Attention and Executive Disorders in Neurofibromatosis 1: Comparison Between NF1 With ADHD Symptomatology (NF1 + ADHD) and ADHD Per Se

2017 ◽  
Vol 24 (13) ◽  
pp. 1807-1823 ◽  
Author(s):  
Laurence Lion-François ◽  
Vania Herbillon ◽  
Emeline Peyric ◽  
Catherine Mercier ◽  
Daniel Gérard ◽  
...  

Objective: To compare children with Neurofibromatosis type 1 and associated ADHD symptomatology (NF1 + ADHD) with children having received a diagnosis of ADHD without NF1. The idea was that performance differences in tasks of attention between these two groups would be attributable not to the ADHD symptomatology, but to NF1 alone. Method: One group of children with NF1 + ADHD ( N = 32), one group of children with ADHD ( N = 31), and one group of healthy controls ( N = 40) participated in a set of computerized tasks assessing intensive, selective, and executive aspects of attention. Results: Differences were found between the two groups of patients in respect of several aspects of attention. Children with NF1 + ADHD did not always perform worse than children with ADHD. Several double dissociations can be established between the two groups of patients. Conclusion: ADHD symptomatology in NF1 does not contribute to all attention deficits, and ADHD cannot account for all attention impairments in NF1.

2019 ◽  
Vol 2019 ◽  
pp. 1-10
Author(s):  
Magdalena Heimgärtner ◽  
Sofia Granström ◽  
Karin Haas-Lude ◽  
Robert A. Leark ◽  
Victor-Felix Mautner ◽  
...  

Aims. Attention deficit hyperactivity disorder (ADHD) is one of the most frequent neurocognitive impairments in neurofibromatosis type 1 (NF1) and a well-known risk factor for intellectual dysfunction in general. Since NF1 is per se associated with intellectual difficulties, this comorbidity may be crucial for the cognitive development of affected patients. In our study, we investigated if attention deficits are associated with intellectual functioning in NF1 and if children with NF1 plus ADHD differ in their intellectual and attention profiles from children affected by NF1-only or ADHD only. Methods. 111 children aged between 6 and 12 years (53 NF1 plus ADHD, 28 NF1-only, 30 ADHD-only) performed the German version of the intelligence test WISC-IV and a continuous performance test (T.O.V.A.) to assess attention functions. Parents completed questionnaires about everyday attention and executive functions (Conners 3®, BRIEF). Results. Children with NF1 plus ADHD showed significantly lower intelligence test scores (full-scale IQ: 89.39 [1.40]) than patients with NF1-only (full-scale IQ: 101.14 [1.98]; p<.001), and intellectual functioning correlated significantly with attention performance in NF1 (p<.001). As compared to NF1-only, attention, and executive functioning were impaired on several dimensions (T.O.V.A., Conners 3® and BRIEF) in NF1 plus ADHD. ADHD-only was associated with significantly higher problem scores regarding hyperactivity/impulsivity and inattention (Conners 3®). NF1-only was associated with inattentiveness when compared to the normative sample of the T.O.V.A. Conclusion. NF1 is associated with variable attention problems. Severe attention deficits appear to be a risk factor for intellectual dysfunction in NF1, more than NF1 without attention deficit. NF1 plus ADHD presents a specific cognitive profile, which differs from that of NF1 and from neurotypical ADHD.


1995 ◽  
Vol 287 (5) ◽  
pp. 413-416 ◽  
Author(s):  
Ingrid Eisenbarth ◽  
Sven Hoffmeyer ◽  
Dieter Kaufmann ◽  
G�nter Assum ◽  
Winfrid Krone

2005 ◽  
Vol 15 (21) ◽  
pp. 1961-1967 ◽  
Author(s):  
Weidong Li ◽  
Yijun Cui ◽  
Steven A. Kushner ◽  
Robert A.M. Brown ◽  
J. David Jentsch ◽  
...  

Hand Surgery ◽  
2012 ◽  
Vol 17 (03) ◽  
pp. 379-381
Author(s):  
Hiroyoshi Hagiwara ◽  
Suguru Hagiwara ◽  
Takashi Ajiki ◽  
Yuichi Hoshino

We present a case of phalangeal deformity in a 17-year-old boy. The deformity was caused by a neurofibroma associated with neurofibromatosis type 1, affecting the left ring finger. The mass was surgically resected. Recurrence had not occurred at three-year follow-up.


