Summary

This document is a scholarly paper on reading and writing abilities in patients, reviewing neuropsychological and cognitive aspects. It explores various types of reading and writing disorders.

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**Reading and writing** **Reading and writing abilities** Neuropsychology tries to explain: - the processes involved in reading and writing in healthy subjects - reading (dyslexia, alexia) and writing (agraphia) deficits in patients - Reading = the conversion of a sequence of grapheme...

**Reading and writing** **Reading and writing abilities** Neuropsychology tries to explain: - the processes involved in reading and writing in healthy subjects - reading (dyslexia, alexia) and writing (agraphia) deficits in patients - Reading = the conversion of a sequence of graphemes (printed letters) into a sequence of sounds (phonemes) Reading aloud tasks - Writing = the conversion of a sequence of sounds (phonemes) into a sequence of graphemes (printed letters) Dictation exercises **Reading and writing neural correlates** **Reading and writing cognitive correlates** ![](media/image2.png) **We can distinguish between**: - Peripheral stages = processing of perceptive features (visual aspect of the stimulus) or selection of motor pattern (non-linguistic processes) E.G: analysis of single letters while reading, actual movement planning while writing - Central stages = based on lexico-semantic knowledge and grapheme-phoneme conversion mechanisms +-----------------------------------+-----------------------------------+ | Reading | Writing | +===================================+===================================+ | [Peripheral deficits] | [Peripheral deficits] | | include: | include: | | | | | - Neglect dyslexia | - Impairments of the graphemic | | | buffer | | - Attentional dyslexia | | | | - Apraxic agraphia | | - Pure alexia | | | | (with or without ideomotor | | (non-linguistic deficits) | apraxia) | | | | | | - Spatial agraphia | | | | | | (non-linguistic deficits) | +-----------------------------------+-----------------------------------+ | [Central deficits] | [Central deficits] | | include: | include: | | | | | - Phonological /Sublexical | - Phonological /Sublexical | | dyslexia | agraphia | | | | | - Lexical/Surface dyslexia | - Lexical/Surface agraphia | | | | | - Deep dyslexia | - Semantic agraphia | | | | | | - Deep agraphia | +-----------------------------------+-----------------------------------+ Please note: Acquired disorders versus developmental learning disorders **Alexia** **The cortical network for reading** Immagine che contiene mappa, diagramma, schermata Descrizione generata automaticamente Green: semantic aspects Orange: Broca's area → wide spread network working **A dual-route model for reading** ![Immagine che contiene testo, diagramma, linea, schermata Descrizione generata automaticamente](media/image4.png) **Peripheral alexia** **ALEXIA WITHOUT AGRAPHIA (OR PURE ALEXIA)** = disruption of a system specialized for the recognition of visually presented words left occipito-temporal lesion plus posterior part of the corpus callosum (e.g., Dejerine) or even in the left fusiform gyrus (the visual word form area, Shallice and Warrington) - "disconnection" of the left hemisphere (word recognition system) from the visual information (presented only to the right hemisphere) - naming letters in a serial fashion (letter-by-letter reading), effect of word length - performance is not influenced by linguistic effects as regularity of word orthography, imaginability of words, etc. BUT, some patients may show implicit reading (comprehension of words they cannot explicitly identify) → reading through the right hemisphere **NEGLECT DYSLEXIA** Patients commit consistently lateralized letter omission, addition, and substitution errors when reading individual words. In patients with left-sided neglect, failure in the identification of the initial portion of a letter string (e.g. reading "lend" for "blend"), in right-sided neglect the opposite pattern of errors different sub-types: retino-centric, Stimulus-centered and Word-centered \- - \> attentional impairment that may be specific for words (i.e., word-centered impairment) **ATTENTIONAL DYSLEXIA** Relative preservation of single-word reading in the context of a gross disruption of reading when words are presented in a text / crowded environment (surrounded by other words) or deficiency in identifying letters within a word interpreted as a pre-lexical reading deficit **Central deficits** **DEEP ALEXIA** [Reading real words] - Semantic errors ('knight' for castle, or 'canary' for bird) - Visual errors ('scale' for skate) - Morphologically errors: prefix/suffix is added/deleted/substituted ('government' for governor') - Better reading for high frequency and high imaginable words - Part-of-speech effects, nouns \> adjectives/adverbs \> verbs [Impaired reading of nonwords] - Deficit in print-to-sound conversion → lexicalization ('flag' for flig) **Example** [Coltheart et al. 1980, Saffran et al., 1980] Following a large left perisylvian lesion extending into the frontal lobe residual reading ability mediated by the right hemisphere (?) \- - \> it could be Associated with Broca or global aphasia **PHONOLOGICAL/SUBLEXICAL ALEXIA** [Reading real words] - Only mildly impaired (85-95% correct) - Not influenced by the regularity of print-to- sound correspondence [Deep deficit in reading nonwords] - Almost 90% of errors - Patients usually substitute the target with a real word (e.g., 'phone' for phope) Cannot be simply attributed to a broader deficit in phonology (dissociation with aphasic symptoms) [Hypothesis of a continuum with deep dyslexia:] - Milder form - Similar sites but more restricted lesions (left superior temporal lobe, angular and supramarginal gyri) **SURFACE/LEXICAL ALEXIA** [Reading real words] - Reading regular word is spared (state, hand, mosquito) - Reading irregular words is deeply impaired (yacht, island, have) → pronunciation cannot be derived by sounding-out strategies [Spared reading of nonwords] [Usually not observed in patients with focal lesions but in those with semantic dementia] (variant of frontotemporal dementia) → (progressive degenerative disease) atrophy in the anterior temporal lobe, sometimes left- sided predominance progressive loss of semantic knowledge as well **Reading with the right hemisphere** The right hemisphere was classically considered 'word blind' (Dejerine, 1892, Gerschwind, 1965) instead, it seems to support some reading abilities (Coltheart, 1980, Saffran, 1980) see for instance a patient (Patterson et al., 1989) who underwent a left hemispherectomy at the age of 15 for treatment of epilepsy: after the operation she was able to read about 30% of single words, exhibited an effect of part-of-speech, was unable to apply grapheme-to-phoneme conversion → similar to deep dyslexia see also the performance of split-brain patients (resection of the corpus callosum) in case of lateralized word presentation: - unable to determine the sound of words presented in the left hemifield (i.e., to the right hemisphere) e.g., impairment in rhyme task - BUT they are able to match the word with the appropriate object - the right hemisphere lexical-semantic system primarly represents high frequency and high imaginable nouns **Agraphia** **A dual-route model for writing** Immagine che contiene testo, diagramma, schermata, linea Descrizione generata automaticamente **Classification of agraphia** [Peripheral deficits include:] - Impairments of the graphemic buffer - Apraxic agraphia (with or without ideomotor apraxia) - Spatial agraphia [Central deficits include:] - Phonological/Sublexical agraphia - Lexical/Surface agraphia - Semantic agraphia - Deep agraphia **Peripheral forms of agraphia** **DEFICITS IN THE ORTHOGRAPHIC (OR GRAPHEMIC) BUFFER** = working-memory deficit - Impairment in the ability to temporarily store letters, which is a required step for letter selection and association - Errors: substitutions, deletions, transportions, insertion of single letters deficit is not affected by linguistic factors (word class, regularity of spelling, imaginability) deficits are influenced by words length (more errors in longer words) - Lesions in fronto-parietal networks **APRAXIC AGRAPHIA** = praxic deficit - Inability to correctly (plan and) perform the movements required for forming written letters (graphemes), without a peripheral motor impairment (no apraxia) - Usually due to a parietal damage illegible handwriting, but correct spelling while using the keyboard oral spelling is preserved (non-linguistic deficit) **SPATIAL AGRAPHIA** = visuo-constructive deficit - Impairment in visuo-spatial skills → produce malformed letter components - Inability to properly plan and use visual and kinaesthetic feedbacks to correct - Associated with nondominant parietal or frontal damage may be part of the neglect syndrome - Correct spelling while using the keyboard **Phonological/Sublexical agraphia** [Spelling real words] - Is spared / only mildly impaired - Not influenced by the regularity of print-to-sound correspondence → regular and irregular spelling is preserved [Deep deficit in spelling nonwords] - Patients usually substitute the target with a real word Associated with lesions in perisylvian regions Frequently, it co-occurs with aphasia, but it may dissociate [Shares many features with phonological dyslexia:] - When they co-occur, they