genellikle 45-65 yaşları arasında sinsi bir şekilde başlayan, her iki cinsiyette eşit sıklıkta görülen, kişilik, davranış ve duygulanım değişikliklerinin yanında içgörü kaybı, perseveratif ve stereotipik davranışlar ve yeme alışkanlıklarında değişikliklerin görülebildiği bir hastalıktır. Bu hastalarda nörolojik muayene, rutin elektroensefalogram (EEG) normaldir ve beyin görüntülemede, frontotemporal loblarda fokal anormallikler vardır. Yürütücü işlevlerde belirgin yetersizlikler vardır. Bu yazıda, hastalığı ellili yaşlarda sinsice başlayan, kişilik değişikliği ve davranışta bozulmanın en çarpıcı belirtiler olduğu, içgörü kaybı, hiperoralite ve diyetle ilişkili değişiklikler, kompülsif ve stereotipik davranışlar, künt duygulanım, konuşma miktarında azalma ve dışa vuran davranışlarda yavaşlama, kişisel bakımda bozulma, altına idrar ve gaita kaçırmanın eşlik ettiği, demans için pozitif aile öyküsünün bulunduğu bir erkek FTD olgusu sunulmuştur. Hastada nörolojik muayene ve rutin EEG normaldi. Kraniyal Manyetik Rezonans Görüntüleme’de (MRG), sağ hemisferde belirgin olmak üzere, dorsolateral ve orbitofrontal bölgede asimetrik atrofi vardı. Hastaya yapılan Tc-99 HMPAO tek fotonlu emisyon tomografisinde (SPECT), sağ frontal ve paryetal lobları içine alan geniş bir alanda asimetrik hipoperfüzyon saptandı. Frontal yürütücü işlevlerde yetersizlik vardı. Hastada, ketiapin 300 mg/gün tedavisiyle davranışsal belirtilerde kısmen düzelme görüldü. Bu olgu, orta yaşlarda sinsi şekilde psikiyatrik belirtilerle başlayan olgularda mutlaka ayrıntılı öykü, fizik ve nörolojik muayene ve beyin görüntüleme dahil tıbbi inceleme yapılması gerektiğini göstermektedir.

Frontotemporal dementia: A case presentation

Frontotemporal dementia (FTD) is, next to Alzheimer disease, the most frequently encountered form of primary degenerative dementia among middle-aged subjects. It generally begins insidiously between the ages of 45 and 65 years, and is seen in both genders with equal frequency. It is characterized by changes in personality, behavior, and affect, in addition to loss of insight, perseverative and stereotypical behaviours, and changes in eating habits. These patients appear normal upon neurological examination and routine electroencephalography (EEG), but brain imaging reveals focal abnormalities in the frontotemporal lobes. There is significant deficiency in executive functions. This study describes a male patient with FTD that had began insidiously in his fifties, and in whom the changes in personality and impairment in behavior were the striking symptoms (loss of insight, hyperorality, and dietary changes; compulsive and stereotypical behaviours; blunted affect, decrease in the amount of speech and retardation in expressional behaviours; impairment in personal hygiene; urinary and fecal incontinence) along with a family history of dementia. The neurological examination and routine electroencephalogram (EEG) of the patient were normal. His cranial magnetic resonance imaging (MRI) revealed asymmetric atrophy, particularly in the right hemisphere at the dorsolateral and orbitofrontal regions. Tc-99 HMPAO single photon emission tomography (SPECT) detected asymmetric hypoperfusion within an extended region, including the right frontal and parietal lobes. There was deficiency in frontal executive functions. Partial improvement in behavioral symptoms was achieved by treating the patient with quetiapine 300 mg/day. The present case study showed that detailed history, as well as medical examination including physical and neurological examination and brain imaging, must be performed in the case of middle-aged patients with insidious onset of psychiatric symptoms.

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