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Suggest Treatment For Chronic Myopathic Muscle Disorder
can i get advice from you to help my sister , her report:
A 32old age female started 3 years after normal delivery of twin in 2005,show muscle tenderness, with pain in calf muscle , progressive to weakness ,difficulty in getting upstairs ,LL>UL.SOB on walking .pacemaker for heart block 5 years ago . Weakness decreased during the day ,difficulty in swallowing, Tensolin test:negative ,vision OK ,no skin rash ,no parasthesia ,no dysphonia ,increase hair loss recently ,thyroid function normal ,no family history .O/E: weakness LL 2/5, UP:4/5 (proximal worse than distal) , no sensory loose, facial muscle 4/5 . She used many analgesic drugs with out benefits, her condition worsted and undergo difficulties in walking.
The electromyography(EMG) study show picture of myopathic MUPs with spontaneous activity of fibrillation in the distal muscle, the serum alkalinphokinase (c.p.k.) was (1090) with high ESR value.
She given prednisolone 40 mg daily for one month the c.p.k. become (270)
she still on prednisolone 40 mg daily for second month the c.p.k. becom (252)and ESR (8 mm\h)
she still taken prednisolone 40mg daily for 3rd month the c.p.k. becom (243)and the ESR(9)
on prednisolone 40 mg for 4th month the c.p.k. became (252) and ESR(8)
She still with symptoms of her disease only little improvement in walking with no significant improvement of muscle function, the tenderness and difficult in walking still.
than her doctor withdrawal of prednisolone to 30mg daily with adding immuran(azathioprine) 100mg daily for tow month than increased the immurane dose to 150mg daily with prednisolone 30mg for one month the c.p.k. was(363) without change or improvement in her state.
She do another EMG show sever, chronic, diffuse polymyositis in compared to the previous study there is no improvement in muscle function.
than her doctor change immuran to methotrexate 7.5 mg weekly with prednisolone 20mg daily for one month of this regimen the c.p.k. was (350) and patient state still worse,
than increased methotrexate dose to 10mg weekly with prednisolone 20mg daily for one month the c.p.k. value become (400) and the ESR(36) and she still on this therapy.
With checking of renal and liver function test was normal but with out change of patient disease only appear of side effects of prednisolone and chemotherapy. than increase dose of methotrexate to 20mg weekly with 10mg prednisolone for about 3 month and show same lab. Results with out any improvement of her condition ,after that she given i.v immunoglobuline 200 ml daily for 5 days she have little improvement specially the pain and she withdrwal the prednisolone in duration of about 6 month and stop taking methotrexate but her condition still worse and in some time she have leg edema and difficulty in breathing,
After some months she go to Jordan in 1/12/2007 her doctor decided to give her rituximab ,and he send her for musle biopsy in first medical labs. By dr. Hsussam Abu Farsak (American Board of pathology and Cytopathology) in Jordan. Procedure: needle biopsy from right deltoid. The biopsy diagnosis : change suggestive of peripheral neuropathy pattern and absence of any inflammation. but her doctor don’t accept the biopsy result and do new EMG and show 1- normal motor and sensory NCVa from Rt median and ulnar NN including F-wave latencie 2-absent sutral and sup. Peroneal sensory responses bilaterally 3- needle EMG show typical picture of inflammatory myopathy in both Quadricept Femoris,Rt Bicept and EDC. , changes stuudestive of mild to moderate chronic denervation are ssen in both Tib. Anterior and EDB ,the conclusion of EMG : picture of inflammatory myopathy suggests by needle EMG finding in proximal muscle of lower and upper limbs, distal sensory motor polyneuropathy ,involving exclusively lower limbs with mild to moderate chronic denervation in distal muscle of lower extremities.
