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Introduction: Ideomotor apraxia, a disorder of skilled movements affecting limbs and/or face, can be seen in patients with Parkinson’s disease (PD), yet tests of apraxia in PD are rare. The aim of this project was to evaluate the psychometric properties and validity of the Dementia Apraxia Test (DATE) in a PD sample. Methods: 118 PD patients were included. Besides DATE performance, motor and non-motor burden, cognition, and activity of daily living (ADL) function were assessed. Patients were classified as cognitively impaired (n = 41) or non-cognitively impaired (n = 77). Results: Interrater reliability of the DATE (sub-)scores between video ratings and on-site ratings by the investigator was good (0.81 ≤ rk ≤ 0.87). Items were mostly easy to perform, especially the buccofacial apraxia items, which had also low discriminatory power. DATE scores were associated with cognition and ADL function. DATE performance was confounded by motor impairment and patients’ age; however, when analysed for both cognitive groups separately, the correlation between DATE and motor performance was not significant. Discussion/Conclusion: The DATE seems to be an objective and predominantly valid apraxia screening tool for PD patients, with a few items needing revision. Due to the potential effect of motor impairment and age, standardized scores adjusting for these confounders are needed.

Marchiafava-Bignami disease (MBD) is a rare complication of chronic alcoholism that typically causes demyelination and necrosis of the corpus callosum. Here, we report a man with probable MBD with callosal and right medial paracentral lesions who presented with abnormal reaching behavior and ideomotor apraxia of the left hand. He exhibited difficulty in reaching with the left hand when a target object was placed on his right-hand side, and he exhibited rightward bias when using his right hand in a line bisection task. These disturbances in reaching suggest disruption of the top-down control of motor intention and spatial attention at the corpus callosum.

Introduction Apraxia is a core clinical feature of corticobasal syndrome (CBS). Among the subtypes of apraxia, ideomotor and imitation apraxia are frequently found in CBS. However, little is known about the brain networks that are characteristic of each apraxia subtype or their clinical implication. In this study, we used 18F-fluorodeoxyglucose positron emission tomography (FDG-PET) to explore the specific patterns of glucose hypometabolism that are characteristic of apraxia subtypes by focusing on ideomotor and imitation apraxia. Methods We compared the areas of glucose hypometabolism in the brains of 52 patients with CBS and 13 healthy controls, both as a whole and according to apraxia subtypes. In addition, we investigated the relationship between the apraxia subtypes and the clinical phenotype of CBS. Results In patients with CBS, common hypometabolism was observed in the frontal gyrus, precentral gyrus and caudate regardless of apraxia subtypes. In particular, ideomotor apraxia was associated with hypometabolism in the angular gyrus, while imitation apraxia was associated with hypometabolism in the posterior part including the postcentral gyrus, precuneus, and posterior cingulate gyrus. Patients who showed both ideomotor and imitation apraxia were more likely to show the typical features of CBS and progressive supranuclear palsy compared with patients showing only one type of apraxia. Conclusion Group comparison analysis using FDG-PET revealed distinct pathways of ideomotor and imitation apraxia in CBS. These findings add to our understanding of the brain networks underlying apraxia in association with the clinical features of CBS.

To analyze the effectiveness of a home-based restorative and compensatory upper limb apraxia (ULA) rehabilitation program.Randomized controlled trial.Neurology Unit of San Cecilio Hospital and 2 private and specialized health care centers.Community dwelling participants (N=38) between the ages of 25 and 95 years old (sex ratio, 1:1) with unilateral mild-to-moderate poststroke lesions (time of evolution since stroke, 12.03±8.98mo) and secondary ULA.Participants were randomly assigned to an 8-week combined ULA functional rehabilitation group (n=19) 3 days per week for 30 minutes or to a traditional health care education protocol group (n=19) once a month for 8 weeks. Both interventions were conducted at home.Sociodemographic and clinical data, Barthel Index (primary outcome), Lawton and Brody Scale, observation and scoring activities of daily living, the De Renzi tests for ideational and ideomotor apraxia and imitating gestures test, recognition of gestures, test for upper limb apraxia , and stroke-specific quality of life scale were assessed at 3 time points: baseline, posttreatment (8wk), and follow-up (8wk).There were statistically significant differences among the groups regarding ideomotor apraxia, imitating gestures, global recognition of gestures, intransitive gestures, and comprehension of gesture production (P.05) in favor of the experimental group. However, no statistically significant differences were found between the groups regarding functionality or quality of life (P.05). Regarding the within-group effect, statistically significant differences were found in all neuropsychological outcomes at posttreatment and follow-up (P.05).A functional rehabilitation program was found to be superior to a traditional health care education program and resulted in improvements in neuropsychological functioning in ULA poststroke. Conventional education showed an insufficient effect on apraxia recovery. Further studies with larger sample sizes are needed to determine the effect of rehabilitation strategies on functionality and quality of life of poststroke ULA patients.

