March Newsletter 2002

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PERSPECTIVES

SPECIAL ISSUE

Vol. 2, Issue 12

March 2002

IN THIS SPECIAL ISSUE:

Translated Text of Italian Neurological Society Meeting

Rehabilitation Management of Peripheral Neuropathies

Presented by Paolo Vinci, M.D., and Ph.D.

( Editor's Note: The article presented by Dr. Vinci has been

translated and made available with special consideration for all

members. Our March 2002 issue of PERSPECTIVES presents the entire

article which includes CMT. It was originally presented at the Italian

Neurological Society meeting October 2001. It has been published in

Neurological Sciences 2001; 22: S-443-446 (in Italian). The CD will be

prepared by the Italian Neurorehabilitation Society and will be

presented at their next meeting in Venice. Below, we bring you the

English translation. We at , thank Paolo, for his generosity in

sharing his presentation with us, along with his own life's CMT journey

as well. We encourage you to spend time reading this important text. As

we have seen formerly with his book, the very words " rehabilitation

management " suggests " hope " .)

REHABILITATION MANAGEMENT OF PERIPHERAL NEUROPATHIES

By Paolo Vinci, M.D., Ph.D.

Neuropathies are pathological processes of nerves, interfering with the

normal transportation of the nervous impulse from the central nervous

system to the muscle fibers (through somatic and visceral motor fiber),

and from peripheral receptors to the center (through sensitive fiber).

The main consequence of the involvement of the motor nerve fiber is

muscle weakness, while the alteration of sensory fibres causes deficit

of sensation and, sometimes, pain.

Neuropathies may involve a single nerve or root or plexus

(mononeuropathies) or may be symmetrically widespread to all nerves

(polyneuropathies). In this latter case, with the exception of

Guillain-Barrè syndrome (GBS) and few other disorders, the process is

length-dependent, that is, the fibers reaching the distal parts of the

limbs are involved more severely than the ones for the proximal

districts.

Because the main task of the rehabilitator is to preserve or restore the

functions necessary to guarantee each person the widest independence, it

is very important to assess the severity and extension of the muscular

and sensitive involvement, and to understand the rule of each weakened

muscular group in the movements which are necessary to carry out each

function. In fact, the weakening of a muscle (or muscle group) alters a

movement according to its role (agonist, antagonist, neutralizer,

stabilizer) in that specific movement.

The specification of the function is also very important: for example,

the plantarflexor muscles, in the first stage of Charcot-Marie-Tooth

disease (CMT), must be considered as " strong " for ambulation and " weak "

for running. With the exception of GBS and few other disorders in which

the patients' functional deficits are due only to the muscle weakness,

in the majority of polyneuropathies the functions are altered not only

by the muscle weakness caused by the neuropathy but also, and in some

cases especially, by the muscle and joint alterations following the

muscle weakening and by compensations.

The sensory deficit that affects the most important daily living

functions, such as normal stance and ambulation, is the one involving

proprioception. However, in some prevalent sensory neuropathies, it is

hypoesthesia, since it may cause trofic changes in the feet, with

consequent difficulty to wear shoes and to walk.

In order to plan an effective rehabilitation program, it is very

important to know whether the neuropathic process, responsible for the

motor and/or sensitive deficit, is reversible, spontaneously or as a

result of a therapy, or if it is not reversible and not progressive

(stabilized) or, worst case, progressive.

In reversible neuropathies, it is necessary to minimize the impact of

muscle weakening and sensitive deficit on functionality until sprouting

(in case of axonotmesis or neurotmesis) or conduction (in neuroapraxia)

take place, and, at the same time, to prevent muscle and articular

damage caused by inactivity and by use of pathological motor engrams,

which would make it difficult or impossible the restore normal functions

even in case of complete nerve healing. Therefore, compensations should

not be encouraged until any possibility of recovery is lost.

An example of mononeuropathy is the one affecting the peroneal nerve,

that's a very common event in traumatology.

The weakening of the foot dorsiflexor muscles, as a result of it, causes

footdrop, with alteration of the swing phase of gait and risk of

tripping, as well as unbalance of strength between them and their

preserved antagonists (plantarflexors) which undergo a contracture.

The first problem is solved by an orthotic for footdrop, such as a

Codivilla's spring, allowing the function ambulation to be performed

safely while awaiting a reinnervation and therefore a power recovery in

the dorsiflexor muscles.

The use of a dorsiflexion assisting device also avoids the adoption of

compensations which might cause pain to the spine and persist even after

distal recovery.

The plantarflexor contracture must be addressed by passive kinesitherapy

(stretching), to avoid it to become permanent (equinus deformity), which

would cause a deficit of dorsiflexion, even in case of complete strength

recovery in the dorsiflexor muscles.

When reinnervation starts, the dorsiflexor muscles will be stimulated to

contract, in the beginning in a favorable contest (in water), then in

the gym both as an exercise and in the context of a functional

re-education to walk normally. Only if the dorsiflexor strength

recovery will not be sufficient, permanent compensatory solutions will

be adopted, such as shoes with higher heels or orthotics for foot drop

or compensatory motory patterns.

An example of polyneuropathy susceptible of improvement is GBS. In this

disease, there is generally a first phase of fast ascending paralysis,

followed, after a short stationary time, by a phase of recovery, either

fast or slow, that leads to complete healing in 60% of cases, and to

permanent severe deficits in 10% of patients.

In the first phase, it is important to monitor the respiratory function,

in order to ventilate the patient promptly in case of respiratory

failure due to severe diaphragm weakening; besides, communication must

be facilitated, a psychological support should be provided and the

consequences of prolonged bed rest, such as joint rigidity, venous

stasis and pressure sores, must be prevented.

