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Background Patients with ideomotor apraxia (IMA) present with selective impairments in higher-order motor cognition and execution without damage to any motor or sensory pathways. Although extensive research has been conducted to determine the regions of interest (ROIs) underlying these unique impairments, previous models are heterogeneous and may be further clarified based on their structural connectivity, which has been far less described. Objective The goal of this research is to propose an anatomically concise network model for the neurophysiologic basis of IMA, specific to the voluntary pantomime, imitation and tool execution, based on intrinsic white matter connectivity. Methods We utilized meta-analytic software to identify relevant ROIs in ideomotor apraxia as reported in the literature based on functional neuroimaging data with healthy participants. After generating an activation likelihood estimation (ALE) of relevant ROIs, cortical parcellations overlapping the ALE were used to construct an anatomically precise model of anatomic substrates using the parcellation scheme outlined by the Human Connectome Project (HCP). Deterministic tractography was then performed on 25 randomly selected, healthy HCP subjects to determine the structural connectivity underlying the identified ROIs. Results 10 task-based fMRI studies met our inclusion criteria and the ALE analysis demonstrated 6 ROIs to constitute the IMA network: SCEF, FOP4, MIP, AIP, 7AL, and 7PC. These parcellations represent a fronto-parietal network consisting mainly of intra-parietal, U-shaped association fibers (40%) and long-range inferior fronto-occipital fascicle (IFOF) fibers (50%). These findings support previous functional models based on dual-stream motor processing. Conclusion We constructed a preliminary model demonstrating the underlying structural interconnectedness of anatomic substrates involved in higher-order motor functioning which is seen impaired in IMA. Our model provides support for previous dual-stream processing frameworks discussed in the literature, but further clarification is necessary with voxel-based lesion studies of IMA to further refine these findings.

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.

Objectives: The potential value of gestures needs to be evaluated as an unaided alternative and augmentative communication (AAC) method to improve the naming ability of persons with Alzheimer's disease (AD). Considering the possible effect of ideomotor apraxia (IMA) in AD patients, the current study...

Objectives: The cardinal motor deficits seen in ideomotor limb apraxia are thought to arise from damage to internal representations for actions developed through learning and experience. However, whether apraxic patients learn to develop new representations with training is not well understood. We studied the capacity of apraxic patients for motor adaptation, a process associated with the development of a new internal representation of the relationship between movements and their sensory effects. Methods: Thirteen healthy adults and 23 patients with left hemisphere stroke (12 apraxic, 11 nonapraxic) adapted to a 30-degree visuomotor rotation. Results: While healthy and nonapraxic participants successfully adapted, apraxics did not. Rather, they showed a rapid decrease in error early but no further improvement thereafter, suggesting a deficit in the slow, but not the fast component of a dual-process model of adaptation. The magnitude of this late learning deficit was predicted by the degree of apraxia, and was correlated with the volume of damage in parietal cortex. Apraxics also demonstrated an initial after-effect similar to the other groups likely reflecting the early learning, but this after-effect was not sustained and performance returned to baseline levels more rapidly, consistent with a disrupted slow learning process. Conclusions: These findings suggest that the early phase of learning may be intact in apraxia, but this leads to the development of a fragile representation that is rapidly forgotten. The association between this deficit and left parietal damage points to a key role for this region in learning to form stable internal representations. (JINS, 2017, 23, 139–149)

Previous neuropsychological studies of ideomotor apraxia (IMA) indicated impairments in pantomime actions for tool use for both right and left hands following lesions of parieto-premotor cortices in the left hemisphere. Using functional magnetic resonance imaging (fMRI) with multi-voxel pattern analysis (MVPA), we tested the hypothesis that the left parieto-premotor cortices are involved in the storage or retrieval of hand-independent representation of tool-use actions. In the fMRI scanner, one of three kinds of tools was displayed in pictures or letters, and the participants made pantomimes of the use of these tools using the right hand for the picture stimuli or with the left hand for the letters. We then used MVPA to classify which kind of tool the subjects were pantomiming. Whole-brain searchlight analysis revealed successful decoding using the activities largely in the contralateral primary sensorimotor region, ipsilateral cerebellum, and bilateral early visual area, which may reflect differences in low-level sensorimotor components for three types of pantomimes. Furthermore, a successful cross-classification between the right and left hands was possible using the activities of the left inferior parietal lobule (IPL) near the junction of the anterior intraparietal sulcus. Our finding indicates that the left anterior intraparietal cortex plays an important role in the production of tool-use pantomimes in a hand-independent manner, and independent of stimuli modality.

