Can Hirayama disease be cured?
Can Hirayama disease be cured?
There is no permanent cure for this neurological disease but wearing a cervical collar can stop its progress if it is in the benign stage.
What does Amyotrophy mean?
Amyotrophy is an asymmetric lower limb motor neuropathy also known as diabetic lumbosacral plexus neuropathy and Bruns-Garland syndrome. Patients typically present with an asymmetric, painful muscle wasting and weakness affecting the lower limbs and loss of reflexes and objective weakness on examination.
What causes Hirayama disease?
The condition is caused by a tight dural sac in the cervical canal that leads to chronic ischemic changes to the anterior horn cells. Although commonly considered a non-progressive and self-limiting disease, this has been noted to be a source of significant disability for some affected individuals.
How common is Hirayama disease?
Hirayama’s disease is a rare, clinical variant of amyotrophic lateral sclerosis where distal muscles are involved, more compared to proximal muscles and vice-versa occurs only in 10% cases,[4,5] just like in our case which makes our case even more interesting.
How is Hirayama disease diagnosed?
The key to diagnose this disease is based on the typical clinical features and dynamic MRI study when the neck is flexed. MR studies in flexion show not only the anterior displacement of the posterior wall but also a well-enhanced crescent-shaped lesion in the posterior epidural space of the lower cervical canal.
What is Polymyopathy?
Overview. Myopathy is a general term referring to any disease that affects the muscles that control voluntary movement in the body. Patients experience muscle weakness due to a dysfunction of the muscle fibers. Some myopathies are genetic and can be passed from parent to child.
What causes Amyotrophy?
Males are more likely to develop the disease than females. The exact cause of monomelic amyotrophy is unknown. It is possible that the disease is caused by movement of the sac that surrounds the spinal cord due to repeated downward movement (flexion) of the neck.
How do you treat Hirayama?
Application of cervical collar for 3 to 4 years has been generally advocated for the treatment of Hirayama’s disease because progression of signs and symptoms is usually expected to cease within several years.
Is Hirayama disease genetic?
There is a debate as to whether Hirayama disease is an unusual neck flexion induced cervical myelopathy or an intrinsic motor neuron disease. Despite being a sporadic disorder, familial forms have been occasionally described, with either autosomal recessive or dominant inheritance.
When does Hirayama stop?
As “Hirayama disease” is considered a self-limited disease and often stops progressing after 1–5 years of onset, the mainstay of treatment consists of preventing neck flexion using a cervical collar to halt further progression.
What do you need to know about monomelic amyotrophy?
View Full Treatment Information. Definition. Monomelic amyotrophy (MMA) is characterized by progressive degeneration and loss of motor neurons, the nerve cells in the brain and spinal cord that are responsible for controlling voluntary muscles.
What is the medical definition of monomelic tumors?
Medical Definition of monomelic : relating to or affecting only one limb monomelic tumors of the leg Learn More About monomelic Dictionary Entries Near monomelic
What is the treatment for monomelic amyotrophy ( MMA )?
Treatment consists of muscle strengthening exercises and training in hand coordination Monomelic amyotrophy (MMA) is characterized by progressive degeneration and loss of motor neurons, the nerve cells in the brain and spinal cord that are responsible for controlling voluntary muscles.
