A Systematic Review on Visual-Processing Deficits in Neurofibromatosis Type 1: What Possible Impact on Learning to Read?

This systematic review aimed to examine the possible implication of visual-perceptual, visuo-attentional and oculomotor processing in the reading deficits frequently experienced by children with Neurofibromatosis type 1 (NF1), as previously shown in dyslexia. Using PRISMA methodological guidelines, we examined 49 studies; most of these reported visual-processing deficits in this population, raising the importance of directly studying the visuo-perceptual and visuo-attentional processes and eye-movement control involved in the learning-to-read process in NF1. The discussion provides a reflection for a better understanding of how visual-processing skills interact with reading deficits in NF1, as well as new avenues for their screening and care.

Visual-processing deficits in children with neurofibromatosis type 1: A clinical marker of reading difficulties.

Today's estimates indicate that nearly 50% of children with Neurofibromatosis type 1 (NF1) suffer from reading disabilities, with a high impact on their academic achievement. In addition to the well-documented importance of phonological skills in reading acquisition and neurodevelopmental disorders, visual-attention processes also appear as important factors in learning to read. The present study aimed at assessing the role of visual-processing dysfunction in the high prevalence of reading disabilities in NF1 children and providing a useful tool for clinician in the early detection of reading impairment in this neurogenetic disorder. Forty-two children with NF1 and 42 typically developing children (TD) participated in the study. All were right-handed and did not present intellectual disability or attention deficit hyperactivity disorder. Visual-attention processes were assessed with the Developmental Eye Movement (DEM) test, together with the NF1 children's reading level. NF1 children with and without reading disabilities were then compared. The results showed that visual-processing deficits were highly present among the NF1 children included in our study. Furthermore, poor readers with NF1 presented an increased risk of visual-processing deficits compared to peers. This finding supports the role of visual-processing deficits in the reading difficulties encountered in nearly half of children with NF1. Finally, in NF1 children without intellectual or attention disability, visual-processing deficits emerge as one of the clinical markers of reading disabilities. The study holds important clinical implications both for the identification, by providing a useful screening tool, and the management of reading disabilities in NF1 children.

Relação entre avaliações de leitura em escolares e queixas relatadas pelos responsáveis / Relationship between reading assessments in schoolchildren and complaints reported by guardians / Relación entre evaluaciones de lectura en escolares y denuncias reportadas por tutores

Introdução: Ler significa processar informações e transformar material escrito em fala e em significado. As pessoas que tiverem aprendido a ler terão desenvolvido um sistema mental de processamento de informações capaz de realizar essas transformações. Objetivo: correlacionar as habilidades de leitura e as queixas relacionadas à leitura de estudantes do terceiro ano do ensino fundamental. Método: 40 escolares de ambos os gêneros, idade média de 8,16 anos. Avaliou-se decodificação, fluência e compreensão textual, e as queixas relacionadas à leitura foram levantadas. Resultados: houve 37,5% de incidência de pelo menos uma queixa relacionada à leitura, prevalecendo a leitura lenta; acertos nos estímulos regulares foram estatisticamente superiores aos demais estímulos; os acertos na fluência variaram entre 0 e 45, o desempenho na compreensão leitora foi alto, sem diferença estatística entre questões inferenciais e literais; houve correlação entre as habilidades de leitura entre si, e entre a queixa "não lê" com estas habilidades. Conclusão: confirmou-se que a melhor decodificação favorece a fluência, que otimiza a compreensão leitora; a queixa "não lê" correlacionou-se com todas as avaliações de leitura, indicando os pais sensíveis à leitura de seus filhos.

