Complete monoplegia due to limb-kinetic apraxia in a patient with traumatic brain injury: A case report.

RATIONALE: Limb-kinetic apraxia (LKA) is a disorder of movement execution that is a result of injury to the corticofugal tracts (CFTs) from the secondary motor area. We report on a patient with traumatic brain injury (TBI) and complete monoplegia due to LKA, which was mainly ascribed to injury of the CFT from the secondary motor area using diffusion tensor tractography. PATIENT CONCERNS: A 35-year-old male was struck by a car from the side during riding an autocycle and received direct head trauma as a result of falling to ground. He lost consciousness for approximately 1 month and experienced continuous post-traumatic amnesia after the accident. The patient's Glasgow Coma Scale score was 3 and he showed quadriparesis including complete monoplegia of his left arm since the onset of TBI. DIAGNOSES: The patient diagnosed complete monoplegia due to LKA after traumatic brain injury. INTERVENTIONS: He underwent conservative management for TBI followed by rehabilitation at approximately 2 months after onset. OUTCOMES: At 32-month after onset, weakness on left arm (Manual Muscle Test [MMT]:0) and partial weakness of left leg (MMT:3). OUTCOMES: Results of electromyography and nerve conduction studies of left extremities were normal. Motor evoked potential values obtained from the abductor pollicis brevis muscle (APB) were: right APB latency 22.3msec, amplitude 1.6mV; left APB latency 22.8msec, amplitude 1.5mV. After 2 weeks of administration of dopaminergic drugs for improvement of LKA, left arm weakness had recovered to level that permitted movement against gravity (MMT:3). Diffusion tensor tractography at 32-month after onset showed right corticospinal tract discontinuation at the pontine level and partial tearing of the left corticospinal tract at the subcortical white matter. In addition, the left CFT from the supplementary motor area showed partial tearing at the subcortical white matter. LESSONS: The LKA due to injury of the left supplementary motor area-CFT was demonstrated in a patient with complete monoplegia following TBI. Accurate diagnosis of LKA is important for successful rehabilitation because LKA is known to respond to dopaminergic drug treatment.

Callosal ideomotor apraxia in Alzheimer's disease.

BACKGROUND/OBJECTIVE: Impaired ability to perform skilled movements with the left upper limb in patients with corpus callosum injury has been well described (callosal apraxia) with some displaying spatial-temporal errors primarily in response to verbal commands (verbal callosal disconnection apraxia), with imitation, and when using actual tools (callosal ideomotor apraxia). Additionally some patients with callosal injury also make content errors when selecting and using the incorrect tool with their left upper limb (callosal conceptual apraxia). Interestingly, patients with Alzheimer's disease (AD) reveal anatomic evidence of callosal degeneration but callosal apraxia in AD has not been described. The purpose of this study was to learn whether patients with AD display forms of callosal apraxia. METHOD: Participants were 22 right-handed patients with AD and 24 matched controls. Both upper limbs were tested by having subjects pantomime transitive movements to command and imitation. Participants also viewed pictures of an incomplete task and attempted to pantomime the action needed to complete the task. RESULTS AND CONCLUSIONS: When compared to controls, the participants with AD demonstrated ideomotor and conceptual apraxias of both upper limbs; however, ideomotor apraxia of their left hand was more robust than that of their right hand, suggesting a hemispheric disconnection.

Novel SETX variants in a patient with ataxia, neuropathy, and oculomotor apraxia are associated with normal sensitivity to oxidative DNA damaging agents.

BACKGROUND: Homozygous and compound heterozygous mutations in SETX are associated with AOA2 disease, a recessive form of ataxia with oculomotor apraxia and neuropathy with onset of ataxia between the first and second decade of life. The majority of the AOA2 mutated cell lines tested show hypersensitivity to oxidative DNA damaging agents, with one exception. RESULTS: We describe a patient presenting with early-onset progressive ataxia, oculomotor apraxia, axonal sensory-motor neuropathy, optic atrophy, delayed psychomotor development, and a behavior disorder. The patient carries two novel missense variants in the SETX gene. Based on the hypothesis that the patient's clinical phenotype may represent an atypical form of the AOA2 disease, we tested the patient-derived cell line for hypersensitivity to oxidative DNA damaging agents, with negative results. CONCLUSIONS: The lack of hypersensitivity we observed may be explained either by considering the atypical clinical picture of the patient analyzed or, alternatively, by hypothesizing that the variants detected are not the cause of the observed phenotype. Consistent with the first hypothesis of an atypical AOA2 form and based on the multiple functions of senataxin reported so far, it is likely that different sets of SETX mutations/variants may have variable functional effects that still need to be functionally characterized. The possibility that the severe and complicated clinical picture presented by the patient described here represents a clinical entity differing from the known recessive ataxias should be considered as well.

