1/13. Difficulty in writing Japanese semantic characters in a 9-year-old boy with williams syndrome. A 9-year-old boy diagnosed as having williams syndrome was evaluated using psychological test batteries in order to clarify his ability in language and visual cognition. The subject had difficulty in writing some of the Japanese semantic characters (called Kanji) which he could otherwise read and understand. Although he could write the small components of which the Kanji characters were composed, he could not locate these correctly. This phenomenon is considered to be very similar to the difficulty in copying a figure observed clinically. The Kaufmann Assessment Batteries for Children clearly revealed that the boy had difficulty with the sub-test of spatial memory compared to his average score for simultaneous processing. This result is considered to be closely related to the difficulty in copying figures or writing Kanji characters. On the illinois Test of Psycholinguistic Abilities, the present authors found that the subject's vocabulary was relatively good, although semantic and pragmatic problems remained. Clarifying the strong and weak points of the abilities of such patients will help to determine the most appropriate mode of education for them. ( info) |
2/13. Apraxia of lid opening is alleviated by pallidal stimulation in a patient with Parkinson's disease. Apraxia of lid opening (ALO) is a syndrome characterized by a non-paralytic inability to open the eyes at will in the absence of visible contraction of the orbicularis oculi muscle. Here we report that globus pallidus internus deep brain stimulation on the right side markedly alleviates ALO as well as gait freezing in a patient with Parkinson's disease. ( info) |
3/13. The role of the dynamic body schema in praxis: evidence from primary progressive apraxia. On an influential model of limb praxis, ideomotor apraxia results from damage to stored gesture representations or disconnection of representations from sensory input or motor output (Heilman & Gonzalez Rothi, 1993; Gonzalez Rothi et al., 1991). We report data from a patient with progressive ideomotor limb apraxia which cannot be readily accommodated by this model. The patient, BG, is profoundly impaired in gesturing to command, to sight of object, and to imitation, but gestures nearly normally with tool in hand and recognizes gestures relatively well. In addition, performance is profoundly impaired on imitation of meaningless gestures and on tasks requiring spatiomotor transformations of body-position information. We provide evidence that BG's apraxia is largely attributable to impairments external to the stored gesture system in procedures coding the dynamic positions of the body parts of self and others; that is, the body schema. We propose a model of a dynamic, interactive praxis system subserved by posterior parietal cortex in which stored representational elements, when present, provide "top-down" support to spatiomotor procedures computed on-line. In addition to accounting for BG's performance, this model accommodates a common pattern of ideomotor apraxia more readily than competing accounts. ( info) |
4/13. Corticobasal syndrome with tau pathology. Six cases with a clinical corticobasal syndrome (progressive asymmetric apraxia and parkinsonism unresponsive to levodopa) and tau pathology were selected from 97 brain donors with parkinsonism. Postmortem volumetric measures of regional brain atrophy (compared with age/sex-matched controls) were correlated with clinical features and the degree of underlying cortical and subcortical histopathology. At death, no significant asymmetry of pathology was detected. All cases had prominent bilateral atrophy of the precentral gyrus (reduced by 22-54%) with other cortical regions variably affected. Subcortical atrophy was less severe and variable. Two cases demonstrated widespread atrophy of basal ganglia structures (44-60% atrophy of the internal globus pallidus) and substantial subcortical pathology consistent with a diagnosis of progressive supranuclear palsy (PSP). The remaining four cases had typical pathology of corticobasal degeneration. In all cases, neuronal loss and gliosis corresponded with subcortical atrophy, while the density of cortical swollen neurons correlated with cortical volume loss. atrophy of the internal globus pallidus was associated with postural instability, while widespread basal ganglia histopathology was found in cases with gaze palsy. This study confirms the involvement of the precentral gyrus in the corticobasal syndrome and highlights the variable underlying pathology in these patients. ( info) |
5/13. Truncal and limb apraxia in corticobasal degeneration. We report a patient with probable corticobasal degeneration with prominent truncal apraxia, and review the literature. ( info) |
| We present two cases of progressive early-onset dementia with apraxia and visuospatial disability as initial manifestations. In the later stages of the illness Gerstmann's and Balint's syndromes developed. Structural neuroimaging demonstrated parieto-occipital atrophy and functional imaging revealed bilateral hypometabolism and hypoperfusion in these areas. These cases fulfil the diagnostic criteria of posterior cortical atrophy (PCA). frontal lobe involvement became evident as the disease progressed. Alzheimer's disease also typically features this anterior spread and possibly this is the underlying pathological substrate for this clinical syndrome, although definite pathology is lacking. In this report, we describe longitudinal evolution in these two cases of PCA. ( info) |
7/13. Apraxia of tool use: an autopsy case of biparietal infarction. Although disorders in the use of single objects have been reported, there have been few detailed analyses. We describe the autopsy case of a 65-year-old, right-handed male patient with severe impairment of actual tool use which was caused by biparietal infarctions. He persistently and stably showed a severely defective use of actual objects, single or multiple, and relatively well-preserved pantomimes of object use and intransitive gestures. He did not have aphasia or dementia, and his ability for tool naming and function description was completely preserved. The author identified similar patterns of errors about a tool-action(-target) relationship both in single-object use and multiple-object use. Lesion analysis showed cortical infarcts mainly located in the bilateral inferior parietal lobules, extending into the temporal lobe on the right side. ( info) |
8/13. Ideomotor and ideational apraxia in corticobasal degeneration: a case study. Corticobasal degeneration (CBD) is a progressive disorder that can be characterised by asymmetrical akinetic rigidity, involuntary movements, cortical sensory loss, alien limb syndrome and asymmetrical apraxia (Gibb et al., 1989; Rinnie et al., 1994). diagnosis of praxic disabilities is thought to be essential for distinguishing CBD, in its early stage, from other akinetic-rigid syndromes. However, the nature of apraxia in CBD, and the relations between ideomotor and ideational apraxia, are not well understood. For example, if there is an ideational deficit in a given patient, does this deficit occur independently of any ideomotor disorder, or are the two impairments linked in some manner? In the present paper we report a case study of a patient with apraxia due to CBD. We examine whether the disorder is confined to production tasks, or whether there is also a related deficit in recognising the correct actions performed with objects (an ideational deficit). We also evaluate whether a disorder found for action with single objects dissociates from the ability to link multiple actions into more complex, everyday tasks. The performance of our patient showed an impairment in both action production and action recognition system, suggesting a component of ideational as well as ideomotor apraxia in CBD. ( info) |
9/13. Pathological heterogeneity of clinically diagnosed corticobasal degeneration. Two patients fulfilling suggested clinical diagnostic criteria for corticobasal degeneration (CBD) are presented, who were found at postmortem to have alternative pathological diagnoses not suspected during life, namely, Alzheimer's disease and Pick's disease, respectively. The nosological position of these cases is considered in light of a literature review of previous reports of clinically diagnosed corticobasal degeneration with atypical (not corticobasal degeneration) pathology. Since such phenocopies may be common, we suggest that all clinically diagnosed cases of corticobasal degeneration should initially be labelled as "corticobasal degeneration syndrome" (CBDS) to emphasize that this is a diagnosis based on clinical phenotype, with the term corticobasal degeneration being reserved for the specific neuropathological phenotype, which itself may have a variety of clinical presentations. ( info) |
10/13. 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. ( info) |