Creutzfeldt-Jakob disease (CJD) is a rare, rapidly progressive, and fatal neurodegenerative disease with neuropsychological sequelae. This study highlighted a rare presentation of CJD (e.g. corticobasal syndrome [CBS]), reviewed updated diagnostic criteria and procedures for CJD (e.g. diffusion weighted imaging [DWI], real-time quaking-induced conversion [RT-QuIC]), and discussed differential diagnoses.Case report methodology focused on a 68-year-old, Hispanic, right-handed man with 11 years of education. He presented with a 1-2-month history of gait and motor difficulties (e.g. rigidity, myoclonus).After evaluation, a 'cortical ribboning' pattern on DWI and positive RT-QuIC was integrated with performance on neurobehavioral exam (i.e. alien limb phenomenon, unilateral ideomotor apraxia) and neuropsychological testing (i.e. frontal-parietal dysfunction pattern) to reach a diagnosis of sCJD-CBS. The patient expired 3 months after onset of symptoms.This literature review and case report highlighted the importance of staying abreast of developments in neurological literature and the added value of neuropsychology, when integrated with newer procedures, for confirming and excluding diagnostic considerations.

Stroke patients with ideomotor apraxia (IMA) have difficulties controlling voluntary motor actions, as clearly seen when asked to imitate simple gestures performed by the examiner. Despite extensive research, the neurophysiological mechanisms underlying failure to imitate gestures in IMA remain controversial. The aim of the current study was to explore the relationship between imitation failure in IMA and mirror neuron system (MNS) functioning. Mirror neurons were found to play a crucial role in movement imitation and in imitation-based motor learning. Their recruitment during movement observation and execution is signaled in EEG recordings by suppression of the lower (8–10 Hz) mu range. We examined the modulation of EEG in this range in stroke patients with left (n = 21) and right (n = 15) hemisphere damage during observation of video clips showing different manual movements. IMA severity was assessed by the DeRenzi standardized diagnostic test. Results showed that failure to imitate observed manual movements correlated with diminished mu suppression in patients with damage to the right inferior parietal lobule and in patients with damage to the right inferior frontal gyrus pars opercularis—areas where major components of the human MNS are assumed to reside. Voxel-based lesion symptom mapping revealed a significant impact on imitation capacity for the left inferior and superior parietal lobules and the left post central gyrus. Both left and right hemisphere damages were associated with imitation failure typical of IMA, yet a clear demonstration of relationship to the MNS was obtained only in the right hemisphere damage group. Suppression of the 8–10 Hz range was stronger in central compared with occipital sites, pointing to a dominant implication of mu rather than alpha rhythms. However, the suppression correlated with De Renzi's apraxia test scores not only in central but also in occipital sites, suggesting a multifactorial mechanism for IMA, with a possible impact for deranged visual attention (alpha suppression) beyond the effect of MNS damage (mu suppression).

Purpose The present article aimed to inform clinical practice on whether people with aphasia (PWA) deploy pantomime techniques similarly to participants without brain damage (PWBD) and if not, what factors influence these differences. Method We compared 38 PWA to 20 PWBD in their use of 6 representation techniques ( handling, enact, object, shape, deictic, and other ) when pantomiming objects, and determined whether PWA used the same defaults as PWBD. We assessed the influence of (non-)dominant arm use, ideomotor apraxia, semantic processing, aphasia severity, and oral naming. Results PWA used various pantomime techniques. Enact, deictic, and other were used infrequently. No differences were found for the use of shape techniques, but PWA used fewer handling and object techniques than PWBD and they did not use these for the same objects as PWBD did. No influence was found for (non-)dominant arm use. All other variables correlated with the use of handling, object, and defaults. Conclusion In our study, PWA were able to use various pantomime techniques. As a group, they used these techniques differently from PWBD and relied more heavily on the use of shape techniques. This was not influenced by a hemiparesis, but seemed dependent on semantic processing. Clinical implications are discussed.