Ideomotor apraxia in agrammatic and logopenic variants of primary progressive aphasia.

There are few studies examining praxis in subjects with primary progressive aphasia. The aim of this study was to examine the pattern and severity of ideomotor apraxia in subjects with logopenic and agrammatic variants of primary progressive aphasia and to determine if the presence of ideomotor apraxia correlated with specific neuroanatomical structural abnormalities. Subjects with primary progressive aphasia were prospectively recruited and classified according to published criteria. Using the apraxia subtest of the Western Aphasia Battery, pattern and severity of ideomotor apraxia was examined in all subjects diagnosed with agrammatic and logopenic variants of primary progressive aphasia. The study included 47 subjects, 21 diagnosed with agrammatic variant of primary progressive aphasia and 26 with logopenic variant primary progressive aphasia. Subjects with agrammatic aphasia were older at onset than the logopenic variant (67.2 vs. 61.7 years, p = 0.02), but there was no difference in illness duration prior to evaluation. Those with logopenic aphasia showed more cognitive impairment on the Mini-Mental Status Examination (agrammatic = 26.7/30, logopenic = 22/30, p = 0.002), and a trend for more severe language impairment as measured by the Western Aphasia Battery-Aphasia Quotient (agrammatic = 82.3, logopenic = 75.2, p = 0.11). Strong correlations were found between Western Aphasia Battery-Aphasia Quotient and total apraxia, instrumental apraxia, and complex apraxia, while average to modest correlations were seen with upper limb apraxia and facial apraxia. After adjusting for age, mental status performance, and Western Aphasia Battery-Aphasia Quotient score, those with agrammatic aphasia had a higher degree of total apraxia (p = 0.004), facial apraxia (p = 0.03), instrumental apraxia (p = 0.0006), and complex apraxia (p = 0.0006) than those with logopenic aphasia. The agrammatic variant of primary progressive aphasia was associated with greater praxis deficits but less cognitive impairment than the logopenic variant. The presence of ideomotor apraxia was associated with grey matter loss in the left lateral premotor cortex with extension into the motor cortex. These findings suggest that although some affected areas in the agrammatic and logopenic variants of primary progressive aphasia overlap, there exists an area that is more affected in the agrammatic variant than the logopenic variant that accounts for the greater association of agrammatic aphasia with ideomotor apraxia.

Impaired finger dexterity in Parkinson's disease is associated with praxis function.

A controversial concept suggests that impaired finger dexterity in Parkinson's disease may be related to limb kinetic apraxia that is not explained by elemental motor deficits such as bradykinesia. To explore the nature of dexterous difficulties, the aim of the present study was to assess the relationship of finger dexterity with ideomotor praxis function and parkinsonian symptoms. Twenty-five patients with Parkinson's disease participated in the study. Their left and right arms were tested independently. Testing was done in an OFF and ON state as defined by a modified version of the Movement Disorder Society-Unified Parkinson's Disease Rating Scale (MDS-UPDRS). Finger dexterity was assessed by a coin rotation (CR) task and ideomotor praxis using a novel test of upper limb apraxia (TULIA), in which the patients were requested to imitate and pantomime 48 meaningless, as well as communicative and tool-related gestures. Coin rotation significantly correlated with TULIA irrespective of the motor state and arm involved, but not with the MDS-UPDRS. This association was significantly influenced by Hoehn and Yahr stage. The strong association of finger dexterity with praxis function but not the parkinsonian symptoms indicates that impaired finger dexterity in Parkinson's disease may be indeed apraxic in nature, yet, predominantly in advanced stages of the disease when cortical pathology is expected to develop. The findings are discussed within a cognitive-motor model of praxis function.

A new bedside test of gestures in stroke: the apraxia screen of TULIA (AST).

