Didi Shirah-Hereditary Inclusion Body Myopathy-(Israel)-Posted on April 9th, 2018

Name: Didi Shirah
Sex: Female
Nationality: Israeli
Age: 28Y
Diagnosis: Hereditary Inclusion Body Myopathy (HIBM)
Date of Admission: Jan. 8th, 2017
Treatment hospital/period: Wu Medical Center/14 days

Before treatment:
The patient felt it was easy for her to fall down 6 years ago and it was also hard  to raise her legs. Her condition got worse day by day and she had a waddling gait so she went to a local hospital and did an EMG. The doctor thought she may have muscle disease and she was then diagnosed with HIBM 6 years ago by doing a gene test. Her disease progressed fast and her leg functions got worse. At present it is hard for her to get up from bed, turn her body over or sit up. She is able to walk with help but she is unable to go up or down stairs.
Her spirit, diet and sleep are good. Her urination and defecation functions are normal.

Admission PE:
Bp: 120/70mmHg, Hr: 72/min, breathing rate: 18/min, body temperature: 36.8 degrees. Height was 166cm, weight was 64Kg. Nutrition status is good with normal physical development. There is no injury or bleeding spots of her skin and mucosa and no tonsil swelling. The chest development is normal and breathing sounds of both lungs were clear with no obvious dry or moist rales. The heart beat is powerful with regular cardiac rhythm and no obvious murmur in the valves. The abdomen was flat and soft with no masses or tenderness. The liver and spleen were normal by touch, shifting dullness was negative, the spinal column was normal and there was mild edema of the left ankle joint.

Nervous System Examination:
Patient was alert, mental status was fine and her speech was clear. Her memory, orientation and calculation abilities were normal . Both pupils were equal in size and round,  diameter of 3 mm, react well to light and the eyeballs can move freely. Bilateral forehead wrinkle and nasolabial fold are symmetrical, she could close eyes powerfully, showing the teeth is normal and she could bulge her cheek without air leakage. The bilateral soft palate could lift as normal but she could not turn her head or shrug powerfully. Muscle power of  the arms were 5- degrees, and of the legs 2+ degrees. The muscle tone  and tendon reflex of the 4 limbs were normal. Abdominal reflex could not be induced by examination, the leg muscle volume was not good enough. Her bilateral palm-jaw reflex were negative, the Hoffman sign, Rossilimo sign and Babinski sign of both sides were negative, her sensory examinations were normal by gross measure. The finger to nose test, finger opposite movement and fast alternate movement were normal. She could not perform the heel-knee-tibia test and the meningeal irritation sign was negative.

Treatment:
After the admission she received related examinations and received 3 neural stem cell injections and 3 mesenchymal stem cell injections to repair her damaged nerves, replace dead nerves with new injected stem cells, nourish nerves, regulate her immune system and improve blood circulation. This was done with rehabilitation training.     

Post-treatment:
After 14 days treatment her arms were flexible, the muscle power of the legs increased 30% and her endurance improved. The muscle power of her back and waist were increased, she could stand up from a sitting position much easier, she was able to raise legs faster and easier and she could walk independently for a much longer distance.

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