We are the combination of four hospitals: the General Hospital, the Children’s Hospital, the Women’s Hospital and the Traumatology, Rehabilitation and Burns Hospital. We are part of the Vall d’Hebron Barcelona Hospital Campus: a world-leading health park where healthcare plays a crucial role.
Patients are the centre and the core of our system. We are professionals committed to quality care and our organizational structure breaks down the traditional boundaries between departments and professional groups, with an exclusive model of knowledge areas.
Would you like to know what your stay at Vall d'Hebron will be like? Here you will find all the information.
The commitment of Vall d'Hebron University Hospital to innovation allows us to be at the forefront of medicine, providing first class care adapted to the changing needs of each patient.
Electroencephalography (EEG) records the electrical activity in the brain. During an EEG various electrodes are placed on the patient's skin to record brain activity.
An EEG can be performed under different conditions: With the patient asleep or even subjected to stimulus such as a flashing light, or after breathing deeply to introduce metabolic changes that reveal an area of the brain with unusual brain activity.
There are different types of EEG waveforms according to their frequency and bandwidth. In a healthy person, a normal EEG will produce alpha waveforms with a frequency of 8 to 12 Hz, and a voltage of 50 microvolts. In the same way, beta and delta waveforms may be detected corresponding to different pathologies. Neurological conditions that may benefit from an EEG are: epilepsy, brain tumours, brain abscesses, brain injuries, and cerebrovascular diseases such as heart attacks or haemorrhaging. EEGs can also be used during neurosurgery to detect and distinguish healthy and diseased brain tissue.
Sensors (electrodes) are placed on different parts of the head. This usually takes a few minutes and, as it is non-invasive, can be repeated as often as necessary.
An EEG is a routine test that poses no risks and can be repeated several times during a neurological illness.
Brain imaging tests are also useful and may allow more accurate diagnosis and treatment when combined with an EEG.
Spinal taps are used to extract samples from the cerebrospinal fluid, which is a substance that surrounds the brain and spinal column and protects them from possible injuries. This contact means that when there is a process that affects these body parts, the liquid is altered and we can detect it by analysing it.
Using cerebrospinal fluid, we can measure pressure and take samples to perform different tests.
Thanks to these tests, several diseases can be diagnosed, such as neurological disorders and infections that affect the brain or bone marrow.
Spinal taps, or lumbar punctures, involve taking a sample of cerebrospinal fluid through the puncture with a needle in the lumbar area.
It is usually done with patients in the foetal position: lying on their sides with legs flexed so the knees touch the chest. Sometimes, the puncture is done with the person sitting and leaning forward.
Once the patient is in place, the area where the puncture is to be done is cleansed and disinfected. After administering anaesthesia, the puncture is performed between the two lumbar vertebrae with a spinal needle.
After this, we measure the pressure of the cerebrospinal fluid and take the sample, which will be from 1 to 10 ml.
The needle is then removed, the puncture zone cleaned and a bandage placed over the puncture area. Sometimes patients need to lie down for a while.
Spinal taps are the least aggressive way to get a sample of cerebrospinal fluid. Study of this liquid is essential to detect certain diseases that affect the brain and the spinal cord.
Alzheimer’s disease is a progressive neurodegenerative illness.
Nowadays, Alzheimer’s disease is the most common form of dementia in older people. The families of people with this illness often have to adapt to the new situation. If you are caring for someone with Alzheimer’s, here are some tips:
This illness manifests itself in cognitive deterioration and behavioural disorders, resulting in a high degree of dependency. In the majority of cases, the family looks after the person and within the family, in 76% of cases, a woman is the main carer. If you are caring for someone with Alzheimer’s, bear in mind these tips:
Amyotrophic lateral sclerosis (ALS) causes muscular degeneration that can affect motor autonomy, oral communication, swallowing and breathing, but the senses, intellect and eyes muscles remain intact. It can therefore affect the respiratory muscles, which is why respiratory care is essential for patients’ quality of life.
In order to improve the respiratory difficulties in patients, ventilation therapy can be used through non-invasive ventilation.
Ventilation therapy refers to breathing support using a ventilator, usually at night during sleep, to achieve:
Ventilation is carried out non-invasively, by means of a patient-adjusted mask (nasal or full face) connected by a tube to the ventilator or respirator.
When patients need this therapy, the place and time it is started, whether outpatient or hospital admission, is planned in a personalised way with the consent of the patient and the person caring for them.
