WHAT IS GUILLAIN–BARRÉ SYNDROME?
Guillain–Barré Syndrome – GBS is a rare, acute immune-mediated disease of the Peripheral Nervous System, involving the nerves that connect the brain and spinal cord to the rest of the body.
It occurs when the body’s immune system, which normally protects us from infections, mistakenly attacks the peripheral nerves and nerve roots.
Guillain–Barré Syndrome is not an infection of the nerves and the syndrome itself is not transmitted from person to person. In some people, however, a preceding infection appears to trigger the abnormal immune response.
Damage to the peripheral nerves disrupts the transmission of motor and sensory nerve signals, causing muscle weakness, sensory disturbances and, in more severe cases, extensive paralysis.
It can occur at any age. It is often preceded, several days or weeks earlier, by a respiratory or gastrointestinal infection.
HOW DOES IT BEGIN AND HOW DOES IT PROGRESS?
In the typical form, the first symptoms are usually numbness, tingling or weakness in the legs.
The weakness may progress rapidly over hours or days and extend upwards, affecting the upper limbs, the face and, in more severe cases, the muscles involved in swallowing and breathing.
Other symptoms may include:
- difficulty walking or inability to stand,
- reduced or absent reflexes,
- pain, particularly in the legs or lower back,
- difficulty swallowing or speaking,
- weakness of the facial muscles,
- disturbances of blood pressure or heart rate.
The severity varies considerably between patients. In some, the disease remains relatively mild, while in others it may progress to extensive paralysis.
Guillain–Barré does not always present with the typical ascending weakness. There are different clinical forms, which may predominantly affect the cranial nerves, balance or particular muscle groups.
WHY IS IMMEDIATE HOSPITAL MANAGEMENT NECESSARY?
Guillain–Barré Syndrome can progress rapidly and affect breathing and swallowing. It may also affect the autonomic nervous system, which regulates bodily functions that we do not consciously control, causing, among other effects, significant changes in blood pressure and heart rate.
For this reason, every patient with suspected Guillain–Barré should be assessed promptly in hospital and monitored closely.
Systematic monitoring of respiratory function is particularly important because respiratory deterioration can progress rapidly.
In severe cases, admission to an Intensive Care Unit and temporary respiratory support with mechanical ventilation may be required.
HOW IS IT DIAGNOSED?
Diagnosis is based primarily on the clinical presentation and neurological examination.
Important investigations include:
- neurophysiological assessment, with nerve conduction studies and electromyography,
- lumbar puncture with examination of the cerebrospinal fluid,
- and, where necessary, additional investigations to exclude other causes.
In the early stages, some investigations may still be normal.
For this reason, when the clinical picture is suggestive, necessary treatment should not be delayed while waiting for characteristic laboratory or neurophysiological findings to appear.
In most cases, neurological deterioration reaches its maximum within four weeks of symptom onset.
If neurological deterioration continues for more than eight weeks after the onset of symptoms, the diagnosis should be reassessed and other immune-mediated neuropathies investigated, such as the acute-onset form of Chronic Inflammatory Demyelinating Polyradiculoneuropathy – CIDP/A-CIDP.
HOW IS THE ACUTE PHASE TREATED?
When immunotherapy is indicated, particularly in patients with significant or rapidly progressive weakness, the two established treatments are intravenous immunoglobulin (IVIg) and therapeutic plasma exchange.
The two treatments have similar effectiveness when given to appropriate patients at the appropriate time.
Sequential treatment with plasma exchange followed immediately by intravenous immunoglobulin is not routinely recommended.
At the same time, close monitoring of breathing, swallowing, cardiac function and blood pressure is essential, together with prevention of complications associated with immobility.
Corticosteroids, unlike in other immunological neuropathies, are not an effective treatment for Guillain–Barré.
WHAT HAPPENS AFTER THE ACUTE PHASE?
Once progression of the neurological deficits has stabilised, the recovery phase gradually begins.
