Clinical dimensions along the non-fluent variant primary progressive aphasia spectrum.

It is debated whether primary progressive apraxia of speech (PPAOS) and progressive agrammatic aphasia (PAA) belong to the same clinical spectrum, traditionally termed non-fluent/agrammatic variant primary progressive aphasia (nfvPPA), or exist as two completely distinct syndromic entities with specific pathologic/prognostic correlates. We analysed speech, language and disease severity features in a comprehensive cohort of patients with progressive motor speech impairment and/or agrammatism to ascertain evidence of naturally occurring, clinically meaningful non-overlapping syndromic entities (e.g. PPAOS and PAA) in our data. We also assessed if data-driven latent clinical dimensions with aetiologic/prognostic value could be identified. We included 98 participants, 43 of whom had an autopsy-confirmed neuropathological diagnosis. Speech pathologists assessed motor speech features indicative of dysarthria and apraxia of speech (AOS). Quantitative expressive/receptive agrammatism measures were obtained and compared with healthy controls. Baseline and longitudinal disease severity was evaluated using the Clinical Dementia Rating Sum of Boxes (CDR-SB). We investigated the data's clustering tendency and cluster stability to form robust symptom clusters and employed principal component analysis to extract data-driven latent clinical dimensions (LCD). The longitudinal CDR-SB change was estimated using linear mixed-effects models. Of the participants included in this study, 93 conformed to previously reported clinical profiles (75 with AOS and agrammatism, 12 PPAOS and six PAA). The remaining five participants were characterized by non-fluent speech, executive dysfunction and dysarthria without apraxia of speech or frank agrammatism. No baseline clinical features differentiated between frontotemporal lobar degeneration neuropathological subgroups. The Hopkins statistic demonstrated a low cluster tendency in the entire sample (0.45 with values near 0.5 indicating random data). Cluster stability analyses showed that only two robust subgroups (differing in agrammatism, executive dysfunction and overall disease severity) could be identified. Three data-driven components accounted for 71% of the variance [(i) severity-agrammatism; (ii) prominent AOS; and (iii) prominent dysarthria]. None of these data-driven LCDs allowed an accurate prediction of neuropathology. The severity-agrammatism component was an independent predictor of a faster CDR-SB increase in all the participants. Higher dysarthria severity, reduced words per minute and expressive and receptive agrammatism severity at baseline independently predicted accelerated disease progression. Our findings indicate that PPAOS and PAA, rather than exist as completely distinct syndromic entities, constitute a clinical continuum. In our cohort, splitting the nfvPPA spectrum into separate clinical phenotypes did not improve clinical-pathological correlations, stressing the need for new biological markers and consensus regarding updated terminology and clinical classification.

Neural basis of speech and grammar symptoms in non-fluent variant primary progressive aphasia spectrum.

The non-fluent/agrammatic variant of primary progressive aphasia (nfvPPA) is a neurodegenerative syndrome primarily defined by the presence of apraxia of speech (AoS) and/or expressive agrammatism. In addition, many patients exhibit dysarthria and/or receptive agrammatism. This leads to substantial phenotypic variation within the speech-language domain across individuals and time, in terms of both the specific combination of symptoms as well as their severity. How to resolve such phenotypic heterogeneity in nfvPPA is a matter of debate. 'Splitting' views propose separate clinical entities: 'primary progressive apraxia of speech' when AoS occurs in the absence of expressive agrammatism, 'progressive agrammatic aphasia' (PAA) in the opposite case, and 'AOS + PAA' when mixed motor speech and language symptoms are clearly present. While therapeutic interventions typically vary depending on the predominant symptom (e.g. AoS versus expressive agrammatism), the existence of behavioural, anatomical and pathological overlap across these phenotypes argues against drawing such clear-cut boundaries. In the current study, we contribute to this debate by mapping behaviour to brain in a large, prospective cohort of well characterized patients with nfvPPA (n = 104). We sought to advance scientific understanding of nfvPPA and the neural basis of speech-language by uncovering where in the brain the degree of MRI-based atrophy is associated with inter-patient variability in the presence and severity of AoS, dysarthria, expressive agrammatism or receptive agrammatism. Our cross-sectional examination of brain-behaviour relationships revealed three main observations. First, we found that the neural correlates of AoS and expressive agrammatism in nfvPPA lie side by side in the left posterior inferior frontal lobe, explaining their behavioural dissociation/association in previous reports. Second, we identified a 'left-right' and 'ventral-dorsal' neuroanatomical distinction between AoS versus dysarthria, highlighting (i) that dysarthria, but not AoS, is significantly influenced by tissue loss in right-hemisphere motor-speech regions; and (ii) that, within the left hemisphere, dysarthria and AoS map onto dorsally versus ventrally located motor-speech regions, respectively. Third, we confirmed that, within the large-scale grammar network, left frontal tissue loss is preferentially involved in expressive agrammatism and left temporal tissue loss in receptive agrammatism. Our findings thus contribute to define the function and location of the epicentres within the large-scale neural networks vulnerable to neurodegenerative changes in nfvPPA. We propose that nfvPPA be redefined as an umbrella term subsuming a spectrum of speech and/or language phenotypes that are closely linked by the underlying neuroanatomy and neuropathology.

