OBJECTIVE: To develop and standardize the Limb and Oral Apraxia Test (LOAT) for Korean patients and investigate its reliability, validity, and clinical usefulness for patients with stroke. METHODS: We developed the LOAT according to a cognitive neuropsychological model of limb and oral praxis. The test included meaningless, intransitive, transitive, and oral praxis composed of 72 items (56 items on limb praxis and 16 items on oral praxis; maximum score 216). We standardized the LOAT in a nationwide sample of 324 healthy adults. Intra-rater and inter-rater reliability and concurrent validity tests were performed in patients with stroke. We prospectively applied the LOAT in 80 patients and analyzed the incidence of apraxia. We also compared the clinical characteristics between the apraxia and non-apraxia groups. RESULTS: The internal consistency was high (Cronbach’s alpha=0.952). The inter-rater and intra-rater reliability and concurrent validity were also high (r=0.924–0.992, 0.961–0.999, and 0.830, respectively; p<0.001). The mean total, limb, and oral scores were not significantly different according to age and education (p>0.05). Among the 80 patients with stroke, 19 (23.8%) had limb apraxia and 21 (26.3%) had oral apraxia. Left hemispheric lesions and aphasia were significantly more frequently observed in the limb/oral apraxia group than in the non-apraxia group (p<0.001). CONCLUSION: The LOAT is a newly developed comprehensive test for limb and oral apraxia for Korean patients with stroke. It has high internal consistency, reliability, and validity and is a useful apraxia test for patients with stroke.
Adult ; Aphasia ; Apraxias ; Dominance, Cerebral ; Education ; Extremities ; Humans ; Incidence ; Prospective Studies ; Psychometrics ; Reproducibility of Results ; Stroke

Crossed aphasia (CA) is defined as language impairment following right-hemispheric brain lesion in right-handed person. Exact mechanism responsible for CA is ambiguous, and recently several brain lesions have been proposed to be associated with aphasia using lesion mapping method. Corpus callosum has dual bloody supply which makes it less vulnerable to infarction. Speech difficulties such as stuttering after corpus callosum infarction have been reported in the past, but aphasia is rare, which makes CA more unique. We report an extraordinary case of CA after right corpus callosum infarction. A 74-year-old female patient with a previous history of right thalamus infarction with no neurologic sequela has developed language disturbance without apraxia 1 month ago and a diffusion-weighted magnetic resonance imaging showed newly developed infarction at right corpus callosum. The aphasia quotient of the Korean version of the Western Aphasia Battery was 2.5, implying severe global aphasia. Positron emission tomography-computed tomography showed decreased metabolism in right corpus callosum and left frontal and temporal cortex, suggesting that interhemispheric diaschisis may be responsible for the CA. This is an extraordinary case report of an isolated manifestation of CA secondary to right corpus callosum infarction.
Aged ; Aphasia ; Apraxias ; Brain ; Brain Infarction ; Corpus Callosum ; Electrons ; Female ; Humans ; Infarction ; Magnetic Resonance Imaging ; Metabolism ; Methods ; Stuttering ; Temporal Lobe ; Thalamus

No abstract available.
Gait Apraxia ; Gait

OBJECTIVE: Dysphagia is a common consequence of stroke with a negative effect on the clinical outcome. Given these potential outcomes, it is important to identify the precursors to dysphagia after stroke. The aims of this study were to identify lesions associated with dysphagia after an ischemic supratentorial stroke using voxel-based lesion symptom mapping (VLSM) and compare the difference in the lesion pattern between the oral and pharyngeal phase dysphagia. METHODS: Stroke patients who met the following inclusion criteria were screened retrospectively between January 2012 and November 2014: a first-ever stroke, supratentorial lesion and who underwent brain MRI and functional dysphagia scale (FDS) from videofluoroscopic swallowing study (VFSS). Finally, the MRI data of 83 patients were analyzed. Statistical maps of the lesion contribution related to dysphagia were generated using VLSM. RESULTS: VLSM showed that FDS was associated with damage to the putamen, caudate, insula, frontal precentral gyrus, and inferior frontal gyrus. The lesions were distributed more widely in the left than right hemisphere. Lesions correlated with the FDS oral score were distributed mainly in the frontal lobe and insula. Otherwise, the associated lesion with the FDS pharyngeal score was mainly the basal ganglia. CONCLUSION: In these results, lesions that correlated with dysphagia were distributed more widely in the left hemisphere, reflecting the possibility of lateralization of the swallowing function. Oral phase dysphagia was associated with left frontal lobe and insula; the lesion correlated with the cognitive function or apraxia. On the other hand, VLSM revealed the lesions associated with pharyngeal dysphagia to be the basal ganglia, which is a structure that plays a role in the automatic motor control network.
Apraxias ; Basal Ganglia ; Brain ; Brain Mapping ; Cognition ; Deglutition ; Deglutition Disorders ; Frontal Lobe ; Hand ; Humans ; Magnetic Resonance Imaging ; Neuroanatomy ; Prefrontal Cortex ; Putamen ; Retrospective Studies ; Stroke

No abstract available.
Apraxias ; Parkinson Disease ; Transcranial Magnetic Stimulation