A previous exposition of apraxia in Practical Neurology 1 offered an account of underlying impairment and accompanying clinical assessment based on a distinction between ideational, ideomotor and limb-kinetic apraxia. This conceptualisation stems from 19th-century models of higher cortical motor, language and visual function, revived by Geschwind2 3 in the 1960s. Liepmann in 19004 had posited a hierarchical model of action control over three discrete levels. At the top, movement formulae (‘visual engrams’ of the action) provided overall targets, which activate innervatory patterns to stimulate the appropriate muscles, coordinated contraction of muscles then led to execution of the action. (1) Ideational apraxia arose when movement formulae were either impaired or disconnected from the innervatory patterns. (2) Ideomotor apraxia resulted from disruption to innervatory patterns. (3) Limb-kinetic apraxia , assumed to be a disruption to the smooth neural transmission of the motor commands, was, even for Liepmann, not a full apraxia. It existed between apraxia and paresis only within a broader view of dysfunction. Liepmann’s model proved valuable in studying apraxia. However, despite surviving in several neurology textbooks, the ideational–ideomotor dichotomy has now been replaced as a way of understanding and classifying apraxias,5–8 similar to the disbandment of Broca’s versus Wernicke’s aphasia, or the dichotomy of associative versus apperceptive agnosia9 (although these distinctions, too, still persist in some areas of clinical practice). A radical rethink was needed, owing to issues with the underlying theoretical model of apraxia and difficulties in distinguishing between ideational and ideomotor clinically. Goldenberg7 made the plea to ‘relegate the dichotomy of ideational …

We report a rare case of a patient with corticobasal degeneration (CBD) who was also diagnosed with complex regional pain syndrome type I (CRPS I), which has similar clinical characteristics. A 76-year-old man who had been diagnosed with CBD several years prior presented with asymmetric severe pain, postural instability, limb rigidity, limb dystonia, tremor, ideomotor apraxia, and bradykinesia especially on his left upper extremity on admission at our rehabilitation center. Additional physical examination showed darkened skin color change, edema, reduced skin elasticity, cold skin temperature, wet skin, and limited range of motion (ROM) of the left side compared to the right side. A three-phase bone scan was done resulting CRPS I. Therefore, we initiated treatment for CRPS I, including steroid pulse therapies and non-steroidal anti-inflammatory drugs (NSAID); subsequently, his left extremity pain reduced from a visual analogue scale (VAS) score of 8-9 to 3 and his functional level also improved. To the best of our knowledge, this is the first case report of a CBD patient being also diagnosed with CRPS I. Due to the similar clinical characteristics that two diseases share, we would like to inform the physicians the importance of differentiating the CRPS I from CBD for the quick proper management.

Clinical history A 57-year old woman presented with left hand pain, periodic leg movement during sleep, gradual onset of stiffness, clumsiness, and falls. Neurological examination showed: generalized rigidity and bradykinesia. There was left hand dystonic posturing and ideomotor apraxia, as well as mirror movements of upper limbs and stimulus-sensitive myoclonus. The patient had a high-pitched voice and hypophonia (Video S1). Discussion Experts discuss localization and the syndromic diagnosis and predict the underlying pathology. The pathological diagnosis is then provided and clinical learning points are considered.

A 74 year-old woman (MD), free of previous neurological history, presented with difficulty in handling cutlery, clothes, writing with what was initially described as an atypical apraxia in acts related to space. Initial neurological evaluation revealed mixed, asymmetric pyramidal, and extrapyramidal semiology. Νeuropsychological testing revealed dressing and constructional deficits, ideomotor apraxia and signs of executive dysfunction in absence of memory, language, and visual perception pathology. The final diagnosis was that of a corticobasal degeneration, where the rare occurrence of a progressively emerging syndrome of self-management loss within peripersonal space is observed.

Limb apraxia is a heterogeneous disorder of skilled action and tool use that has long perplexed clinicians and researchers. It occurs after damage to various loci in a densely interconnected network of regions in the left temporal, parietal, and frontal lobes. Historically, a highly classificatory approach to the study of apraxia documented numerous patterns of performance related to two major apraxia subtypes: ideational and ideomotor apraxia. More recently, there have been advances in our understanding of the functional neuroanatomy and connectivity of the left-hemisphere “tool use network,” and the patterns of performance that emerge from lesions to different loci within this network. This chapter focuses on the left inferior parietal lobe, and its role in tool and body representation, action prediction, and action selection, and how these functions relate to the deficits seen in patients with apraxia subsequent to parietal lesions. Finally, suggestions are offered for several future directions that will benefit the study of apraxia, including increased attention to research on rehabilitation of this disabling disorder.

