Upper Thoracic Myelopathy Caused by Delayed Neck Extensor Weakness in Myotonic Dystrophy.
10.5535/arm.2012.36.4.569
- Author:
Han Kyeong SON
1
;
Young Sun CHA
;
Hwi SUH
;
Chang Seok KI
;
Yong Beom SHIN
Author Information
1. Department of Rehabilitation Medicine, Pusan National University School of Medicine, Busan 602-739, Korea. yi0314@gmail.com
- Publication Type:Case Report
- Keywords:
Myotonic dystrophy;
Neck weakness;
Thoracic myelopathy
- MeSH:
Adult;
Animals;
Female;
Humans;
Hypertrophy;
Ligamentum Flavum;
Longitudinal Ligaments;
Lordosis;
Magnetic Resonance Imaging;
Muscular Diseases;
Muscular Dystrophies;
Myotonic Dystrophy;
Neck;
Neck Muscles;
Spinal Cord Compression;
Spinal Cord Diseases;
Spine;
Stress, Mechanical;
Stress, Psychological;
Urinary Incontinence
- From:Annals of Rehabilitation Medicine
2012;36(4):569-572
- CountryRepublic of Korea
- Language:English
-
Abstract:
Myotonic dystrophy is the most common autosomal dominant myopathy in adults. Our patient, a 41 year-old female suffering from myotonic muscular dystrophy, developed upper thoracic myelopathy due to hypertrophy of the ligamentum flavum and the posterior longitudinal ligament. She had a typical hatchet face and ptosis with "head hanging forward" appearance caused by neck weakness. Motor weakness, sensory changes and severe pain below T4 level, along with urinary incontinence began 3 months ago. Genetic and electrodiagnostic studies revealed myotonic dystrophy type 1. Magnetic resonance imaging of the spine showed loss of cervical lordosis and spinal cord compression due to hypertrophied ligamentum flavum and posterior longitudinal ligament at T1 to T3 level. We concluded that her upper thoracic myelopathy was likely related to the thickness of the ligamentum flavum and posterior longitudinal ligament due to repetitive mechanical stress on her neck caused by neck muscle weakness with myotonic dystrophy.
