Neurology in children. The Role of Child Neuropsychiatry (PART II)

Neurology in children. The Role of Child Neuropsychiatry (PART II)

In the second part of the article, we will provide some insights into four of the most prevalent neuropsychiatric conditions as follows:
1.-Epileptic syndromes: Temporal Lobe Epilepsy (TLE)
2.-Neurodevelopmental disorders:
2.a-Guilles Tourette Syndrome (GTS).
2.b-Autism Spectrum Disorders (ASD).
2.c-Attention Deficit and Hyperactivity Disorder (ADHD).

1.-TEMPORAL LOBE EPILEPSY

Temporal lobe epilepsy (TLE) is the most common of the anatomically defined syndromes accounting for around 60% of all patients with localisation-related epilepsy. Temporal lobe seizures produce varied and complex symptoms. It can become challenging for psychiatrists to distinguish these symptoms from similar presentations in other psychiatric conditions.

The most frequent cause of TLE is mesial temporal sclerosis (50-70% of cases), which is the slimming of the cortical hippocampal region of the brain. Mesial sclerosis is strongly associated with a history of childhood febrile convulsions, although in most cases aetiology remains unclear, that is, primary.

Temporal lobe seizures may take the form of simple (level of consciousness is preserved) or complex (reduced level of consciousness) and can be displayed as partial (movements of one part of the body) or generalized seizures (movements of the whole body).

A variety of autonomic features and visceral sensations have been described in so-called temporal lobe auras, being epigastric auras the most common of such presentations, that is, ill-defined sensations rising from epigastrium towards the throat. Other autonomic symptoms include changes in skin color, blood pressure or heart rate.

From a mental health perspective, it is worth noting that affective experiences have been commonly observed prior to temporal lobe seizures. Specifically, patients tend to complain about anxiety-related symptoms and subjective feelings of depression, guilt and/or anger, which are, of course, unpleasant. In addition, deja vú phenomena, that is, the patient erroneously reports having experienced the same reality before, have been frequently observed in those suffering from TLE. On the other hand, pleasurable effects of joy, elation or ecstasy can occur, although less frequently. In this regard, it has been speculated that Santa Teresa de Jesus may have suffered from TLE.

Of note, patient’s family members should be aware of the impact of aura on emotions, which can be associated with hallucinatory experiences and/or disturbed behaviour. More specifically, temporal lobe epilepsy sufferers can present with auditory, olfactive and/or visual hallucinations, which can be, of course, very stressful.

Not only patients with temporal lobe epilepsy can present hallucinations, but also visual and olfactive hallucinations can be displayed by people with dementia. Although auditory hallucinations can be considered as cardinal symptoms of schizophrenia, auditory hallucinations in TLE are an intrinsic part of the seizure, usually stereotyped, brief, evolving and lack an emotional response, that is, distress.

2.- NEURODEVELOPMENTAL DISORDERS

2.A Guilles de la Tourette Syndrome (GTS):
Guilles Tourette Syndrome (GTS) has been currently categorised as a neurodevelopmental disorder in the DSM 5, which is the most up-to-date classification of mental disorders by the American Psychiatric Association. GTS onset usually occurs at age 6 and there is a higher predominance in males than in females (3:1).
The aetiology (i.e., causes) of GTS remains poorly understood, although a combination of genetic, epigenetic and environmental factors have been postulated to underlie this syndrome. Truly, most patients with GTS have first-degree relatives with symptoms, which would provide support for a genetic component.
Regarding GTS presentations, the core characteristic is the presence of multiple tics which tend to go with forced involuntary vocalisations, which usually takes the form of obscene words or phrases (which is known as coprolalia). Specifically, GTS symptoms can be summarised as follows:
1.-INVOLUNTARY MOVEMENTS AND TICS:
Multiple motor tics (simple or complex) and at least one vocal tic is required for making a GTS diagnosis. Tics occur very frequently throughout the day almost every day for more than a year.
Tics are simple non-purposeful movements of functionally related muscle groups, which can be divided into simple tics which affect specific muscle group (for instance, eye-blinking) and complex tics, which involve several muscle groups, such as touching parts of the body. Phonatory muscles have been linked with vocal tics, such as sounds or grunt.
2.-BEAHAVIOURAL ALTERATIONS:
Psychiatric symptoms and behavioural disturbances in GTS patients can mirror features of obsessive-compulsive disorder (OCD), which may raise issues about the differential diagnosis between the two. More specifically, up to 28-65% of patients with GTS have been reported to have intrusive thoughts, which tend to focus on order and symmetry.
3.- COGNITIVE ISSUES:
To make matters more complicated, GTS patients can present with comorbid ADHD, which occurs in 31-91% of cases. Occurrence of ADHD is not associated with severity of the tic disorder, however. Other cognitive issues, such as executive dysfunction, have been commonly associated with GTS, which can have a very negative impact on their academic performance.