Introduction: Reading means processing information and transforming written material into speech and meaning. People who have learned to read will have developed a mental information processing system capable of carrying out these transformations. Objective: To correlate reading skills and reading-related complaints of third grade students. Method: 40 students of both genders, mean age 8.16 years. Decoding, fluency and textual comprehension were evaluated, and reading-related complaints were raised. Results: there was a 37.5% incidence of at least one reading-related complaint, with slow reading prevailing; hits on regular stimuli were statistically superior to other stimuli; fluency scores ranged from 0 to 45, reading comprehension performance was high, with no statistical difference between inferential and literal questions; There was a correlation between reading skills among themselves, and between the complaint "not reading" with these skills. Conclusion: the incidence of reading-related complaints was high, and slow reading prevailed. It was confirmed that the best decoding favors fluency, which optimizes reading comprehension; the "not reading" complaint correlated with all reading assessments, indicating parents sensitive to their children's reading.

Introducción: Leer significa procesar información y transformar el material escrito en habla y significado. Las personas que han aprendido a leer habrán desarrollado un sistema de procesamiento de información mental capaz de realizar estos cambios. Objetivo: correlacionar las habilidades de lectura y las quejas relacionadas con la lectura de los estudiantes en el tercer año de la escuela primaria. Método: 40 estudiantes de ambos sexos, edad media 8,16 años. Se evaluaron la decodificación, fluidez y comprensión textual, y se plantearon quejas relacionadas con la lectura. Resultados: hubo una incidencia del 37,5% de al menos una queja relacionada con la lectura, prevaleciendo la lectura lenta; los golpes en estímulos regulares fueron estadísticamente superiores a otros estímulos; la fluidez correcta varió entre 0 y 45, el rendimiento de comprensión de lectura fue alto, sin diferencias estadísticas entre preguntas inferenciales y literales; existía una correlación entre las habilidades de lectura entre ellos y entre la queja "no leer" con estas habilidades. Conclusión: se confirmó que la mejor decodificación favorece la fluidez, lo que optimiza la comprensión de lectura; la queja "no lee" se correlacionó con todas las evaluaciones de lectura, lo que indica que los padres son sensibles a la lectura de sus hijos.

A sibling-comparison study of smoking during pregnancy and risk for reading-related problems.

This research examines the relationship between smoking during pregnancy (SDP) and risk for reading related problems in siblings discordant for exposure to SDP. Data (N = 173 families) were drawn from the Missouri Mothers and Their Children study, a sample, identified using birth records (years 1998-2005), in which mothers changed her smoking behavior between two pregnancies (Child 1 [older sibling]: M = 12.99; Child 2 [younger sibling]: M = 10.19). A sibling comparison approach was used, providing a robust test for the association between SDP and reading related outcomes in school-aged children. Results suggested within-family (i.e., potentially causal) associations between SDP and reading and language/comprehension factor scores, as well as between SDP and specific reading-related skills, including reading accuracy and receptive language, with increased exposure to SDP associated with decreased performance. SDP was not associated with spelling, reading rate, or receptive vocabulary. Initial within-family associations between SDP and word-letter identification, phonetic/decoding skills, and reading comprehension were fully attenuated following partial control for genetic and environmental confounding of the associations. These findings indicate that exposure to SDP is associated with poorer performance on some, but not all skills assessed.

Profiling the Word Reading Abilities of School-Age Children with Neurofibromatosis Type 1.

OBJECTIVE: Reading difficulties are one of the most significant challenges for children with neurofibromatosis type 1 (NF1). The aims of this study were to identify and categorize the types of reading impairments experienced by children with NF1 and to establish predictors of poor reading in this population. METHOD: Children aged 7-12 years with NF1 (n = 60) were compared with typically developing children (n = 36). Poor word readers with NF1 were classified according to impairment type (i.e., phonological, surface, mixed), and their reading subskills were compared. A hierarchical multiple regression was conducted to identify predictors of word reading. RESULTS: Compared to controls, children with NF1 demonstrated significantly poorer literacy abilities. Of the 49 children with NF1 classified as poor readers, 20 (41%) were classified with phonological dyslexia, 24 (49%) with mixed dyslexia, and 5 (10%) fell outside classification categories. Children with mixed dyslexia displayed the most severe reading impairments. Stronger working memory, better receptive language, and fewer inattentive behaviors predicted better word reading skills. CONCLUSIONS: The majority of children with NF1 experience deficits in key reading skills which are essential for them to become successful readers. Weaknesses in working memory, receptive language, and attention are associated with reading difficulties in children with NF1.

