GUNTUR: As many as 10 cases of Hirayama disease, a rare neurological disease, were detected in the district, NV Sundarachary, head of the neurology department of the Guntur GGH, said. He added that number of such cases was on the rise in recent times and the patients were being treated at the hospital. First detected in Japan in 1959, there are only 1,500 reported cases of people suffering from Hirayama disease in the entire world, the doctor added.
It was first understood that the disease was dreadful. However, it does not affect the life expectancy of a human, it was found later.
The doctor explained that the disease starts while the person is in his 20s and 30s and causes atrophic weakness in forearms and hands. However, muscles around the shoulder area stay unaffected.
Sundarachary explained that the progress of the diseases is slow and, at first, it affects only one hand. The disease becomes severe in a period of 10-20 years and then becomes stable. It sometimes affect the other hand too, he added. The disease is also known as monomelic amyotrophy or MMA and is more common in young males.
MMA symptoms usually progress slowly for two to five years and then remain stable, he added. The weakness may or may not progress to the opposite limb. The doctor said the disease affects front portion of the cervical cord, which has muscles supplied to the hands.
Doctory Sundarachary said that impaired micro-circulation at the front portion of lower cervical spinal cord is the reason for the damange in C7, C8 and T1 myotomes. He explained that the atrophy of forearm is called oblique atrophy.
The disease is thought to be due to the disproportionate growth of spinal cord at cervical level, he said. Hirayama disease is a self-limiting disorder. Early diagnosis is necessary to check progress of the disease, which can be done by using a cervical collar.
‘Only 1,500 such cases’
The disease starts while the person is in his 20s and 30s and causes atrophic weakness in forearms and hands. However, muscles around the shoulder area stay unaffected. First detected in Japan in 1959, there are only 1,500 reported cases of people suffering from it, NV Sundarachary said.