A 70-year-old man presented with slow gait and a clumsy hand on the right side. Neurological examination revealed dystonia and ideomotor apraxia in the right hand. A mask-like face, rigidity in the right arm, and shuffling gait were noticed. Treatment with L-dopa had no effect. He was ......

Feasibility of portable neuroimaging of cerebellar transcranial direct current stimulation (ctDCS) effects on the cerebral cortex has not been investigated vis-a-vis cerebellar lobular electric field strength. We studied functional near-infrared spectroscopy (fNIRS) in conjunction with electroencephalography (EEG) to measure changes in the brain activation at the prefrontal cortex (PFC) and the sensorimotor cortex (SMC) following ctDCS as well as virtual reality-based balance training (VBaT) before and after ctDCS treatment in 12 hemiparetic chronic stroke survivors. We performed general linear modeling (GLM) that putatively associated the lobular electric field strength with the changes in the fNIRS-EEG measures at the ipsilesional and contra-lesional PFC and SMC. Here, fNIRS-EEG measures were found in the latent space from canonical correlation analysis (CCA) between the changes in total hemoglobin (tHb) concentrations (0.01-0.07Hz and 0.07-0.13Hz bands) and log10-transformed EEG bandpower within 1-45 Hz where significant (Wilks' lambda>0.95) canonical correlations were found only for the 0.07-0.13-Hz band. Also, the first principal component (97.5% variance accounted for) of the mean lobular electric field strength was a good predictor of the latent variables of oxy-hemoglobin (O2Hb) concentrations and log10-transformed EEG bandpower. GLM also provided insights into non-responders to ctDCS who also performed poorly in the VBaT due to ideomotor apraxia. Future studies should investigate fNIRS-EEG joint-imaging in a larger cohort to identify non-responders based on GLM fitting to the fNIRS-EEG data.

Recent advancements in radiological techniques have enabled the observation of the topographic distribution of the human corpus callosum. However, its functional connectivity remains to be elucidated. The symptoms of callosal disconnection syndrome (CDS) can potentially reveal the functional connections between the cerebral hemispheres. Herein, we report a patient with CDS, whose callosal lesion was restricted to the posterior midbody, isthmus, and an anterior part of the dorsal splenium. A 53-year-old right-handed woman demonstrated CDS following cerebral infarction associated with subarachnoid hemorrhage. She exhibited CDS including ideomotor apraxia, and tactile anomia with the left hand, cross-replication of hand postures, cross-localization of the fingers, and constructional impairment with the right hand. Six months after onset, the left-handed ideomotor apraxia on imitation improved, but that to command did not, which indicated the difference in the nature of the transcallosal connections between ideomotor apraxia on imitation and ideomotor apraxia to command. Longitudinal CDS observation and corpus callosum tractography will prove useful in expanding our understanding of the nature of the organization of interhemispheric information transference.

Rationale: We report the possible therapeutic efficacy of immersive virtual reality (VR) rehabilitation for the treatment of ideomotor apraxia in a patient with stroke. Patient concerns: A 56-year-old man with sudden weakness of his left side caused by right frontal, parietal, and corpus callosal infarction was transferred to rehabilitation medicine center for intensive rehabilitation. Although his left-sided weakness had almost subsided 10 days after the onset of symptoms, he presented difficulty using his left hand and required assistance in most activities of daily living. Diagnoses: Ideomotor apraxia in a patient with right hemispheric infarction. Interventions: VR content was displayed to the study participants using a head-mounted display that involved catching of moving fish in the sea by grasping. Before and after of rehabilitative intervention including VR, functional measurements incorporating the Test of Upper Limb Apraxia (TULIA) were conducted. To directly compare therapeutic potencies under different conditions, success rates of consecutive grasping gesture performance were observed in VR, conventional occupational therapy setting, and augmented reality intervention. Outcomes: The patient demonstrated remarkable amelioration of apraxic symptoms while performing the task in the VR environment. At 1 and 3 months after the training, he showed significant improvement in most functions, and the TULIA score increased to 176 from 121 at the initiation of therapy. The number of successful grasps during 30 trials of each grasp trial was 28 in VR, 8 in the occupational therapy setting, and 20 in augmented reality. Lessons: This case report suggests the possible therapeutic efficacy of immersive VR training as a rehabilitative measure for ideomotor apraxia.

