-
Brain and Nerve = Shinkei Kenkyu No... Oct 2023The patient was a 66-year-old man brought to the emergency room with impaired consciousness due to hypercarbonemia, managed on a respirator, and diagnosed with...
The patient was a 66-year-old man brought to the emergency room with impaired consciousness due to hypercarbonemia, managed on a respirator, and diagnosed with amyotrophic lateral sclerosis (ALS). MRI showed atrophy of the anterior and medial surfaces of the bilateral temporal lobes that was more severe in the right side. The patient had dysgraphia in both kana and kanji. Detailed examinations of the language function revealed impaired single-word comprehension, impaired naming, and surface dysgraphia, leading to the diagnosis of semantic variant primary progressive aphasia (svPPA). ALS patients with atrophy of the anterior temporal lobe and surface dysgraphia of kanji may have svPPA as a complication. (Received April 14, 2023; Accepted June 21, 2023; Published October 1, 2023).
Topics: Male; Humans; Aged; Agraphia; Semantics; Amyotrophic Lateral Sclerosis; Language; Magnetic Resonance Imaging; Aphasia, Primary Progressive; Atrophy
PubMed: 37849367
DOI: 10.11477/mf.1416202493 -
Journal of Alzheimer's Disease : JAD 2023Handwriting is a complex process involving fine motor skills, kinesthetic components, and several cognitive domains, often impaired by Alzheimer's disease (AD).
BACKGROUND
Handwriting is a complex process involving fine motor skills, kinesthetic components, and several cognitive domains, often impaired by Alzheimer's disease (AD).
OBJECTIVE
Provide a systematic review of handwriting changes in AD, highlighting the effects on motor, visuospatial and linguistic features, and to identify new research topics.
METHODS
A search was conducted on PubMed, Scopus, and Web of Science to identify studies on AD and handwriting. The review followed PRISMA norms and analyzed 91 articles after screening and final selection.
RESULTS
Handwriting is impaired at all levels of the motor-cognitive hierarchy in AD, particularly in text, with higher preservation of signatures. Visuospatial and linguistic features were more affected. Established findings for motor features included higher variability in AD signatures, higher in-air/on-surface time ratio and longer duration in text, longer start time/reaction time, and lower fluency. There were conflicting findings for pressure and velocity in motor features, as well as size, legibility, and pen lifts in general features. For linguistic features, findings were contradictory for error patterns, as well as the association between agraphia and severity of cognitive deficits.
CONCLUSIONS
Further re-evaluation studies are needed to clarify the divergent results on motor, general, and linguistic features. There is also a lack of research on the influence of AD on signatures and the effect of AD variants on handwriting. Such research would have an impact on clinical management (e.g., for early detection and patient follow-up using handwriting tasks), or forensic examination aimed at signatory identification.
Topics: Humans; Alzheimer Disease; Handwriting; Agraphia; Cognition Disorders; Cognitive Dysfunction
PubMed: 37718808
DOI: 10.3233/JAD-230438 -
Journal of Neurovirology Oct 2023Intensive immunosuppression has enabled liver transplantation even in recipients with preformed donor-specific antibodies (DSA), an independent risk factor for graft...
Intensive immunosuppression has enabled liver transplantation even in recipients with preformed donor-specific antibodies (DSA), an independent risk factor for graft rejection. However, these recipients may also be at high risk of progressive multifocal encephalopathy (PML) due to the comorbid immunosuppressed status. A 58-year-old woman presented with self-limited focal-to-bilateral tonic-clonic seizures 9 months after liver transplantation. She was desensitized using rituximab and plasma exchange before transplantation and was subsequently treated with steroids, tacrolimus, and everolimus after transplantation for her preformed DSA. Neurological examination revealed mild acalculia and agraphia. Cranial MRI showed asymmetric, cortex-sparing white matter lesions that increased over a week in the left frontal, left parietal, and right parieto-occipital lobes. Polymerase chain reaction (PCR) of the cerebrospinal fluid for the JC supported the diagnosis of PML. Immune reconstitution by reducing the immunosuppressant dose stopped lesion expansion, and PCR of the cerebrospinal fluid for the JC virus became negative. Graft rejection occurred 2 months after immune reconstitution, requiring readjustment of immunosuppressants. Forty-eight months after PML onset, the patient lived at home without disabling deficits. Intensive immunosuppression may predispose recipients to PML after liver transplantation with preformed DSA. Early immune reconstitution and careful monitoring of graft rejection may help improve outcomes.
