30 yr old male, having atrophy of calf muscles in rt lower limb which gradually increased in the last 1 year, now complaining of weakness in that leg. o/e pulse:60b min, b.p. 94/64, pallor present. no other significant history present. anybdd? mean while i hav sent for ncs and mri ls spine, cbc, echo. docs please give ur valuable opinion. thank u



theThanks a lot dear Adnan for posting a good case for discussion. The above mentioned case seems to be a case of MUSCULAR DYSTROPHY. By definition MD is group of diseases that cause progressive weakness and loss of muscle mass. In MD abnormal genes mutations interfere with the proteins required to form a healthy muscle. Very unfortunately there is no cure for MD. But medicines and physical therapy will help manage the symptoms and slow down the course of the disease. Complications of MD. 1) Inability to walk 2) Shortening of muscles or tendons around the joints in the form of Contractures. This can further limit the mobility. 3) Breathing problems These patients may need a ventilator support initially in the night and later on during day time too. 4) Scoliosis 5) Heart problems 6) Swallowing problems. Investigations in MD. 1) No trauma, no statin and a high CK Creatinine Phosphokinase indicates muscle disease MD. 2) EMG Changes in the electrical activity confirms MD 3) Genetic testing 4) Muscle Biopsy 5) ECG 6) 2D Echo heart. 7) PFT NCS and MRI have no role in MD. Treatment There is no cure for MD. Treatment includes 1) Medical therapy 2) Physical therapy 3) Surgical and other procedures. Medical Therapy This includes 1) of Steroids 2) Heart drugs like ACEI Beta blockers Physical therapy 1) Range of motion and stretching exercises 2) Low impact aerobic exercise like Walking Swimming 3) Braces 4) Mobility Aids 5) Breathing exercises. Surgery Surgery for Scoliosis may further worsen breathing. Prognosis is very poor. Better counsel the patient and family members about the pros and cons to avoid further complications.

Thank you Krishna sir for making this particular topic look easy and simple.

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Muscular dystrophy is always bilateral except in very few case report of osteo arthritis of ankle and knee joint. There are various Muscular dystrophy identified like Duchene's which is the most common variant, hereditary inclusion body myositis (myopathy) type 1 (HIBM1), Gowers-Laing distal myopathy, Finnish (tibial) distal myopathy etc. The above case is most like of Focal muscular atrophy (FMA). Muscle wasting is probably the presenting symptom and the onset is insidious. The organ ultimately affected is the muscle, although the pathology may be anywhere along the lower motor neuron (LMN) or, at times, secondary to non neurological disorders. Etiologic factors include the following: Infection, Trauma, Inflammation, Spinal cord disorders, Vasculitis, Nerve Entrapment, physical agents, such as electrical or radiation injury, Genetic and enzyme defects. etc. EMG & MRI- spine will be useful to know the level of nerve injury.

About MND 1) Per se MND motor neuron disease is Bilateral. 2) MND is a very rare disease like the incidence is 2 cases out of 1,00,000 population. 3) Progressive muscular atrophy is a v uncommon form of MND. 4) The muscles affected first are the small muscles of hand and foot. 5) Muscle spasticity is absent in MND. 6) Primary Lateral Sclerosis is a rare variant of MND which may cause weakness in the leg muscles.

There is significant wasting of the right leg,straight forward case of wasted leg syndrome where in such cases will have assymetrical slow,non progressive lmn type of weakness which involves the posterior crural -calf muscles followed by anterior crural and quadriceps.

To the point and precisely answered.

Contd. 7) There is no diagnostic test for MND. 8) There is no specific treatment for MND 9) The prognosis is very poor for MND.

thank u all for such good replies. pateint will come with reports on monday so as soon as he comes ill surely share it.

Any sensory symptoms /finding? If no with unilateral atrophy consider motor neuron disease. Rule out dm, Hansen.

Rule out root compression by mri

SMA or a wasted leg syndrome-variants of mnd,SMA has a better prognosis.EMG to confirm the diagnosis .

Thanks for the ipput

Dear Adnan, Can you please share your reports ???

I agree with Dr. Krsna Mohan sir

Thanks dear Nanda Kumar
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