Measuring Sentence Information via Surprisal: Theoretical and Clinical Implications in Nonfluent Aphasia.

OBJECTIVE: Nonfluent aphasia is characterized by simplified sentence structures and word-level abnormalities, including reduced use of verbs and function words. The predominant belief about the disease mechanism is that a core deficit in syntax processing causes both structural and word-level abnormalities. Here, we propose an alternative view based on information theory to explain the symptoms of nonfluent aphasia. We hypothesize that the word-level features of nonfluency constitute a distinct compensatory process to augment the information content of sentences to the level of healthy speakers. We refer to this process as lexical condensation. METHODS: We use a computational approach based on language models to measure sentence information through surprisal, a metric calculated by the average probability of occurrence of words in a sentence, given their preceding context. We apply this method to the language of patients with nonfluent primary progressive aphasia (nfvPPA; n = 36) and healthy controls (n = 133) as they describe a picture. RESULTS: We found that nfvPPA patients produced sentences with the same sentence surprisal as healthy controls by using richer words in their structurally impoverished sentences. Furthermore, higher surprisal in nfvPPA sentences correlated with the canonical features of agrammatism: a lower function-to-all-word ratio, a lower verb-to-noun ratio, a higher heavy-to-all-verb ratio, and a higher ratio of verbs in -ing forms. INTERPRETATION: Using surprisal enables testing an alternative account of nonfluent aphasia that regards its word-level features as adaptive, rather than defective, symptoms, a finding that would call for revisions in the therapeutic approach to nonfluent language production. ANN NEUROL 2023;94:647-657.

Massive MCA stroke requiring alteplase followed by thrombectomy in a 34-year-old female with alport syndrome.

34-year-old-female with a medical history significant for Alport's syndrome, chronic kidney disease on dialysis, and hypertension, was brought to the emergency department for sudden onset aphasia and facial droop that began 30 min prior to arrival. She denied a history of prior strokes, recent illness, or fever. The vital signs on arrival as follows: blood pressure 151/71 mmHg, temperature of 98.4F, pulse of 77 beats/min, and respirations of 16 breaths/min. Upon examination, she appeared in mild distress with a left sided facial droop, right sided hemiparesis, and expressive aphasia, only answering to yes/no. Neurological examination revealed: expressive aphasia, intact sensation throughout the face and bilateral extremities, no effort in the right arm against gravity, some effort against gravity of the right leg, left arm and left leg had muscle strength of 5/5. Patient had an NIH stroke scale of 8. The remainder of the exam was unremarkable. Radiographic imaging with CT revealed no intracranial hemorrhage (Fig. 1) and the patient was given alteplase (tPA) injection 5.6 mg within 1 h of her arrival to the Emergency Department. After administration of tPA a CT perfusion scan was performed (Fig. 2). Imaging demonstrated decreased cerebral blood flow and prolonged mean transit time within the majority of the left middle cerebral artery territory, sparing the basal ganglia. This indicated a left middle cerebral artery M1 occlusion. Neurosurgery was consulted and the patient underwent thrombectomy. Her hospital course was complicated by hemorrhagic transformation (HT) on hospital day 2. The patient underwent MRI that showed a large left MCA distribution acute infarction with focal reperfusion hemorrhage and parenchymal hematoma measuring approximately 3 cm in each dimension (Fig. 3). This finding prompted emergent decompression and hemicraniectomy on day 2 of hospitalization. The patient was discharged on hospital day 17 to a rehab center.