Topics: Humans; Female; Middle Aged; Leukoencephalopathy, Progressive Multifocal; Liver Transplantation; Living Donors; JC Virus; Immunosuppressive Agents
PubMed: 37668873
DOI: 10.1007/s13365-023-01171-x -
Rinsho Shinkeigaku = Clinical Neurology Aug 2023A 72-year-old male developed neurological symptoms such as difficulty in charging his electronic money card and making his mobile-phone call ten months before admission....
[Classical cortical superficial siderosis presenting as extensive higher brain dysfunction with hypoperfusion in the frontoparietal lobe on the I-IMP-SPECT: a case report].
A 72-year-old male developed neurological symptoms such as difficulty in charging his electronic money card and making his mobile-phone call ten months before admission. On admission, neurological examination revealed extensive higher brain dysfunction such as impairment in recent memory, executive function disorders, constructional disturbance, agraphia and acalculia. Brain MRI revealed a low intensity lesion on the surface of the cerebral cortex diffusely and symmetrically on T*-weighted images. MRI images are consistent with superficial siderosis. However, the lack of hemosiderin deposition in the brain stem and cerebellar hemisphere was atypical of the classical type of superficial siderosis. I-IMP-SPECT revealed hypoperfusion dominantly in the left hemisphere, particularly in the left frontal and parietal lobes. According to the Boston criteria, the patient with the cerebral microbleeds and cortical superficial siderosis was diagnosed with probable CAA (cerebral amyloid angiopathy).
Topics: Male; Humans; Aged; Siderosis; Brain Diseases; Cerebral Amyloid Angiopathy; Cerebral Cortex; Magnetic Resonance Imaging; Tomography, Emission-Computed, Single-Photon; Cerebral Hemorrhage
PubMed: 37518020
DOI: 10.5692/clinicalneurol.cn-001828 -
Cortex; a Journal Devoted To the Study... Sep 2023It has been suggested that Gerstmann's syndrome is the result of subcortical disconnection rather than emerging from damage of a multifunctional brain region within the...
It has been suggested that Gerstmann's syndrome is the result of subcortical disconnection rather than emerging from damage of a multifunctional brain region within the parietal lobe. However, patterns of white matter tract disconnection following parietal damage have been barely investigated. This single case study allows characterising Gerstmann's syndrome in terms of disconnected networks. We report the case of a left parietal patient affected by Gerstmann's tetrad: agraphia, acalculia, left/right orientation problems, and finger agnosia. Lesion mapping, atlas-based estimation of probability of disconnection, and DTI-based tractography revealed that the lesion was mainly located in the superior parietal lobule, and it caused disruption of both intraparietal tracts passing through the inferior parietal lobule (e.g., tracts connecting the angular, supramarginal, postcentral gyri, and the superior parietal lobule) and fronto-parietal long tracts (e.g., the superior longitudinal fasciculus). The lesion site appears to be located more superiorly as compared to the cerebral regions shown active by other studies during tasks impaired in the syndrome, and it reached the subcortical area potentially critical in the emergence of the syndrome, as hypothesised in previous studies. Importantly, the reconstruction of tracts connecting regions within the parietal lobe indicates that this critical subcortical area is mainly crossed by white matter tracts connecting the angular gyrus and the superior parietal lobule. Taken together, these findings suggest that this case study might be considered as empirical evidence of Gerstmann's tetrad caused by disconnection of intraparietal white matter tracts.
Topics: Humans; Gerstmann Syndrome; White Matter; Parietal Lobe; Brain; Agnosia
PubMed: 37478549
DOI: 10.1016/j.cortex.2023.05.016 -
Zhurnal Nevrologii I Psikhiatrii Imeni... 2023The review is devoted to one of the current problems of pediatric neurology - reading and writing disorders in children as part of a partial developmental disorder. With...
The review is devoted to one of the current problems of pediatric neurology - reading and writing disorders in children as part of a partial developmental disorder. With the development of neuroscience, the paradigm of «brain damage» in the understanding of a number of pathological conditions was replaced by the concept of «evolutionary neurology». The dominance of the ontogenetic approach caused the appearance of a new section in ICD-11 - «Neurodevelopmental disorders». Twenty-one genes associated with the acquisition of reading and writing skills have been identified. Modern studies demonstrate the connection of neuropsychological prerequisites for reading and writing, and clinical phenotypes of dyslexia with changes in specific loci. It is assumed that there are different molecular genetic bases for dyslexia and dysgraphia depending on ethnicity, orthographic features of language, including logographic features. Pleiotropy of genes is a cause of comorbidity of reading and writing disorders with attention deficit and hyperactivity disorder, specific speech articulation disorders, and dyscalculia. A key function of many of the identified genes is their involvement in the processes of neurogenesis. Their dysfunctions cause atypical neuronal migration, ectopic formation, inadequate axonal growth, and dendrite branching at the early stage of brain development. Morphological changes can distort the correct distribution and/or integration of linguistic stimuli in critical brain areas, leading to abnormalities in phonology, semantics, spelling, and general reading comprehension. The knowledge gained can form the basis for the development of risk models for dysgraphia and dyslexia formation and be used as a diagnostic and/or screening tool, which is important for evidence-based correction, optimization of academic performance, and mitigation of psychosocial consequences.
