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Yehudit Dayan-Hereditary Inclusion Body Myopathy-(Israel)-Posted on June 11th, 2018

Author Zhangqi Views Posted at 2018/06/11

Name: Yehudit Dayan
Sex: Female
Nationality: Israeli
Age: 34Y
Diagnosis: Hereditary Inclusion Body Myopathy(HIBM)  
Date of Admission: Jan. 8th, 2018
Treatment hospital/period: Wu Medical Center/14 days

Before treatment:
The patient felt weakness in her legs 16 years ago. She fell down sometimes so she went to a local hospital and did some treatment and by doing a gene test she was diagnosed with HIBM 15 years ago. She continue to do rehabilitation training but was unable to walk 9 years ago. Her arms, neck and back muscles were also affected 6 years ago. At present she is unable to take care of herself, is unable to sit up, turn over or walk. Her swallowing function and respiration functions are normal.
Her spirit, diet , sleep, urination and defecation functions are all normal.

Admission PE:
Bp: 115/73mmHg, Hr: 67/min, breathing rate: 19/min, body temperature: 36.2 degrees. 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, 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 and the spinal column was normal. There was edema of the legs and dorsalis pedis pulse was reduced.

Nervous System Examination:
Patient was alert and her mental status was fine. Her memory, orientation and calculation abilities were normal . Both pupils were equal in size and round, diameter of 3 mm, react well to light, no nystagmus and the eyeballs can move freely. Bilateral forehead wrinkle and nasolabial fold are symmetrical, showing the teeth is normal, her tongue is in middle and can move freely. The tongue could touch cheek powerfully and she could bulge her cheek as normal. The bilateral soft palate could lift as normal, she could close eyes as normal, the neck was soft but she could not turn her head or shrug powerfully. She had obvious limitation to turn her head to the right side. The muscle power of the arms were 1+ degree, she only could rotate the forearm in bed, left hand grip force was 3 degrees, right hand grip force was 3- degrees. muscle power of the legs was 0. There was only mild toe movement and the 4 limbs muscle tone was low with left side strephenopodia. The 4 limbs tendon reflex and Achilles tendon reflex could not be induced by examination, the bilateral palm-jaw reflex, Hoffmann sign, Babinski sign were negative. Patient could not perform the finger to nose test, fast alternate movement, finger opposite movement or heel-knee-tibia test because of weakness. 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 muscle cells, 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 forearms could move with much more flexibility, the right hand could hold things much better, the grip force was now around 3 degrees, her right hand could perform the finger opposite movement test, her arm function was improved 10% and we can see the feet dorsal now have part movement.

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