Effectiveness of a Verbal Expressive Skills Management Program for Pakistani Patients with Broca Aphasia: A Randomized Clinical Trial.

Background: It is estimated that 25% of the patients in Pakistan experience stroke resulting in problems with language. Among many of the conditions, problem with verbal expressive production (Broca's Aphasia) is one of the main problem faced by people having stoke. Many traditional therapies are incorporated to treat symptoms of Aphasia including fluent and non- fluent Aphasia. Objectives: The primary objective of the current study was to determine the effectiveness of Verbal Expressive Skill Management Program in Urdu (VESMP-U) with convention speech therapy, Melodic Intonation therapy (MIT) in enhancing the verbal expressive skills in patients with severe Broca's Aphasia. Another objective of this study was to compare the efficacy of Verbal Expressive Skill Management Program in Urdu (VESMP-U) with traditional therapy, as well as the quality of life of patients with severe Broca's Aphasia. Methods: A randomized control trial (NCT03699605, clinicaltrials.gov) was conducted from November 2018 - June 2019 in Pakistan railway Hospital (PRH). Patients having a three-month history of severe Broca's Aphasia, aged between 40-60 years, bilingual (Urdu and English language) and having the ability to use a smart phone were included in the study. Patients with cognitive impairments were excluded. Total of 77 patients were evaluated for eligibility criteria according to the G Power software for sample size. Out of 77, 54 individuals fulfilled the inclusion criteria. The participants were divided into 2 groups (27 each) through sealed envelope method. Patients of both groups were assessed pre and post intervention using the Boston Diagnostic Aphasia Examination (BADE) battery (Primary outcome measure). Experimental group n = 25 received VESMP-U therapy and control group n = 25 (2 drop out in each group) received MIT for 16 weeks i.e. 4 days per week having 64 sessions altogether. Each intervention session lasted up to 30-45 minutes for both groups. Results: Within and between group analysis after intervention showed that the VESMP-U group had significantly improved BDAE scores (P = .001; 95% CI) than the MIT group for all variables (articulatory intelligibility, phrase length, grammatical form, prosody/intonation, spontaneous speech, word finding, repetition, and auditory comprehension). The BDAE scores of participants in experimental group having VESMP-U therapy pre- and post-intervention were statistically significant (P = .001; 95% CI), which indicates that participant's communication skills were enhanced by use of VESMP-U. Conclusion: Android based application VESMP-U has been found to be effective in improving expression and quality of life of patients with severe Broca's aphasia.

The role of verbal short-term memory in complex sentence comprehension: An observational study on aphasia.

BACKGROUND: The comprehension profile of people with agrammatism is a debated topic. Syntactic complexity and cognitive resources, in particular phonological short-term memory (pSTM), are considered as crucial components by different interpretative accounts. AIM: To investigate the interaction of syntactic complexity and of pSTM in sentence comprehension in a group of persons with aphasia with and without agrammatism. METHODS & PROCEDURES: A cohort of 30 participants presenting with aphasia was assessed for syntactic comprehension and for pSTM. A total of 15 presented with agrammatism and 15 had fluent aphasia. OUTCOMES & RESULTS: Linear nested mixed-model analyses revealed a significant interaction between sentence type and pSTM. In particular, participants with lower pSTM scores showed a reduced comprehension of centre-embedded object relatives and long coordinated sentences. Moreover, a significant interaction was found between sentence type and agrammatism, with a lower performance for passives within the agrammatic group. CONCLUSIONS & IMPLICATIONS: These results confirm that pSTM is involved in the comprehension of complex structures with an important computational load, in particular coordinated sentences, and long-distance filler gap dependencies. On the contrary, the specific deficit of the agrammatic group with passives is a pure syntactic deficit, with no involvement of pSTM.

