How many people have Hirayama?
As of 2014 there had been less than 1500 described cases, starting with the patients in Hirayama’s 1959 study. The condition is disproportionately high in Asia but no conclusive reason has been found for this. To date the largest studies recorded are Japan (333 cases), India (279 cases), and China (179 cases).
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.
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.
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 causes Hirayama?
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.
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.
What is Hirayama disease?
Hirayama disease, also known as monomelic amyotrophy (MMA), is a rare cervical myelopathy that manifests itself as a self-limited, asymmetrical, slowly progressive atrophic weakness of the forearms and hands predominantly in young males.
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 causes Hirayama disease?
Is Hirayama disease a disability?
The person affected by the disease experiences a certain level of disability due to the disease. On rare occasion, the weakness associated with Hirayama’s disease may progress to the person’s opposite limb.
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 hereditary?
What kind of disease does Hirayama disease have?
Hirayama disease, also known as monomelic amyotrophy (MMA), is a rare cervical myelopathy that manifests itself as a self-limited, asymmetrical, slowly progressive atrophic weakness of the forearms and hands predominantly in young males.
How old do you have to be to have Hirayama disease?
Segmental denervation pattern further narrows the diagnosis to Hirayama disease (HD). HD is a sporadic and focal form of SMA that affects predominantly males between the ages of 15 and 25 years.
What kind of disease is Hirayama muscular atrophy?
Hirayama disease is also known as juvenile segmental muscular atrophy of distal upper extremity and brachial monomelic atrophy (MMA). This disease is prevalent in Asian countries and men seem to be more affected by this as compared to women.