BACKGROUND: Apraxia in patients with stroke may be overlooked, as clumsiness and deficient gestural communication are often attributed to frequently coexisting sensorimotor deficits and aphasia. Early and reliable detection of apraxia by a bedside test is relevant for functional outcome in patients with stroke. The present study was aimed at constructing a new bedside screening test for apraxia, called the Apraxia Screen of TULIA (AST), based on the comprehensive standardised Test for Upper-Limb Apraxia (TULIA). METHODS: First, an item-reduction analysis of the TULIA (48 gestures) was performed, based on the methods of classical test theory and on a larger sample of patients with stroke (n=133) and matched healthy controls (n=50). Stepwise elimination of items resulted in a set of 12 items, demonstrating high internal consistency (Cronbach alpha=0.92). The six-point scoring method of the TULIA was dichotomised to the score levels pass and fail. In the second part of this study the validity of the AST was assessed prospectively in a new cohort of patients with stroke (n=31) by using the Pearson correlation analysis and binary classification display with the TULIA. RESULTS AND DISCUSSION: Validation of the 12-item AST with the TULIA showed a remarkable diagnostic reliability with high specificity, sensitivity and positive predictive value, for the presence and severity of apraxia. The AST is shown to be a reliable and valid bedside test in patients with stroke, allowing a straightforward assessment of apraxia within a few minutes.

Effects of ideomotor apraxia on functional outcomes in patients with right hemiplegia.

The aim of this study was to investigate the effect of ideomotor apraxia on activities of daily living and to determine if the presence of apraxia interferes with rehabilitation. This study was conducted on 47 patients with right hemiplegia. All the patients were assessed at their admission and discharge, respectively, for apraxia by Ideomotor Apraxia Test, for daily living activities by Functional Independence Measure (FIM, Santa Clara Valley Medical Center, San Jose, California, USA), for cognitive functions by Mini Mental State Examination (MMSE), and for language components by Gulhane Aphasia Test (GAT). The effects of apraxia presence and time course on FIM, MMSE, and GAT scores were investigated. Presence of apraxia was found to have significant effect on all test scores (P<0.05). Time course had the main significant effect on FIM, MMSE, and GAT scores (P<0.05). Interaction effect of both presence of apraxia and time course on the test scores was not significant either. In other words, apraxic and nonapraxic patients seemed to gain benefits from the neurological rehabilitation. However, mean FIM scores of apraxic patients during discharge have failed to reach the mean FIM scores of nonapraxic patients during admission. Apraxia is considered as an important determinant in the dependence of patients with stroke in their activities of daily living. For this reason, during the initial assessment of patients with right hemiplegia, apraxia should be tested, and the presence of apraxia as well as its severity should be determined.

Ovarian failure in ataxia with oculomotor apraxia type 2.

Ataxia with oculomotor apraxia type 2 (AOA2) is an autosomal recessive disorder associated with mutations in the Senataxin (SETX) gene. Clinical manifestations (ataxia, peripheral neuropathy, oculomotor apraxia) of this disease have previously been limited to the nervous system. We describe a patient homozygous for a novel mutation of SETX who manifested not only ataxia but also ovarian failure.

Posture recognition in Alzheimer's disease.

BACKGROUND: Apraxia is neurologically induced deficit in the ability perform purposeful skilled movements. One of the most common forms is ideomotor apraxia (IMA) where spatial and temporal production errors are most prevalent. IMA can be associated Alzheimer's disease (AD), even early in its course, but is often not identified possibly because the evaluation of IMA by inexperienced judges using performance tests is unreliable. The purpose of this study, therefore, is to learn if the Postural Knowledge Test (PKT), a praxis discrimination test that assesses knowledge of transitive (PKT-T subtest) and intransitive (PKT-I subtest) postures and does not require extensive training, is as sensitive and specific as the praxis performance tests. METHODS: We studied 15 subjects with probable AD as well as 18 age-matched controls by having them perform transitive and intransitive gestures to command and imitation, as well as having them discriminate between correct and incorrect transitive and intransitive postures. RESULTS: Overall on all tests, the control subjects performed better than those with AD. In addition all subjects had more trouble with transitive than intransitive gestures. Using a stepwise discriminative analysis, 81.8% of the subjects could be classified according to Group (94.4% of Controls, 66.7% of AD subjects). In this analysis, the PKT-T (transitive posture subtest) was the only measure that contributed to the discrimination of subjects. CONCLUSION: We found that having subjects select the correct transitive hand postures in this "booklet test" was more sensitive than grading their praxis performances even when using judges with extensive training. This suggests that this discrimination test might be an excellent means for diagnosing and screening patients for AD. The reason why recognition of transitive postures is relatively more difficult for our AD subjects is not known. Two possibilities are that the representations for intransitive movements are stronger than those for transitive movements, and hence, more resistant to degradation, or that intransitive acts are stored in parts of the brain not affected by AD.

Ideational action impairments in Alzheimer's disease.

