CIDP

Physiotherapy Management
A physiotherapist's role in the treatment of CIDP needs to be very specialized to each
individual. Physiotherapists prescribe gait aids that may assist with balance and
ambulation during ADL’s. Manual therapy may also be provided when indicated to
prevent joint contractures and maintain available ROM. Since many individuals with CIDP
have difficulty with balance and walking, gait retraining and exercise programs prove to
be beneficial at increasing efficiency and endurance [13].
Exercises that promote muscle strengthening and aerobic conditioning are important
once patients have received clearance from a physician for an exercise program [21].
Exercise parameters for CIDP patients also need to be individually tailored as it is
important that individuals don’t over exert themselves. As a result of the demyelination of
axons the body is unable to recruit as many muscle fibres to complete a task, which may
cause the muscle fibres that are engaged to be overworked. Some delayed onset muscle
soreness is expected to occur, but pain that persists longer than 12-48 hours, with or
without a loss of strength, is an indication that the F.I.T.T. parameters need to be
adjusted because the patient was overexerted[21]. It also crucial to assess that CIDP
patients can complete a motion against gravity before apply external resistance to the
movement, as it is important to have baseline muscle control before progressing
exercises[21].
Recent studies on the effects of exercise programs for patients with CIDP have provided
good insight and direction for future physiotherapy treatments. Janssen et al, (2018) [1]
completed an intervention that was designed around the Otago Home Exercise
programme.  This protocol targeted falls prevention, lower limb and core strength
exercises with dynamic balance retraining. Their goal was to maximize walking and
functional movements required for maintenance and recovery. Patients were prescribed
an individualized exercise plan to do three times as well as 30 minute walks twice weekly
for a total of 6 weeks. The physiotherapist was able to check in with participants during
the 6 weeks. The results demonstrated that a tailored exercise program had a positive
effect on participant’s walking speed and balance in patients with CIDP.  More studies
will be need to be completed to determine the long term impact, however these are very
positive results and should be taken into account when treating a patient with CIDP [1].
Markvandsen et al, (2017)[23] examined the effects of a 12 week aerobic or resistance
exercise training programs.  The aerobic exercise program involved patients on an
ergometer bicycle at home or at a local fitness center for 20-30 minutes 3 times a week.
The resistance program, which trained the knee and elbow flexors/extensors on the
patient’s weaker side, was completed 3 times a week under the supervision of the
authors and each exercise was done repeated 12 times in sets of 3.
During the aerobic training period, VO2 max, 6MWT scores and muscle strength
increased. Resistance training also resulted in an increase in muscle strength on the
trained, initially weaker, side. The results from this study demonstrate that physical
exercise training in patients with CIDP is feasible and effective. Their research
determined there was no change in disability, quality of life fatigue severity. The authors
hypothesised the lack of change in quality of life was due to the difficulty of 3 times a
week exercise and exhaustion following exercise[23]. This must be considered when
designing an exercise program for someone with CIDP.
POLYNEUROPATHY
Radiculopathy affects the spinal root, leading to pain, paresthesias, and weakness in
the distribution of the nerve root. It is most often caused by herniation of an
intervertebral disk. Other causes include Lyme disease and neoplasia.

Plexopathy involves either the brachial plexus or the lumbosacral plexus, with
symptoms involving multiple nerves. Causes include trauma, tumor infiltration, bony
or vascular compression, radiation injury, and viral infection. Plexopathy may occur
acutely in-hospital as a procedural complication, as in lumbosacral plexopathy due to
groin hematoma after cardiac catheterization, or brachial plexopathy from stretch
injury after cardiothoracic surgery.

Mononeuropathy is dysfunction of a solitary peripheral nerve. This is typically due to

trauma (as in foot drop from peroneal nerve palsy after fibular fracture), compression
(as in “Saturday night palsy,” compression of the radial nerve in the axilla from
falling asleep with the arm draped over a hard surface, as seen in alcoholics), or
entrapment (median nerve in carpal tunnel syndrome or ulnar nerve in cubital tunnel).
Involvement of several noncontiguous individual nerves is referred to as multiple
mononeuropathies or mononeuropathy multiplex; vasculitis is the most common
cause.

Polyneuropathy affects many peripheral nerves simultaneously, with distal and more or less
symmetric involvement. Symptoms typically begin in the feet, before ascending to involve
the legs and hands. It may involve sensory, motor, and autonomic nerves, either in isolation
or varying combinations.

