Ben Herm-Becker Myodystrophy-(UK)

Name: Ben Herm                         
Sex: Male
Country: UK
Age: 22
Diagnoses: 1. Becker Myodystrophy 2. Dysfunction of liver
Admission Date: August 8, 2012
Days Admitted to Hospital: 27 days

Before treatment:
The patient was falling down when he got up or went to bed without obvious reasons. Then he had pain in his waist. But he didn't receive special treatment. 1 year later, his parents found the patient had less and less active movement. He had poor balance control. He fell down frequently. About 2 years later, the patient suffered from difficulty with standing from the sitting position. He also had difficulty with getting up and downstairs. He suffered pain in his back and went to see a plastic surgeon in 2001. The doctor found he had muscle atrophy. Then the patient went to many hospitals for treatment. He went to London for treatment in 2003. After cellular immunity examination and electromyogram examination, he was diagnosed with Becker Myodystrophy. The disease progressed gradually. The patient suffered from muscle weakness of the limbs. There was obvious muscle atrophy in the bilateral pelvic girdle and lower limbs. The muscle weakness was aggravated. He received physical therapy and his walking ability improved. Before the treatment, the patient could turn over by himself. But he had difficulty with sitting up from clinostatism and needed both hands for support. He had difficulty with standing up. His walking gait presently is duck-like, walking slowly, his endurance was poor. The swallowing ability is weak.

Nervous System Examination:

There was mild muscle atrophy in bilateral deltoid, biceps brachii and triceps brachii. There was severe muscle atrophy in bilateral pelvic girdle, iliopsoas, quadriceps femoris and biceps femoris. Bilateral Gastrocnemius had pseudohypertrophy. The shrug muscle of the right side was weak. The muscle strength of upper limbs' near-end was level 4. The muscle strength of both upper limbs' far-end was level 5-. The muscle strength of both lower limbs' near-end was level 3, the muscle strength of lower limbs' far-end was level 2. Both lower limbs couldn't lift off the bed surface, but he still could bend his knees slowly. He could turn over autonomously, but turning over was slow. He needed assistance with upper limbs to sit up or stand up from clinostatism, at the same time he needed a special position in clinostatism to sit up or stand up. The Gower sign was positive. He could walk independently and the walking gait was abnormal, presenting a waddling position. The walking endurance was poor and exhausted easily. The muscle tone of both upper limbs was almost normal. The muscle tone of both lower limbs was low. The tendon reflex of four limbs was reduced. Left abdominal reflexes were weakened. Right abdominal reflexes were normal. Bilateral palm jaw reflex was negative. Bilateral Hoffmann's sign and Rossolimo's sign were negative. The bilateral Babinski's sign was negative. The deep and shallow sensation throughout the body was normal through course measurement. The patient was able to finish the finger-to-nose test, the rapid rotation test and digit opposition test almost normally. He was unable to finish the Heel-knee-shin test. He had poor balance control. There were no signs of meningeal irritation.

Treatment:
We initially gave Ben Herm a complete examination. The patient received treatment to improve the blood circulation in order to increase the blood supply to the damaged nerves and to nourish the neurons. He also received treatment to protect liver function and reduce creatase. This was accompanied with physical rehabilitation training.

Post- treatment:
Ben Herm's condition has improved. The liver function is better. The muscle strength of both lower limbs is stronger than before. He is able to bend knees quickly. The control ability of adduction and abduction of both lower limbs in curtsey position has increased. He can turn over more quickly. The muscle strength in the waist and back is stronger. He has more ability to control his body. The walking endurance is stronger. The fatigue times are more reduced than before when he does rehabilitation exercises.