In the last series of “approach to pediatric neurology signs and symptoms”, I discussed how to approach children presenting with seizures. The following “CELE” approach has been applied for consistency and rational thinking.
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 shall discuss the approach to children presenting with a variety of movement disorders. Pediatric movement disorders (PMDs) comprise a large group of disorders (tics, stereotypies, myoclonus, tremor, dystonia, chorea, Parkinsonism, etc.), often with mixed phenotypes, involving different parts of the body. They can affect the speed, fluency, quality and ease of movement in children. Those movements are involuntary, and mostly not suppressible, but usually absent while children are sleeping. They may be paroxysmal with a completely symptom free period, however, some movement disorders are persistent while the child is awake. Determination of the type of movement disorder and underlying etiology can be sometimes, difficult especially if they are mixed. This can make the diagnostic process time-consuming and difficult. In this article, I present a diagnostic approach for PMDs, with emphasis on phenomenology of movements.
C – Confirmation
Children with different movement disorders can present with abnormal movements, abnormal posture, abnormal gait, difficulty in performing specific tasks e.g., handwriting, drop things, etc.
To confirm the type of movement disorders, the pattern or phenomenology is the key information. Like in children with seizures, children with those movement disorders come to the doctors, but at the time of their clinic visits, they might be totally normal especially if they are paroxysmal. Therefore, as I mentioned in the previous series, the detailed and sequential description of the phenomenology are critical, history taken from the direct witness is important and if possible, video clips of those episodes are incredibly useful. The phenomenology is not describable, but needs visualization.
Following features in phenomenology will be helpful to confirm the type of movement disorders. Please refer to table 1, for more detailed descriptions.
- site and distribution
- whether those movements are migratory (Moving from one part of the body to another) or not
- velocity, rhythm and range of movements
- whether they are stereotypic (the same pattern) or not
- whether they are suppressible or not
- whether their awareness lost or not
- factors aggravating those movements
- whether they are sleep-related or not
- other associated symptoms
Movement disorders can be classified into three major groups according to their velocity of movement;
- hyperkinetic movement disorders
- hypokinetic movement disorders and
- ataxia.
In children, hyperkinetic movement disorders are more prevalent and include tics, dystonia, chorea with or without athetosis, myoclonus, stereotypies, and tremor. Hypokinetic movement disorders, including Parkinsonism, are rare in children. Ataxia is not a hypo or hyperkinetic movement disorder, but includes disorganized and poorly executed movements, and is relatively common in children.
On the other hand, different types of seizures and normal, age appropriate variants need to be excluded. Associated features such as lost of awareness, altered mental status, eyes opened, eyes and head deviation, tongue bite, tonic/clonic movements, incontinence, brief duration, and subsequent drowsiness or sleepiness are suggestive of seizures. Seizures tend to occur or be worse at sleep, whereas the movement disorders usually disappear. It is also important to distinguish between normal, physiological developmental motor patterns and pathological movement disorders.
For example, Infantile fidgety movements and startles resembling chorea and myoclonus normally disappear around the age of 3 months. Dystonic-like features may appear from birth and can persist until the age of 16 years. It may be difficult to distinguish physiological developmental motor patterns from pathological movement disorders. Children should be monitored to evaluate how the movement pattern develops. In general, physiological patterns will subside with normal psychomotor development.
Many movement disorders are quite characteristic to have a spot diagnosis. And the diagnosis is easier especially if they have the constellation (combination) of associated signs and symptoms, preceding cause – e.g. chorea with migratory in nature and associated hypotonia, in children with other features of acute rheumatic fever.
E – Emergency
Some movement disorders are associated with life threatening conditions or those needing urgent care, therefore, recognition of those conditions are important – e.g. children with underlying infectious encephalitis, autoimmune encephalitis, brain tumors. Therefore, associated features e.g., altered mental status, seizures, focal deficits need to be recognized especially if acute onset. Some movement disorders are not in real emergency, but if their underlying causes are missed at that time, the consequences can be permanent for life with high mortality and morbidity – e.g. Wilson’s disease, Sydenham’s chorea.
L – Localization
Almost all movement disorders except ataxia originate from the basal ganglion and related structures, ataxia originates from the cerebellum and related structures. Those anatomical localization of individual movement disorder is mentioned in table -2.
E – Aetiology
In principle, among the long lists of underlying aetiologies, common or treatable or potentially serious conditions need to be prioritized for the diagnosis. The underlying diagnosis depends on following;
– age,
– type of movement disorder,
– onset, natural history and
– associated signs/symptoms
For example, a child with a history of significant birth asphyxia or severe neonatal jaundice, will initially present with developmental delay and hypotonia in early infancy, then followed by choreoathetoid movements. And the diagnosis will be choreoathetoid or dyskinetic cerebral palsy. Children with autism can frequently present with hand flapping, body rocking or sniffing movements and they are stereotypies. An adolescent girl can develop sub-acute onset of abnormal gait, hemi-dystonia of leg, and tends to be worse in the evening, without other obvious causes and these features are suggestive of dopa-responsive dystonia or Segawa disease.
Sometimes, presenting movement disorders 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
This approach can serve as a framework to lead the clinician through the rational diagnostic process, including recognition of the different movement disorders, and emergency conditions, and identification of an underlying conditions. I summarized the above step by step approach in the following algorithm.
Fig.1 The CELE approach
References
- Mink J. Movement disorders. Pediatr Rev. 2010; 31(7): 287-295.
- Korn-Lubetzki I, Steiner I. Common movement disorders in children: diagnosis, pathogenesis and management. Neurosci Med. 2012; 3: 90-100.
- Brandsma R, van Egmond ME, Tijssen MAJ; Groningen Movement Disorder Expertise Centre. Diagnostic approach to paediatric movement disorders: a clinical practice guide. Dev Med Child Neurol. 2021 Mar;63(3):252-258. doi: 10.1111/dmcn.14721. Epub 2020 Nov 5. PMID: 33150968; PMCID: PMC7894329.
Author Information
Kyaw Linn
Paediatric Neurologist
