DMCN 12476

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tell us how the sample directly compares to international developmental windows, cut-off points, and other history.

populations. They can, however, reassure us that the AIMS Serendipitously, their methodology is well described and
hasn’t changed and results from the AIMS should be uti- could be readily replicated with alternate populations, work
lized as they have been in the past. The authors stress that might contribute additional information on the big
appropriate caution in interpreting the AIMS related to questions of motor development.

REFERENCES
1. Piper M, Darrah J. Motor Assessment of the Developing motor development in infants. Dev Med Child Neurol ged in 20 years? A re-evaluation of the Alberta Infant
Infant. Philadelphia, PA: Saunders, 1994. 2007; 49: 858–67. Motor Scale normative values Dev Med Child Neurol
2. Pin T, Eldridge B, Galea MP. A review of the effects of 3. Darrah J, Bartlett D, Maguire TO, Avison WR, Lacaze- 2014; 56: 877–81.
sleep position, play position, and equipment use on Masmonteil T. Have infant gross motor abilities chan-

Are proprioceptive functions affected in Duchenne muscular


dystrophy?
DIDO GREEN development, such as in Duchenne muscular dystrophy
Centre for Rehabilitation, Oxford Brookes University, Oxford, UK. (DMD), and what impact this may have on functional
motor skills.
doi: 10.1111/dmcn.12476 The study by Troise et al.6 draws attention to potential
This commentary is on the original article by Troise et al. on pages sensory impairments associated with DMD. Their findings
882–887 of this issue. highlight a dependence on visual feedback for manual dex-
terity in the absence of impairments in either two-point
The processing of sensory and perceptual information tactile discrimination or stereognosis. Their results, reflect-
has long been a focus of interest for researchers and cli- ing a greater discrepancy in the manual skills of young
nicians in attempting to understand the motor coordina- males with DMD than control children only when per-
tion difficulties of children with developmental disorders1 forming the manual task with the eyes closed, lead to a
and those with cerebral palsy (CP).2,3 Until recently the number of hypotheses. While muscle weakness may have
assessment of tactile, proprioceptive, and visual functions played a role in the slower speeds for identifying objects,
has formed an important component in the diagnostic this does not provide an explanation for equivalent speeds
formulation of developmental coordination disorder in the manual dexterity task when the eyes were open. The
(DCD). This included the assumption that impaired sen- authors have therefore conjectured that this may be due to
sorimotor integration underpinned the perceptual-motor reduced glucose metabolism although it is not particularly
problems of these children and thus should be the focus evident that this may be due to lateralisation of hypo-
of treatment (European Academy for Childhood Disabil- metabolism to the right hemisphere.
ity recommendations). The evidence for the benefits of Alternatively, Troise et al. also postulate that the depen-
sensory motor treatments for children with DCD how- dence on visual feedback is similar to that in younger
ever, is marginal.4 The sensory deficits of children with children and therefore may reflect more generalized delays
CP have been shown to affect movement production, in motor development in children with DMD. They did
particularly upper limb performance.3 Furthermore, chil- not however explore proprioception directly.
dren with CP with better sensory status have shown pro- It has been shown from both human and animal studies
longed retention of motor skill progress following that the discharge from muscle spindles, which provide
practice.5 the main source of proprioceptive feedback to the CNS,
In both DCD and CP, the predominant disturbance to is modulated by the contractile state of the skeletal mus-
function is linked to atypical structural or functional con- cles in which they are embedded.7 While Ribot-Ciscar
nectivity within the central nervous system (CNS) affecting et al.8 suggest that muscle spindle proprioceptive func-
both sensory and motor systems, although the neural basis tions may in fact be spared in muscular dystrophy, their
of DCD has yet to be defined. Less well known though is sample only included four participants with DMD or
how the sensory system is affected in children with muscle Becker muscular dystrophy, the remaining with myotonic,
weakness that is not due to atypical brain function or fascioscapulohumeral, or limb-girdle dystrophies. The

Commentaries 805
sparing of intrafusal muscle fibres and proprioceptive potential treatment of manual dexterity in DMD. Further
functions may be limited to the more slowly progressive exploration of the underpinnings of both sensory and per-
muscular dystrophies.9 ceptual control of movement across muscular dystrophies
Troise et al. have provided an important basis for con- is warranted, especially considering the implications for
sideration of proprioceptive feedback in the assessment and treatment planning.