[Screening for reading difficulties in Parkinson's disease: An evaluation of the Alouette test]. / Exploration des troubles de la lecture dans la maladie de Parkinson : utilité du test de l'alouette.

INTRODUCTION: Reading disorders in Parkinson's disease (PD) are poorly evaluated due to the lack of validated tests to screen for them. They are often attributed to hand tremors associated with the disease. In this study, we evaluated the "alouette test" validated for dyslexia screening, in PD by comparing the results to healthy patients. METHODS: The "alouette test" was conducted on a fixed surface to avoid errors related to tremor. A fixation and tracking test were then performed. All the tests were filmed to be analyzed later by 2 examiners blinded to the neurological diagnosis. RESULTS: Thirty-eight patients were included, 19 with PD, and 19 healthy age-matched patients. PD patients read on average 250.9±13.7 words correctly vs. 260.3±2.7 words for healthy patients (P=0.008). This difference was greatest for the older patient subgroup (>65 years), who had the disease longer (P=0.014). Tracking and fixation tests were more impaired in PD patients compared to healthy patients. CONCLUSION: This study highlighted many reading disorders in PD. The use of the "alouette test" which can easily be implemented in clinical practice, could help to diagnose these disorders. Better evaluation of these difficulties would allow for better medical care of these patients.

Is hiragana decoding impaired in children with periventricular leukomalacia?

BACKGROUND: There are few studies on hiragana reading skill and phonological awareness in Japanese schoolchildren with periventricular leukomalacia (PVL). METHODS: Three seven-year-old children with PVL who had no intellectual disabilities or dysarthria were recruited. Their perinatal information, brain magnetic resonance image (MRI) at term equivalent age, accompanying neurodevelopmental disorders, ophthalmologic features, Kaufman Assessment Battery for Children (K-ABC), a hiragana reading test (four tasks), and a phonological awareness task (mora reversal tasks) were analyzed. RESULTS: Patient (Pt) 1 and pt2 were male. Pt2 and pt3 were siblings of triplets. Their gestational age was 28 or 32 weeks, and their birth weights were 1196, 1554, and 1848 g, respectively. Their brain MRI revealed cystic or non-cystic periventricular white matter injury involving the deep white matter at the trigone of both lateral ventricles. Pt1 had attention-deficit/hyperactivity disorder and pt3 had pervasive developmental disorder not otherwise specified. All patients had strabismus with spared best-corrected visual acuity. Scores of Reading/Decoding in K-ABC ranged from 89 to 99. As for the single mora reading task or the non-word reading task in the kana reading test, Z scores of their reading time ranged from 2.3 to 5.9 compared to control children. Pt1 and pt3 made significant errors in the mora reversal task of three-mora words, whereas all patients could answer all words correctly in the mora reversal task of two-mora words. CONCLUSION: All children showed significantly prolonged reading time despite their adequate letter recognition. Two patients showed delayed phonological awareness. It was suggested that hiragana decoding impairment due to subcortical and/or cortical injury related to PVL affected their reading ability.

Preliteracy impairments in children with neurofibromatosis type 1.

