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Multiple System Atrophy

Author Admin Views Posted at 2013/12/29

Dr Wang and Wu
MSA (Multiple system atrophy) is a kind of multiple CNS degeneration and atrophy which had no clear cause. Here we can introduce one successful case of MSA patient treatment.

Medical history:
Patient is a 65 year old female, she presented progressive motor disturbance of 4 limbs and speech difficulty more than 2 years, her symptoms aggravated more than half year. She had walking problem in December, 2006, then the disease developed quickly, she had severe tremor of her 4 limbs. Patient had been diagnosed as Parkinson Disease and she had received anti-PD treatment, but the treatment effect was not good. Her disease continued to deteriorate and she had slur speech, choke, swallow difficulty, she had difficult to open her mouth, and her slow movement and balance disturbance aggravated gradually, it was a hard work for her to get up from bed, roll over, walk and turn around. Patient also can not complete fine motor, she had to write slowly, but she can walk few steps slowly with assistant. Patient was diagnosed as MSA on August, 2007. In half year before her treatment, her condition got to be much worse: her movement disturbance was more worsen, she had severe rigidity of 4 limbs, she can not do any voluntary movement completely, she can not walk, difficult to open mouth, she ca not eat any solid food by herself. Patient had hypertension more than 20 years, and she had depression. In February 2005 patient had received thyroidectomy because of thyroid cancer. She had bone fracture of right arm in 2006 and her arm remained malformation. She had received cholecystectomy in 2007. Patient had no medical history of diabetes, CHD, no hepatitis, tuberculosis or other infectious disease. No drug allergy history.

Admission PE:
Bp 130/70mmHg, Hr 83 times/min. No remarkable sign of her heart, lungs and abdomen. There is Mild pitting edema of her lower limbs.
Neural system examination: alert, mask face, slur speech, anarthria, her memory, calculation and orientation ability are all normal. Bilateral pupils are equal and around, diameter is about 2mm. both of eyes had slow reflex to light. The bilateral pupils move slowly, with limitation and mild nystagmus. She had tongue muscle tremor. Muscle force of upper limbs are 4 degree, of left lower limb is 2- degree, of right lower limb is 1 degree. Muscle tone increased, and her lower extremities had severe cogwheel rigidity. Tendon reflex are weaker than normal. Sucking reflex is negative, Palmental reflex is positive, Hoffmann sign of both sides are positive, Babinski sign of right side is positive. Sensor system is OK. She can not cooperate the medical examination of her coordinate movement because of her high muscle tone. She suffered from neck rigidity and she had to maintain a special posture because of her rigidity. Kerning sin is negative, and the Brudainski sign is negative.
Assistant examination:

Brain MRI: Bilateral frontal lobe, pons and cerebellum atrophy.

Diagnosis: MSA

Treatment procedure:
Gave patient treatment to control blood pressure, expand blood vessels, anti-free radical, nourish neurons, stable the cell membrane, combined with neural stem cells activation treatment. After the treatment, patient´´s condition improved very much, her muscle tone decreased quickly, she can lift her head and turn her head to both sides. Her movement ability of 4 limbs improved obviously. Muscle force of upper limbs is 5-, she can do some voluntary movement, and she can life her arm to her head. Muscle force of left lower limb recovered to 4 degree, muscle force of right lower limb is 3+ degree. Patient can open her mouth and speak, she still speak slowly, but the voice his higher than before. She can chew food by herself, and now she can eat like normal.

Case analysis:
MSA is a member of Synuclein Disease. It is a kind of plus of series symptoms of clinical pathological change which includes OPCA (oliva-pons-cerebella atrophy), sporadic degeneration of nigrostriatal system, or companied with vegetative nerve functional disturbance: Parkinson symptoms (tremor, rigidity, start walk difficulty); vegetative nerve functional damage (it is related with the loss of lateral horn cells and brain stem pigment group cells, the clinical symptoms are orthostatic hypotension, swoon, impotence, adiapneustia, thirst, urinary retention,faecal incontinence. In general, paralysis vocal cord is the most important and earliest symptom of vegetative nerve functional disturbance, patient always has hoarseness ); cerebella symptoms and pyramidal signs. The symptoms will be different in different patient, so in 1969, Dr. Graham and Dr. Oppenheimer propose to give a name of the complicated disease as MSA. But to now, doctors still can not find an effective solution.

MSA is a kind of neurodegenerative disorders, from the causal mechanisms research in molecular level, we can see that the amyloid change of CNS lead by the aggregation and aggradation of malconstructed proteins is the most important mechanism. That means in the affected neurons, spongiocyte cells, those high soluble proteins can turn into insoluble fibrous polymer, which can transfer into fibrous amyloid deposit which will deposit in the endochylema, cell nucleus and spatium of extracellulars. These degenerated proteins/ polymer had great neurotoxicity, and they can lead to the neurons damage and death.

According to recent research, neural stem cells can improve patient´s vegetative nerve, cerebellar extrapyramidal symptoms and movement disturbance effective. In one side, those stem cells (neural stem cells and bone marrow mesenchymal stem cells) had a complicated and elaborate self control system, they can prevent the protein´s malconstruction and aggregation: molecular chaperone can help the proper protein folding, and prevent the accumulation of non-natural protein. We can use medication to accelerate those malconstructure proteins degradation and endocytose by ubiquitin-protease bodies system. It can block the development of the disease effective. In another side, those stem cells can located in the surrounding of damage area of neural system, repair the damage and help patient regain more neural function.

For this case, we have patient a comprehensive treatment: 1. improve the internal microenvironment 2. stem cells implantation and treatment to help those stem cells located in the damaged area 3. rebuild the neural system, improve patient´´s neural function. After the 3 steps treatment, patient´´s condition had obvious improvement. In general, the MSA patient will has nonreversible neurodegeneration, while the treatment results showed that neural stem cells implantation treatment can be effective to MSA patient. Now we still need further observation and randomized controlled research to verify it.

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