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Muscular Dystrophy

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S. K.

Age: 10Yrs.
Sex: Male

Diagnosis: Duchenne’s Muscular Dystrophy
Country: India

Date of therapy: On 10/05/2010 underwent Autologous bone marrow derived stem cells + intensive Neurorehabilitation (NRRT).

Brief History:

He is a known case of  DMD since the age of 6. It started with history of bilateral lower limb weakness and difficulty in climbing stairs and getting up from floor. Thereafter, he started walking on toes and having frequent falls. He stopped walking since a year.

Clinical presentation:

Neurologically, he was hypotonic and hyporeflexic. He had all sensations intact. He had grade 1+ strength in bilateral lower limb and grade 3 in bilateral upper limb with predominant proximal muscle weakness >> distal. On examination: he had pseudohypertrophy of bilateral calf.  On investigation: EMG,genetic tests and increased CPK confirm the diagnosis .Functionally, he was totally dependent on mother for all ADL and wheelchair bound for mobility. On FIM   he scored  81.

Investigations till date in Ascending Order:

17/4/10: EMG :  Primary Muscle Disease

8/5/10: MRI – UL, LL - showed marked fatty infiltration.

Condition one week post stem cell therapy:

  1. After stretching, mother reports that both the legs have straightened from knees, earlier both knees were bent a lot, now they are relatively straight.
  2. Strength in his hands has improved, now he can (press) use jet in bathroom, which he could not do before.
  3. His stamina for exercises has increased.
  4. He can now move both his legs from knees below, when he is sitting on bed, which he could not do before.
  5. He can now attempt sitting up from lying position.
  6. Rolling on bed is easier for him.
  7. Now while sitting, he can keep his hip adducted, which he used to keep outward abducted before.
  8. His CPK level pre Stem Cell Therapy was 3076 whereas post Stem Cell Therapy has reduced to 2870.


2 months post therapy:

  1) Mother reports overall stamina has improved.
  2) Sitting balance on ball/unstable surface has improved.
  3) Neck control/strength has improved.
  4) Upper extremity has improved. He can now lift both hands straight overhead with ½ kg wt on.
  5) He can now wash up after passing motions by himself. Grip strength has improved.
  6) He can now sit up from lying position by himself and also shift on bed, by bearing wts on hand.
  7) He can now do lower body dressing independently.
  8) Overall, exercises are easier than before and happy to do it.
  9) Standing tolerance improved (15-20 mins).
  10) He can now voluntarily tighten and relax his knee muscles (since 10 days).
  11) His knee has straightened up (earlier was 8” now 6’’)

6 months post therapy:

  1) Can lift the upper limb with ½ kg weight, keeping the elbow straight.
  2) Can transfer from bed to chair of same height.
  3) Can open the tap (water tap) which he could not do before.
  4) Can stand without support for 10 minutes.
  5) He can dorsiflex the foot which was not there earlier.
  6) While sitting, can bend and lift the objects from floor.
  7) Can take the bath independently.

8 months post therapy:


  1) Grip strength bilaterally has improved. Able to pull theraband, etc with increased force.
                                   Rt       Lt
     Jamar Dynamometer readings - 3 lbs   2.5 lbs
     Jamar Pinchometer readings - 5.5 lbs 6 lbs

  2) Transfer at same height i.e. from bed to chair is independent. However is dependent for all other transfers.
  3) Can stand with help of calipers and walker for 40 minutes.
  4) Can bathe independently in sitting in chair as he can now bend and come up independently.

10 months post therapy:

  1) Mother reports overall stamina has improved.
  2) Sitting balance on ball/unstable surface has improved.
  3) Neck control/strength has improved.
  4) Upper extremity has improved. He can now lift both hands straight overhead with ½ kg weight on.
  5) He can now wash up after passing motions by himself. Grip strength has improved.
  6) He can now sit up from lying position on his own and also shift on bed, by bearing weights on hand.
  7) He can now do lower body dressing independently.
  8) Overall, exercises are easier than before and happy to do it.
  9) Standing tolerance improved (15-20 mins).
  10) He can now voluntarily tighten and relax his knee muscles (since 10 days).
  11) His knee has straightened up (earlier was 8" now 6")


           Right                                   Hip                                     Left

10/5/10           13/7/10                                               10/5/10        13/7/10                                                              

1+            2+                               Flexors                  1                   2+                                                                                                                     

2+   3+                               Extensors              ? 1                1                                   

3              3+                               Abductors              3                  3+

2              3+                                Adductors             2                   3

3              3+                                Knee Flexors                         

?1            1                                 Extensors               ? 1                1       

                                                  Abdominals

1              1++                              Upper  

1              2?                                 Lower        

                                                    Back Extensors

 

                                                     Neck 

 3+           3++                            Flexors                                                        

 3+           3++                            Extensors                                        

 3+           3++                            Trapezius

                                                  Shoulder

3              3+                              Flexors                   2+                 3

2+            3+                              Extensor                 2                  3+

 3             3+                              Abductors               3                  3+

 2             3+                              Adductors               2                  3

                                                  Elbow 

 3             3+                             Bicep                       2                  3?  

3              3+                             Brachialis                3-                  3-

3              3+                            Triceps                     3                   3+

 3             3+                           Brachioradialis          3                   3