Approach to Paediatric Neurology Signs and Symptoms – CELE approach

Neurological symptoms are common in children and are challenging because of complexity, age and development-dependent presentations, information mainly from the caretakers rather than children themselves, and wide variation of underlying conditions ranging from benign, self-limited nature to life-threatening diseases3. Paediatric neurology comprises a large number of diseases and conditions in which their etiology, signs and symptoms, and complications very much depend on the age of the child – neonate, infant, toddler, older children, etc. For example, seizures in neonates may be very subtle and never generalized because of the immature development and connections of developing brain, whereas seizures of older children are easily generalized and present as a well-known pattern of generalized tonic clonic seizures. Again, because of the developing nature of the brain, newborn and infant brains are more susceptible to permanent damage causing different patterns of neurodevelopmental, cognitive, and behavioral issues which are mostly irreversible⁴.

Because of the above reasons, many medical professionals are a bit reluctant to manage children with neurological signs and symptoms. To help this issue, I created a simple approach to those children, recognizing characteristic patterns, emergency and life-threatening conditions, excluding age dependent normal variants, localizing possible sites of lesions and evaluating the most possible etiologies. I named this approach as “CELE” approach and representations of each word are as follow;

C – Confirmation of signs and symptoms with looking for Constellations, Characteristic features, and Clues

E – Emergency

L – Localization of site of lesion

E – Evaluation for Etiology

In this article, I am going to explain those steps of “CELE” approach briefly and then I will write subsequent articles with the same approach to groups of paediatric neurological signs and symptoms including;

1. paroxysmal symptoms
2. movement disorders
3. weakness
4. developmental problems
5. altered mental status.

C – Confirmation

Children of different ages come to the doctors with different complaints or symptoms and those symptoms are either from caregivers or from the patients themselves. Caregivers complain of those symptoms either they see or think or hear that information from another source such as other family members, neighbors or school teachers. Since they are so closed to the children, we cannot ignore those concerns and have to pay attention to those complaints, however, on the other hand, the words they mentioned are either inaccurate or ambiguous, especially if the source of information is not from the direct witness. Therefore, second hand description of signs and symptoms are not acceptable in terms of accuracy and reliability. If the complaints come directly from the children themselves, it might be reliable if in older children, however, the younger the child, the less reliable.

For example, the parents and families always complain of “possible seizures or convulsions” if they see any spells or impaired consciousness or stiffening episodes, however, the majority of them are turned out to be “seizures mimics” rather than true seizures. On the other hand, some seizure types especially in newborn and infants like subtle seizures, epileptic spasms, might not be recognized as seizures because of their special patterns different from common seizure types. Therefore, confirmation of these complaints and symptoms are critically important before you approach further for investigations and treatments. That will avoid unnecessary tests, drugs, other interventions, expenses and worries.

How to confirm these complaints, signs and symptoms? Spending more time on history taking, gathering more detailed and relevant information related to that particular complaint, exploring relevant background information like pregnancy, delivery, development and family data, are essential to initially approach a child with neurological signs and symptoms. If possible, for some paroxysmal episodes, like possible seizures, movement disorders, video clips of those episodes are incredibly useful for confirmation of particular complaints, since those cannot be thoroughly explained by the families and also the doctors might not see them in the clinic setting.

In some conditions, you can see characteristic features or clues regarding underlying diagnosis, although the presenting complaints or symptoms are not accurate. Following are some examples⁴;

• hypopigmented macules suggesting possible tuberous sclerosis complex
• KF ring suggesting Wilson’s disease
• positive hyperventilation test suggesting absence seizure
• diurnal variation of symptoms – weakness in myasthenia gravis and dystonia in Segawa disease
• orofacial dyskinesia in anti NMDA receptor encephalitis

Sometimes, although one feature is not enough for the diagnosis, the constellation of signs and symptoms may indicate possible diagnosis and following are some examples4;

• paroxysmal headache, relieved by sleep, with a family history of migraine suggesting migraine
• nystagmus, head nodding and torticollis in infant suggesting spasmus nutans
• delayed walk or frequent fall, positive Gower sign, calf hypertrophy, in a boy suggesting Duchenne’s muscular dystrophy

In summary, Confirmation of presenting complaints with recognizing Characteristic features, Clues and Constellation of signs and symptoms, are critical before approaching for further management.