Left hemisphere stroke often causes a severe communication disorder that is usually attributed to aphasia. While aphasia refers to linguistic problems, communication is also accomplished by volunta...

Introduction: Movement Disorder Society (MDS) new diagnostic criteria for Progressive Supranuclear palsy (PSP) identifying different disease phenotypes were recently released. The aim of the present study is to report on the cognitive and behavioral features of the different phenotypes diagnosed according to the MDS criteria. Methods: Forty-nine PSP patients underwent an extensive battery of clinical assessments. Differences between PSP subtypes were computed with χ2 or ANOVA tests. Using the z scores, subjects were classified as having normal cognition, mild cognitive impairment, single or multiple domain, and dementia. A logistic regression model was implemented to investigate the major determinants of PSP non-Richardson’s syndrome phenotype. Results: Half of the cohort presented Richardson’s syndrome (46.9%), followed by PSP with parkinsonism and corticobasal syndrome (22.4% and 14.2%, respectively). Richardson’s syndrome and PSP with corticobasal syndrome presented a similar burden of disease. The only cognitive testing differentiating the phenotypes weresemantic fluency and ideomotor apraxia. The majority of our cohort was either affected by dementia or presented normal cognition. Richardson’s syndrome presented the highest rate of dementia. The only marker of PSP non-Richardson’s syndrome phenotype was better performance in visuo-spatial testing, implying worse visuo-spatial abilities in PSP Richardson’s syndrome. Conclusion: Available clinical assessments hardly capture differences between PSP phenotypes. The cognitive testing differentiating the PSP phenotypes were semantic fluency and ideomotor apraxia. In PSP, mild cognitive impairment likely represents an intermediate step from normal cognition to dementia. The only marker of PSP non-Richardson’s syndrome phenotype was better performance in visuo-spatial testing.

Background Neurodegenerative disorders are characterized by insidious progression with poorly-delineated long latent period. Antecedent clinical insult could rarely unmask latent neurodegenerative disorders. Here, we report an autopsy-proven case of corticobasal degeneration which was preceded by a lacunar infarction. Case presentation A 58-year-old man presented with acute ataxia associated with a lacunar infarction in the right paramedian pons. His ataxia persisted with additional progressive gait difficulty and left arm clumsiness. Six months later, a follow-up neurological examination showed asymmetrical bradykinesia, apraxia, dystonic posturing, postural instability, and mild ataxia of the left limbs. Cognitive examination revealed frontal executive dysfunction and visuospatial difficulties. Dopamine transporter imaging scan demonstrated bilateral reduced uptakes in mid-to-posterior putamen, more prominent on the right side. Levodopa-unresponsive parkinsonism, asymmetric limb dystonia, and ideomotor apraxia became more conspicuous, while limb ataxia gradually vanished. The patient became unable to walk without assistance after 1 year, and died 4 years after the symptom onset. Autopsy findings showed frontoparietal cortical atrophy, ballooned neurons, and phosphorylated tau-positive astrocytic plaques and neuropil threads with gliosis and neuronal loss, confirming the corticobasal degeneration. Conclusions The case illustrates that precedent clinical events such as stroke might tip a patient with subclinical CBS into overt clinical manifestations.

