Developmental coordination disorder: disruption of the cerebello-cerebral network evidenced by SPECT.
Little is known about the neurobiological substrate of developmental coordination disorder (DCD), a neuro-developmental syndrome with significant, negative impact on the motor, cognitive and affective level throughout lifespan. This paper reports the clinical, neurocognitive and neuroradiological findings of a 19-year-old patient with typical DCD. As demonstrated by mild ataxia and a close semiological correspondence with the recently acknowledged 'cerebellar cognitive affective syndrome', clinical and neurocognitive investigations unambiguously indicated functional disruption of the cerebellum. Structural MRI of the brain confirmed cerebellar involvement revealing a slight anterior/superior asymmetry of vermal fissures consistent with rostral vermisdysplasia. Although this abnormality of vermal fissuration is generally considered an incidental neuroradiological finding without any clinical relevance, a potentially subtle impact on the developmental level has never been formally excluded. In addition to a generally decreased perfusion of the cerebellum, a quantified Tc-99m-ECD SPECT disclosed functional suppression of the anatomoclinically suspected supratentorial regions involved in the execution of planned actions, visuo-spatial processing and affective regulation. Based on these findings, it is hypothesised that the cerebellum is crucially implicated in the pathophysiologcial mechanisms of DCD, reflecting disruption of the cerebello-cerebral network involved in the execution of planned actions, visuo-spatial cognition and affective regulation.
Mariën
Peter
P
Department of Neurology, ZNA Middelheim General Hospital, Lindendreef 1, 2020 Antwerp, Belgium. peter.marien5@telenet.be
Wackenier
Peggy
P
De Surgeloose
Didier
D
De Deyn
Peter P
PP
Verhoeven
Jo
J
D002531:Cerebellum / Q000000981:diagnostic imaging* / Q000473:pathology / Q000503:physiopathology; D002540:Cerebral Cortex / Q000000981:diagnostic imaging* / Q000473:pathology / Q000503:physiopathology; D003545:Cysteine / Q000031:analogs & derivatives; D005260:Female; D006801:Humans; D019957:Motor Skills Disorders / Q000000981:diagnostic imaging* / Q000473:pathology / Q000503:physiopathology; D009434:Neural Pathways / Q000000981:diagnostic imaging* / Q000473:pathology / Q000503:physiopathology; D009483:Neuropsychological Tests; D015609:Organotechnetium Compounds; D019275:Radiopharmaceuticals; D015899:Tomography, Emission-Computed, Single-Photon; D055815:Young Adult
D015609:Organotechnetium Compounds; D019275:Radiopharmaceuticals; C059659:technetium Tc 99m bicisate; D003545:Cysteine
Neuroradiology. 2002 Aug;44(8):639-46
12185541
Cortex. 2010 Jul-Aug;46(7):831-44
20152963
Child Care Health Dev. 2006 Nov;32(6):741-50
17018049
J Child Neurol. 2009 Oct;24(10):1273-81
19687388
Brain. 1998 Apr;121 ( Pt 4):561-79
9577385
Neural Plast. 2003;10(1-2):141-53
14640315
Hum Mov Sci. 2007 Jun;26(3):491-509
17509709
Clin Neurol Neurosurg. 2008 Sep;110(8):763-73
18602745
Rocz Akad Med Bialymst. 2005;50 Suppl 1:87-90
16119635
Mov Disord. 2009 Sep 15;24(12):1820-8
19562773
Acta Psychol (Amst). 2004 Feb-Mar;115(2-3):271-89
14962404
Clin Neurol Neurosurg. 2006 Jul;108(5):518-22
16055261
Cortex. 2008 Jan;44(1):54-67
18387531
Neurology. 2009 Aug 18;73(7):566-7
19687458
Brain Res. 2008 Dec 9;1244:89-102
18718456