AIM: This cross-sectional study aimed to examine the preliteracy abilities of young children with neurofibromatosis type 1 (NF1) and to identify which of these abilities best predicted conventional literacy (spelling). METHOD: Forty-two children with NF1 (23 males, 19 females; mean age [SD] 5y 6mo [6mo]) were compared with 32 unaffected children (15 males, 17 females; mean age [SD] 5y 4mo [6mo]). All children completed a comprehensive cognitive assessment including measures of phonological processing (phonological awareness, phonological memory, rapid automatic naming) and letter-sound knowledge. RESULTS: Children with NF1 performed significantly poorer than the comparison group across all cognitive and preliteracy domains, with specific weaknesses evident in phonological awareness (F1,68 =14.13, p<0.001, partial η2 =0.17), phonological memory (F1,68 =13.87, p<0.001, partial η2 =0.17), and letter-sound knowledge (F1,71 =5.65, p=0.020, partial η2 =0.07). Within the group with NF1 group, over a third of children demonstrated impairment in at least one phonological processing domain and the risk of phonological impairment was 5.60 times that of unaffected children. Children's letter-sound knowledge was the strongest predictor of conventional literacy (spelling). INTERPRETATION: This study establishes that preliteracy deficits are present and detectable in young children with NF1. As a result of the high incidence of preliteracy impairment, we recommend screening phonological awareness and letter-sound knowledge to identify risk of future learning disorders. WHAT THIS PAPER ADDS: Young children with neurofibromatosis type 1 are at elevated risk of preliteracy deficits. The most affected domains are phonological awareness and phonological memory. Letter-sound knowledge is the strongest predictor of conventional literacy (spelling).

Genetic and environmental risk factors for developmental dyslexia in children: systematic review of the last decade.

Despite advances in the characterization of developmental dyslexia (DD), several questions regarding the interplay between DD-susceptibility genes and environmental risk factors remain open. This systematic review aimed at answering the following questions: What has been the impact of new resources on the knowledge about DD? Which questions remain open? What is the investigative agenda for the short term? Forty-six studies were analyzed. Despite the growing literature on DD candidate genes, most studies have not been replicated. We found large effects on causative genes and smaller environmental contributions, involving maternal smoking during pregnancy, SES and the DYX1C1-1259C/G marker. Implications are discussed.

Reading in children with temporal lobe epilepsy: A systematic review.

OBJECTIVE: Children with epilepsy have higher rates of reading difficulties compared to the general population. Reading difficulties are associated with lower academic attainments, higher school drop-out rates, greater risk of unemployment, lower income, and poorer adjustment. We examined the literature dealing with reading in children with the most common type of focal epilepsy, temporal lobe epilepsy (TLE), in relation to: presence of reading difficulties, contributing factors, and efficacy of treatments for reading difficulties. METHODS: We searched databases (MEDLINE, EMBASE, PsycINFO and PubMed) for studies published before September 2016. Included studies (i) reported on a group of children with TLE, (ii) used a standardized reading test or included a control group, (iii) involved original research published in peer reviewed journals in the English language. RESULTS: Of 2018 citations obtained through literature searches, six met inclusion criteria. Reading accuracy and/or reading comprehension were assessed using different tests. All but one study found statistical evidence of reading difficulties in children with TLE. Only two studies examined relations between cognitive deficits and reading. One found that memory contributed to reading accuracy and comprehension. Another found evidence of a small decline in reading accuracy, which was not associated with a decline in memory post-surgery. Several studies were underpowered, giving false negative findings and not allowing relations between epilepsy factors, underlying cognitive deficits, and reading to be adequately examined. No study examined efficacy of reading intervention in this patient population. SIGNIFICANCE: We showed that reading difficulties that are present in children with TLE are under researched, yet they have significant functional consequences through childhood and into adulthood. There is an urgent need to identify risk factors and investigate efficacy of treatments for reading difficulties in children with TLE, as this will enable early identification and evidence-based treatment to be delivered in clinical practice.

Phonics Training Improves Reading in Children with Neurofibromatosis Type 1: A Prospective Intervention Trial.