We report data from a group of patients with mild Alzheimer's disease on a range of tasks requiring either stored semantic knowledge about objects (e.g., naming object use) or the execution of action to objects (e.g., miming and using objects). We found that the patients were impaired at miming in response to objects, even when they could describe the object's function. On the other hand, copying gestures was not impaired relative to naming gestures, indicating that an ideomotor deficit in action execution, per se, was unlikely to explain the impairments in object use. We suggest instead that the patients had an impairment in stored motor programmes for action, over and above their deficits in semantic knowledge. Despite this, the patients were better at using than at miming to objects, consistent with the view that proprioceptive input (when using objects) can directly constrain selection of the appropriate motor programme for action.

Corticobasal syndrome with novel argyrophilic glial inclusions.

A 42-year-old, left-handed woman first noted impaired dexterity of the dominant hand, soon followed by dysarthria and cognitive decline. Over a 4-year period, she developed severe left-sided apraxia with eventual neglect of the left arm and progressive extrapyramidal signs. Cognitive testing showed progressive executive, visuospatial, fluency, and naming impairment with relative preservation of memory. Single-photon emission computed tomography demonstrated asymmetric right posterior frontal and superior parietal hypoperfusion. The clinical impression was corticobasal degeneration. At autopsy, severe atrophy was seen in the perirolandic and frontal regions. There was marked neuronal loss and gliosis in the posterior frontal and precentral regions and less severe pathology in prefrontal, temporal, and parietal areas. Mild to moderate gliosis and neuronal loss were also seen in the putamen, globus pallidus, subthalamic, and dentate nuclei. Gallyas silver stain revealed numerous inclusions adjacent to oligodendrocyte nuclei in white and gray matter of affected cortical and subcortical regions. The gracile inclusions were wavy, slender, and stained positively with antibodies to ubiquitin and alphaB-crystallin but not to microtubule-associated proteins (tau, MAP1B, MAP2), tubulin, neurofilaments, glial fibrillary acidic protein, or alpha-synuclein. The argyrophilic inclusions identified in this case are distinct from those previously described in neurodegenerative diseases.

Apraxia of speech: an overview.

Apraxia of speech (AOS) is a motor speech disorder that can occur in the absence of aphasia or dysarthria. AOS has been the subject of some controversy since the disorder was first named and described by Darley and his Mayo Clinic colleagues in the 1960s. A recent revival of interest in AOS is due in part to the fact that it is often the first symptom of neurodegenerative diseases, such as primary progressive aphasia and corticobasal degeneration. This article will provide a brief review of terminology associated with AOS, its clinical hallmarks and neuroanatomical correlates. Current models of motor programming will also be addressed as they relate to AOS and finally, typical treatment strategies used in rehabilitating the articulation and prosody deficits associated with AOS will be summarized.

Apraxia of lid opening: dose-dependent response to carbidopa-levodopa.

A 67-year-old woman with an 8-year history of Parkinson's disease and Lewy body dementia experienced difficulty in opening her eyelids (apraxia of lid opening [ALO]); she could close them without difficulty. This problem emerged 2 weeks after the patient's dosage of carbidopa 50 mg-levodopa 200 mg 3 times/day was decreased to twice/day. Two weeks after the onset of ALO the patient visited her physician, who suspected carbidopa-levodopa of causing the problem; the drug was discontinued. When the patient's condition worsened rather than improved, she was referred to a neuro-ophthalmologist, who confirmed the diagnosis of ALO. However, the neuro-ophthalmologist noted that this may not have been a true apraxia but rather a form of sustained blepharospasm that prevented the eyelid from opening. Carbidopa-levodopa was restarted, and her condition improved dramatically when her dosage was increased gradually to carbidopa 50 mg-levodopa 200 mg in the morning and at noon, and carbidopa 25 mg-levodopa 100 mg in the evening. Clinicians should be aware of adverse reactions, such as AOL, in patients taking carbidopa-levodopa who have dementia of the Lewy body type.

Corticobasal degeneration as a cognitive disorder.