Education for the patient and their main carer should begin as soon as possible, both from the point of view of managing secretions and the resulting care, as well as the emotional support they need to receive. This means that during the patient’s admission or outpatient visit, the patient and their carer will be trained in:
The patient and the carer must take care to keep the airway in good condition to allow secretions to be managed. It is important to preserve the ability to cough where possible, but if coughing is no longer effective, the patient and carer will need to start learning how to use mechanical aids (cough assist or mechanically assisted cough). In certain cases secretion suction may also be used.
To improve the quality of life of patients it is important to follow the advice below:
Patients with Asperger’s syndrome need a stable and predictable environment that can be easily adapted. It is key to their well-being to establish routines according to their interests, organise their time, avoid inactivity or over intense activity as well as sudden changes. Although the syndrome has no cure, appropriate treatment and involving family members can improve the quality of life of patients.
People with Asperger’s syndrome may have different requirements depending on their age, surroundings and the awareness that they have of their difficulties. For this reason, they need a tailor-made programme that responds to their specific case.
The aim of these customised programmes is to:
It is important to manage their development through different disciplines. These may include cognitive treatments, social skills programmes and occupational therapy for the patient. You also have to consider guidelines on how to resolve conflicts and how to manage pyschoeducational groups for families or caregivers.
In infants, from an emotional and attitudinal point of view, it is important to learn to identify the warning signs in their mood. In this way, we can prevent difficulties in anger management and low tolerance to frustration, since they are patients with a high degree of sensitivity to criticism. Avoid punishment as much as possible and establish more positive reinforcement strategies.
All these guidelines must be established in a space where the differences the child or adolescent presents are valued positively, including their limitations, but also their possibilities and positive aspects.
In adults, many of these characteristics continue, as Asperger’s cannot be cured. In any case, personalised treatment, involving family members and good communication with professionals can allow a better quality of life.
Dystonia is a neurological disorder characterised by involuntary muscle contractions that cause repetitive movements and tortuous and painful stances every time the patient makes a learned movement, such as walking or speaking.
Dystonia that is detected in childhood can progress rapidly and interfere in the development of the child's language and mobility, causing a physical disability that will affect them throughout life.
Dystonia is a very heterogeneous disease, which can occur for a variety of reasons:
Due to its incidence, dystonia is considered a rare disease.
Dystonia in childhood can occur in isolation or associated with other neurological and development problems. It can cause difficulty in everyday tasks such as walking, speaking, feeding oneself and taking care of personal hygiene.
When presented in isolation it is called primary dystonia and often has a genetic origin. Children who suffer from it do not usually have other health problems and their neurological development is normal. At first it manifests itself in actions such as walking, running, or writing and, later, can spread to other parts of the body and cause widespread dystonia.
Myoclonic dystonia is one of the most frequent hereditary forms of dystonia in childhood. It is characterised by the presence of sustained (dystonic) and abrupt (myoclonic) muscle contractions and psychiatric disorders such as anxiety, depression and obsessive-compulsive features. The first symptoms appear in childhood and affect the lower limbs of children, who have difficulties walking, running and with sports. This disease also affects the social relationships of these children, who have problems speaking in public or eating and drinking with friends.
Dystonia can also be associated with other neurological problems, such as spasticity, ataxia, weakness, delay in neurological development and intellectual disability. In this case we call it secondary dystonia and it is necessary to rule out neurometabolic and neurodegenerative causes.
It affects children, adolescents, and adults of all ages.
First of all, patients are subjected to a series of clinical, metabolic, neurophysiological and neuroimaging studies to classify the type of dystonia, before carrying out genetic studies. In patients with myoclonic dystonia, first of all, a Sanger sequencing study is carried out to determine the gene that causes it, and in the other patients, a complete family exome sequencing(parents and patient) is carried out or the index case (patient) based on the DNA samples available.
Being a rare and very heterogeneous disease it is difficult to reach a correct diagnosis and treatment plan. It is important to distinguish hereditary dystonia from childhood cerebral palsy, caused by brain damage at birth, since its diagnosis has very important consequences for treating the disease later.
A diagnosis in time decreases the need to carry out more diagnostic tests, making it possible to form a prognosis and to advise families on avoiding future diseases. It also has a very positive psychosocial impact on the patient and family. And most importantly, an exact diagnosis of the cause that generates the dystonia makes it possible to guide the best possible treatment for each patient, in what we call personalised medicine.
Dystonia in childhood is progressive and debilitating, but can be prevented with early diagnosis and the use of specific therapies depending on the identified genetic defect.
