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It is important to remember that MS is only one part of a person and not the person.
MS does not represent them, it is not their identity, they are as normal as the next person.
Children
Authors -
Introduction
Multiple sclerosis (MS) in children is being recognized with increasing frequency. The first descriptions of MS in children were published by Charcot between 1829 and 1849, though it was not for another 50 years that MS in children was again described in the literature (Hanefeld, 2007). There are now several national programs focused on the research and clinical management of children with MS. Recently, an International Pediatric Multiple Sclerosis Study group was constituted with the goal of fostering collaborative efforts (for more information, email: info@ipmssg.org).
Demographics and Epidemiology of Pediatric Multiple Sclerosis
How common is MS in children?
Analysis suggests that 2% to 5% of all patients with MS are diagnosed before their 16th birthday (Ness et al., 2007). These estimates, however, are based on retrospective review of established adult MS populations and may underestimate the true prevalence of the disease in the pediatric population. The annual average incidence of a first demyelinating event in Canadian children is 0.9/100,000, but has been reported as lower in other parts of the world (Banwell et al., 2007; Pohl, 2008). The incidence of MS diagnosis following an acute demyelinating event is the subject of ongoing research.
Genetics of MS
Genetic factors clearly influence the risk of developing MS, as MS can “run” in families.
The risk of developing MS is approximately 30% if you have an identical twin with
MS, 5% if you have a first degree relative (parent or non-twin) with MS, but only
0.1% if no one in the family has MS (Sadovnick, Dircks, & Ebers, 1999). Furthermore,
carefully documented family histories reveal that approximately 20% of people with
MS will have at least one first degree or distant relative with MS (Sadnovnick, Baird,
& Ward, 1988). Family history data obtained from a large international study of MS
demonstrated that 6% to 8% have a positive history of MS (Banwell et al., 2007).
It is important to remember that the first degree relatives of pediatric patients
with MS are still young, and may still be at risk to develop MS in the future.
A female preponderance in MS is well-
Immunological Studies
In order for the immune system to “attack”, it must first recognize the “target.” Scientists are very interested in learning what is initially targeted in MS. Complicating this search, however, is the fact that once the immune system is active, it will not only attack the initial target, but over time will also attack the injured tissue in the brain/spinal cord as well.
We found that children with MS harbour T-
Environmental Triggers
Infectious Triggers
Children with MS have been shown to have a significantly increased likelihood, relative
to healthy age-
Sunlight Exposure, Vitamin D, and MS
The increased prevalence of MS in temperate regions has prompted consideration of
the role of vitamin D and sunlight exposure as potential non-
Clinical Features of Acute Demyelination in Children
The First Attack
MS in both children and adults is characterized by multiple episodes of neurological dysfunction secondary to inflammatory demyelination of the central nervous system (CNS). Just as in adults, however, not all children who experience an initial acute demyelinating syndrome (ADS) will develop MS. The term “clinically isolated syndrome or CIS” has also been applied to persons experiencing a first demyelinating event, although many authors restrict the term CIS to patients with an initial demyelinating event at high risk for future diagnosis of MS. As such, the term “CIS” is not universally applied across the entire spectrum of ADS events, particularly those considered to have a low risk of relapses.
An ADS is classified as “monofocal” if the clinical features were referable to a single CNS lesion, such as optic neuritis, transverse myelitis, or brainstem, cerebellar, or hemispheric dysfunction; and as polyfocal if the clinical features are localized to more than one CNS location. This is based on the physician’s clinical examination, rather than MRI findings (which could show asymptomatic lesions). “Polyfocal” features refer to more than one CNS lesion and when accompanied by problems with thinking, is termed, acute disseminated encephalomyelitis (ADEM) (Krupp, Banwell, & Tenembaum, 2007).
Specific ADS presentations include:
Transverse Myelitis: Transverse myelitis (TM), or attack of the immune cells on the spinal cord, leads to loss of strength and sensation of the limbs and difficulty with bowel and bladder control. TM was the presenting feature of MS in only 14% of children enrolled in a multinational pediatric MS Study (Banwell, Teller et al., 2005).
Optic Neuritis: Optic neuritis (ON), an attack of the immune system on the optic nerve from the eye, results in reduced vision, pain with eye movements, and difficulty seeing color. It has been thought that bilateral ON is more common in children and unilateral ON more common in adults. This may simply reflect, however, that young children may not notice or report loss of vision in one eye. In one study of childhood ON, in which some patients were followed for 40 years, 26% were ultimately diagnosed with MS (Lucchinetti et al. 1997). In a review of ON at SickKids (www.sickkidsfoundation.com), bilateral ON was more common than monocular ON, and was associated with a greater likelihood of MS diagnosis (Wilejto et al., 2006). Of the 36 children enrolled, 13 (36%) were diagnosed with MS within the two years of ON, an outcome that was highly correlated with MRI evidence of white matter lesions in the brain. Optic neuritis
Acute Disseminated Encephalomyelitis (ADEM): For a diagnosis of ADEM, there must be a multiple neurological symptoms plus trouble thinking (encephalopathy). The demyelinating event in some children may be accompanied by fever, drowsiness or even coma, and neck stiffness.