Comparing the efficiency of speech and language therapy and transcranial magnetic stimulation for treating Broca's aphasia.

OBJECTIVES: Aphasia is an acquired language-cognitive disorder that highly affects an individual's speech, language, and communication skills. Recovery from aphasia requires attentive treatment since it is a long and dynamic process. This study aimed to show interactive benefits of combining classical intervention strategies with new technological approaches and demonstrating their effectiveness. MATERIALS AND METHODS: A total of 40 individuals with Broca's aphasia were included in the study. The participants were divided into Application-1 Speech and Language Therapy, Application-2 Transcranial Magnetic Stimulation, Application-3 (consecutive Transcranial Magnetic Stimulation and Speech and Language Therapy), and Application-4 (Control Group) experimental groups, with 10 participants in each group. RESULTS: Analysis indicated that individuals in the group in which Transcranial Magnetic Stimulation and Speech and Language Therapy were applied consecutively had further increases in speech fluency, repetition, and naming scores from pre-test to post-test (p<0.01). Picture naming and quality-of-life communication scores of individuals in the group in which Speech and Language Therapy was performed increased further from pre-test to post-test (p<0.01). CONCLUSIONS: The results of the study showed a positive effect on language skills, naming scores, and participation in social life of Turkish-speaking aphasic individuals with the Speech and Language Therapy and Transcranial Magnetic Stimulation methods. The use of Transcranial Magnetic Stimulation alone is insufficient in this context. Although Speech and Language Therapy alone is effective in naming ability, Transcranial Magnetic Stimulation in addition to Speech and Language Therapy significantly increases the gain obtained with therapies.

Case study: use of sentence completion with a person with aphasia and apraxia of speech.

Several interventions for acquired apraxia of speech (AOS) involve models, imitation, or repetition to elicit production of words. Early researchers wrote about anecdotal experiences, in which sentence completion facilitated speech production for individuals with AOS. The use of sentence completion to elicit production of words during intervention has not been systematically researched. The purpose of this research was to evaluate the effectiveness of sentence completion to elicit correct productions of words, as part of an intervention for one individual with aphasia and AOS. Training occurred three times a week for six weeks. In each session, the participant answered 20 simple questions, for which the answer was one of 20 target words. Ten of these 20 words were used for training, while the other set of 10 words remained untrained. During training, the participant produced words, as the final word of a sentence. The participant improved production of the trained target words. By the end of training, the participant produced more words, as the final word in a sentence and as the answer to a simple question. For this case study, an individual with severe Broca's aphasia and severe AOS increased the number of personally-relevant words produced following training using sentence completion to elicit production.

Pure Prosodic Type of Primary Progressive Apraxia of Speech Mimicking Nonfluent Aphasia and Later Progressing to Corticobasal Syndrome.

Primary progressive apraxia of speech (PPAOS), a rare neurodedegenerative disorder, can be subdivided into predominant phonetic or prosodic type. Pure prosodic type of PPAOS as an isolated disorder has been hardly found. We present 2 cases of patients with pure prosodic PPAOS who initially were misdiagnosed as nonfluent variant of primary progressive aphasia and later turned out to be corticobasal syndrome. A 65-year-old woman and a 72-year-old man were referred to our speech-language clinic under the clinical impression of nonfluent variant of primary progressive aphasia. The neurological examinations revealed no definite abnormalities except for slow and effortful speech with the production of simple sentences. However, their receptive and expressive language abilities were normal. Their brain magnetic resonance imaging was unremarkable. We initially entertained the diagnosis of pure prosodic type of PPAOS. During several years of follow up, they gradually developed extrapyramidal symptoms which are compatible with corticobasal syndrome. The characteristics of the patients and the results of neuroimaging studies are discussed.