Topics: Humans; Agraphia; Dyslexia; Genetic Background; Brain; Language
PubMed: 37315241
DOI: 10.17116/jnevro202312305148 -
Sensors (Basel, Switzerland) May 2023Handwriting learning disabilities, such as dysgraphia, have a serious negative impact on children's academic results, daily life and overall well-being. Early detection...
Handwriting learning disabilities, such as dysgraphia, have a serious negative impact on children's academic results, daily life and overall well-being. Early detection of dysgraphia facilitates an early start of targeted intervention. Several studies have investigated dysgraphia detection using machine learning algorithms with a digital tablet. However, these studies deployed classical machine learning algorithms with manual feature extraction and selection as well as binary classification: either dysgraphia or no dysgraphia. In this work, we investigated the fine grading of handwriting capabilities by predicting the SEMS score (between 0 and 12) with deep learning. Our approach provided a root-mean-square error of less than 1 with automatic instead of manual feature extraction and selection. Furthermore, the SensoGrip smart pen SensoGrip was used, i.e., a pen equipped with sensors to capture handwriting dynamics, instead of a tablet, enabling writing evaluation in more realistic scenarios.
Topics: Child; Humans; Deep Learning; Handwriting; Agraphia; Algorithms; Machine Learning
PubMed: 37299942
DOI: 10.3390/s23115215 -
Brain & NeuroRehabilitation Mar 2023Gerstmann syndrome (GS) is a rare syndrome that occurs when there is a lesion of the dominant inferior parietal lobule (IPL), causing agraphia, acalculia, finger...
Gerstmann syndrome (GS) is a rare syndrome that occurs when there is a lesion of the dominant inferior parietal lobule (IPL), causing agraphia, acalculia, finger agnosia, and right-left disorientation. A 49-year-old right-handed male was diagnosed as GS after left parieto-occipital lobe hemorrhage. The patient showed mild anomic aphasia with agraphia in the language test and the neuropsychological test revealed acalculia, impaired right-left discrimination, and finger agnosia. In diffusion tensor tractography, the tracts of left superior longitudinal fasciculus (SLF), middle longitudinal fasciculus, U-fibers and posterior corpus callosum (CC) were disrupted around the left IPL. In addition, fractional anisotropy (FA) values were markedly decreased in left SLF, and posterior CC when compared to twelve healthy control subjects. Our clinical and neuroimaging findings support that GS is a disconnection syndrome caused by lesion in the white matter pathway surrounding IPL. In future, more studies of the correlation between the white matter disconnection and the development of GS including high quality imaging technique are needed.
PubMed: 37033002
DOI: 10.12786/bn.2023.16.e3 -
Journal of Neurosurgery. Case Lessons Mar 2023Apraxia of speech is a disorder of speech-motor planning in which articulation is effortful and error-prone despite normal strength of the articulators. Phonological...
BACKGROUND
Apraxia of speech is a disorder of speech-motor planning in which articulation is effortful and error-prone despite normal strength of the articulators. Phonological alexia and agraphia are disorders of reading and writing disproportionately affecting unfamiliar words. These disorders are almost always accompanied by aphasia.
OBSERVATIONS
A 36-year-old woman underwent resection of a grade IV astrocytoma based in the left middle precentral gyrus, including a cortical site associated with speech arrest during electrocortical stimulation mapping. Following surgery, she exhibited moderate apraxia of speech and difficulty with reading and spelling, both of which improved but persisted 6 months after surgery. A battery of speech and language assessments was administered, revealing preserved comprehension, naming, cognition, and orofacial praxis, with largely isolated deficits in speech-motor planning and the spelling and reading of nonwords.
LESSONS
This case describes a specific constellation of speech-motor and written language symptoms-apraxia of speech, phonological agraphia, and phonological alexia in the absence of aphasia-which the authors theorize may be attributable to disruption of a single process of "motor-phonological sequencing." The middle precentral gyrus may play an important role in the planning of motorically complex phonological sequences for production, independent of output modality.
PubMed: 37014023
DOI: 10.3171/CASE22504