What happens to children with ADS
In a review of 296 children with acquired demyelination in France, 57% were diagnosed
with MS, while the remaining 43% appeared to have a monophasic illness (Mikaeloff,
Suissa et al., 2004). The children in this study were followed for a mean of 2.9
years (range 0.5– 14.9 years). Since patients can develop their second MS-
Recurrent Attacks -
Pediatric MS requires multiple episodes of CNS demyelination separated in time (by four weeks or more) and space (involving new areas of the CNS) just as is specified for adults. MRI evidence of new lesions in new CNS locations can be used to meet the requirement for disease dissemination in time (Polman et al., 2005).
Approximately 95% of pediatric patients with MS have recurrent attacks followed by
periods of clinical recovery or stability (Banwell, Ghezzi et al., 2007; Boiko et
al., 2002). This form of MS is known as relapsing-
Figure 1 illustrates the typical MRI features of MS in children.
How do children with MS do?
The time from the initial acute attack to the second, MS-
In a multinational study of 137 children with MS, 13% of children with MS showed fixed neurological deficits that limited their ambulation (EDSS >4.0) after a mean disease duration of 5 years. Mikaeloff and colleagues, (Mikaeloff et al., 2006) documented EDSS scores of 4 or higher in 15% of children with MS enrolled in the French KIDSEP study after a median observation of 4.8 years (from second demyelinating event).
While physical disability may occur relatively infrequently in the first decade in
pediatric-
Disease Course
In a study reviewing the disease course of 116 patients with MS onset under age 16
years, 53% of the 116 patients ultimately progressed to SPMS at 23 years post-
Symptoms
Many symptoms may accompany an MS relapse, which by definition, lasts at least 24 hours.
Sensory symptoms: The most common sensory symptoms are numbness and paresthesias
(tingling) in one or more limbs. The sensory symptoms can be due to a myelopathy,
which can produce a spinal sensory level. Sensory deficits that arise from lesions
in the sensory cortex or the supraspinal pathways lead to numbness. Patients may
also have radicular symptoms due to a lesion at the dorsal root entry zone of the
spinal cord or the brainstem, although this is very rare. Patients with sensory deficits
involving the dorsal column pathways subserving vibration and propioception, can
experience a “useless hand syndrome” in which motor movement is preserved, but the
ability to manipulate the arm in space is impaired (El-
Motor symptoms: Weakness can occur in any extremity, singly or in combination. The
most dramatic of the acute motor syndromes is an acute transverse myelitis. In most
children with MS, TM manifests as a partial cord syndrome. Longitudinally extensive
lesions that traverse the cross-
Spasticity: Spasticity or stiffness of the limbs during attempted limb movement occurs in patients following severe relapses associated with residual damage to motor pathways, and occurs as a core component of the progressive disability seen in the secondary progressive phase of MS. As such, it is relatively rare as a major symptom in children with RRMS. When present, spasticity is disabling, causes disruption of sleep, and contributes to pain.
Bladder and sexual function: Lesions of the distal spinal cord can impair both bladder
and sexual function. While such deficits are rarely reported in children and adolescents
with MS, recognition of these issues is critical. Impaired bladder emptying can lead
to retention of urine, infection, and potential life-
Bladder impairment most commonly results from overactivity of the detrusor muscle
of the bladder. This produces the sensation or urgency despite low bladder volume.
Urge incontinence occurs if high intravesical pressure results in the loss of some
urine. Detrusor-
Bladder problems -
Fatigue: Fatigue or a “sense of physical tiredness and lack of energy, distinct from sadness or weakness,” is reported by approximately 40% of children and adolescents with MS. (Banwell, Ghezzi et al., 2007). Fatigue of sufficient severity to compromise participation normal activities, such as sports, social events, or completion of academic tasks is considered worthy of treatment.
Dysarthria: Children with MS can have different forms of dysarthria or impaired speech
production. Dysarthria of the cerebellar type results in scanning speech which is
characterized as monotonous speech interspersed with explosive consonants, resulting
in irregular volume and indistinct articulation tremor of the voice. As cerebellar
involvement occurs relatively commonly in pediatric-
Tremors and other movement disorders: Tremors in MS are usually most notable when
the child is reaching for an object or attempting to perform purposeful movements
of the upper limbs. Tremor in MS is associated with greater impairment and functional
disability due to impairments in hand-
Pain: A significant number of adults with MS, and a lesser proportion of pediatric patients with MS, experience pain
Pain -
Fig 1.
MRI image of a 13 year old boy presenting with his first demyelating event. Numerous lesions are noted (see arrows), and the patient subsequently developed numerous MS relapses. The image highlights the potential for marked CNS involvement in pediatric MS.
Source -