E – Emergency

The most important part of a patient’s management in clinical practice is to recognize emergency or life-threatening signs and symptoms and treat accordingly. This also applies to paediatric neurology conditions since many underlying diseases like CNS infections, inflammation, demyelination, tumors, seizures, etc. are potentially serious and need urgent management. Therefore, doctors and pediatricians need to be familiar with emergency signs and symptoms, and red flags indicating possible serious conditions. For example, in a child with headache, features such as, signs of raised intracranial pressure, progressively severe nature, disturbance of daily activities and sleep, are red flags indicating needs for urgent work up of underlying cause¹.

In my opinion, not only those life-threatening conditions, but also some diseases which can cause lifelong severe, irreversible neurodevelopmental problems unless early diagnosis and intervention provided, can be assumed as relatively emergency conditions. One example is an autism spectrum disorder; therefore, medical professionals should know early features of autism.

In summary, this “E” step of “Emergency” approach can detect emergency, life threatening and potentially serious conditions, so that early resuscitation, treatment and intervention can be possible.

L – localization

The beauty of the nervous system is systematically arranged networks of central and peripheral neuronal pathways. Whenever they are damaged, their individual presenting signs and symptoms are unique and therefore, localization of site lesion is quite easy if we understand those pathways, networks and their functions. When we know the site of lesion, again, we can consider and narrow down possible etiologies in those areas.

Following table shows common sites of lesions and presenting signs and symptoms².

• Weakness with signs of lower motor neuron lesion (LMNL)
  In summary, this “L” step of “Localization” approach will guide us to the site of lesion and that will help again for evaluation of underlying etiology.

E – Etiology

After the initial steps of Confirmation of complaints, signs and symptoms, trying to look for Characteristic features, Clues and Constellation of signs and symptoms, detecting Emergency and life-threatening conditions, and Localizing the site of lesion, evaluation for the possible Etiology is the last, but not the least step of CELE approach. Following information are helpful in looking for the etiology;

• age of the child
• site of lesion
• onset of signs and symptoms – acute/ subacute/insidious
• time course – improving/static/progressively worse/paroxysmal
• associated signs and symptoms

For example, when toddlers presented with ataxia (complaint), the site of lesion will be the cerebellum and if that presentation is subacute or gradually progressive (onset and time-course), with signs and symptoms of raised intracranial pressure (associated features), posterior fossa tumors (etiology) will be the most possible diagnosis. Paraplegic (complaint) children with bowel and bladder symptoms (associated features) might be because of spinal cord lesion (localization) and if of insidious onset with constitutional symptoms (other associated features), tuberculosis (etiology) of the spine will be the top differential diagnosis⁴.

When we consider the possible etiologies, we pay attention to common causes as well as treatable conditions so that necessary specific treatment can be prescribed as early as possible. Sometimes, presenting signs and symptoms are very vague, not severe and not always present, not disturbing the child’s activities of daily living and underlying etiology cannot be explained. In that case, if the child is otherwise normal (normal development, normal neuro-exam), that child should be just followed up without any investigations and medications. Those symptoms might go away or that will be found out as normal variants rather than pathological conditions. During the follow up period, the families should be given safety advice and reassurance.

Conclusion

I have explained briefly about the “CELE” approach to paediatric neurological signs and symptoms, with some simple examples. I believe principles are more important than knowledge and a rational approach is more practical than a text-written battery of tests and management options. Therefore, I would encourage the junior doctors to learn patients at bed sides with a rational approach and principles that can be applied to every patient in any situation.

References

1. Fenichel, GM. 2005. “Clinical pediatric neurology, a signs and symptoms approach” (5th edition). Elsevier Saunders.
2. Ralston, S. H., Penman, I. D., Strachan, M. W. J., & Hobson, R. (Eds.). 2018.“Davidson’s principles and practice of medicine (23rd ed.)”. Elsevier Health Sciences.
3. Rasmussen LA, Grégoire MC. 2015 “Challenging neurological symptoms in paediatric palliative care: An approach to symptom evaluation and management in children with neurological impairment.” Paediatric Child Health ;20(3):159-65. doi: 10.1093/ pch/20.3.159. Erratum in: Paediatric Child Health. 2015 Nov-Dec;20(8):466-7. PMID: 25914579; PMCID: PMC4403283.
4. Swaiman, K. F., Ashwal, S., Ferriero, D. M., Schor, N. F., Finkel, R. S., Gropman, A. L., Pearl, P. L., &Shevell, M. I. 2017.“Swaiman’s Pediatric Neurology: Principles and Practice”:(6^thEdition). Elsevier Inc.

Author Information

Kyaw Linn
Paediatric Neurologist