2.B. Autism spectrum disorders (ASD):
Today’s psychiatry has expanded autism boundaries to include a more heterogeneous group of patients that can present with a wide variety of symptoms and relevant differences in functional outcomes. Accordingly, the term spectrum has been adopted. In other words, ASDs include from patients with mild deficits to severely affected children and adolescents.
ASD core symptoms affect three domains:
1.- RECIPROCAL SOCIAL COMMUNICATION ISSUES:
ASD patients usually suffer reciprocal social communication issues, that is, problems in setting up bidirectional language. More specifically, verbal and non-verbal communication deficits have been described, such as catching doble senses and jokes, being able to create stories or the ability to flexibilise the language, poor eye contact, abnormalities in tone, intonation and rhythm of the conversation and/or limited range of gestures.
2.-STEREOTYPED AND REPETITIVE BEHAVIOURS:
ASD children tend to struggle to regulate their own behaviours, that is, changing routines and activities. As a result, ASD patients show restrictive and stereotyped behaviours, and an inappropriate lack of cognitive flexibility which affects their quality of life, including interests, habits and play. Patients with ASD usually lack creativity and imagination which turns in repetitive play more focused on objects and sequences.
Additionally, abnormal specific sensory interests have been reported in children with ASD. For example these patients tend to struggle with new colours, textures, smells and/or sounds, which can even trigger an outburst at home. Although the neurobiological mechanisms underlying these sensory issues remain unknown, the role of hyperreactivity in specific brain regions has been widely accepted.
Also, parents and caregivers should be aware of repetitive non purposeful movements, such as balancing or flapping, as major symptoms of classic ASD.
3.-SOCIAL INTERACTION DEFICITS
Most importantly, ASD has a significant impact on social day-to-day life. These children tend to become socially isolated as a result of difficulties in making and maintaining social relationships, which is a core symptom of the disorder. To make matters worse, lack of understanding of conventional social rules (for example grief, parties or celebrations) may result in inappropriate behaviours. Therefore, all the stakeholders involved in the care of ASD children have a role in mitigating their social isolation. This noted, early detection leading to early intervention has been demonstrated to be the most successful strategy to improve short- and long-term outcomes in ASD patients, thus improving their quality of life as well as reducing the burden of their family members and careers.

2.C. Attention deficit and hyperactivity disorder (ADHD):
Diagnosis of ADHD tends to be confirmed at around 6-7 years old, although earlier symptoms have been observed, which may be linked with worse prognosis. Up to 5% of children may suffer from ADHD and approximately half of them will continue to display ADHD symptoms in adulthood, particularly if untreated.
ADHD core symptoms can be categorised in three main domains, which are detailed below. Although most ADHD patients present with abnormalities in the three domains, more rare types of ADHD with deficits in a single domain have been described. Also, it is worth noting that many day-to-day behaviours displayed by ADHD children actually involve several domains, which therefore overlap each other.
As alluded to above, currently available pharmacological and non-pharmacological interventions can alleviate these symptoms and reduce their impact on functioning. Moreover, if (early) treated, ADHD can get ‘cured’. Hence, no ADHD patient should be prevented from receiving such a successful treatment, which, otherwise may have long-term implications.
1.- ATTENTION:
ADHD patients have significant difficulties in staying focused on cognitive processes, such as following multiple-step instructions and/or picking up specific details during a conversation. This inattention is not related to motivation. Thus, not only inattention can be observed when carrying out school tasks, but also during leisure time.
In addition, these children seem to get easily distracted by stimuli so they may not listen to others when directly questioned. Moreover, poor compliance with instructions in ADHD patients have been linked with poorer academic performance. Consistent with this, parents complain of their tendency to losing personal belongings and avoidance of those tasks requiring high levels attention/concentration.
2.- HYPERACTIVITY:
Hyperactivity observed in most of these children led to the conceptualization of ADHD decades ago. In particular, ADHD children are unable to keep still without moving different parts of the body, e.g. restless legs. Fidgetiness can be easily picked up by non-professionals, e.g. restless legs, rolling or rubbing their hands and/or having tics. Sometimes these features can be observed during sleep time.
Parents of children with ADHD have also reported that their children tend to choose games which do not involve cognitive processes. Rather, they prefer to play with more physical-based games and they appear to be unable to wait turn, e.g. queuing, travelling by plane or going to the cinema. These behaviours are frequently picked up by teachers who observed their inability to keep seated and to follow lessons at school. Moreover, ADHD children are usually described as talkative people who can become disruptive by interrupting others in conversations, which may contribute further to worsening their academic performance.
3.- IMPULSIVITY:
Impulsivity in ADHD refers to a tendency towards a decision-making style characterised by not reflection on consequences. As a result, they tend to take much risk, e.g. crossing the road without checking the traffic, climbing high places or walking around closed areas. These decisions can result in accidents (for instance, while cycling) and fractures.
ADHD-related impulsivity also involves emotional regulation and communication issues. Examples of these behaviours include interrupting others in conversations, prompting inadequate responses, lending others’ belongings and/or jumping queues.
Similarly, impulsivity is observed in terms of emotional regulation and understanding their own emotions, which they can express inadequately.