The presence of cognitive impairment in corticobasal degeneration (CBD) is now widely recognised. Our review of the literature reveals that, although the pattern and severity of neuropsychological impairments can be highly variable across patients, several general trends can be identified. The most characteristic impairments are limb apraxia (usually ideomotor), constructional and visuospatial difficulties, acalculia, frontal dysfunction, and nonfluent aphasia. The limb apraxia is associated with deficits in drawing, copying, and handwriting, but there is emerging evidence that the problems with handwriting are not due exclusively to the apraxia. The findings with respect to episodic memory are more variable, but when there is impairment in this area, it tends to be milder than that seen in Alzheimer's disease. Semantic memory functioning appears relatively preserved but has been poorly studied. Problems with speech are common, and may be due to dysarthria or buccofacial apraxia. Aphasia, although initially considered rare, is in fact a common accompaniment of CBD, may be the presenting feature, and is typically nonfluent in type. More systematic investigation of the clinical and neuropathological overlap between progressive nonfluent aphasia (generally considered to be a form of frontotemporal dementia) and CBD is needed.

Ecological implications of ideomotor apraxia: evidence from physical activities of daily living.

OBJECTIVE: To learn if ideomotor apraxia (IMA) adversely influences skilled acts in the environment and interferes with independent functioning after stroke. METHODS: The relationship between IMA severity, based on scores from a verbal gesture-to-command (pantomime) task, and the dependency score, as defined by increased caregiver assistance on the Physical Self-Maintenance Scale (PSMS), was investigated in 10 unilateral left hemisphere-damaged stroke patients and 10 matched control subjects. RESULTS: There was a significant relationship between apraxia severity and dependency in physical functioning (PSMS). Impairment on the PSMS in the patients with IMA could not be accounted for based on overall cognitive impairment, poststroke depression, content-conceptual errors, elementary motor impairment, lesion size, or stroke-test interval. Analysis of categories composing the PSMS revealed that the patients with apraxia had increased dependency in grooming, bathing, and toileting relative to age-matched control subjects. CONCLUSIONS: These findings emphasize the ecological implications of apraxia and the need for rehabilitation strategies to improve the execution and efficiency of coordinated skilled movements in stroke patients with left hemisphere damage.

The neurological bases of apraxia of speech.

Which site(s) of brain damage are associated with apraxia of speech (AOS)? There appears to be little agreement. The article first considers some reasons why not. Even allowing for factors that may have influenced findings, a definitive answer to the question of the neurological bases of AOS is not currently possible. The article goes on to look briefly at developments in the field of motor control, and limb and buccofacial apraxia in particular, that may hold clues to an answer or at least to asking the right questions. In particular, if AOS is to be understood as a motor disorder, then models compatible with motor control and its neurophysiological underpinnings must be sought. Current models of motor control and apraxia stress the sensorimotor, distributed, interactive nature of control across multiple brain areas.

Psycholinguistic and motor theories of apraxia of speech.

This article sketches the relationships between modern conceptions of apraxia of speech (AOS) and current models of neuromotor and neurolinguistic disorders. The first section is devoted to neurophysiological perspectives of AOS, and its relation to dysarthrias and to limb apraxia is discussed. The second section introduces the logogen model and considers AOS in relation to supramodal aspects of aphasia. In the third section, AOS with the background of psycholinguistic models of spoken language production, including the Levelt model and connectionist models, is discussed. In the fourth section, the view of AOS as a disorder of speech motor programming is discussed against the background of theories from experimental psychology. The final section considers two models of speech motor control and their relation to AOS. The article discusses the strengths and weaknesses of these approaches.

Ability to learn inhaler technique in relation to cognitive scores and tests of praxis in old age.

Clinical observations have shown that some older patients are unable to learn to use a metered dose inhaler (MDI) despite having a normal abbreviated mental test (AMT) score, possibly because of dyspraxia or unrecognised cognitive impairment. Thirty inhaler-naive inpatients (age 76-94) with an AMT score of 8-10 (normal) were studied. Standard MDI training was given and the level of competence reached was scored (inhalation score). A separate observer performed the minimental test (MMT), Barthel index, geriatric depression score (GDS), ideational dyspraxia test (IDT), and ideomotor dyspraxia test (IMD). No correlative or threshold relationship was found between inhalation score and Barthel index, GDS, or IDT. However, a significant correlation was found between inhalation score and IMD (r = 0.45, p = 0.039) and MMT (r = 0.48, p = 0.032) and threshold effects emerged in that no subject with a MMT score of less than 23/30 had an inhalation score of 5/10 or more (adequate technique requires 6/10 or more), and all 17/18 with an inhalation score of 6/10 or more had an IMD of 14/20 or more. The three patients with a MMT >22 and inhalation score <6 had abnormal IMD scores. Inability to learn an adequate inhaler technique in subjects with a normal AMT score appears to be due to unrecognised cognitive impairment or dyspraxia. The MMT is probably a more useful screening test than the AMT score in this context.