WHAT IS CIDP?
Chronic Inflammatory Demyelinating Polyradiculoneuropathy – CIDP is a rare, immune-mediated disorder of the Peripheral Nervous System in which the body’s immune system, which normally protects us from infection, mistakenly attacks the peripheral nerves and nerve roots.
The damage primarily affects myelin, the protective covering around many nerve fibres that allows nerve impulses to be conducted rapidly and efficiently.
When myelin is damaged, conduction of nerve impulses becomes slowed or blocked. In severe or prolonged disease, secondary damage to the nerve fibres themselves may also occur — axonal damage — which can limit complete neurological recovery.
This may result in muscle weakness, sensory disturbances, unsteadiness and difficulty walking.
HOW DOES CIDP PRESENT AND PROGRESS?
In typical CIDP, there is progressive, usually symmetrical muscle weakness affecting both the lower and upper limbs.
The weakness may involve muscles closer to the trunk, such as those of the thighs and shoulders, as well as the more distal muscles of the hands and feet.
Common associated features include:
- numbness or tingling in the hands and feet,
- reduced sensation,
- unsteadiness and impaired balance,
- difficulty climbing stairs, rising from a chair or walking,
- weakness of the hands and difficulty with fine movements,
- reduced or absent reflexes,
- neuropathic pain,
The disease may follow a progressive course or may present with relapses and remissions.
Unlike Guillain–Barré Syndrome, which usually progresses acutely over days or a few weeks, CIDP is generally characterised by progression of symptoms over a period of at least eight weeks.
There are, however, cases with acute onset (A-CIDP) which may initially resemble Guillain–Barré Syndrome and only become distinguishable from it through their subsequent clinical course.
FORMS OF CIDP
CIDP does not present in the same way in every patient.
In addition to typical CIDP, recognised CIDP variants include distal, multifocal or focal patterns, as well as motor and sensory forms.
In recent years, Autoimmune Nodopathies have also been recognised. In these conditions, specific autoantibodies target proteins located at the nodes of Ranvier and the paranodal regions of peripheral nerves, which are crucial for normal conduction of nerve impulses.
These disorders may resemble CIDP clinically and electrophysiologically, but they are now considered a separate category because they have different pathophysiological mechanisms and may respond differently to treatment.
HOW IS CIDP DIAGNOSED?
There is no single test that can diagnose CIDP on its own.
Diagnosis is based on the combination of the clinical presentation, neurological examination and, most importantly, neurophysiological assessment.
Motor and sensory nerve conduction studies are central to the diagnostic evaluation and can demonstrate electrophysiological features of demyelination, such as marked slowing of nerve conduction or impaired transmission of nerve impulses.
Depending on the individual case, further investigations may include:
- electromyography,
- lumbar puncture and examination of the cerebrospinal fluid,
- haematological and immunological investigations,
- assessment for other causes of polyneuropathy,
- specific testing for autoantibodies against nodal and paranodal proteins when clinically indicated,
- specialised imaging of peripheral nerves and nerve plexuses using ultrasound or MRI in selected cases.
When the clinical and neurophysiological findings are not sufficient on their own, additional supportive evidence may be used where appropriate.
An objectively documented response to immunotherapy may provide supportive diagnostic evidence in selected cases, but is not sufficient on its own to establish the diagnosis of CIDP.
Response to treatment should be documented using objective measures of functional ability and neurological status, rather than relying only on the subjective assessment of the patient or doctor.
Accurate diagnosis is particularly important because other neuropathies may cause similar symptoms but require different treatment.
HOW IS CIDP TREATED?
CIDP is an immune-mediated neuropathy for which effective treatments are available. Early treatment is important in limiting neurological damage and preserving function.
Established first-line treatments include:
- intravenous immunoglobulin (IVIg),
- corticosteroids.
In motor CIDP, IVIg is usually preferred as initial treatment.
Therapeutic plasma exchange is also an effective treatment and is used particularly when the above treatments are ineffective or cannot be given.
For long-term maintenance treatment, intravenous immunoglobulin (IVIg), subcutaneous immunoglobulin (SCIg) or, where indicated, corticosteroids may be used, depending on the form of the disease, the patient’s response to treatment and their needs.
Treatment is individualised according to the form and severity of the disease, response to treatment, adverse effects and the needs of each patient.
More recently developed targeted immunotherapies include efgartigimod alfa, an inhibitor of the neonatal Fc receptor (FcRn), which has been approved in the European Union as monotherapy for adults with active CIDP with a progressive or relapsing course, following prior treatment with corticosteroids or immunoglobulins.
In patients with Autoimmune Nodopathies, the treatment strategy may differ and depends on the specific autoantibody, clinical presentation and response to treatment.
Some of these disorders respond poorly to conventional CIDP therapies and may require a different immunotherapeutic approach.
WHY IS REHABILITATION IMPORTANT?
While immunotherapy aims to control the disease, Rehabilitation addresses its functional consequences.
Muscle weakness, sensory disturbances, unsteadiness, reduced endurance, pain and fatigue may significantly limit walking, independence in daily activities, work and everyday life.
For this reason, Rehabilitation should form part of the overall management of the patient.
Therapeutic exercise should be individualised, progressive and systematically monitored, with the aim of improving muscle strength, endurance and functional ability without causing excessive exertion.
Particular attention is given to the assessment and management of fatigue, which is common in CIDP and may significantly affect function and quality of life.
HOW DO WE MANAGE CIDP AT ANAPLASI?
