Monomelic Amyotrophy (Hirayama Disease): Clinical Findings, EMG Characteristics and Differential Diagnosis
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Tarih
2015
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Erişim Hakkı
info:eu-repo/semantics/openAccess
Özet
Giriş ve Amaç: Monomelik amyotrofi (MA) üst ekstremitede bir veya birkaç myotomal tutuluşla giden ve bir süre sonra progresyonu duran benign motor nöron hastalığıdır. Semptom ve bulguların başlangıcında, benzer tabloyla seyredebilen hastalıklarla ayırımı yapmak zor olabilmektedir. Bu amaçla MA'nin klinik-EMG özellikleri ve ayırıcı tanılarını gözden geçirdik. Gereç ve Yöntem: 2005-2014 yılları içerisinde, Ege Üniversitesi Nörofizyoloji EMG laboratuvarına müracaat eden ve monomelik amyotrofi tanısı alan 14 olgu; yakınma, başlama yaşı, cinsiyet, tutulan myotom, EMG ve klinik özellikleri ile birlikte retrospektif değerlendirilmiştir. Takiplerde ALS gelişen, motor ileti bloğu saptanan veya mononöropatilere neden olabilecek olası etyolojik tanılı olgular çalışmaya alınmamıştır. Bulgular: On dört olgunun tümü üst ekstremite başlangıçlıydı. Kadın/ Erkek oranı 4/10, yakınmaların başlama yaşı ortalama 24±8.64 (ortalama ± standart sapma), minimum- maksimum başlangıç yaşı 16-40 yaş, ortanca değeri ise 21 idi. Bir olgu hariç tüm olgularda C8-T1 segmenti tutulmuştu. EMG takiplerinde denervasyon, fasikulasyon ve birleşik kas aksiyon potansiyel amplitudlerinde küçülme gözlenmiştir. Karşı ekstremitede yakınması olmayan asemptomatik üç olgumuzda elektrofizyolojik olarak ılımlı etkilenme bulguları saptanmıştır. Sonuç: MA' lı olgular, ALS'den, daha genç yaş erkeklerde başlaması, benign seyirli olması ve genelde 2-4 yıl içerisinde stabil klinik ve EMG bulgularıyla seyretmesi ile ayırt edilebilir. Asemptomatik görülen tarafta elektrofizyolojik olarak etkilenmiş olabileceği düşünülmeli.
Introduction and Objective: Monomelic amyotrophy (MA) is a benign motor neuron disease with a stationary stage after a progressive course, involving one or more myotomes in the upper limb. In the early stages, it may be difficult to discriminate the diseases presenting with similar clinical course. We reviewed clinical and EMG characteristics and differential diagnosis of MA for this purpose. Methods: Fourteen cases admitted to the Neurophysiology EMG laboratory of Medical School of Ege University, and diagnosed as having monomelic amytrophy between 2005 and 2014, were evaluated retrospectively along with complaints, age at onset, gender, the involved myotome, EMG and clinical characteristics. Cases with possible etiological diagnoses that might cause mononeuropathy or detected motor conduction block or developed ALS during follow-ups were excluded from the study. Results: All fourteen patients had onset in the upper limb. The female to male ratio was 4:10, the mean age of the onset was 24±8.64 years (mean ± Standard deviation), minimum- maximum age of onset was 16-40 years, with median value being 21. C8-T1 segments were involved in all cases except one. Follow-up EMG studies demonstrated denervation, fasciculations, and reduced compound muscle action potential amplitudes. Electrophysiologically, our three asymptomatic cases had moderate involvement in the contralateral limb without any complaints. Conclusion: Cases with MA can be distinguished from ALS by symptom onset at younger ages in men, its benign course and generally with stabilization of clinical and EMG findings in 2 to 4 years. Asymptomatic side should also be considered to be affected electrophysiologically.
Introduction and Objective: Monomelic amyotrophy (MA) is a benign motor neuron disease with a stationary stage after a progressive course, involving one or more myotomes in the upper limb. In the early stages, it may be difficult to discriminate the diseases presenting with similar clinical course. We reviewed clinical and EMG characteristics and differential diagnosis of MA for this purpose. Methods: Fourteen cases admitted to the Neurophysiology EMG laboratory of Medical School of Ege University, and diagnosed as having monomelic amytrophy between 2005 and 2014, were evaluated retrospectively along with complaints, age at onset, gender, the involved myotome, EMG and clinical characteristics. Cases with possible etiological diagnoses that might cause mononeuropathy or detected motor conduction block or developed ALS during follow-ups were excluded from the study. Results: All fourteen patients had onset in the upper limb. The female to male ratio was 4:10, the mean age of the onset was 24±8.64 years (mean ± Standard deviation), minimum- maximum age of onset was 16-40 years, with median value being 21. C8-T1 segments were involved in all cases except one. Follow-up EMG studies demonstrated denervation, fasciculations, and reduced compound muscle action potential amplitudes. Electrophysiologically, our three asymptomatic cases had moderate involvement in the contralateral limb without any complaints. Conclusion: Cases with MA can be distinguished from ALS by symptom onset at younger ages in men, its benign course and generally with stabilization of clinical and EMG findings in 2 to 4 years. Asymptomatic side should also be considered to be affected electrophysiologically.
Açıklama
Anahtar Kelimeler
Nörolojik Bilimler
Kaynak
Journal of Neurological Sciences (Turkish)
WoS Q Değeri
Scopus Q Değeri
Cilt
32
Sayı
3