REFERENCES
1. Mon-Williams MA, Wann JP, Pascal E. Visual-proprio- recommendations on the definition, diagnosis and inter- 7. Wise AK, Fallon JB. The effect of muscle contraction
ceptive mapping in children with developmental coordi- vention of developmental coordination disorder (long on kinaesthesia. Adv Exp Med Biol 2002; 508: 87–94.
nation disorder. Dev Med Child Neurol, 1999; 41: 247– version). Dev Med Child Neurol, 2012; 54: 54–93. 8. Ribot-Ciscar E, Trefouret S, Aimonetti JM, Attarian S,
54. 5. Robert MT, Guberek R, Sveistrup H, Levin MF. Motor Pouget J, Roll JP. Is muscle spindle proprioceptive
2. Yekufiel M, Jariwala M, Stretch P. Sensory deficit in the learning in children with hemiplegic cerebral palsy and function spared in muscular dystrophies? A muscle
hands of children with cerebral palsy: a new look at the role of sensation in short-term motor training of tendon vibration study. Muscle Nerve 2004; 29:
assessment and prevalence. Dev Med Child Neurol 1994; goal-directed reaching. Dev Med Child Neurol, 2013; 55: 861–6.
36: 619–24. 1121–8. 9. Aimonetti JM, Ribot-Ciscar E, Rossi-Durand C, Attari-
3. Auld ML, Boyd RN, Moseley L, Ware RS. Impact of 6. Troise D, Yoneyama S, Resende MB, Reed U, Xavier an S, Pouget J, Roll JP. Functional sparing of intrafusal
tactile dysfunction on upper-limb motor performance in G, Hasue R. The influence of visual and tactile muscle fibers in muscular dystrophies. Muscle Nerve
children with unilateral cerebral palsy. Arch Phys Med perception on hand control in children with Duchenne 2005; 32: 88–94.
Rehabil, 2012; 93: 696–702. muscular dystrophy. Dev Med Child Neurol, 2014; 56:
4. Blank R, Smits-Engelsman B, Polatajko H, Wilson P. 882–87.
European Academy for Childhood Disability (EACD):

Ketogenic diet in children with intractable epilepsy: what about


resting energy expenditure and growth?
SIMONA BERTOLI 1 | ALBERTO BATTEZZATI 1 | From the available research it is hard to draw firm con-
2 clusions on the effect of the ketogenic diet on growth.
ANNA TAGLIABUE
1 Department of Food Environmental and Nutritional Sciences (DeFENS), Quite often results are confounded by ketogenic dietary
International Center for the Assessment of Nutritional Status (ICANS), Milano; management, follow-up protocol, duration of treatment
2 Department of Public Health, Experimental and Forensic Medicine, Human exposure, and pre-existing malnutrition (as in our own pre-
Nutrition and Eating Disorder Research Center, University of Pavia, Pavia, Italy. vious investigation3). In this regard, the present study pro-
vides an advance in this area. In line with previous research,
doi: 10.1111/dmcn.12474
results show that height z-score decreased overall from
This commentary is on the original article by Groleau et al. on pages baseline to 3 and 15 months, indicating height velocity
898–904 of this issue. deceleration, particularly in children with CP. This could
suggest differential mechanisms underlying the association
The study by Groleau et al.1 looks at the effect of ketogen- between the ketogenic diet and growth, possibly linked with
ic diet treatment on resting energy expenditure (REE) and the putative effects of ketone bodies plasma level and
growth in children with intractable epilepsy with and with- chronic ketosis on intermediate metabolism and hormone
out cerebral palsy (CP) over 15 months. It is an attempt to secretion (i.e. growth hormone and insulin-like growth
better understand the metabolic and nutritional long-term factor I) according to different neurological diseases.
effects of the ketogenic diet in children at different degrees Moreover, the ketogenic diet could act differently on
of risk for growth and body composition abnormalities. nutritional status across the different developmental ages.
In the last decade, only one study has focused on the In adults affected by glucose transporter 1 deficiency syn-
effects of the ketogenic diet on REE2 in children with intrac- drome (Glut1-DS) it was recently shown that the keto-
table epilepsy without CP for over 6 months of dietary treat- genic diet over 5 years did not have any major negative
ment. The authors stated that ketogenic diet treatment impact on nutritional status.4 From this point of view,
increases fat oxidation without significant changes in REE. growth retardation could reflect the potential compensa-
These findings are confirmed in the present study tory mechanisms of the neuro-endocrine axis to cope with
suggesting that the ketogenic diet does not change daily the ketogenic diet-induced alteration in protein metabo-
basal metabolic rate both in the short- or in long-term. lism.

806 Developmental Medicine & Child Neurology 2014, 56: 801–807

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