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Aleksandar - Progressive muscular dystrophy (Macedonia) Posted on March 10, 2014

Author Julia Views Posted at 2014/03/12

Name: Aleksandar Mishev

Sex: Male

Age:1 9 years old

Country: Macedonia

Diagnoses: Progressive muscular dystrophy, Dysfunction of liver

Admission Date: 2014-01-25

Days Admitted to the Hospital: 30

Before treatment:

The parents found the patient needing great effort when he climbs to bed when he was 4 years old. Then his parents found the patient's four limbs were weak and he falls frequently. He received a series of examination and was diagnosed with muscular dystrophy. Then the patient received rehabilitation. The disease progressed gradually and the muscle atrophy aggravated. He was unable to walk completely and couldn't take care of himself. The disease still progressed. Before the treatment, the patient suffered from joint contracture of elbow joints, hip joints and knee joints. The movement of joints was limited. There was no obvious breathing difficulty. He could maintain sitting position in wheelchair. He still could control head. He couldn't turn over, sit-up, stand or walk. There was no cough with diet. There was no same disease in his family member.

Admission PE:

Bp: 94/69mmHg; Hr: 76/min, temperature: 36.2 degrees. Br: 19/min. The skin and mucosa were normal, with no yellow stains or petechia. Through auscultation, the respiratory sounds in both lungs were clear, with no dry or moist rales. The heart sounds were strong, the cardiac rate was normal, with no obvious murmur. The abdomen was enlarged and soft. There was no pressing pain or rebound tenderness. We didn't touch the liver or spleen under the ribs. He had scoliosis. There was severe skeletal muscle atrophy of shoulder girdle, pelvic girdle and both upper limbs and both lower limbs. There was tendon contracture deformity of hip joints, knee joints and elbow joints. Both knee joints and elbow joints couldn't be straightened completely. Left elbow joint presented 90 degrees. Right elbow joint presented 90 degrees. The knee joint presented 110 degrees. Bilateral wrist and both palm articulationes digitorum manus presented with extension. The wrist and both palm articulationes digitorum manus couldn��t be bent. Bilateral ankle joint presented 90 degrees. The ankle joints presented varus deformity. The range of motion of both ankle joints was poor.

Nervous System Examination:

Aleksandar Mishev was alert, and his speech was unclear. He had good spirit. His memory, calculation ability, orientation and comprehension were all normal. Both pupils were equal in size and round, the diameter was 2.5mms. Both eyeballs could move freely and flexibly and the pupils were sensitive to light stimulus. The forehead wrinkle pattern was symmetrical. The ability to close his eyes was strong. The bilateral nasolabial sulcus was equal in depth. The tongue was centered in the oral cavity, the teeth couldn't close up. He was unable to show his teeth. He couldn't drum gills. He could raise the soft palate symmetrically and strongly. The muscle strength of his head and neck was slightly poor. He couldn��t shrug his shoulders. The muscle strength of both upper limbs near-end was at level 1 and the far-end was at level 2. The grasp power of hands was at level 3-. The muscle strength of both lower limbs was at level 0-1. The muscle tone of four limbs was low. The tendon reflex of both upper limbs was not elicited. The tendon reflex of both lower limbs was elicited slightly. Bilateral abdominal reflex was not elicited. Bilateral palm jaws reflex was negative, bilateral Hoffmman sign and Rossilimo sign were negative. Bilateral Babinski sign was negative. The deep, shallow sensation and fine sensation was normal through loose measure. He was unable to finish the finger-nose test, digital opposition test and the rapid rotation test. He had difficulty with the heel-knee-shin test. There were no signs of meningeal irritation. The laboratory test showed obvious increment of the creatase level.

Treatment:

We initially gave Aleksandar Mishev a complete examination. He received treatment to enhance liver function, nourish the nerves, promote the muscle growth and stabilize the organs function. He also received treatment to improve the blood circulation in order to increase the blood supply to the damaged neurons. This was accompanied with physical rehabilitation treatment.

Post-treatment:

Aleksandar Mishev has shown good improvement. His appetite has improved. The diet is normal. The urine and defecation are normal. The patient felt that he can swallow better now. The muscle strength to shrug his shoulders has increased. The muscle volume of both upper limbs' near-end and scapular region has increased than before. The muscle strength of both upper limbs' near-end has improved to level 2. Both hands can point thumbs with other fingers in a flexible manner. The activity range of both wrist joints and digital joints has increased too. The grasp power of both hands is at level 3+ now. Both knees can do adduction and abduction in lying position more easily. The contracture of elbow joints and knee joints is alleviated. The range of motion of joints has increased obviously. Bilateral elbow joints present 100 degrees. The knee joints present 145 degrees. The muscle group volume of both lower limbs has increased. The muscle strength of both lower limbs has increased from level 0 to level 2. He does not complain of early tiredness when he does exercises. The exercise endurance has increased. The creatase level has reduced.

 


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