Lesions of the Corpus Callosum in Children With Neurofibromatosis 1

Abstract Objective Neurofibromatosis 1 (NF1) is a rare autosomal dominant disease that causes the dysregulated growth of Schwann cells. Most reported studies of brain morphology in NF1 patients have included only children, and clinical implications of the observed changes later in life remain unclear. In this study, we used MRI to characterize brain morphology in adults with NF1. Methods Planar (2D) MRI measurements of 29 intracranial structures were compared in 389 adults with NF1 and 112 age- and sex-matched unaffected control subjects. The 2D measurements were correlated with volumetric (3D) brain measurements in 99 of the adults with NF1 to help interpret the 2D findings. A subset (n = 70) of these NF1 patients also received psychometric testing for attention deficits and IQ and was assessed for clinical severity of NF1 features and neurological problems. Correlation analysis was performed between the MRI measurements and clinical and psychometric features of these patients. Results Four of nine corpus callosum measurements were significantly greater in adults with NF1 than in sex- and age-matched controls. All seven brainstem measurements were significantly greater in adults with NF1 than in controls. Increased corpus callosum and brainstem 2D morphology were correlated with increased total white matter volume among the NF1 patients. No robust correlations were observed between the 2D size of these structures and clinical or neuropsychometric assessments. Conclusion Our findings are consistent with the hypothesis that dysregulation of brain myelin production is an important manifestation of NF1 in adults.

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Alterations in Brain Morphology by MRI in Adults with Neurofibromatosis 1

10.21203/rs.3.rs-711404/v1 ◽

2021 ◽

Author(s):

Su Wang ◽

Victor-Felix Mautner ◽

Ralph Buchert ◽

Stephane Flibotte ◽

Per Suppa ◽

...

Keyword(s):

Corpus Callosum ◽

Psychometric Testing ◽

Neurofibromatosis 1 ◽

Clinical Severity ◽

Brain Morphology ◽

Consistent Finding ◽

Unaffected Control ◽

Dominant Disease ◽

Brain Measurements ◽

Mri Measurements

Abstract Objective: Neurofibromatosis 1 (NF1) is a rare autosomal dominant disease that causes the dysregulated growth of Schwann cells. Most studies focused on brain morphology changes in NF1 were small and only included children, making clinical implications unclear. One consistent finding in children with NF1 has been increased corpus callosum area. We aimed to characterize alterations in brain morphology by MRI in adults with neurofibromatosis 1 (NF1). Methods: Planar (2D) MRI measurements of 29 intracranial structures were compared in 389 adults with NF1 and 112 age- and sex-matched unaffected control subjects. The 2D measurements were correlated to volumetric (3D) brain measurements for 99 of the adults with NF1. A subset of adults with NF1 (n = 70) was also assessed for clinical severity of NF1 features and neurological problems and received psychometric testing for attention deficits and IQ. Correlation analyses were performed between principal components of the intracranial measurements and clinical and psychometric features of these patients. Results:Four of nine corpus callosum measurements were significantly greater in adults with NF1 than in sex- and age-matched controls. All seven brainstem measurements were significantly greater in adults with NF1 than in controls. Increased corpus callosum and brainstem 2D morphology were correlated with increased total white matter volume among the NF1 patients. No robust correlations were observed between the 2D size of these structures and clinical or neuropsychometric assessments.Interpretation:Our findings are consistent with the hypothesis that dysregulation of brain myelin production is an important manifestation of NF1 in adults.

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Decision letter for "Comparative morphology of the corpus callosum across the adult lifespan in chimpanzees ( Pan troglodytes ) and humans"

10.1002/cne.25039/v1/decision1 ◽

2020 ◽

Keyword(s):

Corpus Callosum ◽

Pan Troglodytes ◽

Comparative Morphology ◽

Adult Lifespan

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Review for "Comparative morphology of the corpus callosum across the adult lifespan in chimpanzees ( Pan troglodytes ) and humans"

10.1002/cne.25039/v2/review1 ◽

2020 ◽

Keyword(s):

Corpus Callosum ◽

Pan Troglodytes ◽

Comparative Morphology ◽

Adult Lifespan

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Corpus callosum may be similar in children with ADHD and siblings of children with ADHD

Developmental Medicine & Child Neurology ◽

10.1017/s0012162200000037 ◽

2000 ◽

Vol 42 (01) ◽

pp. 8 ◽

Cited By ~ 13

Author(s):

S Overmeyer ◽

A Simmons ◽

J Santosh ◽

C Andrew ◽

S C R Williams ◽

...

Keyword(s):

Corpus Callosum ◽

Children With Adhd

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Kommentar: Das Corpus callosum und der interhemisphärische Informationsaustausch: Ein Mysterium?

Zeitschrift für Neuropsychologie ◽

10.1024//1016-264x.11.4.216 ◽

2000 ◽

Vol 11 (4) ◽

pp. 216-219

Author(s):

Lutz Jäncke

Keyword(s):

Corpus Callosum

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Interhemispheric transfer evaluation in multiple sclerosis 1The authors would like to thank Claude-Alain Hauert and Christoph Michel for their assistance in the evaluation of motor tapping and Michel Habib for his suggestions and comments. This work was supported by a grant from the Swiss Society of Multiple Sclerosis.

Swiss Journal of Psychology ◽

10.1024//1421-0185.59.3.150 ◽

2000 ◽

Vol 59 (3) ◽

pp. 150-158 ◽

Cited By ~ 2

Author(s):

Nadia Ortiz ◽

Michael Reicherts ◽

Alan J. Pegna ◽

Encarni Garran ◽

Michel Chofflon ◽

...

Keyword(s):

Multiple Sclerosis ◽

Corpus Callosum ◽

Lexical Decision ◽

Dichotic Listening ◽

Neurological Impairment ◽

Interhemispheric Transfer ◽

Decision Task ◽

Swiss Society ◽

Listening Test ◽

Relapsing Remitting

Patients suffering from Multiple Sclerosis (MS) have frequently been found to suffer from damage to callosal fibers. Investigations have shown that this damage is associated with signs of hemisphere disconnections. The aim of our study was to provide evidence for the first signs of interhemispheric dysfunction in a mildly disabled MS population. Therefore, we explored whether the Interhemispheric Transfer (IT) deficit is multi-modal and sought to differentiate two MS evolution forms, on the basis of an interhemispheric disconnection index. Twenty-two patients with relapsing-remitting form of MS (RRMS) and 14 chronic-progressive (CPMS) were compared with matched controls on four tasks: a tachistoscopic verbal and non-verbal decision task, a dichotic listening test, cross tactile finger localization and motor tapping. No overall impairment was seen. The dichotic listening and lexical decision tasks were the most sensitive to MS. In addition, CPMS patients' IT was more impaired and was related to the severity of neurological impairment. The different sizes of the callosal fibers, which determine their vulnerability, may explain the heterogeneity of transfer through the Corpus Callosum. Therefore, evaluation of IT may be of value as an index of evolution in MS.

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