And her cpk 857 u/l and some change in liver enzyme and creatine kinase, ANA:negative, ENA:negative, DNA:negative, Anti_MAG:positive, anti_gangliosides:negative, anti_neuronal:negative. After that her doctor decided to give her rituximab and he say he have typical polymositis because her symptom lab. Result and EMG and say the biobsy may be incorrect .she admitted in Jordan hospital and given 500mg i.v methylprednisolone followed by 1000 mg rituximab at first and given 20 mg oral prednisolone daily , after 2 weeks she admitted to hospital the cpk was 360 u/l and given 500 mg i.v methyprednisolone and 1000 mg i.v rituximab, after 2 week her pain was less ,walking became slightly good her hair grow ,hands dermatitis disappear, and withdrawal prednisolne in 6 weeks her condition still with out any improvement and she fell pain in back ,legs, weaknees,shortnees of breath.
After that she don’t show improvement and don’t take any medication for one year , her condition began to worse and she need help to stand and to walk , after that she go to neurologist and the EMG show neuropathy and her doctor said she have chronic inflammatory demyeilinated polyneuropathy (cidp) and he decided to do plasmapheresis 4 courses after the plasmaphersis she show some improvement like the pain disappear but with out benefit in movement after that she still worse and have muscle and back pain and generalized weakness her doctor told her to do pasmaphersis 10 cycle after it she show little improve like she fell her leg become lighter than past and no pain but with out significant improvement and her doctor give her prednisolone 15 mg each alternative day along with the plasmapheresis now she also show crosses albumin urea with normal renal function , she do another biopsy(open biopsy 1cm from deltoid muscle was taken)show muscle with preserved structure(perimyseal and endomyseal structures) associated with focal area of endomyseal fibrosis and some fibrils show central nuclei no evidence of inclusion bodies seen neither inflammatory cell infiltrate in between muscle fibers. Picture is consistent with chronic myopathic muscle disorder and report say (enzyme histochemistry is recommended) ,she taken 15 mg prednisolone each alternative day from 2 month ago with her generalized weakness still cpk about 400 and ESR 8. The CSF finding was normal, she also developed cross albumin urea with recurrent UTI and now she didn’t taken any drugs.
A 32old age female started 3 years after normal delivery of twin in 2005,show muscle tenderness, with pain in calf muscle , progressive to weakness ,difficulty in getting upstairs ,LL>UL.SOB on walking .pacemaker for heart block 5 years ago . Weakness decreased during the day ,difficulty in swallowing, Tensolin test:negative ,vision OK ,no skin rash ,no parasthesia ,no dysphonia ,increase hair loss recently ,thyroid function normal ,no family history .O/E: weakness LL 2/5, UP:4/5 (proximal worse than distal) , no sensory loose, facial muscle 4/5 . She used many analgesic drugs with out benefits, her condition worsted and undergo difficulties in walking.
The electromyography(EMG) study show picture of myopathic MUPs with spontaneous activity of fibrillation in the distal muscle, the serum alkalinphokinase (c.p.k.) was (1090) with high ESR value.
She given prednisolone 40 mg daily for one month the c.p.k. become (270)
she still on prednisolone 40 mg daily for second month the c.p.k. becom (252)and ESR (8 mm\h)
she still taken prednisolone 40mg daily for 3rd month the c.p.k. becom (243)and the ESR(9)
on prednisolone 40 mg for 4th month the c.p.k. became (252) and ESR(8)
She still with symptoms of her disease only little improvement in walking with no significant improvement of muscle function, the tenderness and difficult in walking still.
than her doctor withdrawal of prednisolone to 30mg daily with adding immuran(azathioprine) 100mg daily for tow month than increased the immurane dose to 150mg daily with prednisolone 30mg for one month the c.p.k. was(363) without change or improvement in her state.
She do another EMG show sever, chronic, diffuse polymyositis in compared to the previous study there is no improvement in muscle function.
than her doctor change immuran to methotrexate 7.5 mg weekly with prednisolone 20mg daily for one month of this regimen the c.p.k. was (350) and patient state still worse,
than increased methotrexate dose to 10mg weekly with prednisolone 20mg daily for one month the c.p.k. value become (400) and the ESR(36) and she still on this therapy.