Peripheral nerves need time to restore their function. Improvement may be substantial, but it does not occur at the same rate in every patient.
Most patients achieve significant functional recovery. However, recovery may take months and, in some cases, longer.
Muscle weakness, fatigue, sensory disturbances or neuropathic pain may persist for a considerable period.
WHY IS REHABILITATION IMPORTANT?
Rehabilitation is an essential part of recovery following Guillain–Barré Syndrome.
It begins during the acute phase, as soon as the patient’s clinical condition permits, with appropriate positioning, joint mobilisation, respiratory care and prevention of complications associated with immobility.
As the neurological condition stabilises and muscle strength returns, the therapeutic programme progresses gradually and includes progressive muscle strengthening, functional training, balance and gait exercises, as well as aerobic exercise and respiratory physiotherapy, according to the patient’s abilities and endurance.
The rehabilitation team systematically adjusts the intensity and duration of exercise to the patient’s muscle strength, endurance and fatigue.
In patients with autonomic dysfunction, mobilisation and standing are introduced progressively, with monitoring of blood pressure and heart rate.
FATIGUE AND GRADUAL RETURN TO ACTIVITY
Fatigue may persist even when muscle strength has improved substantially and may affect everyday activities, work and social life.
The aim is neither excessive exertion nor unjustified avoidance of exercise, but the progressive recovery of strength, endurance and functional ability.
HOW DO WE MANAGE GUILLAIN–BARRÉ AT ANAPLASI?
At ANAPLASI, the Multidisciplinary Rehabilitation Team systematically assesses muscle strength, sensation, balance, gait, endurance, respiratory function and the patient’s ability to perform activities of daily living.
Based on these findings, an individualised Rehabilitation programme is developed, with specific and measurable therapeutic goals and systematic reassessment of the patient’s progress and achievement of those goals.
The programme includes:
- physiotherapy,
- progressive muscle strengthening and exercises to improve endurance,
- balance and gait training,
- fatigue management,
- specialised aquatic therapy in the Hydrotherapy Pool,
- occupational therapy and training in activities of daily living,
- management of neuropathic and musculoskeletal pain,
- speech and language therapy for disorders of speech and swallowing,
- Clinical Psychology, including assessment and, where necessary, psychological support for the patient and their family,
- dietetic assessment and follow-up,
- neuro-urological assessment when urinary problems are present,
- assessment for appropriate aids and orthoses and training in their use,
- 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 is used when the patient’s general, respiratory and cardiovascular condition permits. Buoyancy supports body weight and may facilitate mobilisation, balance and gradual gait retraining.
At the ANAPLASI Neurophysiology Laboratory, nerve conduction studies and electromyography are performed when neurophysiological investigation or reassessment is required.
ROBOTIC REHABILITATION
In appropriately selected patients, robot-assisted standing and gait training may be used, with the level of assistance adapted to the patient’s abilities and endurance.
In patients with weakness of the upper limbs and hands, robotic retraining of the upper limb and hand may also be used, according to the patient’s abilities and specific functional goals.
Robotic technology is used as an adjunct and does not replace active therapeutic exercise.
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.
CARE OF THE SEVERELY AFFECTED PATIENT
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.
In ANAPLASI Inpatient Rehabilitation, advanced therapeutic beds are used, allowing controlled and programmed changes of position, alternating lateral rotation, specialised pressure management, a full-chair position 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, when clinically indicated, may form part of the patient’s respiratory care.
These features are used as 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 for safer transfers from the bed to the wheelchair and to treatment areas.
The programme is reassessed systematically and adapted as nerve function recovers, muscle strength returns and the patient’s abilities and endurance change.
SEXUAL FUNCTION AND REHABILITATION
Prolonged weakness, fatigue, pain, autonomic dysfunction and the psychological consequences of a severe acute illness may also affect sexual life.
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 of the disease, the patient’s functional status and the stage of recovery.
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.