OBJECTIVE: To examine the efficacy of a phonics-training program in children with neurofibromatosis type 1 (NF1) and reading difficulties. STUDY DESIGN: Thirty children (7-12 years of age) with NF1 completed a double-baseline, 24-week intervention trial. Literacy outcome measures were assessed at 4 time points: (1) at baseline; (2) after an 8-week no-treatment period; (3) immediately post-treatment; and (4) at follow-up 8 weeks post-treatment. Repeated-measures ANOVA were conducted to examine change over time for all outcome measures, and significant main effects were explored with planned comparisons. Predictors of treatment effects were examined by linear regressions. RESULTS: Ninety percent of participants completed the intervention. Intervention-specific improvements were observed across a range of literacy outcomes, including reading accuracy (nonword reading, Cohen d = 1.10; regular-word reading, Cohen d = 0.32), letter-sound knowledge (Cohen d = 0.80), blending (Cohen d = 0.88), repetition of nonsense words (Cohen d = 0.94), phonemic decoding fluency (Cohen d = 0.55), and reading comprehension (Cohen d = 0.31). Improvements were maintained 8 weeks post-treatment. Age (P = .03) and working memory (P = .02) significantly influenced efficacy, with greatest improvements observed in older children with stronger verbal working memory capacity. CONCLUSIONS: Home-based, computerized reading intervention was effective in improving the reading and reading-related abilities of children with NF1 and reading difficulty. TRIAL REGISTRATION: Australian and New Zealand Clinical Trials Registry: ACTRN12611000779976.

Deterioration of dyslexia after non-dominant temporal lobectomy for drug-resistant epilepsy.

We present a patient with drug-resistant right-sided temporal lobe epilepsy, caused by a ganglioglioma of the parahippocampal gyrus. Preoperatively, the patient was also known to have dyslexia. A right-sided anterior temporal lobectomy, including complete lesionectomy, was performed. Several months after the otherwise uncomplicated procedure, the patient complained about visual memory disturbances, accompanied by increased reading and spelling problems. Postoperative neuropsychological examination revealed deterioration of the visual memory functions, compared to the preoperative assessment, and consequently provided a possible explanation for worsening of the pre-existing dyslexia. In this case report, we hypothesize on the cause of this unusual deterioration and present recommendations to be included in the preoperative epilepsy surgery evaluation for patients with verbal or reading disorders such as dyslexia.

Glioblastoma Presenting with Pure Alexia and Palinopsia Involving the Left Inferior Occipital Gyrus and Visual Word Form Area Evaluated with Functional Magnetic Resonance Imaging and Diffusion Tensor Imaging Tractography.

BACKGROUND: Rapid word recognition and reading fluency is a specialized cortical process governed by the visual word form area (VWFA), which is localized to the dominant posterior lateral occipitotemporal sulcus/fusiform gyrus. A lesion of the VWFA results in pure alexia without agraphia characterized by letter-by-letter reading. Palinopsia is a visual processing distortion characterized by persistent afterimages and has been reported in lesions involving the nondominant occipitotemporal cortex. CASE DESCRIPTION: A 67-year-old right-handed woman with no neurologic history presented to our emergency department with acute cortical ischemic symptoms that began with a transient episode of receptive aphasia. She also reported inability to read, albeit with retained writing ability. She also saw afterimages of objects. During her stroke workup, an intra-axial circumscribed enhancing mass lesion was discovered involving her dominant posterolateral occipitotemporal lobe. Given the eloquent brain involvement, she underwent preoperative functional magnetic resonance imaging with diffusion tensor imaging tractography and awake craniotomy to maximize resection and preserve function. CONCLUSIONS: Many organic lesions involving these regions have been reported in the literature, but to the best of our knowledge, glioblastoma involving the VWFA resulting in both clinical syndromes of pure alexia and palinopsia with superimposed functional magnetic resonance imaging and fiber tract mapping has never been reported before.

Functional MRI in medulloblastoma survivors supports prophylactic reading intervention during tumor treatment.