Damage to Broca's area does not contribute to long-term speech production outcome after stroke.

Broca's area in the posterior half of the left inferior frontal gyrus has long been thought to be critical for speech production. The current view is that long-term speech production outcome in patients with Broca's area damage is best explained by the combination of damage to Broca's area and neighbouring regions including the underlying white matter, which was also damaged in Paul Broca's two historic cases. Here, we dissociate the effect of damage to Broca's area from the effect of damage to surrounding areas by studying long-term speech production outcome in 134 stroke survivors with relatively circumscribed left frontal lobe lesions that spared posterior speech production areas in lateral inferior parietal and superior temporal association cortices. Collectively, these patients had varying degrees of damage to one or more of nine atlas-based grey or white matter regions: Brodmann areas 44 and 45 (together known as Broca's area), ventral premotor cortex, primary motor cortex, insula, putamen, the anterior segment of the arcuate fasciculus, uncinate fasciculus and frontal aslant tract. Spoken picture description scores from the Comprehensive Aphasia Test were used as the outcome measure. Multiple regression analyses allowed us to tease apart the contribution of other variables influencing speech production abilities such as total lesion volume and time post-stroke. We found that, in our sample of patients with left frontal damage, long-term speech production impairments (lasting beyond 3 months post-stroke) were solely predicted by the degree of damage to white matter, directly above the insula, in the vicinity of the anterior part of the arcuate fasciculus, with no contribution from the degree of damage to Broca's area (as confirmed with Bayesian statistics). The effect of white matter damage cannot be explained by a disconnection of Broca's area, because speech production scores were worse after damage to the anterior arcuate fasciculus with relative sparing of Broca's area than after damage to Broca's area with relative sparing of the anterior arcuate fasciculus. Our findings provide evidence for three novel conclusions: (i) Broca's area damage does not contribute to long-term speech production outcome after left frontal lobe strokes; (ii) persistent speech production impairments after damage to the anterior arcuate fasciculus cannot be explained by a disconnection of Broca's area; and (iii) the prior association between persistent speech production impairments and Broca's area damage can be explained by co-occurring white matter damage, above the insula, in the vicinity of the anterior part of the arcuate fasciculus.

Complexity and Feedback During Script Training in Aphasia: A Feasibility Study.

OBJECTIVE: To explore the effect of complexity and feedback on script training outcomes in aphasia DESIGN: Randomized balanced single-blind 2 × 2 factorial design. SETTING: Freestanding urban rehabilitation hospital. PARTICIPANTS: Adults with fluent and nonfluent aphasia (at least 6 months post onset). INTERVENTIONS: Experimental treatment was AphasiaScripts, a computer-based script training program. Scripts were 10-turns long and developed at different complexity levels to allow for comparison of high vs low complexity. The program was modified to contrast high vs low feedback conditions during sentence practice. Participants were instructed to practice three 30-minute sessions per day, 6 days per week for 3 weeks. MAIN OUTCOME MEASURES: Gains achieved from baseline in accuracy and rate of production of trained and untrained script sentences at post treatment and at 3-, 6-, and 12-weeks after the end of treatment. RESULTS: Sixteen participants completed the intervention. On the trained script, gains were statistically significant for both accuracy and words per minute at post treatment and 3-, 6-, and 12-week maintenance. Gains on the untrained script were smaller than on the trained script; they were statistically significant only for accuracy at post treatment and 3-week maintenance. Complexity had an influence on accuracy at post-treatment (F1=4.8391, P=.0501) and at maintenance (F1=5.3391, P=.0413). Practicing scripts with high complexity increased accuracy by 11.33% at post treatment and by 9.90% at maintenance compared with scripts with low complexity. Participants with nonfluent aphasia made greater gains than those with fluent aphasia. There was no significant effect of feedback. CONCLUSIONS: This study reinforces script training as a treatment option for aphasia. Results highlight the use of more complex scripts to better promote acquisition and maintenance of script production skills. There is a need for further investigation of these variables with larger samples and with other types of aphasia treatments.

