Dilek KAZANCI, Büşra TEZCAN, Çilem BAYINDIR DİCLE, İbrahim MUNGAN, Derya ADEMOĞLU, Müçteba CAN

Fahr Sendromu Olan Bir Hastada Yoğun Bakımdaki Sedasyon Problemi

Nadir görülen nörolojik bir sendrom olan Fahr Sendromu, sporadik ya da genetik geçişli bazal ganglion kalsifikasyonuyla karakterizedir. Fahr Sendromu’nun patofizyolojisiyle ilgili olarak; kalsiyum metabolizma bozukluğu, metastatik kalsiyum depozitleri ve artmış serbest radikal üretimi gibi bazı hipotezler mevcuttur. Hastalar genellikle ekstrapiramidal semptomlarla tanı alsa da, serebellar disfonksiyon, konuşma bozuklukları, demans ve nöropsikiyatrik semptomlarla da başvurabilirler. Bu olgu sunumunda amacımız, intihar amaçlı karbamazepin alımı sonrası yoğun bakımımıza kabul edilen 49 yaşındaki kadın hastadaki sedasyon yetersizliğinden bahsetmektir. Hastaya 1.5 mcg kg-1 sa-1 deksmedetomidin infuzyonu ve 1.5 mg midazolam bolusları uygulandığı halde yeterli sedasyon düzeyine ulaşılamamıştır. Bu durum uzun süreli antiepileptik ve antidepresan kullanımına bağlı olabilir. Diğer yandan da, benzersiz bir sedatif ajan olan deksmedetomidin spesifik ve selektif bir α2 agonistidir. Hastamızdaki yaygın serebral kalsifikasyon, α2 reseptör aktivitesini bozmuş olabilir. Bunun yanında Fahr Sendromu’nun muhtemel nedenlerinden biri olan kalsiyum metabolizma bozukluğu da α2 adrenoreseptör aracılıklı kalsiyum ilişkili nörotransmitter salınımını etkilemiş ve böylece deksmedetomidinin etkinliğini azaltmış olabilir

Sedation Failure in a Patient with Fahr Syndrome in the Intensive Care Unit

Fahr Syndrome, which is a rare neurologic syndrome, is characterized by sporadic or geneticallyinherited basal ganglion calcification. There are some hypotheses about the pathophysiology ofFahr Syndrome related to a defect in calcium metabolism, metastatic calcium deposits andincreased free radical production. Although patients are usually diagnosed with extrapyramidalsymptoms, they may also present with cerebellar dysfunction, speech disorders, dementia andneuropsychiatric symptoms. We aimed to discuss sedation failure with dexmedetomidine andmidazolam in a 49-year-old female patient with Fahr Syndrome who was admitted to our intensive care unit after suicidal carbamazepine overdose in this case report. Adequate sedation levelscould not be reached although infusion of 1.5 µg kg-1 h-1 dexmedetomidine and bolus injectionsof 1.5 mg midazolam were administered. This may be due to the tolerance to sedatives developedby long-term use of antidepressant and antiepileptic agents. On the other hand; the uniquesedative agent dexmedetomidine is a specific and selective α2 agonist and the widespread intracerebral calcification in our patient may have impaired α2 receptor activity. Besides, calciummetabolism disorder, one of the probable causes of Fahr Syndrome, may affect calcium-mediatedinhibition of neurotransmitter release through α2 adrenoreceptors and reduced the effectivenessof dexmedetomidine.

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