At ANAPLASI, the Multidisciplinary Rehabilitation Team systematically assesses each patient’s neurological and functional status and develops an individualised Rehabilitation programme with specific and measurable therapeutic goals and regular reassessment of the patient’s progress and achievement of those goals, in collaboration with the treating neurologist.
The programme includes:
- physiotherapy,
- progressive muscle strengthening and exercises to improve endurance,
- fatigue management,
- specialised aquatic therapy in the Hydrotherapy Pool,
- occupational therapy and training in activities of daily living,
- management of neuropathic and musculoskeletal pain,
- balance and gait training,
- assessment for appropriate aids and orthoses and training in their use,
- speech and language therapy for disorders of speech and swallowing,
- Clinical Psychology, including assessment and, where required, psychological support for the patient and their family,
- guidance on returning to work and social life,
- assessment and management of sexual dysfunction.
Information, education and guidance are also provided to the patient and their family on the course of the disease, the Rehabilitation process and the safe management of functional difficulties.
For inpatients, respiratory physiotherapy is provided from the time of admission, with the aim of maintaining and improving respiratory function, promoting better lung expansion and facilitating the mobilisation and clearance of bronchial secretions.
Specialised aquatic therapy in the Hydrotherapy Pool, when the patient’s general condition permits, uses buoyancy and the other physical properties of water to facilitate movement, balance and functional exercise with reduced loading.
SPEECH AND SWALLOWING
When swallowing is impaired, specialist assessment and treatment are provided.
At the ANAPLASI Dysphagia Unit, when clinically indicated, Flexible Endoscopic Evaluation of Swallowing (FEES) is performed to assess the safety and efficiency of swallowing and to guide the individualised planning of treatment, feeding and hydration.
When speech or communication has been affected, the corresponding specialist assessment and therapeutic intervention are provided.
NEUROPHYSIOLOGICAL AND FUNCTIONAL ASSESSMENT
At the ANAPLASI Neurophysiology Laboratory, when required, neurophysiological assessment is performed using motor and sensory nerve conduction studies and electromyography.
Functional assessment includes systematic and, where possible, quantified evaluation of muscle strength, sensation, balance, gait, endurance, fatigue and the patient’s ability to perform activities of daily living.
Where appropriate, progress is documented using standardised outcome measures such as I-RODS and INCAT, the MRC sum score, and objective measures of muscle strength, including grip strength, so that disease progression and response to therapeutic interventions can be recorded with greater accuracy.
ROBOTIC REHABILITATION
In appropriately selected patients, modern robotic rehabilitation technology may be used for controlled and repetitive training in standing, balance and walking and, where a corresponding functional deficit is present, for retraining of the upper limb and hand.
Robotic technology is used as an adjunct and does not replace active therapeutic exercise.
MANAGEMENT OF NEUROPATHIC PAIN
Neuropathic and musculoskeletal pain are also addressed as part of the overall programme.
In selected patients with chronic neuropathic pain that is not adequately controlled by established treatments, Navigated Repetitive Transcranial Magnetic Stimulation – navigated rTMS may be considered as an adjunctive intervention.
This application is aimed at the management of neuropathic pain and is not a treatment for CIDP itself.
FATIGUE AND GRADUAL RETURN TO ACTIVITY
Fatigue is common in CIDP and may persist even when muscle strength has improved substantially.
For this reason, therapeutic exercise and functional training are adjusted individually and progressively, according to the patient’s muscle strength, endurance, fatigue and response to the therapeutic programme.
The aim is to improve strength, endurance and functional ability without excessive exertion or unjustified restriction of activity.
CARE OF PATIENTS WITH SEVERE FUNCTIONAL LIMITATION
In patients with severe muscle weakness or very limited mobility, Rehabilitation and prevention of complications begin early, as soon as the patient’s medical condition permits, even before the patient is able to get out of bed.
During Inpatient Rehabilitation at ANAPLASI, advanced therapeutic beds are used, allowing controlled and programmed changes of position, alternating lateral rotation, specialised pressure management, full-chair positioning and, where indicated, gradual progression towards a more upright position.
These functions also contribute to both the prevention and management of pressure ulcers.
The beds also provide percussion and vibration functions which, where clinically indicated, may form part of the patient’s respiratory care.
These features form part of the overall medical, nursing and therapeutic management and do not replace active mobilisation or the structured Rehabilitation programme.
In patients with a high level of physical dependence, electrically powered patient hoists may also be used to facilitate safer transfers from the bed to the wheelchair and to treatment areas.
The programme is reassessed systematically and adapted according to the course of the disease, response to immunotherapy and changes in functional status.
SEXUAL FUNCTION AND REHABILITATION
CIDP may affect sexual life through muscle weakness, fatigue, sensory disturbances, neuropathic pain, reduced mobility and the psychological consequences of chronic disease.
When a relevant problem is present and the patient wishes to address it, sexual rehabilitation may include individualised assessment, management of factors affecting sexual function, practical guidance, counselling and psychological support and, where required, specialist medical treatment.
The involvement of the patient’s partner may be helpful, if the patient wishes.
HOW IS REHABILITATION PROVIDED?
Rehabilitation may be provided through Inpatient Rehabilitation, Day Rehabilitation or Rehabilitation at Home, according to the severity and course of the disease, the patient’s functional status and individual needs.
WHAT IS THE AIM OF THE REHABILITATION PROGRAMMES?
At ANAPLASI, the aim is to achieve the greatest possible recovery of the patient’s functional ability and independence and to support their active return to personal, family, social and working life.
