Hirayama disease/Hirayama Hastaligi

Hirayama disease (HD) is a rare benign lower motor neuron disorder in the young with a male preponderance and usually affecting one upper extremity. It is characterized by the insidious onset and progressive weakness and wasting of a distal extremity. Generally, this disease is considered as a benign and nonprogressive motor neuron disease that stabilizes within five years of onset. We describe a 20-year-old male patient who experienced left distal upper extremity amyotrophy with no sensorial abnormality. Keywords: Hirayama hastaligi, monomelik amyotrofi, motor noron hastaligi Hirayama Hastaligi (HH), genc erkeklerde daha sik gorulen, genellikle tek ust ekstremitreyi tutan, nadir gorulen benign bir alt motor noron hastaligidir. Distal ekstremiteyi tutan ilerleyici gucsuzluk ve sinsi baslangic ile karakterizedir. Genellikle baslangicindan sonra 5 yil icinde stabilize olan, benign ve nonprogresif bir hastalik olarak kabul edilir. Biz burada duysal yakinmanin eslik etmedigi sol ust ekstremite distal amyotrofi bulgulari olan 20 yasinda bir erkek hastayi sunuyoruz. Anahtar Kelimeler: Hirayama disease, monomelic amyotrophy, motor neuron disease