OBJECTIVE: To investigate the characteristics and risk factors of dysphagia using the videofluoroscopic dysphagia scale (VDS) with a videofluoroscopic swallowing study (VFSS) in patients with acute cerebral infarctions. METHODS: In this retrospective study, the baseline VFSS in 275 stroke patients was analyzed. We divided patients into 8 groups according to lesion areas commonly observed on brain magnetic resonance imaging. Dysphagia characteristics and severity were evaluated using the VDS. We also analyzed the relationship between clinical and functional parameters based on medical records and VDS scores. RESULTS: In comparison studies of lesions associated with swallowing dysfunction, several groups with significant differences were identified. Apraxia was more closely associated with cortical middle cerebral artery territory lesions. Vallecular and pyriform sinus residue was more common with lesions in the medulla or pons. In addition, the results for the Korean version of the Modified Barthel Index (K-MBI), a functional assessment tool, corresponded to those in the quantitative evaluation of swallowing dysfunctions. CONCLUSION: A large cohort of patients with cerebral infarction was evaluated to determine the association between brain lesions and swallowing dysfunction. The results can be used to establish a specific treatment plan. In addition, the characteristic factors associated with swallowing dysfunctions were also confirmed.
Apraxias ; Brain* ; Cerebral Infarction* ; Cohort Studies ; Deglutition ; Deglutition Disorders* ; Evaluation Studies as Topic ; Fluoroscopy ; Humans ; Magnetic Resonance Imaging ; Medical Records ; Middle Cerebral Artery ; Pons ; Pyriform Sinus ; Retrospective Studies ; Risk Factors ; Stroke

BACKGROUND AND PURPOSE: Autosomal recessive cerebellar ataxias constitute a highly heterogeneous group of neurodegenerative disorders. This study was carried out to determine the clinical and genetic causes of ataxia in two families from Pakistan. METHODS: Detailed clinical investigations were carried out on probands in two consanguineous families. Magnetic resonance imaging was performed. Exome sequencing data were examined for likely pathogenic variants. Candidate variants were checked for cosegregation with the phenotype using Sanger sequencing. Public databases including ExAC, GnomAD, dbSNP, and the 1,000 Genome Project as well as ethnically matched controls were checked to determine the frequencies of the alleles. Conservation of missense variants was ensured by aligning orthologous protein sequences from diverse vertebrate species. RESULTS: Reverse phenotyping identified spinocerebellar ataxia, autosomal recessive 1 [OMIM 606002, also referred to as ataxia oculomotor apraxia type 2 (AOA2)] and ataxia telangiectasia (OMIM 208900) in the two families. A novel homozygous missense mutation c.202 C>T (p.Arg68Cys) was identified within senataxin, SETX in the DNA of both patients in one of the families with AOA2. The patients in the second family were homozygous for a known variant in ataxia-telangiectasia mutated (ATM) gene: c.7327 C>T (p.Arg2443Ter). Both variants were absent from 100 ethnically matched control chromosomes and were either absent or present at very low frequencies in the public databases. CONCLUSIONS: This report extends the allelic heterogeneity of SETX mutations causing AOA2 and also presents an asymptomatic patient with a pathogenic ATM variant.
Alleles ; Apraxias* ; Ataxia Telangiectasia ; Ataxia* ; Cerebellar Ataxia ; DNA ; Exome ; Genome ; Humans ; Magnetic Resonance Imaging ; Movement Disorders ; Mutation, Missense ; Neurodegenerative Diseases ; Pakistan ; Phenotype ; Population Characteristics ; Spinocerebellar Ataxias ; Vertebrates

We present a case of beta-propeller protein-associated neurodegeneration, a form of neurodegeneration with brain iron accumulation. The patient harbored a novel mutation in the WDR45 gene. A detailed video and description of her clinical condition are provided. Her movement disorder phenomenology was characterized primarily by limb stereotypies and gait dyspraxia. The patient's disability was advanced by the time iron-chelating therapy with deferiprone was initiated, and no clinical response in terms of cognitive function, behavior, speech, or movements were observed after one year of treatment.
Brain ; Chelation Therapy ; Cognition ; Extremities ; Gait Apraxia ; Humans ; Iron ; Movement Disorders

With recent advancement in amyloid imaging, diagnostic application of this new modality has become a great interest among researchers. New ligands, such as 18F- florbetaben, florbetapir and flutemetamol, have been discovered to overcome limitations of preexisting ligand Pittsburgh compound B. We report here a case of a 37-year-old male patient whose initial complaints comprised of gradual cognitive decline, apraxia, disorientation and sleep disturbances. 18F-Florbetaben amyloid imaging of the patient showed diffuse amyloid retention with prominent striatal uptake. This finding supports the clinical utility of amyloid imaging in diagnostic process of early-onset AD. Moreover, striatal dominant uptake pattern demonstrated in this patient include some meaningful clinical implications that warrant special attention among clinicians.
Adult* ; Alzheimer Disease* ; Amyloid* ; Apraxias ; Diagnostic Imaging ; Humans ; Ligands ; Male

Cerebellar mutism (CM) is a rare neurological condition characterized by lack of speech due to cerebellar lesions. CM is often reported in children. We describe a rare case of CM after spontaneous cerebellar hemorrhage. The patient showed mutism, irritability, decreased spontaneous movements and oropharyngeal apraxia. Diffusion tensor imaging revealed significant volume reduction of medial frontal projection fibers from the corpus callosum. In Tracts Constrained by UnderLying Anatomy (TRACULA) analysis, forceps major and minor and bilateral cingulum-angular bundles were not visualized. Cerebello-frontal pathway reconstructed from the FMRIB Software Library showed continuity of fibers, with decreased number of fibers on qualitative analysis. These results suggest that cerebello-frontal disconnection may be a neuroanatomical mechanism of CM. Damage of brain network between occipital lobe, cingulate and cerebellum caused by hemorrhage may also have role in the mechanism of CM in our case.
Akinetic Mutism ; Apraxias ; Brain ; Cerebellum ; Child ; Corpus Callosum ; Diffusion Tensor Imaging ; Hemorrhage ; Humans ; Mutism* ; Occipital Lobe ; Stroke* ; Surgical Instruments