With checking of renal and liver function test was normal but with out change of patient disease only appear of side effects of prednisolone and chemotherapy. than increase dose of methotrexate to 20mg weekly with 10mg prednisolone for about 3 month and show same lab. Results with out any improvement of her condition ,after that she given i.v immunoglobuline 200 ml daily for 5 days she have little improvement specially the pain and she withdrwal the prednisolone in duration of about 6 month and stop taking methotrexate but her condition still worse and in some time she have leg edema and difficulty in breathing,
After some months she go to Jordan in 1/12/2007 her doctor decided to give her rituximab ,and he send her for musle biopsy in first medical labs. By dr. Hsussam Abu Farsak (American Board of pathology and Cytopathology) in Jordan. Procedure: needle biopsy from right deltoid. The biopsy diagnosis : change suggestive of peripheral neuropathy pattern and absence of any inflammation. but her doctor don’t accept the biopsy result and do new EMG and show 1- normal motor and sensory NCVa from Rt median and ulnar NN including F-wave latencie 2-absent sutral and sup. Peroneal sensory responses bilaterally 3- needle EMG show typical picture of inflammatory myopathy in both Quadricept Femoris,Rt Bicept and EDC. , changes stuudestive of mild to moderate chronic denervation are ssen in both Tib. Anterior and EDB ,the conclusion of EMG : picture of inflammatory myopathy suggests by needle EMG finding in proximal muscle of lower and upper limbs, distal sensory motor polyneuropathy ,involving exclusively lower limbs with mild to moderate chronic denervation in distal muscle of lower extremities.
And her cpk 857 u/l and some change in liver enzyme and creatine kinase, ANA:negative, ENA:negative, DNA:negative, Anti_MAG:positive, anti_gangliosides:negative, anti_neuronal:negative. After that her doctor decided to give her rituximab and he say he have typical polymositis because her symptom lab. Result and EMG and say the biobsy may be incorrect .she admitted in Jordan hospital and given 500mg i.v methylprednisolone followed by 1000 mg rituximab at first and given 20 mg oral prednisolone daily , after 2 weeks she admitted to hospital the cpk was 360 u/l and given 500 mg i.v methyprednisolone and 1000 mg i.v rituximab, after 2 week her pain was less ,walking became slightly good her hair grow ,hands dermatitis disappear, and withdrawal prednisolne in 6 weeks her condition still with out any improvement and she fell pain in back ,legs, weaknees,shortnees of breath.
After that she don’t show improvement and don’t take any medication for one year , her condition began to worse and she need help to stand and to walk , after that she go to neurologist and the EMG show neuropathy and her doctor said she have chronic inflammatory demyeilinated polyneuropathy (cidp) and he decided to do plasmapheresis 4 courses after the plasmaphersis she show some improvement like the pain disappear but with out benefit in movement after that she still worse and have muscle and back pain and generalized weakness her doctor told her to do pasmaphersis 10 cycle after it she show little improve like she fell her leg become lighter than past and no pain but with out significant improvement and her doctor give her prednisolone 15 mg each alternative day along with the plasmapheresis now she also show crosses albumin urea with normal renal function , she do another biopsy(open biopsy 1cm from deltoid muscle was taken)show muscle with preserved structure(perimyseal and endomyseal structures) associated with focal area of endomyseal fibrosis and some fibrils show central nuclei no evidence of inclusion bodies seen neither inflammatory cell infiltrate in between muscle fibers. Picture is consistent with chronic myopathic muscle disorder and report say (enzyme histochemistry is recommended) ,she taken 15 mg prednisolone each alternative day from 2 month ago with her generalized weakness still cpk about 400 and ESR 8. The CSF finding was normal, she also developed cross albumin urea with recurrent UTI and now she didn’t taken any drugs.
