Revach Sarit-Spinal Muscular Atrophy-(Israel)

Name: Revach Sarit
Sex: Female
Nationality: Israeli
Age: 11Y
Diagnosis: Spinal Muscular Atrophy(SMA)

Before treatment:
The patient felt weak in her walking at the age of 3. Her disease progressed slowly and her four limbs got weaker and weaker. She also had muscle atrophy and was taken to a local hospital and diagnosed with SMA. She used a non-invasive ventilator at night initially then 6 months ago she needed the non-invasive ventilator for the whole day. She had a PEG 1 year ago due to slow eating. At present she is unable to stand, uses a wheelchair for moving, is unable to turn over her body and is not  able to take care of herself.
Her spirit is good, she eats less food by mouth, her PEG tube is used to inject nutrient solution, her urination and defecation functions are normal, her weight is lower than normal.

Admission PE:
Bp: 110/70mmHg, Hr: 110/min, body temperature: 36.5 degrees. Breathing rate: 19/min. weight: 20Kg. There is no broken or bleeding spots on her skin and mucosa,  the chest development was normal, the respiratory sounds in both lungs were clear and there were no 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 4 limbs have muscle atrophy with scoliosis, there was obvious joint contracture of the bilateral knee joints, elbow joints, right finger joints, etc. Other joint movement was normal. There was no edema of the legs.

Nervous System Examination:
Patient was alert and had clear speech. Her orientation, cognitive function and calculation abilities were normal. Both pupils were equal and round, diameter of 2.5 mm, react well to light and the eyeballs can move freely. The eyesight and visual field were basically normal. Bilateral forehead wrinkle and nasolabial fold are symmetrical, showing the  teeth was normal, she could make her tongue extend out normally, her chewing ability was decreased, she can turn her head powerfully but she cannot shrug powerfully. Right arm muscle power was 2- degrees, left arm muscle power was 0, her right hand thumb and index finger can bend slightly, left hand fingers can not grasp. Muscle power of the legs was 2- degrees. The feet dorsal flexor muscle power was 0, the 4 limbs muscle tone was decreased. The abdomen reflex and ankle reflex disappeared, bilateral biceps reflex, triceps reflex, radial periosteal reflex, patellar tendon reflex can not be induced by examination. The bilateral palm-jaw reflex was negative, grasp reflex was negative. The Hoffmann sign of both sides were negative, bilateral Babinski sign were negative. The sensory examination was normal. Patient could not perform the coordinated movement, finger to nose test, finger opposite movement, fast alternate movement  etc because of weakness. 

After the admission she received related examinations and  received 3 times nerve regeneration treatment to repair her damaged nerves, replace dead nerves, nourish nerves, regulate her immune system and improve blood circulation. This was done with rehabilitation training.     

After 12 days treatment her swallowing function was improved, she ate faster than before and ate more. The muscle power of her left arm was increased 1 level, her right arm was more flexible, the muscle power of both arms was increased 20%, contracture angle of her bilateral knee joints  was higher than before, the muscle power of her legs was increased 20% and the muscle strength of her left arm was also increased.

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