Resting-State Network Changes Following Transcranial Magnetic Stimulation in Patients With Aphasia-A Randomized Controlled Study.

BACKGROUND: Although repetitive transcranial magnetic stimulation (rTMS) has exhibited promising efficacy in treating stroke-related aphasia, changes in neuroimaging in response to this therapy remain unclear. MATERIALS AND METHODS: By using resting-state functional magnetic resonance imaging (rsfMRI), we examined brain activations associated with language recovery in patients with poststroke nonfluent aphasia during an rTMS intervention. Twenty-six stroke patients with nonfluent aphasia were recruited in this randomized double-blinded study. The patients received real (n = 13) or sham (n = 13) 1-Hz inhibitory rTMS to the right pars triangularis (PTr) for ten consecutive weekdays. They underwent rsfMRI and completed the Concise Chinese Aphasia Test (CCAT) before and after the rTMS intervention. RESULT: The fractional amplitude of low-frequency fluctuation (fALFF) was calculated to investigate spontaneous neural activity in the brain. After treatment, the language function in the experimental group was higher than that in the sham group in terms of total CCAT score (p = 0.014) and the CCAT subscores of conversation (p = 0.012), description (p = 0.006), and expression (p = 0.003). Postintervention intergroup comparisons revealed that fALFF was significantly increased in the right superior temporal gyrus, right dorsolateral prefrontal gyrus, insular cortex, and caudate nucleus. Clusters in the right thalamus exhibited suppressed fALFF. The enhanced clusters in the frontotemporal region were significantly correlated with CCAT score improvements. CONCLUSIONS: Our findings provide empirical evidence for the vital role of the right frontotemporal and subcortical regions in language recovery after rTMS interventions in patients with aphasia. Inhibitory rTMS may improve language expression by promoting involvement of the right frontotemporal region. The results can be further used to refine rTMS protocols and optimize brain stimulation treatments. CLINICAL TRIAL REGISTRATION: The Clinicaltrials.gov registration number for the study is NCT03059225.

The Efficacy of Structural Priming in the Production of Sentences with Prepositions in Persian Patients with Poststroke Aphasia.

INTRODUCTION: Problems with the production of sentences with prepositions are one of the most common language problems in people with aphasia (PWA). Structural priming (SP) is one of the theory-based therapeutic approaches to improve these deficits. Although several studies have been conducted on the sentence construction problems of PWA, there is no study on the SP protocol for the production of sentences with Persian prepositions. This study aimed to investigate the effectiveness of SP in the production of these sentences among Persian-speaking PWA. METHODS: This study, with a single-subject ABA design, evaluated the efficacy of SP in the production of trained and untrained sentences containing Persian prepositions in four aphasic individuals with agrammatism and investigated the maintenance effects at 4 weeks post-training. RESULTS: Two subjects (A.G. and M.S.) showed notable changes in the production of trained sentences with Persian prepositions (50% to 91.67% for A.G. and 0% to 66.67% for M.S.). This significant improvement was generalized to sentences with untrained prepositions (50% to 91.67% for A.G. and 0% to 83.33% for M.S.). The treatment effects were also maintained in the 4-week follow-up (d2 = 10.3 for A.G. and d2 = 19.06 for M.S.). However, the other two participants only showed a slight increase in these variables after treatment. CONCLUSION: The findings of the present study revealed that SP for sentences with prepositions could be useful to varying degrees for Persian-speaking PWA with agrammatism and lead to improved language skills in producing these sentences. Overall, individuals with better language skills and cognitive status in early assessments showed better treatment outcomes than others.

Production of time reference in Turkish Broca's aphasia: The effect of morphological complexity.

