Cerebral metabolic alterations and cognitive dysfunction in children with chronic kidney disease using magnetic resonance spectroscopy and Wechsler intelligence scale

Youssef, Doaa Mohammed; Mohamed, Ahmed Hosny; Attia, Wafaa Mahmoud kamel; Mohammad, Faten Fawzy; Fatah, Nelly Rafaat Abd El; El‐Shal, Amal S.

doi:10.1111/nep.13085

Cited by 3 publications

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References 10 publications

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“…More precisely, a mean difference of − 10.5 was noticed between CKD children and the general population [45,47,49,50]. A significant negative correlation was found between CKD stages and Wechsler IQ test [51]. Longer CKD duration has been associated with poorer performance on attention regulation and inhibitory control [52].…”

Section: Discussionmentioning

confidence: 96%

Neuropsychological and neuroanatomical phenotype in 17 patients with cystinosis

Curie

Touil

Gaillard

et al. 2020

Orphanet J Rare Dis

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Background: Cystinosis is a rare autosomal recessive disorder caused by intracellular cystine accumulation. Proximal tubulopathy (Fanconi syndrome) is one of the first signs, leading to end-stage renal disease between the age of 12 and 16. Other symptoms occur later and encompass endocrinopathies, distal myopathy and deterioration of the central nervous system. Treatment with cysteamine if started early can delay the progression of the disease. Little is known about the neurological impairment which occurs later. The goal of the present study was to find a possible neuroanatomical dysmorphic pattern that could help to explain the cognitive profile of cystinosis patients. We also performed a detailed review of the literature on neurocognitive complications associated with cystinosis. Methods: 17 patients (mean age = 17.6 years, [5.4-33.3]) with cystinosis were included in the study. Neuropsychological assessment was performed including intelligence (Intelligence Quotient (IQ) with Wechsler's scale), memory (Children Memory Scale and Wechsler Memory Scale), visuo-spatial (Rey's figure test) and visuoperceptual skills assessments. Structural brain MRI (3 T) was also performed in 16 out of 17 patients, with high resolution 3D T1-weighted, 3D FLAIR and spectroscopy sequences. Results: Intellectual efficiency was normal in patients with cystinosis (mean Total IQ = 93). However the Perceptual Reasoning Index (mean = 87, [63-109]) was significantly lower than the Verbal Comprehension Index (mean = 100, [59-138], p = 0.003). Memory assessment showed no difference between visual and verbal memory. But the working memory was significantly impaired in comparison with the general memory skills (p = 0.003). Visuospatial skills assessment revealed copy and reproduction scores below the 50th percentile rank in more than 70% of the patients. Brain MRI showed cortical and sub-cortical cerebral atrophy, especially in the parieto-occipital region and FLAIR hypersignals in parietal, occipital and brain stem/cerebellum. Patients with atrophic brain had lower Total IQ scores compared to non-atrophic cystinosis patients. Conclusions: Patients with cystinosis have a specific neuropsychological and neuroanatomical profile. We suggest performing a systematic neuropsychological assessment in such children aiming at considering adequate management.

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