may be due to the same impairment in the conversion mechanism - While, when they dissociate, the deficit may be due to lesions that disrupt information as it enters/exits the conversion system (this would affect reading or writing in isolation) **Lexical/Surface agraphia** [Spelling real words] - Is spared for regularly spelled words - Impaired for words with irregular spelling (regularization errors, e.g., yacht → YAT) Spared spelling of nonwords Associated with more posterior lesions: left posterior angular gyrus or parieto-occipital lobule [Shares many features with surface dyslexia:] - When they co-occur, they may be due to the same impairment in the lexical route - Disrupted flow of information out of the orthographic lexicon would produce lexical agraphia, while disrupted flow of information into this system would produce surface dyslexia, in isolation **Semantic agraphia** - Patients with dementia - Semantic dementia or Alzheimer's disease - Lose the ability to write with meaning - disconnection of the lexicon with the semantic system (or semantic deficit itself) - Able to spell both nonwords and irregularly spelled words, but impaired comprehension - Association with aphasia and/or alexia **Deep agraphia** [Spelling real words] - Semantic paragraphic errors: writing a semantically related (but not visually similar) word instead of the target ('knight' for castle, or 'canary' for bird) - Better spelling for high frequency and high imaginable words - Part-of-speech effects, more problems in writing function words and grammatical suffixes E.G: eating but not ceiling [Impaired spelling of nonwords] [When comprehension is spared] the deficit is interpreted as a disruption in the lexical route (access from semantic to the orthographic output lexicon) \- - \> often associated with deep dyslexia (left perisylvian lesion) **Graphemic buffer** = lesions in fronto-parietal network - Temporary store for letters → responsible for letter selection and association - Impairment → errors in: - spontaneous writing - writing to dictation - writing naming - delayed copy - at the end of the word (especially if long) - Influence by word length errors: substitutions, deletions, transportations, insertion of single letters **Assessment** First, take into account premorbid abilities (as reported by the patients and family members), which may be inferred from developmental problems, lack of opportunities to learn reading and writing, academic records, knowledge on the patient's occupation and job responsibilities [Linguistic components for dyslexia and dysgraphia] → read aloud and write - letters - real words (familiar and unfamiliar) - irregular words - nonwords - phrases and sentences [Motor component for writing]: - spontaneous writing - writing to dictation - copying [Semantic component]: word-picture or picture-picture matching !! Always consider the following components: - length - lexical status (real/non words) - frequency of use - grammatical category (nouns, verbs, adjectives, functors,...) - imageability: concreteness vs. abstractness - orthographic regularity **Treatment** = recover skills already acquired and widely used by the subject in the past two main rehabilitation programs: a. Specifically aimed at reading or writing whole words b. Directed at the damaged lexical component. - For the PHONOLOGICAL APPROACH, the aim is to reconstruct the ability to transform single phonemes or single syllables into graphemes (or vice versa) - For the LEXICAL APPROACH, activities as - Tachistoscopic presentation of letter, word, or phrases of increasing difficulty - Identification of irregular groups within words - Recognition of parts of words - Division of compound words - Completion of words - Semantic approach, activities as matching written word to a picture (alexia) or to write words for a presented picture (agraphia) **Acalculia** Acalculia refers to an acquired neuropsychological condition in which patients with previously normal calculation abilities develop impairments in processing numbers as a consequence of an acquired brain dysfunction left inferior parietal lobule lesion often in association with aphasia Calculation is crucial in our daily life e.g., paying bills, addressing letters, counting out money, understanding measurements for a recipe... impairments in calculation are very common in patients with focal lesions (occuring in about 30% of stroke patients with left hemispheric damage), and are frequent in some types of dementia (e.g., early symptom in Alzheimer's disease) We can distinguish between - Primary (pure) deficits = acalculia cannot be accounted for by deficits in other cognitive domains, usually associated with left parietal damage anarithmetria (Hécaen et