Diagnosis of Distal Spinal Muscular atrophy

My daughter has been diagnosed with spinal muscular atrophy , the neurologist is saying it is likely distal type.  I am very confused and afraid for my daughters future.



The disease seems to be progressing rapidly, does not seem to fit into any neat diagnostic category which alarms me further, I am terrified that there is something else here but am feeling very powerless to know what to do, what questions to ask , what to read , anything , consequently i am surfing the net in a haphazard and random way trying to do , I dont know what.  

I see that distal refers to furthest away from the centre and it seems that distal sma should affect the lower parts of the legs and arms, hands and feet , this seems to not be happening to ella so i do not understand why it is being called this , is it because the symtoms (symptoms) do not fit type 1,2,3,or 4, i have noticed a disease called  Hirayama disease which affects the upper limbs only  but i think only the lower arms and hands are effected.  ella is effected in other places also it seems.

I have never met the neurologist and have been in email contact with the child neurologist , I am not living in the same country as my daughter but will be there soon.

I have emailed the neurologist twice for specific information which he has given, I want to know much more but am afraid to ask,  my daughters mother has seen him 3 times in the 2 months or so .  Ella was diagnosed two months ago after a number of tests were carried out.   I have asked the neurologist about these he gave the following information.

Her workup consisted of  a normal MRI of the brain and spinal cord, normal spinal fluid examination,
and normal bloodwork, including CK, and transaminases.  Electromyography and nerve conduction tests showed  denervation pattern in all muscles tested, and normal nerve conduction velocities in both motor and sensory nerves. A biopsy of a thigh muscle
did not show any abnormality, but unfortunately a more affected muscle was not sampled.

I do not know what muscles were tested by emg and nerve conduction but have asked him by email along with other information,   since now blood tests that were sent to the U.S  which I believe were for genetic confirmation have come back inconclusive, i am presuming that the test was confirmation of a faulty smn1 gene,etc ,I think more tests have been ordered but the neurologist has basically said (so ellas mum says) that it wont effect anything anyway.   Ellas mother says that the biopsy was taken from the leg, there was no abnormality it says.  Ella can walk still and does not show any weakness in this area,

she has lost the use of left arm and her right is very weak and worsening. a month or so she could still lift light hoops and place them over their holder but no long can lift anything. you maybe able to see that the arm is very floppy , there is no muscle tone.  I have read about frog leg like and her arm to me looks similar.   would the fact that she cannot move her arm indicate that the deltoids are affected?


I believe that the back is affected and her mum says her ribs are beginning to show,

The neurologist says that there is a problem now with her tongue and she also will need a feeding tube I think, this is all very scary and i am really upset for her future , it seems that she will not have the use of her hands or be able to talk.

based on the results of the tests and information I have listed  can you suggest any disease apart from sma that could show those results, i realise that the neurologist may have exluded many other diseases based on other observations and maybe the results are not detailed enough.

can you offer any advice on dealing with the neurologist, how can i ask questions without causing a nuisance or offence, what questions can i reasonably ask , i have just emailed him asking for 'a case summary for me detailing your observations, findings and  reasonings so that i can try to understand ellas condition '  is this a reasonable request?.  

here is a link to a short clip of ella taken a few days ago.

[maje]

http://www.youtube.com/watch?v=UFSSaqN1ZJI

here is a short clip , somehow it has been turned upside.

I am sorry there are three identical postings of this question.