Crossover learning may aid rehabilitation in patients with neurological disorders. Ideomotor apraxia (IMA) is a common sequela of left-brain damage that comprises a deficit in the ability to perform gestures to verbal commands or by imitation. This study elucidated whether crossover learning occurred in two post-stroke IMA patients without motor paralysis after gesture training approximately 2 months after stroke onset. We quantitatively analysed the therapeutic intervention history and investigated whether revised action occurred during gesture production. Treatment intervention was to examine how to influence improvement and generalisation of the ability to produce the gesture. This study used an alternating treatments single-subject design, and the intervention method was errorless learning. Results indicated crossover learning in both patients. Qualitative analysis indicated that revised action occurred during the gesture-production process in one patient and that there were two types of post-revised action gestures: correct and incorrect gestures. We also discovered that even when a comparably short time had elapsed since stroke onset, generalisation was difficult. Information transfer between the left and right hemispheres of the brain via commissural fibres is important in crossover learning. In conclusion, improvements in gesture-production skill should be made with reference to the left cerebral hemisphere disconnection hypothesis.

We describe an autopsy case of basophilic inclusion body disease (BIBD), a subtype of frontotemporal lobar degeneration (FTLD) with the appearance of fused in sarcoma (FUS) inclusions (FTLD-FUS), clinically presenting corticobasal syndrome (CBS). A 54-year-old man initially developed worsening of stuttering and right hand clumsiness. Neurological examinations revealed rigidity in the right upper and lower extremities, buccofacial apraxia, and right-side dominant limb-kinetic and ideomotor apraxia. Neuroimaging showed asymmetric left-dominant brain atrophy and a cerebral blood flow reduction in the ipsilateral frontal region. At 56 years, his apraxia had advanced, and ideational apraxia was observed. Furthermore, the asymmetry in the limb-kinetic and ideomotor apraxia had disappeared, and both conditions had become bilateral. He had a new onset of aphasia. His symptoms progressed and he died 9 years after the initial symptoms. The brain weighed 955 g. Diffuse brain atrophy was most obvious in the bilateral frontotemporal regions. The atrophy of the left superior frontal and precentral gyri and bilateral basal ganglia was remarkable. Histologically, there was a marked loss of neurons with gliosis in the affected areas, where basophilic neuronal cytoplasmic inclusions were observed. The inclusions were immunoreactive for FUS, p62, and TATA-binding protein-associated factor 15 (TAF15), but not for phosphorylated tau, transactive response DNA-binding protein of 43 kDa (TDP-43), neurofilament protein, or Ewing sarcoma (EWS). From these pathological findings, this case was diagnosed as having BIBD as an FTLD-FUS variant. Spinal cord lower motor neurons were spared in number, similar to primary lateral sclerosis. Mutations in FUS were undetectable. Common background pathologies for CBS include corticobasal degeneration, Alzheimer's disease, PSP, FTLD with phosphorylated TDP-43 inclusions (FTLD-TDP), Pick's disease, Lewy body disease and CJD. However, FTLD-FUS (BIBD) has been rarely reported. Our case suggested further pathological heterogeneity in CBS than had previously been reported. It is necessary to consider FTLD-FUS (BIBD) as a background pathology for CBS in the future.

We propose STIMA, a short test for ideo-motor apraxia, allowing us to quantify the apraxic deficit according to action meaning and affected body segment. STIMA is based on a neurocognitive model holding that there are two processes involved in action imitation (i.e., a semantic route for recognizing and imitating known gestures, and a direct route for reproducing new gestures). The test allows to identify which imitative process has been selectively impaired by brain damage (direct vs. semantic route) and possible deficits depending on the body segment involved (hand/limb vs. hand/fingers). N = 111 healthy participants were administered with an imitation task in two separated blocks of known and new gestures. In each block, half of the gestures were performed mainly with the proximal part of the upper limb and the remaining half with the distal one. It resulted in 18 known gestures (nine proximal and nine distal) and 18 new gestures (nine proximal and nine distal) for a total of 36. Each gesture was presented up to a maximum of two times. Detailed criteria are used to assign the final imitation score. Cut offs, equivalent scores and main percentile scores were computed for each subscale. Participants imitated better known than new gestures, and proximal better than distal gestures. Age influenced performance on all subscales, while education only affected one subscale. STIMA is easy and quick to administer, and compared to previous tests, it offers important information for planning adequate rehabilitation programs based on the functional locus of the deficit.

