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

Background: Motor Neuron Diseases (MNDs) are progressive neurological disorders characterized by the degeneration of upper and/or lower m otor neurons. T he t erm M ND includes Amyotrophic lateral sclerosis (ALS), Primary Lateral Sclerosis (PLS), Hereditary Spastic Paraparesis (HSP), Progressive Muscular Atrophy (PMA), Spinal Muscular Atrophy (SMA), Hirayama Disease, Kennedy Syndrome, Poliomyelitis and Postpolio syndrome (PPS). While both upper and lower motor neurons are involved in ALS, only upper motor neurons are involved in Primary Lateral Sclerosis (PLS), Hereditary Spastic Paraparesis (HSP), and only lower motor neurons are involved in Progressive Muscular Atrophy (PMA), Spinal Muscular Atrophy (SMA), Hirayama Disease, Kennedy Syndrome, Poliomyelitis and Postpolio syndrome (PPS). We aimed to investigate the clinical, demographic, and electrophysiological characteristics of MND patients who presented to our university hospital in Şanlıurfa 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. 18 patients were excluded from the study because of insufficient file data. Patient data, including age, gender, presenting complaints, examination findings, family history, and electrophysiological features, were recorded. Results: Of the 172 patients included in our study, 103 were male and 69 were female. 54 patients were diagnosed with ALS, 3 with HSP, 2 with Kennedy syndrome, 21 with SMA, 4 with Hirayama disease, 82 with poliomyelitis sequelae, and 6 with PPS. 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 HSP 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 i n t he m edian n erve w as observed i n 2 other patients. T he a verage a ge of 6 P PS patients w as 5 4.25±8.057. A mong t he patients diagnosed with Hirayama Disease, 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. Conclusions: Recognizing the symptoms, signs, and risk factors of MND is crucial for physicians to facilitate early diagnosis. [ABSTRACT FROM AUTHOR]