As muscle strength starts to be recovered, rehabilitators will have to

facilitate the recovery of functions which, in the axonal forms, can

require a very long time and cannot be complete. The remaining deficits

will have to be addressed either by facilitating the most suitable

compensatory patterns of movement, or, when compensations cause muscular

or articular overload, or are not sufficient, by orthotics.

In progressive polyneuropaties, such as genetic ones, rehabilitation is

aimed at improving the main functional activities and quality of life,

through a complex intervention involving numerous professionals

(neurologist, orthopedic surgeon, pedorthotist, orthotist,

physiotherapist, occupational therapist, geneticist, psychologist)

coordinated by a physiatrist experienced in neuromuscular

rehabilitation.

Although rehabilitation has no possibility to stop the progression of

the muscular weakening in a distoproximal direction, it is very

important to know in which order the ability to move joints is lost,

both to facilitate muscle contractures that will be useful in the future

and to prevent the consequences of the evolution to the next stage.

For instance, in the majority of CMT cases, dorsiflexion fails before

plantarflexion, so a triceps surae muscle contracture develops. If this

contracture is severe, a poor balance will result; on the contrary if it

is slight and not associated to a severe rotation, it is useful, as it

allows the ankle stabilization on the sagittal plane and the use of

shoes with a small heel, easier to find on the market as compared to

flat ones, even when the plantaflexors will become completely atrophic.

It is up to the rehabilitator to monitor the progression of the

plantarflexor weakening, in order to stop the stretching program at the

right moment allowing the optimal contracture to develop and, not less

important, for patients, whose triceps surae has weakened beyond a

certain degree, continue to wear shoes provided with high heels, so

overloading the quadriceps muscles and risk of falls does not take

place.

On the contrary, in Hereditary Motor Neuropathy (HMN or DSMA)

plantarflexion generally weakens earlier than dorsiflexion. This does

not allow the use of heeled shoes since the first stages and, in many

cases, also causes a rotation of the legs forwards during stance, with

consequent need to keep the knees slightly bent, thanks to the isometric

contraction of the quadriceps that will undergo an overloading.

In case the evolution of the muscle progression is known, the

rehabilitator will encourage a triceps surae muscle shortening before

its complete degeneration, so allowing a more stable stance and the use

of shoes with a small heel.

The goals of rehabilitation management in CMT are: improvement of muscle

strength and balance, prevention of fatigability, prevention and

treatment of joint deformities, promotion of mobility, prevention of

falls, improvement of hand function, solution of psychological problems,

prevention and treatment of pain.

In CMT there is great variability in the extension and severity of

muscle weakening, with cases in which the weakness is restricted to the

foot muscles and cases with progressive involvement of the pelvic girdle

ones.

Also in upper limbs, there are cases in which all the intrinsic hand

muscles are strong even against a moderate resistance and cases in which

they are completely atrophic and, in addition, also the forearm muscles

are weak.

In order to help rehabilitation professionals to evaluate easily the

severity of rehabilitative problems and provide the most appropriate

rehabilitation, we suggest categorization of patients in levels of

increasing severity (seven for the lower limbs, and four for the upper

limbs). The functional classification of the lower limb impairment takes

into account the problems caused by the progressive weakening of muscles

in the lower limbs and by its biomechanical consequences on gait.

In every stage, there is a new problem, typical of the present stage,

which is added to the ones of the previous stages, modifying the pattern

of gait and requiring a different rehabilitative intervention.

In each stage, rehabilitation management, which consists of lifestyle

changes, orthotics (proper shoes, foot orthoses, ankle-foot-orthoses,

knee-ankle-foot-orthoses), stretching, postural kinesitherapy and

orthopedic surgery, is different and specific.

It is very important to stress the importance of aesthetics in choosing

orthotics, since many patients, especially women and young people,

refuse to wear orthopedic shoes and traditional ankle-foot-orthoses: in

these cases, the " aesthetic in-shoe device for footdrop " inserted in

fashionable above-the-ankle shoes or boots is recommended.

Functional classification of the upper limb impairment is based on the

possible type of handgrip. At stage 1, the pinch between the pulps of

thumb and forefinger is preserved, although it is weaker and the thumb

is less rotated than in normal individuals. At stage 2, a weak but

functional pinch between thumb and second or third finger is still

possible, thanks to compensations or tricks. At stage 3, the thumb lies

on the same plane as the other fingers and only a lateral pinch between

thumb and forefinger is possible. At stage 4, the muscles of the hands

are wasted, deformities are often present and only a rough grasp by the

lateral fingers is possible. For each stage there is a different

rehabilitation management, which includes recommendations to avoid

overwork weakness and cold, stretching to prevent or treat permanent

contractures and special tools to retain the ability to perform daily

living activities.

About the possibility of strengthening the muscles weakened by CMT

disease through exercise against resistance; there are still doubts,

both about efficacy (since studies have been carried out on proximal

muscles) and harmlessness (since overwork weakness has been found also

in this disease).

References

· Vinci P (2001). Rehabilitation management of Charcot-Marie-Tooth

disease. Spazio Immagine Editore, Roma.

· Vinci P (2001). La riabilitazione nelle neuropatie periferiche. Neurol

Sci 22: S443-6

Aknowledgements

The Author thanks the Italian Charcot-Marie-Tooth Association (AICMT -

via Pisacane 10 - 00152 Roma - Tel/fax 06 3038338 - www.aicmt.org -

aicmt@...) for active collaboration in rehabilitative research.

Author's e-mail: paolovinci@...