Alstrom syndrome (OMIM#203800) is an ultra-rare autosomal recessive monogenic disease presenting pathogenic variants in ALMS1 (chromosome 2p13). It is characterized by early onset of blindness, hearing loss and systemic comorbidities, with delayed development without cognitive impairment. We aimed to investigate the cognitive functions and describe new pathogenic variants in Alstrom syndrome patients. Nineteen patients (13 adults, 6 children) underwent a thorough clinical, genetic, laboratory, instrumental, and neurocognitive assessment. Six new pathogenic variants in ALMS1 including the first described in exon 6 were identified. Four patients displayed a "mild phenotype" characterized by slow disease onset or absence of complications, including childhood obesity and association with at least one pathogenic variant in exon 5 or 6. At neurocognitive testing, a significant proportion of patients had deficits in three neurocognitive domains: similarities, phonological memory, and apraxia. In particular, 53% of patients showed difficulties in the auditory working memory test. We found ideomotor and buccofacial apraxia in 74% of patients. "Mild phenotype" patients performed better on auditory working memory and ideomotor apraxia test than "typical phenotype" ones (91.9 + 16.3% vs. 41.7 + 34.5% of correct answers, Z = 64.5, p

Neuropsychologia : an international journal in behavioural and cognitive neuroscience 159, 107921 (2021). doi:10.1016/j.neuropsychologia.2021.107921, Published by Elsevier Science, Amsterdam [u.a.]

A 59-year-old right-handed hypertensive diabetic and previously alcoholic man presented acute confusional state followed by apathy, dysexecutive syndrome, clumsy left hand, and apraxic gait. A year later, his wife noticed impaired hearing. Neurologic examination revealed marked callosal apraxia (nondominant limb ideomotor apraxia, disconnection variant) and left stereoagnosis due to callosal disconnection (video). Left hand agraphia was present without aphasia. MRI showed punched out holes through corpus callosum, sparing the calloso-septal interface (figure 1). Audiometry revealed left neurosensorial loss. Fluorescein retinography demonstrated hyperfluorescence of arterial vessel wall (figure 2), confirming the hypothesis of Susac syndrome.1

Background: Left hemisphere stroke often causes a severe communication disorder that is usually attributed to aphasia. While aphasia refers to linguistic problems, communication is also accomplished by voluntarily articulate and gestural movements, which may be compromised due to apraxia. Along with aphasia, apraxia is a common disorder in left hemisphere stroke, which in severe cases can limit the use of verbal and nonverbal communication methods. The discussion about apraxia from a communicative perspective is still scarce, although the disorder is regularly experienced among left hemisphere stroke patients with aphasia. The rehabilitation of the disorder in severe apraxia-aphasia is challenging and recovery is slow. Aims: The purpose of this study is to provide an overview of the research on long-term recovery from apraxia and to discuss the meaning of these findings in observing the recovery of communication abilities in a person with a severe apraxia-aphasia. The search was not restricted to any specific type of apraxia, as this review assumes that communication may be influenced by apraxia in its different manifestations. The review is based on a systematic literature search, which includes English-language studies retrieved from the databases of Ovid Medline, PsycINFO, and Scopus. Main Contribution: Seven long-term follow-up studies of apraxia were found; one case study of apraxia of speech (AOS), four group studies of ideomotor apraxia (IMA), one case study of IMA (and aphasia), and one group study of limb apraxia. Conclusions: The reviewed group studies of patients with left hemisphere stroke indicate that apraxia is a persistent disorder, but the steepest recovery occurs within the first few months post-stroke. Imitation skills and actions involving real-tool use in activities of daily functions show the best recovery. Real-tool use also continues to improve longer, while recovery of gesturing after verbal command may not show clear signs of recovery in the chronic stage post-stroke. There is some evidence that the pace of recovery from oral apraxia and limb apraxia is comparable, and recovery from apraxia and aphasia would not correlate. Some of the studies used only imitation to assess changes in gesturing, which cannot be regarded as an ecologically valid measure to compare gesturing in natural communicative situations or even gesturing after verbal command. Finally, no follow-up studies were found that would have discussed apraxia from a communicative perspective. Overall, the field is lacking research on long-term follow-up of patients with apraxic-aphasic disorder.