Summary and conclusions

Through this article we have intended to provide an overview of the role of Neuropsychiatry, which as a branch of psychiatry remains unknown to many people, in the management of children suffering from neuropsychaitric conditions. 

In particular, four highly prevalent neuropsychiatric conditions, which have been detailed above and have a relevant impact on child neurodevelopment, highlight the importance of better understanding neuropsychiatry. On reflection, professional staff should undertake specific training in neuropsychiatry, which should result in high-quality multidisciplinary teams delivering care to these complex children in need.

To sum up, highly qualified neuropsychiaty professionals should be prioritised in order to better manage children and adolescents suffering from neuropsychiatric conditions. In particular, the aforementioned complex needs of these patients and the high prevalence of neuropsychiatric disorders such as ASD and ADHD require integrative treatments delivered by multidisciplinary teams working collaboratively from a multiagency approach.

Division of Medicine
Dra. Encarna Domínguez
Neurologist
Psychiatrist

Children, adolescents and adults
Languages: English and Spanish
See Resumé

Child and adolescent neurology

Neurology in children. The Role of Child Neuropsychiatry (PART I)

As parents, it is important to ensure the health and safety of our children. With advances in health science, today more than ever, we have the tools and professionals that help us even with less common health conditions, including diseases and pathologies associated with the nervous system, which many times , develops from an early age.

Therefore, it is crucial to know what neurology is, how some neurological conditions can affect children, the role of health professionals such as neuropsychiatrists when treating these conditions and some of the most common pathologies associated with the nervous system and can affect the development of our children. In the following article we will explore this subjects.

Child and adolescent neurology

Neurology is a branch of medicine concerning especially with the structure, function and diseases of the nervous system. Our nervous system is divided in Central Nervous System (CNS) and Periferic Nervous System (PNS). The first one includes the brain, cerebellum, medulla oblongata and spinal cord. The periferic nervous system includes all the extensions from central organs to muscles and periferic senses. eurology involves a wide range of CNS-PNS related diseases from cognitive conditions (related to memory processes and other brain functions such as writing, reading or calculations) to neuromuscle or movement diseases.

Child Neurology is also a widely diverse and extensive science. Child Neurology includes genetic conditions (such as Rett Syndrome or metabolic myopathies) nerodegenerative issues (such as multiple sclerosis) structural and biological illnesses (such as complex epilepsies) infectious diseases (such as meningoencephalitis) or neurodevelopmental conditions (such as Guilles Tourette Syndrome)

Neuropsychiatry is a branch of Neurology linked to Psychiatry. Currently, this subspecialty does not exist as such in Spain. However, neuropsychiatrists are widely recognised across most European countries including the UK and Germany. In these countries, all the professionals dedicated to Neuropsychiatry undertake specific training programmes for acquiring necessary skills for the manegement of patients with neuropsychiatric disorders. As a result, there is a shortage of Neuropsychiatrists in Spain, which is even more concerning in terms of neuropsychiatrists in children and adolescents. This results in an unacceptably high number of children and adolescents with undiagnosed/untreated neuropsychiatric conditions who also have very complex unmet needs in Spain. In other words, these patients seem to be neglected by the Spanish medical health system. The interface between Neurology and Psychiatry is a random consensus by modern science, a simple response to professionals´ needs of categorizing conditions. Over the last few centuries, clinicians have made big efforts to better understand human body functioning. In order to do so, medicine has been artificially split up in different specialties. However, such an approach may have ignored close relationship between mind and body, which particularly applies to neuropsychiatric conditions.

So far, the division between Neurology and Psychiatry can be summarized as follows: Conditions in which organic alterations can be objectively shown by diagnostic tests (for example MRI, EEG or blood tests) belong to Neurology (such as epilepsy) and Conditions with no evidence of structural damage would fall under the scope of Psychiatry (such as bipolar disorder). As noted above, bipolar disorder is one of the most common “mental” disorders. However, it is intriguing that first line treatment for bipolar disorder, that is so called mood stabilizers, includes several antiepileptic drugs such as valproate, carbamacepine and lamotrigine. Hence, it could be speculated that both conditions may share some neurobiological underpinnings. This said the link between neuropathology and psychiatry appears to be stronger tan previously thought. Also, well-known genetic vulnerability to bipolar disorder, including some candidate genes, suggests that currently available tests may fail to identify its neuroanatomical correlates. In summary, our daily clinical practice in psychiatry is full of examples in which the interface between Neurology and Psychiatry is far from clear. Thus, the vast majority of patients with neurologicval and psychiqtric conditions present with very complex symptoms, which reflect the complexity of human brain rather than an arbitrary diagnostic category.