Development of reading skills is vulnerable to disruption in children treated for brain tumors. Interventions, remedial and prophylactic, are needed to mitigate reading and other learning difficulties faced by survivors. A functional magnetic resonance imaging (fMRI) study was conducted to investigate long-term effects of a prophylactic reading intervention administered during radiation therapy in children treated for medulloblastoma. The fMRI study included 19 reading-intervention (age 11.7 ± 0.6 years) and 21 standard-of-care (age 12.1 ± 0.6 years) medulloblastoma survivors, and 21 typically developing children (age 12.3 ± 0.6 years). The survivors were 2.5 [1.2, 5.4] years after completion of tumor therapies and reading-intervention survivors were 2.9 [1.6, 5.9] years after intervention. Five fMRI tasks (Rapid Automatized Naming, Continuous Performance Test using faces and letters, orthographic and phonological processing of letter pairs, implicit word reading, and story reading) were used to probe reading-related neural activation. Woodcock-Johnson Reading Fluency, Word Attack, and Sound Awareness subtests were used to evaluate reading abilities. At the time of fMRI, Sound Awareness scores were significantly higher in the reading-intervention group than in the standard-of-care group (p = 0.046). Brain activation during the fMRI tasks was detected in left inferior frontal, temporal, ventral occipitotemporal, and subcortical regions, and differed among the groups (p < 0.05, FWE). The pattern of group activation differences, across brain areas and tasks, was a normative trend in the reading-intervention group. Standardized reading scores and patterns of brain activation provide evidence of long-term effects of prophylactic reading intervention in children treated for medulloblastoma.

[Cognitive and linguistic abilities of a boy with PVL showing relatively higher VIQ compared to PIQ].

In this study, we investigated the cognitive processing and language abilities of a 13-year-old boy with moderate periventricular leukomalacia (PVL), spastic diplegia and exotropia who had discrepant scores in the verbal intelligence quotient (VIQ) and performance intelligence quotient (PIQ) in the Wechsler Intelligence Scale for Children, third edition (VIQ; 82 > PIQ; under 40). In the Kaufman Assessment Battery for Children and Das-Naglieri Cognitive Assessment System, his performance was poor at simultaneous processing compared to sequential processing. He could not copy three-dimensional figures, and he could place only two out of eight blocks correctly in the second level models of Benton three-dimensional block construction test, showing visuospatial impairment typical of patients with PVL. Despite the relatively high score in VIQ, there was a gap among the scores of the subtests in the Illinois Test of Psycholinguistic Abilities. He tended to get low scores in tests that required visual abilities. In addition, there was also an impairment in reading fluency tested by the Diagnostic Criteria and Medical Guideline for Specific Developmental Disorders. He was much less fluent in reading syllables, words or sentences (6.0 SD or more compared to 12-year-old boys). The relatively higher score in VIQ superficially suggests adequate language ability. However, in the present study, precise investigation revealed some discrepancies even within the field of language. Thus, defining stronger and weaker points of a patient is important in order to determine optimal medical or educational approaches.

Teaching reading to children with neurofibromatosis type 1: a clinical trial with random assignment to different approaches.

AIM: Neurofibromatosis type 1 (NF1) is a genetic disorder with a cognitive profile that includes visual-spatial perception deficits and a high incidence of reading disability. There is a paucity of information about how this cognitively complex population responds to mainstream reading interventions. The clinical trial goals were to determine whether children and adolescents with NF1 and reading deficits (NF+RD) benefit from mainstream remedial reading programs and whether responsiveness varies with differences in program-related visual-spatial demands. METHOD: Forty-nine participants (28 males, 21 females; aged 8-14y) with either NF+RD (n=17, 11 males, six females) or idiopathic reading deficit (IRD) (n=32, 17 males, 15 females) were randomly assigned to intensive remedial teaching using one of two multisensory reading programs: one with greater kinesthetic demands and the other with greater visual-spatial demands. Two control groups - wait-list IRD (n=14, 11 males, three females) and typically developing readers (n=26, 13 males, 13 females) - received no treatment. Repeated measures and multivariate ANOVA analyses compared each group's growth in reading achievement from pre- to post-testing. RESULTS: Treated groups showed significant growth whereas untreated groups did not. Comparing treated groups, the IRD group responded equally well to both interventions, whereas the NF+RD group showed a better response to the more kinesthetic approach. INTERPRETATION: Results suggest that multisensory remedial reading teaching that emphasizes kinesthetic demands more than visual-spatial demands is suitable for students with NF+RD.