al., 1961) - Secondary acalculia = calculation disturbances are attributable to deficits in attention, memory, or language i. acalculia with alexia and agraphia for numbers: can be associated with agraphia as there's an impairment in reading or writing numbers due to left inferior parietal lesions ii. acalculia of the spatial type: deficit in spatial organization of numbers due to right hemisphere lesions (association with neglect) **Number processing** Although acalculia often co-occurs with other cognitive deficits (language first) - **Calculation** is an independent and multicomponential cognitive function - **Numbers** represent a highly specific semantic category patients may show highly specific deficits and dissociations with language and memory deficits Two codes can be used for numbers - Arabic code, e.g., 4 → numbers as sequences of digits (inferior temporal areas) - Verbal code, e.g., 'four' → numbers as sequences of letters or words (perisylvian areas) each has its own lexicon (basic meaningful elements) and syntax (how elements can be combined) transcoding rules exist to transform a number from one to the other code TRIPLE CODE MODEL (Dehaene, 1992) Argues for the existence of three representational codes for number, each subserved by functionally dissociated neural substrates: i. Arabic digits → numbers as sequences pf syntactically organized digits ii. Verbal number words → numbers as sequences of syntactically organized words iii. Analogue non-symbolic magnitude representations → numbers located along a mental number line ![](media/image6.png) **Neuro-anatomical model (Dehanene, 2000)** The neurophysiological bases of calculation: discrepancies between functional neuroimaging studies (bilateral fronto-temporo-parietal networks) and data from patients (left posterior parietal regions are crucial) some researchers do not agree with this distinction **Errors made by patients with acalculia** The deficits may include the inability to read, write and understand numbers and numerical quantities, and the inability to perform calculation tasks the deficits can be very selective **Errors in processing numbers** - LEXICAL = errors in selecting the correct elements E.G: 'sixteen' → 18 - SYNTACTIC = inability to identify the correct sequence in order to express magnitude - MIXED ERRORS E.G: 'fourhundredeight' → 3007 **Errors in calculation** - Deficits in numerical facts (e.g., multiplication tables) stored in long-term memory - Deficits in arithmetic comprehension (concepts, e.g., the commutative property) - Deficits in arithmetic procedures (e.g., how to carry the amount over in addition) **Neuropsychological assessment of acalculia** - Take into account premorbid calculation abilities (as reported by the patients and family members), which may be inferred from developmental problems, lack of opportunities to learn calculation skills, academic records, knowledge on the patient's occupation and job responsibilities - Explore the different areas that may be impaired: - written and auditory comprehension and production - use of both the verbal and Arabic code - transcoding of numbers and calculation performance for all operations - Always include a qualitative analysis (classification of error types) ↓ Results must be interpreted in the light of the other deficits shown by the patient (in attention, visuo-spatial skills, language, working-memory...) Gerstmann syndrome (digital agnosia, dysgraphia, acalculia and right-left disorientation) **The Numerical Activities for Daily Living (NADL) battery** The way NADL allows to collect information about the degree of awareness of the deficit by the patient and by her or his caregivers it's divided into four parts: 1. The Patient's Interview, 5 min 2. The Caregiver Interview, 5 min 3. The Informal Test, 10 min: brief clinical assessment to determine whether the Formal Test of numerical abilities needs to be administered 4. The Formal Test, 30 min: Detailed and formal assessment of the numerical abilities **Formal Test of numerical abilities** includes four sections: - Number comprehension E.G: Numerosity Comparison, Number Line Marking , and Digit Comprehension - Reading and writing Arabic numbers - Mental calculation - Written calculation **Treatment** - **[Restitutive approach]**: re-learning through intense practice (relearning arithmetic facts or making the recovery process automatic again) - **[Compensatory approach]**: promoting the use of \"back-up\" strategies based on the patient\'s residual strategies EXAMPLE: patient with impaired ability to retrieve arithmetic facts Compensatory approach: rehabilitation could be based on counting procedures EXAMPLE: 6×5 would become 6+6+6+6+6 **Specific learning disorders** Specific