Objectives A novel method of rehabilitation for ideomotor apraxia (IMA), using a modified version of the mirror box (MB), is proposed. The rationale is based on the theory that disrupted body representation occurs in IMA and that MB training may improve body representation. In the present MB training, patients observed and reproduced movements made by the experimenter in a mirror. The visual perspective gave the illusory sensation of seeing one's own affected hand in the mirror. Methods Thirteen patients were included in the study; apraxia was measured four times: i) at baseline; ii) after a week of unspecific poststroke rehabilitation (rest); iii) after a week of imitation training for apraxia, used as a control; and iv) after a week of MB training. Imitation and mirror box training were presented in counterbalanced order between participants. The effect of the mirror box on a measure of body representation was also assessed. Results The results show that MB training improved apraxia when compared to the outcomes in both the imitation and rest conditions. The improvement correlates with the impact of the mirror box on the body representation (i.e., the degree of embodiment). Conclusions MB training shows promising effects in promoting recovery from apraxia. The hypothesis is that the mirror box triggers a quickly generated sense of embodiment of the reflected moving arm into the observer's body representation. This embodiment of the visuomotor features of the observed movements would positively affect motor programming, promoting motor improvement. Crucially, this effect seems to extend to actions performed outside the mirror box setup, enhancing patients' performance on an apraxia test.

It has now been 100 years since Hugo Liepmann, in his classic 1920 paper described limb kinetic, ideomotor, and ideational apraxia. There are now several rating scales used to assess and grade the signs and symptoms associated with Parkinson’s disease; however, none of these assesses patients for the presence of these disabling forms of upper limb apraxia. This paper, reviews the four types of apraxia that can be associated with Parkinson’s disease, how they can be tested, the disabilities associated with these disorders and possible treatments.

Apraxia is a disorder of the skilled movements that are needed successfully perform transitive or intransitive movements, when this disorder is not caused by failures of comprehension, weakness, sensory loss, or involuntary movements. The following forms of upper limb apraxia have been reported to be associated with Parkinson's disease: (1) limb-kinetic apraxia, a loss of the ability to make precise, independent but coordinated finger-hand movements; (2) ideomotor apraxia, a failure to make the correct joint movements, to coordinate these movements with the correct timing, speed, and force; and (3) ideational apraxia, the incorrect sequencing of a series of actions required to complete a goal. This chapter summarizes the means of testing for these different forms of apraxia, the types of errors made by patients with these forms of apraxia, and the pathophysiology and treatment of these disorders.

BACKGROUND: Many patients with early-onset Alzheimer’s disease (EOAD; age of onset

OBJECTIVEApraxia is a cognitive-motor deficit affecting the execution of skilled movements, termed praxis gestures, in the absence of primary sensory or motor disorders. In patients affected by stroke, apraxia is associated with lesions of the lateral parietofrontal stream, connecting the posterior parietal areas with the ventrolateral premotor area and subserving sensory-motor integration for the hand movements. In the neurosurgical literature to date, there are few reports regarding the incidence of apraxia after glioma surgery. A retrospective analysis of patients who harbored a glioma around the central sulcus and close to the parietofrontal circuits in depth showed a high incidence of long-term postoperative hand apraxia, impairing the patients’ quality of life. To avoid the occurrence of postoperative apraxia, the authors sought to develop an innovative intraoperative hand manipulation task (HMt) that can be used in association with the brain mapping technique to identify and preserve the cortical and subcortical structures belonging to the praxis network.METHODSThe intraoperative efficacy of the HMt was investigated by comparing the incidence of postoperative ideomotor apraxia between patients undergoing mapping with (n = 79) and without (n = 41) the HMt. Patient groups were balanced for all demographic and clinical features.RESULTSIn patients with lesions in the dominant hemisphere, the HMt dramatically reduced the incidence of apraxia, with a higher sensitivity for the ideomotor than for the constructional abilities; patients with lesions in the nondominant hemisphere benefitted from the HMt for both ideomotor and constructional abilities. The administration of the test did not reduce the extent of resection.CONCLUSIONSThe HMt is a safe and feasible intraoperative tool that allowed surgeons to prevent the occurrence of long-term hand apraxia while attaining resection goals for the surgical treatment of glioma.

Apraxic agraphia can be caused by left hemispheric cerebral lesions in the area that contains the spatial representations of the movements required to write, from a lesion in, or connections to, the frontal premotor cortex that converts these spatial representations to motor programs (Exner's area). A right-handed woman with Marchiafava Bignami disease and lesions of the genu and splenium of her corpus callosum had apraxic agraphia without ideomotor apraxia of her left. A disconnection of Exner's area in the left hemisphere from the right hemisphere's premotor and motor areas may have led to her inability to write with her left hand.