More than 100 types of peripheral neuropathy have been identified, each having a
characteristic set of symptoms, a pattern of development, and prognosis

Although some neuropathies may affect all three types of nerves, others primarily affect
one or two types. Therefore, in describing a patient's condition, doctors may use terms
such as:

• Predominantly motor neuropathy

• Predominantly sensory neuropathy

• Sensory-motor neuropathy

• Autonomic neuropathy

ACUTE POLYNEUROPATHY

Acute or subacute polyneuropathies tend to be inflammatory, infectious, or

postinfectious in origin. Acute symmetric polyneuropathy with the rapid onset of
ascending paralysis is usually due to acute inflammatory demyelinating
polyradiculoneuropathy (AIDP), also known as Guillain-Barré syndrome.

. What is the distribution of weakness?

 – Only distal versus proximal and distal
 – Focal/asymmetric versus symmetric
3. What is the nature of the sensory involvement?
 – Temperature loss or burning or stabbing pain (e.g., small fiber)
 – Vibratory or proprioceptive loss (e.g., large fiber)
4. Is there evidence of upper motor neuron involvement?
 – Without sensory loss
 – With sensory loss
5. What is the temporal evolution?
 – Acute (days to 4 weeks)
 – Subacute (4–8 weeks)
 – Chronic (>8 weeks)
 – Monophasic, progressive, or relapsing-remitting
6. Is there evidence for a hereditary neuropathy?
 – Family history of neuropathy
 – Lack of sensory symptoms despite sensory signs
7. Are there any associated medical conditions?
 – Cancer, diabetes mellitus, connective tissue disease or other autoimmune diseases, infection
(e.g., HIV, Lyme disease, leprosy)
 – Medications including over-the-counter drugs that may cause a toxic neuropathy
 – Preceding events, drugs, toxins
INFORMATION FROM THE HISTORY AND PHYSICAL
EXAMINATION: SEVEN KEY QUESTIONS
1. What Systems are Involved?
It is important to determine if the patient’s symptoms and signs are motor, sensory,
autonomic, or a combination of these (Table 459-1). If the patient has only weakness
without any evidence of sensory or autonomic dysfunction, a motor neuropathy,
neuromuscular junction abnormality, or myopathy should be considered. Some
peripheral neuropathies are associated with significant autonomic nervous system
dysfunction. Symptoms of autonomic involvement include fainting spells or
orthostatic lightheadedness; heat intolerance; or any bowel, bladder, or sexual
dysfunction (Chap. 454). There will typically be an orthostatic fall in blood pressure
without an appropriate increase in heart rate. Autonomic dysfunction in the absence of
diabetes should alert the clinician to the possibility of amyloid polyneuropathy.
Rarely, a pandysautonomic syndrome can be the only manifestation of a peripheral
neuropathy without other motor or sensory findings. The majority of neuropathies are
predominantly sensory in nature.

2. What is the Distribution of Weakness?

Delineating the pattern of weakness, if present, is essential for diagnosis, and in this
regard two additional questions should be answered: (1) Does the weakness only
involve the distal extremity, or is it both proximal and distal? and (2) Is the weakness
focal and asymmetric, or is it symmetric? Symmetric proximal and distal weakness is
the hallmark of acquired immune demyelinating polyneuropathies, both the acute
form (acute inflammatory demyelinating polyneuropathy [AIDP], also known as
Guillain-Barré syndrome [GBS]) and the chronic form (chronic inflammatory
demyelinating polyneuropathy [CIDP]). The importance of finding symmetric
proximal and distal weakness in a patient who presents with both motor and sensory
symptoms cannot be overemphasized because this identifies the important subset of
patients who may have a treatable acquired demyelinating neuropathic disorder (i.e.,
AIDP or CIDP).

Findings of an asymmetric or multifocal pattern of weakness narrow the differential

diagnosis. Some neuropathic disorders may present with unilateral extremity
weakness. In the absence of sensory symptoms and signs, such weakness evolving
over weeks or months would be worrisome for motor neuron disease (e.g.,
amyotrophic lateral sclerosis [ALS]), but it would be important to exclude multifocal
motor neuropathy that may be treatable (Chap. 452). In a patient presenting with
asymmetric subacute or acute sensory and motor symptoms and signs,
radiculopathies, plexopathies, compressive mononeuropathies, or multiple
mononeuropathies (e.g., mononeuropathy multiplex) must be considered.

3. What is the Nature of the Sensory Involvement?

The patient may have loss of sensation (numbness), altered sensation to touch
(hyperpathia or allodynia), or uncomfortable spontaneous sensations (tingling,
burning, or aching) (Chap. 31). Neuropathic pain can be burning, dull, and poorly
localized (protopathic pain), presumably transmitted by polymodal C nociceptor
fibers, or sharp and lancinating (epicritic pain), relayed by A-delta fibers. If pain and
temperature perception are lost, while vibratory and position sense are preserved
along with muscle strength, deep tendon reflexes, and normal nerve conduction
studies, a small-fiber neuropathy is likely. This is important, because the most likely
cause of small-fiber neuropathies, when one is identified, is diabetes mellitus or
glucose intolerance. Amyloid neuropathy should be considered as well in such cases,
but most of these small-fiber neuropathies remain idiopathic in nature despite
extensive evaluation.