In PWA (people with aphasia) difficulties with sentences that refer to the past compared to non-past time reference have been shown for many languages, including Turkish. However, the impact of morphological complexity on past time reference ability in production has not yet been reported for Turkish-speaking PWA. Turkish, where verb forms have complex inflectional paradigms and exhibit overt and non-overt morphology, facilitates the examination of the effects of morphological complexity. The current study has two objectives: 1) to investigate whether the morphological complexity of the verb form affects time reference production of Turkish-speaking PWA and 2) to provide analysis for the error patterns discovered. Seventeen Turkish individuals with Broca's aphasia who were matched in age with a control group of 17 neurologically intact Turkish individuals were tested with a picture sentence completion task. Test conditions were present progressive, simple past, past perfect, past progressive, and future tense. The task required the participants to complete each sentence frame with a verb. Our findings show that Turkish-speaking PWA were more successful in producing verb forms referring to non-past than verb forms referring to the past time reference. The current study supports previous findings that past is more difficult than non-past time reference for Turkish-speaking PWA. In terms of morphological complexity, we find that PWA were more impaired when producing morphologically complex verb forms rather than morphologically simple forms. We argue that these impairments lie in the realization of overt morphology.

[Symptomatic arachnoid cyst in an elderly patient who developed motor aphasia].

The patient was an 83-year-old woman with a history of breast cancer, distal right radial edge bone fracture, and cervical spine symptoms who had been diagnosed with an arachnoid cyst 9 years previously. She was examined by a nearby doctor, because of an approximately 1-year history of reduced verbal output; she also begun experiencing difficulties with walking. However, she was diagnosed with aging, a history of cervical spondylosis, and the effects of past fractures.At the time of this consultation, she was conscious and lucid, with mild right-sided hemiparesis, was unable to write, and had mild motor aphasia. Head magnetic resonance imaging revealed an arachnoid cyst (longer axis: 10 cm) in the left frontal lobe that did not take up contrast media. There was also a midline shift. The cause of the right hemiparesis and motor aphasia was probably compression of the left frontal lobe by the arachnoid cyst.We performed excision of the cyst wall by craniotomy and placed a cyst-peritoneal shunt under general anesthesia. At approximately one week after surgery, the patient was able to write and her motor aphasia improved. She was discharged 20 days after the operation.It is rare for an arachnoid cyst to increase in size after childhood. In the present case, surgical treatment led to a good outcome in an elderly patient with a symptomatic arachnoid cyst. Arachnoid cysts rarely increase in size. These cysts may become symptomatic in elderly people after lying clinically dormant for a long time.

[Phasic disorders and aphasia in geriatrics]. / Troubles phasiques et aphasie en gériatrie.

Aphasia, resulting from a brain lesion, leads to a partial or total loss of language in the elderly. By affecting communication abilities, it has repercussions on the life of the subject and his family. There are two types of aphasia. The roles of the caregiver and the speech therapist are in all cases essential. Depending on the communication difficulties encountered by the patient, specific supports can be recommended.

Dyke-davidoff-masson syndrome: A case report of an adolescent boy at a tertiary hospital in Adamawa, North-Eastern Nigeria.

Dyke-Davidoff-Masson Syndrome (DDMS) is a rare clinical condition in which atrophy or hypoplasia of one cerebral hemisphere occurs secondary to brain insult during fetal or early childhood, which results in variable clinical manifestations like hemiparesis, seizures, expressive aphasia, and mental retardation. This rare entity mainly presents in childhood and is unusual in adults. DDMS is a rare cause of epilepsy and should be considered and excluded in cases of refractory seizures. Few cases have been reported from a developing nation like Nigeria but not from the north-eastern part of Nigeria to the best of our knowledge. Though fewer specialists exist in Adamawa State, efforts to train more specialists and education of medical officers to manage this rare case need to be strengthened. Herein is a case of an adolescent boy with recurrent generalized tonic-clonic convulsions complicated by left-sided hemiparesis, expressive aphasia, and mental age equivalent of a six-year-old by the Goodenough draw-a-person test. Found to be obese with Body Mass Index (BMI) of 29 kg/m2 (Z-score >2 Standard deviation SD), microcephaly, Occipito-frontal Circumference (OFC) of 45 cm (Z-score > -3 SD), spastic left-sided hemiplegia and hemiplegic gait. Magnetic resonance imaging (MRI) of the brain showed hyper-intensity in the right cerebral hemisphere, extensive atrophy of the right cerebral hemisphere involving the ipsilateral fronto-temporoparietal lobes, cerebral peduncle, and a contralateral megalencephaly, ipsilateral lateral ventricular dilatation, hypertrophic calvarium, hyperpneumatization of sphenoidal sinuses and midline shift due to loss of volume on the right. A diagnosis of Dyke-Davidoff-Masson syndrome was made; the patient did well on carbamazepine and physiotherapy. Caregivers were counseled, and the patient was discharged home and is currently on a follow-up visit.