Several neurological variables have been investigated as premorbid biomarkers of vulnerability for schizophrenia and other related disorders. The current study examined whether childhood dyspraxia predicted later adult nonaffective–psychosis-spectrum disorders. From a standardized neurological examination performed with children (aged 10–13) at genetic high risk of schizophrenia and controls, several measures of dyspraxia were used to create a scale composed of face/head dyspraxia, oral articulation, ideomotor dyspraxia (clumsiness), and dressing dyspraxia (n = 244). Multinomial logistic regression showed higher scores on the dyspraxia scale predict nonaffective–psychosis-spectrum disorders relative to other psychiatric disorders and no mental illness outcomes, even after controlling for genetic risk, χ2 (4, 244) = 18.61, p < .001. Findings that symptoms of dyspraxia in childhood (reflecting abnormalities spanning functionally distinct brain networks) specifically predict adult nonaffective–psychosis-spectrum disorders are consistent with a theory of abnormal connectivity, and they highlight a marked early-stage vulnerability in the pathophysiology of nonaffective–psychosis-spectrum disorders.

Corticobasal syndrome (CBS) is associated with asymmetrical rigidity as well as asymmetrical limb-kinetic and ideomotor apraxia. Stiff person syndrome (SPS) is characterized by muscle stiffness and gait difficulties. Whereas patients with CBS have several forms of pathology, many patients with SPS have glutamic acid decarboxylase antibodies (GAD-ab), but these 2 disorders have not been reported to coexist. We report 2 patients with GAD-ab-positive SPS who also had signs suggestive of CBS, including asymmetrical limb rigidity associated with both asymmetrical limb-kinetic and ideomotor apraxia. Future studies should evaluate patients with CBS for GAD-ab and people with SPS for signs of CBS.

Praxis is a voluntary nonverbal sequence of movements that allows the completion of programmed and organized movements in defined sequences. A type of praxis are the orofacial ideomotor, that allows to perform movements such as open and close the mouth, smiling, make tongue gestures, among others. In the execution of any praxis, a cortical cerebral component and a neuromuscular peripheral structure are involved. There is a limited literature related with the orofacial praxis recording using surface electromyography (sEMG) signals. The purpose of this paper is to describe the electromyographic characteristics of ideomotor orofacial praxis in 20 healthy individuals. Eight electrodes were placed during, the execution of five praxical orofacial movements and we evaluated the root mean square (RMS) of the sEMG signals for the orbicularis oris and masseter muscles. In the five executed praxis we found significant differences in the median values of RMS data, except for the “smile” praxis where median RMS values were similar. The evaluation of praxis is mainly used in the clinical neuropsychology field, done through tests highly dependent of the clinical expertise as a key element in determining the alteration or normal praxic funtions. This EMG characterization forecasts a progress in terms of the objective study of the praxical disorders with future implementation in diagnosis of apraxias secondary to brain lesions.

Poor oral hygiene and dental health are very common among the institutionalized elderly. The purpose of this study was to evaluate the association of apraxia with oral hygiene and/or health.Ninety-two residents of 13 long-term care homes in southwest Germany participated in the study. For each participant, ideomotor apraxia scoring (AS) was conducted and demographic variables were collected. Participants underwent a comprehensive dental examination to assess the oral health indices gingival bleeding index (GBI), community periodontal index of treatment needs (CPITN) and denture hygiene index (DHI). Statistical comparison of dental indices among apraxic and non-apraxic individuals (cut-off45) was performed by use of t-tests. In addition, linear regression models were constructed with the dental indices as dependent variables. Each model was fitted with the dichotomized variable AS (pathologic or healthy) and adjusted for age and sex.Mean (SD) GBI of 48.5 (25.9), CPITN of 2.9 (0.7) and DHI of 82.6 (14.6) were observed among the participants. Statistical analysis revealed AS was significantly associated with all oral health indicators (p0.05). Linear regression models showed apraxia is a predictor of GBI (p = 0.002) and CPITN (p = 0.027), but not of denture hygiene (p = 0.916).Although this pilot investigation has limitations, the results suggest the presence of apraxia should be considered when planning oral healthcare strategies. Further research with larger samples is encouraged to confirm these relationships.