Levodopa is the treatment of choice in dopa-sensitive dystonia, caused by a defect in the synthesis of dopamine. Botulinum toxin is used to control focal dystonia. In the case of widespread dystonia, different drugs are used to decrease tremors, muscle tone, and painful spasms. And in some cases of paroxysmal dystonia, which is characterised by brief and repetitive involuntary movements during the night, antiepileptic drugs are used.
An intrathecal baclofen pump administers liquid medication through a device that is placed under the skin, and is used to treat generalised secondary forms of dystonia. It reduces pain, decreases muscle tone and spasms. It is a treatment we call symptomatic and palliative, since it does not improve the motor function of the patient.
Deep brain stimulation or globus pallidus stimulation, two electrodes are placed in the globus pallidus using a stereotaxic technique, it is the treatment of choice in primary dystonia, especially if they are widespread and do not respond to conventional medication. In these cases children can recover the function of the area affected by dystonia and improve their quality of life. It can also be useful in patients with secondary forms of dystonia, although its effectiveness is less than in primary forms of dystonia.
Performing genetic testing is the best prevention to avoid having more children affected by this disease in the same family.
Campanya MoutePerLaDistonia
Associació de Lluita contra la Distonia Mioclònica a Espanya
Associació de Malalties Neurodegeneratives amb Acumulació Cerebral de Ferro
Associació GNAO Espanya
Poliomyelitis is a highly contagious disease caused by any of the three human poliovirus serotypes, which are part of the enterovirus family. Europe was certified free of poliomyelitis in June 2002. Immunisation and vigilance of the disease continue to ensure the region is free of poliomyelitis. Post-polio syndrome has no defined causal mechanism but it affects between 20% and 80% of patients afflicted with poliomyelitis.
Initial symptoms are those of a influenza-like illness (fever, headache, joint and muscle pain, vomiting, among other things) and can last up to 10 days. Its most serious forms may cause respiratory paralysis leading to death. Post-polio syndrome presents a new neurological weakness that may be progressive or abrupt on muscles previously affected or unaffected. It may or may not be accompanied by new health problems such as excessive fatigue, muscle pain, pain in the joints, intolerance to cold, reduced physical stamina and function, and atrophy.
It mainly affects children and the mechanisms for its transmission may be through faecal-oral channels or a common vehicle (contaminated water or food).
Post-polio syndrome affects patients who have had poliomyelitis for 20 years or more.
Diagnosis is given clinically, supplemented with laboratory and electromyographic (EMG) tests.
Symptomatic treatment with analgaesics, a ventilator where necessary, gentle exercise and possibility of orthopaedic devices to prevent deformities or to enable function.
In acute diagnoses, studying secretions, stools and cerebrospinal fluid. EMG in acute and later stages for diagnosing post-polio syndrome.
Poliomyelitis has no cure but it can be prevent by vaccination.
Amyotrophic Lateral Sclerosis (ALS) is the most common degenerative motor neurone disease in adults. It is also known as Charcot disease after the famous French neurologist Jean-Martin Charcot who discovered it in 1869. In North America, it is known as Lou Gherig’s disease in honour of a famous baseball player who died at 38 years old as a result of this disease.
Amyotrophic Lateral Sclerosis manifests in the form of progressive paralysis that affects most of the muscles in the diaphragm. The life expectancy is less than five years. In rare cases, longer survival times may be observed, especially if artificial ventilation devices are provided.
ALS is a neurodegenerative disease caused by the death of motor neurons in the brain and the spinal cord.
There are two types of motor neuron: upper and lower. The first are found in the motor cortex and establish connections with the lower motor neurons located in the brain stem and spinal cord, which innervate muscles. When the upper motor neurons die, spasticity, weakness and hyperreflexia appear.
When the lower motor neurons die, twitching, weakness and muscle atrophy occur. Other neuron populations can also be affected, such as the temporal and frontal behavioural and executive circuits.
Epidemiologically speaking, ALS has an incidence of 1.5-2 new cases a year per 100,000 people (3 new cases are diagnosed per day in Spain). The total number of cases (prevalence) is 2-5 per 100,000. According to this data, the total number of patient with ALS in Spain is approximately 4,000 cases. This is why it is included in the rare or minority disease group.
90% of cases of ALS are sporadic (no family history). Around 10% of ALS cases are familial, usually inherited as dominant traits. The incorporation of new molecular genetics techniques in the field of research has allowed more than 25 genes involved in ALS to be identified.
