Is Hirayama disease curable?

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.

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.

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).

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.

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 hereditary?

Unlike other forms of lower motor neuron diseases, Hirayama’s disease is not one that is believed to be hereditary. For people who have this disease, ‘fasciculations,’ or involuntary muscle twitches, are rare.

What causes Hirayama disease?

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.