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Does anyone have any experience in treating a patient with muscular dystrophy? My patient is a middle-aged man with progressive muscle weakness primarily affecting his left side, including the pelvis and lower limb. I'd be grateful for management suggestions.
You are going to have to be more specific if you want any advice. Management of what? The muscular dystrophy? The foot problem? What is the presenting complaint?
sorry, podiatric management, ie trying to facilitate mobility in the light of a progressive muscular wasting disease via whatever means are at the disposal of podiatrists. This patient has left sided foot drop and is hip hiking to achieve ground clearance, and expending valuable energy unnecessarily, so other than the obvious AFO I am keen to know whether anyone has any experience in helping these patients from the podiatric perspective.
l only have two clients that l see with MD, but we can treat/help your client the same way we could a stoke victim in many ways.
Once they have the AFO, in this case the left foot, l would place a heel wedge in the right shoe, this will help with the swing through phase and reduce the "Hip Hike" of the left side as the amount of lift required is not as much now due to the heel lift in the right shoe and we are only talking 4mm maybe less, you will have to fiddle with that one yourself.
We would also modify the sole to have a lateral flare plus rocker sole.
If you are unable to do a rocker sole at least grind down and smooth the toe of the left shoe, there is no need for grip at that end of the shoe and the smooth surface will help it slide rather than catch.
Good luck hope this helps !
PS Dont forget the AFO will also add to the overall leg length of the left side wich can be 3mm or more and this needs to be "counterbalanced" before the extra possible 4mm is added
Duchenne Muscular Dystrophy (DMD)
Most common muscular dystrophy; x-linked recessive disorder – mutation of chromosome X at location p21 (dystrophin gene) --> reduced or impaired dystrophin (stabilises cell membrane within muscle cell); affecting boys – usually starts before 5 years (mean age of presentation is 3.5yrs); affects about 1/3500 male births;
Clinical features:
Pelvic girdle affected first with proximal muscles initially showing signs of weakness --> clumsy waddling wide based gait. Shoulder girdle affected next. Delayed motor milestones - tend to walk and run at later age than peers (early motor development often normal or slight delay). May have trouble climbing stairs or getting up from the floor (Gower’s sign). Frequent falls. Protuberant abdomen from lumbar lordosis. Progressive --> distal limbs become affected. Calf muscles enlarged from fat cells replacing muscle.
Usually in wheelchair by 10-12 years and usually die of cardiovascular complications before age 30.
Calf muscle contracture --> equinus and toe walking a common early sign
Laboratory findings – creatine kinase elevated up to 20x
Management:
No cure; adaptive equipment; prednisone can delay onset of loss of ambulation by up to 2 years; surgical management of scoliosis
Lower limb – stretching and night splints for equinus; surgical release of contracture may keep ability to walk
Becker Muscular Dystrophy (BMD)
X-linked recessive. Clinically similar to Duchenne Muscular Dystrophy (can still produce some dystrophin) – but less severe, more protracted and less common (1/30-50 000 live male births) and later onset. Usually begins around 10-15 yrs.
Clinical features:
Usually able to walk until early adulthood – often live to 30’s. Progression is variable.
Associated with cardiac problems. Weakness --> difficulty walking, climbing stairs and getting up from floor.
Cavus or equinus and toe walking is common. Often need AFO’s.
Weight training can increase strength and endurance.
Psu-dohypertrophy of calf muscles common
Emery-Dreifuss Muscular Dystrophy (EDMD):
Rare; x-linked recessive; onset late childhood/early teens with mild muscle weakness; associated with cardiac complications; usually weakness and wasting of shoulder and upper limb muscles; contractures common; can present with toe walking due to calf muscle contracture.
Limb Girdle Muscular Dystrophy (LGMD):
Autosomal recessive or dominant forms; proximal muscle weakness; clinically similar to Becker’s; onset in second or third decade; a number of molecular defects in muscle membrane have been identified; starts in proximal lower limb muscles, then upper; scapular becomes ‘winged’; some develop respiratory muscle weakness;
Fascioscapulohumetal Muscular Dystrophy (Landouzy-Dejerine):
Slow progressive weakness, mainly of shoulder muscles with some involvement of facial and peroneal muscles. Usually onset in second decade. Autosomal dominant.
Clinical features – shoulder muscle weakness; pouting lips; difficulty whistling and drinking through a straw; eye often slightly open during sleep; clavicle prominent; peroneals may be week and have foot drop
Treatment aimed at provision of aids for ADL.
Congenital muscular dystrophy (CMD):
Rare; hypotonic at birth with generalised muscle weakness; limb contractures and joint deformities early; slowly progressive; some have severe cognitive deficits
Myotonic dystrophy (Steinert’s disease):
Onset from childhood to middle age; weakness initially affects the hands, feet, face and neck; delayed relaxation after muscle contraction; slowly progressive – lasts 50-60 years.
__________________
Craig Payne
Department of Podiatry
La Trobe University
Melbourne, Australia http://www.latrobe.edu.au/podiatry
__________________________________________________ ___________________________________ God put me on this earth to accomplish a certain number of things - right now I am so far behind, I will never die.
The views expressed above are those of the author and not that of La Trobe University This is where I am, where are you?
thank you for your advice, these are just the kind of practical suggestions I was hoping for. According to the classification in Craig's lecture notes (thank you Craig) it is the fascioscapulohumeral type which is affecting this patient.
FSH Muscular Dystrophy
Linear Mode
