Motor Nöron Hastalarının Klinik, Demografik ve Elektrofizyolojik Özellikleri: Tek Merkez Deneyimi

Öz

Amaç: Motor nöron hastalığı (MNH) üst ve/veya alt motor nöronların dejenerasyonu ile seyreden progresif nörolojik bozukluklardır. Şanlıurfa ilinde üniversite hastanemize başvuran MNH hastalarının klinik, demografik ve elektrofizyolojik özelliklerinin incelenmesini amaçladık. Gereç ve Yöntem: 2018-2023 yılları arasında Nöroloji ve Nöromusküler polikliniklerinde ve Elektronörofizyoloji laboratuvarında MNH tanısı konarak takip edilen 190 hastanın dosyaları elektronik ortamda retrospektif olarak incelendi. Hastaların yaş, cinsiyet, başvuru şikâyeti, muayene bulgusu, aile öyküsü ve elektrofizyolojik özellikleri kayıt altına alındı. Bulgular: Amyotrofik lateral skleroz (ALS) hastalarının 22’ si kadın, 32’ si erkekti. 15 hastanın başlangıç semptomu bulber iken, 39’ unun spinal başlangıçlıydı. 17 hastanın ilk semptomu alt ekstremiteden, 22’ sinin üst ekstremiteden başlamıştı. 36 hastanın ulnar sinir distal motor latansları (DML) median sinir DML’den kısayken, bu hastaların 24’ünde ulnar sinir birleşik kas aksiyon potansiyeli (BKAP) median sinir BKAP’larından yüksek bulundu. 3 Herediter Spastik Paraparezi hastamızın 1’i komplike tipteydi. Poliomiyelit sekeli nedeniyle Elektromiyografi (EMG)’si yapılan hastaların tibialis anterior kası 55 hastada bilateral, 7 hastada tek taraflı etkilenmişti. 2 hastada ileti hızında yavaşlama saptanırken farklı 2 hastada median sinirde tuzak nöropati izlendi. 6 post polio sendromu hastamızın yaş ortalaması 54.25+8.057’ idi. Hirayama Sendromu tanılı hastalarımızın 1’i kadın, 3’ü erkekti. 3 Hastamiz 20-30 yaş arasındayken, 1 hastamiz 58 yaşındaydı. 1 hastamızın servikal görüntülemesinde C6-7 düzeyinde miyelomalazi saptandı. 2 hastanın semptomu olmadığı halde iki yanlı tutulumu varken diğer 2 hastada tek taraflı tutulum vardı. Sonuç: Hekimlerin MNH’nın belirti, bulgu ve risk faktörlerini bilmeleri erken tanı koyabilmeleri açısından önemlidir.

Anahtar Kelimeler

• nöromüsküler hastalıklar
• elektromiyografi
• motor nöron

Clinical, Demographic and Electrophysiological Characteristics of Motor Neuron Patients: A Single Centre Experience

Öz

Objective: Motor Neuron Diseases (MNDs) are progressive neurological disorders characterized by the degeneration of upper and/or lower motor neurons. We aimed to investigate the clinical, demographic, and electrophysiological characteristics of MND patients who presented to our university hospital in XXX province. Materials and Methods: 190 patients diagnosed and followed up with MND in the Neurology and Neuromuscular clinics and Electroneurophysiology laboratory were retrospectively analyzed from the medical records of patients between 2018 and 2023. Patient data, including age, gender, presenting complaints, examination findings, family history, and electrophysiological features, were recorded. Results: Among the amyotrophic lateral sclerosis (ALS) patients, 22 were female and 32 were male. Fifteen patients had bulbar-onset symptoms, while 39 had spinal-onset symptoms. The initial symptom in 17 patients originated from the lower extremities, and in 22 patients, it started in the upper extremities. In 36 patients, ulnar nerve distal motor latencies (DML) were shorter than median nerve DML, and 24 of these patients had higher ulnar nerve compound muscle action potentials (CMAP) than median nerve CMAP. One of the 3 patients with Hereditary Spastic Paraparesis had a complicated type. Electromyography (EMG) of the tibialis anterior muscle in patients with sequelae of poliomyelitis revealed bilateral involvement in 55 patients and unilateral involvement in 7 patients. Slowing of conduction velocity was detected in 2 patients, and trap neuropathy in the median nerve was observed in 2 other patients. The average age of 6 post-polio syndrome patients was 54.25±8.057. Among the patients diagnosed with Hirayama Syndrome, 1 was female, and 3 were male. Three patients were between 20-30 years old, while one of them was 58 years old. Myelomalacia at the C6-7 level was detected in the cervical imaging of one patient, and two patients had bilateral involvement without symptoms, while the other 2 had unilateral involvement. Conclusion: Recognizing the symptoms, signs, and risk factors of MND is crucial for physicians to facilitate early diagnosis.

Anahtar Kelimeler

• neuromuscular
• electromyography
• motor neuron

Kaynakça

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