Hirayama Hastalığı (Monomelik Amyotrofi)

Öz

Hirayama Hastalığı (HH), genç erkeklerde daha sık görülen benign bir alt motor nöron hastalığıdır. Distal ekstremiteyi tutan ilerleyici güçsüzlük ve sinsi başlangıç ile karekterizedir. Genellikle başlangıcından sonra 5 yıl içinde stabilize olan, benign ve nonprogresif bir hastalık olarak kabul edilir. Biz bu bildiride duysal yakınmanın eşlik etmediği progresif amyotrofi bulguları olan 23 yaşında bir kadın hastayı sunuyoruz.©2008, Fırat Üniversitesi, Tıp Fakültesi

Anahtar Kelimeler

• Hirayama hastalığı, monomelik amyotrofi, motor nöron hastalığı

Hirayama Disease (Monomelic Amyotrophy)

Abstract

Hirayama Disease (HD) is a benign lower motor neuron disorder in the young with male preponderance. It is characterized by insidious onset and progressive weakness and wasting of a distal extremity . Generally, this disease is considered a 'benign' and non-progressive motor neuron disease, which stabilizes within five years of onset.We describe a 23-year-old famele patient who experienced progressive amyotrophy with no sensorial abnormality.©2008, Firat University, Medical Faculty.

Keywords

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Kaynakça

1. Fetoni V, Briem E, Carrara F, Mora M, Zeviani M. Monomelic amyotrophy associated with the 7472insC mutation in the mtDNA tRNA gene. Neuromusculer Disorders 2004; 14: 723-726.
2. Tunçbay T, Tunçbay E. Nöromüsküler Hastalıklar. İzmir: Ege Üniversitesi Basım Evi, 2000: 136-140. 3. Hirayama K,Toyokura Y, Tsubaki T. Juvenile musculer atrophy unilateral upper extremity- a new clinical entity. Psychiatr Neurol Jpn 1959; 61: 2190-2197.
3. Gourie Devi M, Nalini A. Long term follow-up of 44 patients with brachial monomelic amyotrophy. Acta Neurol Scand 2003; 107: 215-220.
4. Nalini A, Lokesh E, Ratnavalli E. Familial monomelic amyotrophy: a case report from India. Journal of Neurological Sciences 2004; 220: 95-98.
5. Massa R, Scalise A, Iani C, Palmieri MG, Bernardi G. Delayed focal involvement of upper motor neurons in the Madras pattern of motor neuron disease. Electroencephalography and clinical Neurophysiology 1998; 109: 523-526.
6. Hamano T, Mutoh T, Hirayama M, Ito K, Kimura M, and et al. MRI findings of bening monomelic amyotrophy of lower limb. J neurological Sciences 1999; 165: 184-187.
7. Münchau A, Rosenkranz T. Benign Monomelic Amyotrophy of the lower limb- case report and brief review of the Literature. Eur Neurol 2000; 43: 238-240.
8. Gourei Devi M, Nalini A. Sympathetic skin response in monomelic amyotrophy. Acta Neurol Scand 2001; 104: 162-166.

9. Rowin J, Meriggioli MN, Cochran EJ. Monomelic amyotrophy with late progression. Neuromusculer Disorder 2001; 11: 305- 308.
10. Sullivan O, McLeod J. Distal chronic spinal muscular atrophy involving the hands. J Neurol Neurosurg Psychiatry 1978; 41: 653-658.
11. Kiernan MC, Lethlean AK, Blum PW. Monomelic amyotrophy: non progressive atrophy of the upper limb. J.Clinical Neuroscience.1999; 6: 353-355
12. Schroder R, Keller E, Flacke S, Schmidt S, Pohl C, Klockgether T,et al. MRI findings in Hirayama’s Disease: flexion- inducedcervical myelopathy or intrinsic motor neuron disease? J Neurol 1999; 246: 1069-1074.
13. Kabul Tarihi:22.11.2007