As a consequence of the continuous decrease in motor neurons, symptoms of the disease appear. These usually depend on the location of the motor neurons undergoing the most advanced processes of degeneration. In most patients (70%) the first symptom is loss of strength with muscular atrophy in the hands or clumsiness when walking, with frequent falls. In approximately 25% of patients, the first symptom is difficulty talking or swallowing, which indicates that degeneration of the bulbar motor neuron population is the most intense. There are also other possibilities for clinical presentation of this disease, although much less frequent: respiratory failure, weight loss or unexplained lack of energy (asthenia), cramps and twitches in the absence of muscle weakness, spasticity in legs, rapid mood changes or cognitive impairment.
In advanced phases, the disease can also paralyse the eye muscles. In the final stages of the disease, paralysis of the respiratory muscles leads to respiratory failure, which is often the cause of death.
The condition particularly affects people aged between 40 and 70. The incidence is greater in men (3:2.2 per 100,000) in sporadic forms. The age of first onset of symptoms reaches its peak between 58 and 63 years old in sporadic cases and between 47 and 52 years in familial forms. Incidence decreases markedly after the age of 80. The risk of suffering ALS is 1:400 for women and 1:350 for men.
The differing ways in which ALS manifests is one of the two reasons for a delay in suspected diagnosis of the disease, which can be up to 15 months. The other is that there is no test or biomarker to objectively confirm the diagnosis in the initial stages of the condition. A diagnosis of ALS is a diagnosis of exclusion, based on clinical criteria and conducting tests (MRI, clinical analysis, genetic tests, electromyography, EMTC, neuropsychological exam, nuclear medicine techniques and others) to rule out other illnesses with similar clinical findings. In most specialised ALS units, the disease diagnostic criteria used are the revised El Escorial criteria and the Awaji-shima criteria.
There is currently no medication that can cure or stop the disease. Riluzole and Edaravone are the only medications approved for ALS treatment, although their effect on survival is moderate (months).
The European (EFNS) and American (ANA) associations of neurology recommend that patients with ALS be treated in specialised centres, where possible in multidisciplinary units, so that they might be prepared for any complications. These units should offer solutions to control the symptoms, including the use of a feeding tube, control of saliva secretions, cough assist devices, respirators for mechanical ventilation, technology to improve the patient’s ability to move around and facilitate communication in patients who have lost the ability to speak.
These multidisciplinary units are the centres preferred by those running new drug trials.
The reality is that there is currently no effective treatment, although patients and their relatives often desperately search online for miracle drugs that might cure the condition. ALSuntangled, a group made up of 80 international experts in ALS, was born with the aim of protecting these patients from the numerous products advertised. It mission is to review the veracity and safety of the alternative treatments offered online that have not gone through the proper regulatory channels. It publishes its results in the official magazine for the disease and on its website.
Diagnostic imaging techniques (MRI, CAT, PET), electrophysiology (electromyography, EMTC, PESs), laboratory analysis (haematology, biochemistry, antibodies, hormones, enzymes, serology, genetics), respiratory functional tests, gasometry, pulse oximetry, overnight pulse oximetry, capnography, BMI, calorimetry, lumbar puncture, functional scale for the disease (ALS-FRS-R). A muscular biopsy may be required in exceptional cases. It is advisable to admit the patient in order to arrange for testing and offer them a report on discharge detailing the ALS diagnostic category and degree of functional repercussion (ALS-FRS-R).
Although various environmental risk factors have been suggested (geographic, occupational, dietary habits, proximity to electrical channels, contact with pesticides or other neurotoxins), there is no agreement on preventative measures to take.
In family forms, it is possible to offer genetic counselling to people with a desire for offspring.
During the natural course of the disease, complications often appear that may be prevented and treated. Among the most significant are malnutrition, respiratory failure, hypersalivation, spasticity, pain, loss of independent movement and communication, depression, anxiety, sleep disorders, bed sores, cognitive deficits and burden on carers.
The Multidisciplinary ALS Unit in the Neurology Department at Vall d’Hebron University Hospital is accredited by the Generalitat de Catalunya, Spanish Government (CSUR) and by the European Reference Network for Rare Neuromuscular Diseases (EURO-NMD).
Professionals from the following specialisms make up this unit: Case handling, nursing, social care, neurology, pneumology, rehabilitation, nutritional support, neuropsychology, physiotherapy, speech therapy, endoscopy, interventional radiology, technicians for increasing communication (UTAC).
The coordinator is Dr. Josep Gamez.
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