Over the last decade science paradigms have significantly changed. For instance, although genetic has been considered a major risk factor for developing mental disorders, this concept has evolved to so called epigenetic. In short, while genes were suggested to underlie stable markers of diferent diseases, that is, something that can not be modify through intervention, modern reasearch has shown that genes expression can vary depending on the influence of external factors, that is environment. The gen-environment interaction has been labelled as epigenetic. This model has had a real impact on today’s Neuropscyhiatry resulting in a more comprehensive approach to mental and neurological issues.

Child neuropsychiatric conditions are highly prevalent. These conditions involve a wide range of illnesses which share neurobiological factors and mental symptoms. Therefore, an integrative approach to treatment of child neuropsychiatric conditions is required. This approach should be provided by multidisciplinary teams integrated by Child Neurologists, Child Psychiatrists, Child Neuropsychiatrists, Child Psychologists and Family Therapists.

In addition, children present with special features which make the management of neuropsychiatric conditions even more challenging:

  1. CHILD NEURODEVELOPMENT:The majority of neuropsychiatric conditions are characterised by symptoms which partially overlap with features of normal development such as stereotyped movements, regressions or sleeping disturbances. A profound knowledge of child milestones is therefore required for making an accurate diferential diagnosis. In particular, distinguising normal signs of different madurative stages from pathological symptoms should characterised all the members of this multidisciplinary process.
  2. PLASTICITY OF CENTRAL NERVOUS SYSTEM DURING THE DEVELOPMENT: During the childhood CNS is a complex neuronal network with high levels of plasticity and astonishing malleability. That is, children´s brain continuosly changes during the first two decades of life. This means that the same structural damage in an adult brain can cause huge symptoms in comparison with minimal symptoms during the development. However, this results in an increasing complexity in the diagnosis process, management and treatment of child neuropsychiatric conditions.
  3. COLLATERAL INFORMATION FROM PARENTS:: When gathering information from parents, two steps need to be taken: First one being selecting those behaviours which may be associated with pathological symptoms as opposed to normal developmental features. the second step is among potential pathological symptoms, clinicians should be able to make the differential diagnosis and translate these symptoms to potential neuropsychiatric conditions. For instance obssesive-compulsive behaviours vs repetitive play which is normal during the development or abnormal sensory experiences such as hallucinations vs imaginative play such as imaginary Friends. In keeping with this, professionals such as GPs, teachers, counsellors or paeditricians play also an important role in early detection as the main referrals when picking up potential mental health related issues. In order to provide a better understanding of the challenges above, we are going to summarise two major child neuropsychiatric conditions: epileptic syndromes during neurodevelopment and neurodevelopmental disorders.

Neurology in children. The Role of Child Neuropsychiatry (PART I)

In the two parts of this article we are going to develop some of the entities that can be treated by child neuropsychiatry. More specifically, we are going to talk about 4 diseases with a high prevalence in the population, we will start with 2 of the most common conditions:

  1. Epileptic syndromes during the neurodevelopment: these syndromes involve a number of neurological processes in which convulsive or non-convulsive seizures can emerge during the neurodevelopment. The vast majority of these conditions have been reported to have an impact on CNS maturation which suggests that patients with early epileptic syndromes can presetn with a combination of neurological issues namely seizures, epileptic status or postcrisis symptoms, and behavioural problems such as cognitive disorders, emotional dysregulation or communicative issues. Temporal lobe epilepsy is the cornestone of epileptic neuropsychiatric síndromes which is commonly caused by a structural damage in the temporal lobe. Given the involvement of the temporal lobe in major cerebral functions such as audition, smells and emotions, temporal lobe epilepsy is not only characterised by seizures but also by psychiatric symptoms such as auditory and/or olfatory hallucinations, deja vù or deja vie phenomena or behavioral changes.
  2. Neurodevelopmental disorders: While the specific origin of neurodevelopmental disorders remains unclear a number of factors such as genetic predisposition, environmental factors and structural brain damage, have been postulated. The most common age of onset is from 2 to 6-7 years, that is in early development. As a result, this children usually present with cognitive, metacognitive, emotional and communicative issues.

In the second part of this article we will provide more detailed information on three representative examples of neurodevelopmental disorders such as Guilles Tourette Syndrome, ADHD (attention deficit and hiperactivity disorder) and ASD (autism spectrum disorders) ,all of which are clinically characterised by a wide spectrum of symptoms despite their common neurodevelopment related origin.

Division of Medicine
Dra. Encarna Domínguez
Neurologist
Psychiatrist

Children, adolescents and adults
Languages: English and Spanish
See Resumé