Is Hirayama disease curable?

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.

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.

Is Hirayama disease painful?

Technically, it is termed as a lower motor neuron disorder. Classical findings include muscle wasting (atrophy) and weakness of the forearms and hands, either on one or both sides. There is, however, no change in sensation and the patient doesn’t experience any pain.

Is Hirayama disease hereditary?

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.

Can Hirayama disease affect legs?

It has been suggested that Hirayama disease is a variant of motor neurone disease. Some studies have reported that there may be electromyographic damage to the muscles of the thoracic wall and lower limbs lending weight to the hypothesis of a degenerative origin [8].

What is Hirayama?

Hirayama disease, a rare neurological disease, is characterized by insidious unilateral or bilateral muscular atrophy and weakness of the forearms and hands, without sensory or pyramidal signs. [1] The disease primarily affects men in the second to third decades.

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 amyotrophy?

Muscle atrophy can occur due to malnutrition, age, genetics, a lack of physical activity or certain medical conditions. Disuse (physiologic) atrophy occurs when you don’t use your muscles enough. Neurogenic atrophy occurs due to nerve problems or diseases.