Background Attentional and executive dysfunctions are associated with falls in community-dwelling elderly individuals and patients with PD. Frontal cognitive dysfunction and falls are frequent symptoms of PSP. We studied to identify the cognitive domains associated with recurrent falls in patients with PSP. Methods We performed a battery of neuropsychological tests in 59 individuals with probable PSP. We categorized patients into infrequent fall (≤one fall during the last 12 months, n = 29) or recurrent fall (≥two falls during the last 12 months, n = 30) groups. Results UPDRS subscores for axial deficits were significantly higher in the recurrent fall group than the infrequent fall group, but there were no significant differences in UPDRS total motor scores or subscores for bradykinesia, rigidity, and tremor. There was no difference between groups in MMSE scores. ANCOVA with adjustment for confounding factors showed that, recurrent falls were associated with abnormalities in alternating hand movement, alternating square and triangle, RCFT copying task, and ideomotor apraxia. Group difference of abnormalities in Stroop test was marginal (p = 0.054). However, there were no group differences in the frequency of abnormalities in forward or backward digit span, motor impersistence, fist-edge-palm, contrast programming, go-no-go, Luria loop drawing, or Controlled Oral Word Association Tests. Recurrent falls were not associated with memory or language dysfunction. Conclusions Recurrent falls in patients with PSP were associated mainly with executive and visuospatial dysfunctions, including (1) impaired coordinated alternating uni- and bimanual motor programming and execution, (2) deficit of attention and decision making in the presence of interference, (3) visuospatial misperception and (4) ideomotor apraxia.

A 74-year-old right-handed woman without cognitive impairment suddenly developed nonfluent aphasia. Brain MRI showed acute infarction in the right frontal lobe and insula without involvement of the corpus callosum. A neurological examination demonstrated not only transcortical motor aphasia, but also ideomotor apraxia and right hand predominant constructional apraxia (CA). To date, right hand predominant CA has only been reported in patients with corpus callosum lesions. The right hand predominant CA observed in our patient may be associated with the failure to transfer information on the spatial structure from the right hemisphere to the motor cortex of the left hemisphere.

A 62-year-old woman, previously healthy, presented with 3 weeks of progressive short-term memory loss, dyscalculia, dysgraphia, appetite loss, and frequent episodic left arm dystonic posturing and eyebrow elevation. On examination, she also had finger agnosia, dysarthria, agraphesthesia, and astereognosis bilaterally, with myoclonus, ideomotor apraxia (left worse), and wide-based gait. EEG showed slowing and periodic lateralized discharges over the right hemisphere with triphasic morphology, less often involving the left, reflecting clinical asymmetry (figure 1).1 MRI brain showed diffusion restriction in the bilateral basal ganglia with cortical ribboning more prominent on the right involving several gyri, consistent with Creutzfeldt-Jakob disease (figure 2).2

A 65-year-old woman developed progressive apraxic agraphia, characterized by poorly formed graphemes, a kanji (Japanese morphograms) recall impairment, relatively preserved oral spelling of kanji characters, and incorrect stroke sequences on writing accompanied by micrographia over a 3-year period. She also showed minor degrees of rigidity, limb-kinetic apraxia, and ideomotor apraxia of the left hand. Although asymmetric rigidity and limb-kinetic apraxia strongly suggested corticobasal degeneration, (11)C-Pittsburgh compound B positron emission tomography (PiB-PET) showed the predominantly right-sided accumulation of amyloid β in the cortices and striatum. (18)F-fluoro-deoxy-glucose PET and single photon emission computed tomography with a (99m)Tc-ethylcysteinate dimer (ECD-SPECT) also revealed predominantly right-sided hypometabolism and hypoperfusion in the primary sensorimotor cortex, posterior cingulate gyrus, temporoparietal cortices, frontal cortices, thalamus, and basal ganglia, a pattern characteristic of both corticobasal degeneration and Alzheimer's disease. The findings suggest that progressive apraxic agraphia with micrographia presenting as corticobasal syndrome can show an Alzheimer's disease pathology. It is also suggested that ideomotor apraxia of the left hand can occur without a callosal lesion, and is caused by hypometabolism or hypoperfusion in the right frontal and parietal cortices, as revealed by PET and SPECT.