Verbal memory and literacy outcomes one year after pediatric temporal lobectomy: a retrospective cohort study.

OBJECTIVE: In children with temporal lobe epilepsy (TLE), temporal lobectomy (TL) is a treatment of choice for those children with seizure that are difficult to control with medication. Semantic memory is dependent on functional integrity of the temporal lobes and is thought to be critical for development of literacy skills. However, little is known about semantic memory and literacy outcomes post-TL in children. METHOD: In this retrospective cohort study, 40 children with TLE were administered tests of memory and literacy pre-TL and 1year post-TL in one hospital between 1996 and 2011. RESULTS: One year post-TL, 60% of the children became seizure-free. A significant decline was found in one aspect of semantic memory (naming) in children who underwent left TL. In addition, a significant drop was also evident in one aspect of literacy (reading accuracy), irrespective of the side of surgery. These declines were related neither to each other nor to epilepsy variables including seizure outcome. CONCLUSIONS: This is the largest pediatric outcome study of memory and literacy skills to date and shows that TL is associated with a risk of a mild drop in specific aspects of semantic memory (naming, following left TL) and reading accuracy, while other areas of memory and literacy remain unchanged.

Alexia due to ischemic stroke of the visual word form area.

The visual word form area (VWFA) is a region in the posterior left occipitotemporal cortex adjacent to the fusiform gyrus hypothesized to mediate word recognition. Evidence supporting the role of this area in reading comes from neuroimaging studies of normal subjects, case-controlled lesion studies, and studies of patients with surgical resection of the VWFA for tumors or epilepsy. Based on these prior reports, a small discrete lesion to the VWFA would be expected to cause alexia in a literate person without prior brain process, but such a case has not previously been reported to our knowledge. Here, we report the case of a previously-healthy 63-year-old man with the acute onset of alexia without other significant impairments. Magnetic resonance imaging (MRI) of the brain revealed a small ischemic stroke localized to the inferior left occipitotemporal cortex, corresponding to the approximate location of the putative VWFA. Characteristic of pure alexia, testing in the weeks following the stroke revealed a letter-by-letter reading strategy and a word length effect on single word reading. Formal visual field testing was normal. There was no color anomia, or object or face recognition deficits, although a mild agraphia may have been present. This case of acute-onset alexia in a previously normal individual due to a small stroke restricted to the VWFA and sparing occipital cortex and white matter pathways supports the conclusion that the VWFA is crucial for reading.

Complete abolition of reading and writing ability with a third ventricle colloid cyst: implications for surgical intervention and proposed neural substrates of visual recognition and visual imaging ability.

We report a rare case of a patient unable to read (alexic) and write (agraphic) after a mild head injury. He had preserved speech and comprehension, could spell aloud, identify words spelt aloud and copy letter features. He was unable to visualise letters but showed no problems with digits. Neuropsychological testing revealed general visual memory, processing speed and imaging deficits. Imaging data revealed an 8 mm colloid cyst of the third ventricle that splayed the fornix. Little is known about functions mediated by fornical connectivity, but this region is thought to contribute to memory recall. Other regions thought to mediate letter recognition and letter imagery, visual word form area and visual pathways were intact. We remediated reading and writing by multimodal letter retraining. The study raises issues about the neural substrates of reading, role of fornical tracts to selective memory in the absence of other pathology, and effective remediation strategies for selective functional deficits.