Severe proprioceptive loss also narrows the differential diagnosis. Affected patients
will note imbalance, especially in the dark. A neurologic examination revealing a
dramatic loss of proprioception with vibration loss and normal strength should alert
the clinician to consider a sensory neuronopathy/ganglionopathy (Table 459-2, Pattern
8). In particular, if this loss is asymmetric or affects the arms more than the legs, this
pattern suggests a non-length-dependent process as seen in sensory neuronopathies.

4. Is There Evidence of Upper Motor Neuron Involvement?

If the patient presents with symmetric distal sensory symptoms and signs suggestive
of a distal sensory neuropathy, but there is additional evidence of symmetric upper
motor neuron involvement (Chap. 30), the physician should consider a disorder such
as combined system degeneration with neuropathy. The most common cause for this
pattern is vitamin B12 deficiency, but other causes of combined system degeneration
with neuropathy should be considered (e.g., copper deficiency, HIV infection, severe
hepatic disease, adrenomyeloneuropathy).

5. What is the Temporal Evolution?

It is important to determine the onset, duration, and evolution of symptoms and signs.
Does the disease have an acute (days to 4 weeks), subacute (4–8 weeks), or chronic
(>8 weeks) course? Is the course monophasic, progressive, or relapsing? Most
neuropathies are insidious and slowly progressive in nature. Neuropathies with acute
and subacute presentations include GBS, vasculitis, and radiculopathies related to
diabetes or Lyme disease. A relapsing course can be present in CIDP and porphyria.

Is There Evidence for a Hereditary Neuropathy?

In patients with slowly progressive distal weakness over many years with very little in
the way of sensory symptoms yet with significant sensory deficits on clinical
examination, the clinician should consider a hereditary neuropathy (e.g., Charcot-
Marie-Tooth disease [CMT]). On examination, the feet may show arch and toe
abnormalities (high or flat arches, hammertoes); scoliosis may be present. In
suspected cases, it may be necessary to perform both neurologic and
electrophysiologic studies on family members in addition to the patient.

7. Does the Patient Have Any Other Medical Conditions?

It is important to inquire about associated medical conditions (e.g., diabetes mellitus,
systemic lupus erythematosus); preceding or concurrent infections (e.g. diarrheal
illness preceding GBS); surgeries (e.g., gastric bypass and nutritional neuropathies);
medications (toxic neuropathy), including over-the-counter vitamin preparations (B6);
alcohol; dietary habits; and use of dentures (e.g., fixatives contain zinc that can lead to
copper deficiency).

The electrodiagnostic (EDx) evaluation of patients with a suspected peripheral

neuropathy consists of nerve conduction studies (NCS) and needle electromyography
(EMG). In addition, studies of autonomic function can be valuable. The
electrophysiologic data provide additional information about the distribution of the
neuropathy that will support or refute the findings from the history and physical
examination; they can confirm whether the neuropathic disorder is a mononeuropathy,
multiple mononeuropathy (mononeuropathy multiplex), radiculopathy, plexopathy, or
generalized polyneuropathy. Similarly, EDx evaluation can ascertain whether the
process involves only sensory fibers, motor fibers, autonomic fibers, or a combination
of these. Finally, the electrophysiologic data can help distinguish axonopathies from
myelinopathies as well as axonal degeneration secondary to ganglionopathies from
the more common length-dependent axonopathies.

NCS are most helpful in classifying a neuropathy as being due to axonal degeneration
or segmental demyelination. In general, low-amplitude potentials with relatively
preserved distal latencies, conduction velocities, and late potentials, along with
fibrillations on needle EMG, suggest an axonal neuropathy. On the other hand, slow
conduction velocities, prolonged distal latencies and late potentials, relatively
preserved amplitudes, and the absence of fibrillations on needle EMG imply a
primary demyelinating neuropathy. The presence of nonuniform slowing of
conduction velocity, conduction block, or temporal dispersion further suggests an
acquired demyelinating neuropathy (e.g., GBS or CIDP) as opposed to a hereditary
demyelinating neuropathy (e.g., CMT type 1).

Autonomic studies are used to assess small myelinated (A-delta) or unmyelinated (C)
nerve fiber involvement. Such testing includes heart rate response to deep breathing,
heart rate, and blood pressure response to both the Valsalva maneuver and tilt-table
testing and quantitative sudomotor axon reflex testing. These studies are particularly
useful in patients who have pure small-fiber neuropathy or autonomic neuropathy in
which routine NCS are normal.
The electrodiagnostic (EDx) evaluation of patients with a suspected peripheral neuropathy
consists of nerve conduction studies (NCS) and needle electromyography (EMG). In addition,
studies of autonomic function can be valuable

EDx evaluation can ascertain whether the process involves only sensory fibers, motor fibers,
autonomic fibers, or a combination of these. Finally, the electrophysiologic data can help
distinguish axonopathies from myelinopathies as well as axonal degeneration secondary to
ganglionopathies from the more common length-dependent axonopathies.