2017 ◽  
Vol 14 ◽  
pp. 499-505 ◽  
Author(s):  
Annet Bluschke ◽  
Maja von der Hagen ◽  
Katharina Papenhagen ◽  
Veit Roessner ◽  
Christian Beste

Author(s):  
Maria J Ribeiro ◽  
Otília C d’Almeida ◽  
Fabiana Ramos ◽  
Jorge Saraiva ◽  
Eduardo D Silva ◽  
...  

2019 ◽  
Vol 53 (2) ◽  
Author(s):  
Leah Antoinette M. Caro-Chang ◽  
Maria Franchesca S. Quinio ◽  
Georgina C. Pastorfide

This is a case of an 11-year-old male who presented with multiple neurofibromas with hypertrichosis. Classic cutaneous neurofibromas, café-au-lait macules, axillary freckling, Lisch nodules, and scoliosis were also present fulfilling a diagnosis of Neurofibromatosis type 1. This is the first report of multiple neurofibromas with hypertrichosis in the Philippines. Hypertrichosis overlying a neurofibroma is rarely reported and the mechanism remains to be elucidated.


2021 ◽  
Vol 12 ◽  
Author(s):  
Hanlu Tang ◽  
Qiong Wu ◽  
Shiwei Li ◽  
Yehong Fang ◽  
Zhijun Yang ◽  
...  

Background: Cognitive dysfunction is one of the main symptoms of neurofibromatosis type 1 (NF1). As an important cognitive function, working memory (WM) has rarely been systematically analyzed in NF1 by isolating the particular domain of WM, and existing data involving WM in adult patients with NF1 are insufficient. This study aimed to investigate the characteristics of different types of WM in NF1 from the perspective of the adult population.Method: We comprehensively analyzed WM in both verbal and visuospatial WM domains by using the N-back task (including the verbal N-back task and the visuospatial N-back task) in 31 adults with NF1 and 34 healthy controls matched for age, gender, education levels, and general cognitive status. The accuracy and reaction times (RTs) in the N-back task were entered into mixed-design ANOVA.Results: Compared with healthy controls, adults with NF1 presented significantly lower mean accuracy and longer RTs in the visuospatial N-back task. However, no significant difference was found between the NF1 group and healthy controls in the verbal N-back task.Conclusions: The present study suggested that adults with NF1 might have deficits in visuospatial WM. We did not find evidence for verbal WM deficits in adult patients with NF1. Our findings supplement and refine the existing data on WM in the context of NF1.


2018 ◽  
Vol 17 (2) ◽  
pp. 35-40
Author(s):  
K Kalmantis ◽  
A Petsa ◽  
G Daskalakis ◽  
E Alexopoulos ◽  
E Korovesi ◽  
...  

Introduction: Neurofibromatosis type-1 (NF-1) is one of the most common genetic diseases following an autosomal dominant inheritance pattern. Maternal and fetal complications have been reported. Purpose: To present a very interesting and rare case report regarding neurofibromatosis – 1 (NF-1) in pregnancy and to create a complete review concerning this genetic disease. Materials and Methods: Articles were identified through electronic databases; no date or language restrictions were placed; relevant citations were hand searched. The search was conducted using the following terms: neurofibromatosis, neurofibromatosis type-1, pregnancy. Case presentation: We present a case of a 36-year-old nulliparous pregnant woman affected by NF-1. She presented with café-au-lait spots and cutaneous/subcutaneous neurofibromas, progressively increasing in size and number. The unique obstetric complication was placenta previa, diagnosed in the second trimester. A caesarean section was performed on the 36th week. A healthy male neonate unaffected by NF-1 was born. Both post-operative period and puerperium were uneventful. Conclusions: This case report highlights the fact that a normal pregnancy outcome can occur in pregnant women with NF-1 and proper counselling should be in place so that informed decisions can be made by future parents.


2011 ◽  
Vol 32 (6) ◽  
pp. 2608-2613 ◽  
Author(s):  
Yafit Gilboa ◽  
Sara Rosenblum ◽  
Aviva Fattal-Valevski ◽  
Hagit Toledano-Alhadef ◽  
Albert (Skip) Rizzo ◽  
...  

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