Hereditary Spastic Paraplegia'

1
Hereditary Spastic Paraplegia.

• Every patient is different.
• Many placed in too hard basket.
• No one solution fits all!

2
Essentials before starting!

• Careful assessment of every muscle- its strength
  and /or spasm. Every affected muscle and all
  those in the same limb or body area need to be
  assessed individually.
• Check radiographs for any abnormality of bone,
  that needs correcting.
• If spasm, attempt to reduce spasm medically-
  Baclofen and/or Botox, and gabapentin, can be
  tried, orally or by injection.
• Or use of intra-thecal ( that is into the
  spine) Baclofen.
• These may indicate improvement that may be
  achieved by various methods, including the
  patient doing physiotherapy.

3
Walking problems are due to muscle imbalance,
because some muscles overwork with spasm and
this produces abnormalities in walking, and
deformities.

• Weak acting -- unopposed antagonistic ----- Eff
  ect
• Muscle Muscle.
• Tibialis anterior lt Gastroc )
  -gt Equinus Muscle. peroneus longus )
  /or Cavus
• Peroneus brevis lt Tibialis posterior
  -gt Varus
• muscle muscle
• Toe intrinsic lt Toe extrinsic
  -gt claw toes
• muscles Muscles

4
Not Steven,s feet- but both his feet had a
similar problem to that seen in the right
foot here..
Right foot shows the inversion of heel from over
action of Tibialis posterior
Marked deformity R foot due to overacting Tib
Posterior muscle, underaction of ant tib (that
elevates the foot at ankle ), with overaction of
Peroneal longus and abnormal acting toe
muscles. These raise the arch and deform the
toes. Note L foot less problem, but still has
high arch.
5
On this side see the big muscles at the back of
the leg , that often needs lengthening to get
the heel onto the ground.
On this side see how the big tendon at the back
has been cut like a Z to allow it to be
lengthened,( as marked in black) to get the heel
more on the ground.
6
For Steven, -- adjust dorsiflexion.
This diagram shows the muscles on the front of
the leg that are responsible for raising the
toes off the ground while the heel is on the
ground. In Steves case, the small diagram
shows how part of the tendon that is normally
inserted into the foot over the arch ( the Tib
Ant Muscle) .was split off in the lower few
inches and moved over and attached to the
lateral side so that it will lift the foot
straight and not with that twist seen in this
picture. That tendon , and its split, is shown in
Black on the smaller picture.
7
In diagram below the red lines are the main
muscles that lift the toes (in front) and lift
the heel at the back. We need to get a suitable
balance in their function.
These are typical CMT deformed feet- showing
how the heels are turned in ( inverted) and toes
are clawed due to over-action of residual
muscles when some others are paralysed. Patient
could not lift toes off ground when heel on the
ground, she had a foot drop and typical CMT
deformity.
8
Foot deformity and difficulty in walking Typical
of CMT.
After surgery similar to that already described
. The heels sit straight , the toes are down, and
there is a More stable gait.
9
For Steven, The big muscle at the back The
Tib Post muscle, was prone to spasm. This
caused the heel, and, so the rest of the foot
to turn in. That tendon was cut off
the foot , here and reattached to the back of
the tibia , in here where its spasms would not
produce deformity.
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) These are the most ) common things we do
We must assess every patient and every muscle-
But he must learn to use the good ones correctly.
11
Next problem was the clawing of his toes and
their tendency to turn in, to trip him up.

• For clawing of toes, we take the flexor tendons
  from
• under each toe and swing them up and attach
  them
• on the top of that toe so that
• they make the toe flex at the
• joint where it joins the foot
• ( MTP joint) . We use a wire
• to hold the toe straight
• while it heals. This gives
• more weight bearing surface.
• The tendon that pulls on the dorsum of the
  foot, to lift the
• front of the foot, was split and half moved to
  the lateral side of the foot, to help overcome
  the toes turning in.
• This was described earlier in this presentation.

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• The surgery requires 2-4 hours per foot depending
  on what each person needs.
• As many of you know Dr Ellis and I do both feet
  at the same operation.
• Then with both feet in Walking plaster casts the
  patient walks out of hospital on the 6-7th post
  op day.
• 6-8-12 weeks later the patient is admitted for
  intensive rehabilitation- to learn to walk again
  and resume physiotherapy.
• It may be 4-6-12 months before the patient
• is confident and walking without needing to
  concentrate on each step. But it is essential
  that the new gait must become automatic , so he
  uses it all the time, not just sometimes !

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• These Major transfers improved the function of
  the feet themselves. However the tendency of
  the adductors of the thigh to spasm still
  continues. Also these tendons were short. So he
  is having more injections (botox) to encourage
  relaxation in order for him, by physiotherapy to
  lengthen ( stretch) the tendons so they will not
  be such a problem. After the main surgery they
  were still making the feet tend to hit each
  other with every step. He says that they are
  improving now, walking is becoming easier.
• Yes it can be a long process!

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• It has been shown that deliberate exercise to
  strengthen weakening muscles maintains or
  improves the strength and function of muscles
  that are still functioning.
• This in turn slows the development of
  deformity.
• USE IT OR LOSE IT--- PHYSIOTHERAPY IS VITAL.
• The muscles need to be individually exercised
  against resistance to achieve their maximum
  possible power. The patient has to do that- the
  physiotherapist can only teach him how.

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FURTHER TREATMENT May be recommended to
continue, long term. This is a progressive
disease and continues exercises may help prevent
development of further deformities.,
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Good prolonged physiotherapy is essential to
ensure automatic use of transfer for rest of
patients life. A good set of walking bars will
help many to walk better. The physio needs to
check often to make sure he is doing it right
Initially I do not allow bending of the knees
when walking
17
Orthotics will help many Before /or after
surgery- but need to be correctly designed and
constructed with good Resilient insoles.
Some patients do best with a heel rise in the
shoe, or a wedged shoe.
18
Remember many people with inherited neuropathies
have sensory abnormalities of hands as well as
and feet They need to develop techniques of
regular care of those anaesthetic hands/feet.

• SELF CARE
• Daily routine - soak, scrape , oil, exercise
• Wound prevention
• Protective footwear Protective work tools
• Wound care - use of contact casts for ulcers on
  anaesthetic feet- to be worn full 24 hours)
• Every night look for trauma to limbs .
• Think why? How did it occur.
• And how to prevent recurrence.

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• Your future in in your hands.,

Many people accept their muscle paralysis and
spasms and believe that nothing can be done.
However if you really want to improve I am
sure you can-- BUT it is up to you.! No one
else can do your exercises- The physiotherapist
can only teach you how ! You must exercise every
day.
What do you want?? Well you must work for
it. All the Best!!