Expansion of the GRIA2 phenotypic representation: a novel de novo loss of function mutation in a case with childhood onset schizophrenia.

Childhood-onset schizophrenia (COS) is a rare form of schizophrenia with an onset before 13 years of age. There is rising evidence that genetic factors play a major role in COS etiology, yet, only a few single gene mutations have been discovered. Here we present a diagnostic whole-exome sequencing (WES) in an Israeli Jewish female with COS and additional neuropsychiatric conditions such as obsessive-compulsive disorder (OCD), anxiety, and aggressive behavior. Variant analysis revealed a de novo novel stop gained variant in GRIA2 gene (NM_000826.4: c.1522 G > T (p.Glu508Ter)). GRIA2 encodes for a subunit of the AMPA sensitive glutamate receptor (GluA2) that functions as ligand-gated ion channel in the central nervous system and plays an important role in excitatory synaptic transmission. GluA2 subunit mutations are known to cause variable neurodevelopmental phenotypes including intellectual disability, autism spectrum disorder, epilepsy, and OCD. Our findings support the potential diagnostic role of WES in COS, identify GRIA2 as possible cause to a broad psychiatric phenotype that includes COS as a major manifestation and expand the previously reported GRIA2 loss of function phenotypes.

Language treatment effects on communicative abilities and working memory in Korean-speaking agrammatic Broca's aphasia caused by moyamoya disease: Phase II evidence from a case study.

The present study reports on the language treatment outcomes from sentence- and story-level linguistic facilitation and its generalization effect on communicative abilities, working memory, and sentence processing in the case of an adult with Moyamoya Disease (MMD). After treatment,the patient's overall performance, including the Aphasia Quotient, and sentence processing ability as measured by language testing, were improved. Furthermore, the treatment effects were generalizable to working memory abilities. Our case study conveys clinically meaningful implications since it is the first report on the effects of language treatment on linguistic and cognitive domains for an individual with MMD-induced agrammatic Broca's aphasia.

Plasticity of sentence processing networks: evidence from a patient with agrammatic variant of primary progressive aphasia (PPA).

This study reports the results of a longitudinal study examining the effects of treatment for sentence processing deficits for a 70-year-old gentleman (DK) with the agrammatic variant of Primary Progressive Aphasia (PPA). On entry into the study, he presented with a 2-year history of impaired verb and sentence processing and concomitant neural atrophy in primarily subcortical regions. Spanning an 18-month period, treatment focused on improving comprehension and production of syntactically complex, passive and object cleft, structures, consecutively. Results, derived from extensive behavioral and neurocognitive testing, showed not only improved ability to comprehend and produce both trained and untrained, less complex, linguistically related structures in offline tasks, but also improved online sentence processing strategies as revealed by partially normalized eye movements in online comprehension (i.e., emergence of thematic prediction and thematic integration) and production (i.e., use of incremental processing) tasks. Changes in neural activation from pre- to post-treatment of both structures also were found, with upregulation of tissue in both the left and right hemispheres, overlapping with regions recruited by neurotypical adults performing the same task. These findings indicate that Treatment of Underlying Forms (TUF) is effective for treatment of patients with the agrammatic variant of PPA (as it is for those with stroke-induced agrammatism), and show that unaffected neural tissue in patients with PPA is malleable and may be recruited to support language, providing evidence of experience-based plasticity in neurodegenerative disease.