Learning Disabilities (SpLDs) refer to a group of specific lifelong cognitive impairments identified through the unexpected underachievement in a range of learning processes involving the skills of listening, speaking, spelling, written expression, mathematics, understanding, or reading including decoding and comprehension (APA, 2013; IDEA, 2004; Hammill, 1990) key facts about SpLDs: - SpLDs are distinct from intellectual disabilities since they are specific and not global impairments - Dyslexia is the most common SpLD comprising 80% of all diagnosed SpLDs. - Aside from academic barriers, people with SpLDs can also experience barriers in time management, organisational skills, social perception and social interaction - SpLDs have a variety of presentations and range in severity - The experience of SpLD varies from person to person and can be more disabling when there are greater barriers in the learning environment (Snowling, 2012) - International estimates indicate that SpLDs impact approximately 10% of the population (Firth, 2008) **DYSLEXIA** is associated with specific barriers in reading, writing, spelling or comprehension. Dyslexia is the most common SpLD, affecting approximately 80% of people with an SpLD (Lee, 2008; Mather and Wendling, 2012; Kalanje, 2011; International Dyslexia Association, 2012). Because dyslexia is related to literacy skills, it is most evident in educational settings. Students with dyslexia can experience barriers in one or more of the following symptoms: visual perception of letters and words and their corresponding sounds and phonemes, memory, vocabulary, spelling of spoken words, comprehension. **DYSCALCULIA** is associated with barriers in understanding numbers and mathematical concepts. It occurs in up to 3% of the population. For individuals with dyscalculia, it may be difficult to visualize patterns, different parts of a maths problem or identify critical information needed to solve equations and more complex problems. Dyscalculia can affect a person's ability to think quantitatively, do arithmetic, understand and measure time and space, remember times tables and perform basic calculations. **DYSGRAPHIA** is associated with restrictions in handwriting tasks. Dysgraphia can affect skills associated with holding a pen or pencil such as drawing shapes, numbers or letters. Dysgraphia is a neurological disorder that typically appears when using the complex collection of motor and information processing skills involved in the activity of writing. It requires the ability to get the muscles in the hands and fingers to write. A person with dysgraphia may write their letters in reverse, have trouble recalling how letters are formed, or when to use lower or upper case letters. executive-motor disorder **DYSORTHOGRAPHY** is the difficulty in correctly translating the sounds that make up words into graphic symbols → phoneme-grapheme conversion phonological and morphological errors \- - \> most common errors: - omissions of graphemes or parts of words - substitutions of graphemes - inversions of graphemes no motor deficits often associated to dysgraphia \*White matter tracts as abnormally organized in the dyslexic brain: arcuate fasciculus (AF, in blue) and inferior fronto-occipital fasciculus (IFOF, in green), respectively associated with phonological and orthographic scores on standardized tests \*Left-hemisphere cortical regions have consistent structural (both in grey and white matter) and functional abnormalities **Multilinguals** - Heterogeneity of multilingual individuals: - the number of languages the individual knows - what order they learned them, and thus have them stored in the brain - the age at which they learned them - how frequently each language is used - how proficient the individual is in using those languages. - Language features (regularity, coordinates in reading and writing, morphosintactic rules, etc.) - Classification of multilingual individuals: - [Compound bilingual]: in the early years of life → the person develop 2 or more linguistic codes simultaneously and in the same environment - [Coordinate bilingual]: the individual acquires the two languages in different contexts (e.g., home and school), so the words of the two languages belong to separate and independent systems - [Sub-coordinate bilingual]: learning other language(s) filtering from the native language (e.g., translating) - [Bimodal bilinguals]: people who can speak one sign language and one oral language - Age of acquisition - Early learners = before school. - Late learners = after ten years of age Recruitment of Broca's and Wernicke's areas: - Early learners (Compound bilingual): similar parts for both languages - Later learners (Coordinate bilingual): different