NCS are most helpful in classifying a neuropathy as being due to axonal degeneration or
segmental demyelination.

NERVE BIOPSIES
Nerve biopsies are now rarely indicated for evaluation of neuropathies. The primary
indication for nerve biopsy is suspicion for amyloid neuropathy or vasculitis. In most
instances, the abnormalities present on biopsies do not help distinguish one form of
peripheral neuropathy from another (beyond what is already apparent by clinical
examination and the NCS). Nerve biopsies should only be done if the NCS are
abnormal. The sural nerve is most commonly biopsied because it is a pure sensory
nerve and biopsy will not result in loss of motor function. In suspected vasculitis, a
combination biopsy of a superficial peroneal nerve (pure sensory) and the underlying
peroneus brevis muscle obtained from a single small incision increases the diagnostic
yield. Tissue can be analyzed by frozen section and paraffin section to assess the
supporting structures for evidence of inflammation, vasculitis, or amyloid deposition.
Semithin plastic sections, teased fiber preparations, and electron microscopy are used
to assess the morphology of the nerve fibers and to distinguish axonopathies from
myelinopathies.

SKIN BIOPSIES
Skin biopsies are sometimes used to diagnose a small-fiber neuropathy. Following a
punch biopsy of the skin in the distal lower extremity, immunologic staining can be
used to measure the density of small unmyelinated fibers. The density of these nerve
fibers is reduced in patients with small-fiber neuropathies in whom NCS and routine
nerve biopsies are often normal. This technique may allow for an objective
measurement in patients with mainly subjective symptoms. However, it adds little to
what one already knows from the clinical examination and EDx.
Variants

Typical CIDP is a symmetrical motor and sensory progressive neuropathy affecting

proximal and distal muscles with loss of deep tendon reflexes.

Multifocal Motor Neuropathy is a pure motor disorder in which there is asymmetric

weakness in the distribution of individual nerves that can be confirmed by diagnostic
nerve conduction results.

Lewis-Sumner syndrome is a sensory-motor disorder in which there is sensory loss

and weakness in the distribution of individual nerves. Diagnostic nerve conduction
studies confirm the focal nerve involvement.

Pure sensory CIDP presents with sensory loss, pain, and poor balance with abnormal
gait or walking. There is no weakness but frequently motor nerve conduction studies
are abnormal in addition to sensory conduction studies.

Pure motor CIDP presents with weakness and loss of reflexes without sensory loss.

There are other less well established variants most of which would fall into the
category of CIDP.

It’s not uncommon for CIDP to go undiagnosed for a while due to many factors. The
symptoms may be vague and brushed off until they become more profound and/or
interfere with everyday functioning. Once an individual does go to a physician, a
definitive diagnosis still may not follow.
A diagnosis of CIDP is based on an electrophysiologic pattern of multifocal
demyelination identified through an EMG/nerve conduction study, elevated CSF
(cerebral spinal fluid) protein and, when necessary, nerve biopsy. These tests,
combined with a thorough health history and neurological exam, will help guide the
physician to a correct diagnosis
The classic form of CIDP : fairly symmetric, motor involvement is greater than
sensory.
Weakness is present in both proximal and distal muscles, and this pattern is a
hallmark of acquired demyelinating polyneuropathy.
Cranial nerve and bulbar involvement occur in 10 to 20 percent of patients.
Tremor has been reported as a common symptom in several studies .
Most have sensory involvement and globally diminished or absent reflexes.
Usually greater for vibration and position sense than for pain and temperature sense,
reflecting the involvement of larger myelinated fibers.
Unlike the motor involvement, sensory involvement tends to follow a distal to
proximal gradient, although finger involvement is frequently seen as early as toe and
foot involvement.
Painful dysesthesias can occur. Back pain may also be present. Symptoms of lumbar
spinal stenosis and cauda equina syndrome can occur rarely if there is marked nerve
root hypertrophy, and these problems may require surgical intervention.
Autonomic involvement in CIDP is generally mild and limited in distribution .
Constipation and urinary retention are not usually early symptoms of CIDP, but may
occur in more severe cases.
Most patients with CIDP exhibit a slowly progressive course, but a relapsing-
remitting course is noted in at least one-third, and may be more common in younger
patients.
The advent of early treatment for CIDP has made the temporal progression of the
disease more difficult to characterize, since remissions may be related to therapy
rather than to the natural course of the disease

The long term prognosis of CIDP patients was generally favourable, but 39% of
patients still required immune treatments and 13% had severe disabilities