parts depending on language - Language proficiency 1. Beside age of acquisition, an individual who resides in a bilingual society is more likely to be highly proficient in both languages, as opposed to a bilingual individual who lives in a dominantly monolingual community 2. Higher language proficiency is related also to higher ability in phonological working memory and language switching - Before the 1960s, bilinguism was considered and handicap that slowed a child development by forcing him/her in spending too much energy in distinguish between different languages - Recent studies showed that compared to monolingual individuals: - Alxheimers's disease and dementia may be delayed up to 5 years in multilingual patients - Bilinguals have shown to have a better recovery (better cognitive outcomes) after a stroke - In healthy subjects: higher rate of errors and slower RTs in bilingual students during interlinguistic tests - Multilingualism affects the structure (higher grey matter density + increased myelination of some white matter tracts) → the function of the brain language learning boosts brain plasticity and the brain\'s ability to code new information **Aphasia in multilingualism** Two primary theoretical approaches to study multilingual aphasics: 1. THE LOCALIZATIONALIST APPROACH: different languages are stored in different regions of the brain, explaining why multilingual aphasics may lose one language they know, but not the other(s) 2. THE DYNAMICAL THEORY (OR SHARED REPRESENTATION): the language system is supervised by a dynamic equilibrium between the existing language capabilities and the constant alteration and adaptation to the communicative requirements of the environment **Assessment of aphasia in multilingualism -- BAT** In the past, the assessment of aphasia in bilinguals or multilinguals was only available in the language of the hospital → misjudged the patient\'s recovery progress in the non-native languages → The Bilingual Aphasia Test (BAT) was developed by Michel Paradis and associates The test is available in many different languages, and it is designed to be culturally and linguistically equivalent → the assessment of aphasia allows a direct comparison of the knowledge and performance of each of the patient\'s languages to determine the severity of the aphasia The BAT consists of 3 major sections: [Part A]: evaluation of the patient's multilingual history (50 items) [Part B]: systematic and comparable assessment of language disorders in each language known by the subject (472 items in each known language) [Part C]: assessment of translation abilities and interference detection in each language pair (58 items each) **Recovery of aphasia in multilingualism** Seven patterns of recovery have been outlined: 1. [Selective recovery]: one language remains impaired and the other recovers; the activation threshold for the impaired language is permanently increased 2. [Parallel recovery]: both impaired languages improve to a similar extent and concurrently 3. [Successive recovery]: complete recovery of one language precedes the recovery of the other 4. [Differential recovery]: there is greater inhibition of one language than of another 5. [Alternating recovery]: the language that was first recovered will be lost again due to the recovery of the other language 6. [Alternating antagonistic recovery]: the language that was not used for a time becomes the currently used language (i.e., on one day the patient is able to speak in one language while the next day only in the other) 7. [Blended recovery]: pathological mixing of two languages (i.e., the elements of the two languages are involuntarily mixed during language production) research that compares the prevalence of the different recovery patterns generally shows that the most common pattern of recovery is parallel recovery, followed by differential, blended, selective, and successive EXAMPLE: - Patient (coordinate bilingual): - JRC, male, right-handed, 55 years old, born in Argentina - Native language Spanish = L1; - At the age of 17 emigration to Italy (Italian =L2); - High language proficiency for both languages - Stroke: - Left subcortical hemorrhage → severe aphasia that developed within a few days into a fluent aphasia (not so good in comprehension) (main deficit = anomia, he was not able to get access to the lexicon and the semantic store and name things) - In the acute/post-acute stage, L1 spontaneously recovered better than L2 - Speech therapy for Spanish was not so useful, for Italian a little better Multilinguals are really complex because we have many factors to take in consideration We have a good assessment of different linguistic deficit

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