1/16. A kinematic study of progressive apraxia with and without dementia.BACKGROUND: Prehension is an ideationally simple, cued movement requiring proximal (transport) and distal (manipulation) limb control. patients with this syndrome of progressive apraxia are unable to perform many activities of daily living that require prehension. There is little known about how this syndrome kinematically disrupts such movements or whether concurrent dementia might play a critical role. OBJECTIVES: Using prehension as a paradigm for an ideationally simple, cued functional movement, we sought to (1) characterize the kinematic features of progressive apraxia in general, and (2) contrast the kinematic differences between apraxic patients with and without dementia. methods: Eight patients with the syndrome of progressive apraxia (including five without dementia, one of whom had autopsy-confirmed corticobasal ganglionic degeneration, and three with dementia, one of whom had autopsy-confirmed Alzheimer's disease) were compared with eight age-matched normal control subjects on a prehension task using an Optotrak camera system. RESULTS: Compared with control subjects, apraxic subjects had slowed reaction time, slowed transport and manipulation kinematics, greater lateral deviation from the linear prehension trajectory, greater intermanual asymmetry, motor programming disturbances, and mild transport-manipulation uncoupling. There were minor differences between the apraxia subgroups such as greater intermanual differences and impaired grip aperture velocity in the nondemented group, and overall slower movement in the demented group. CONCLUSIONS: There are major kinematic differences between apraxic and control subjects on a prehension task. The differences between clinical-pathologic subgroups are more subtle, and the movement disorder itself rather than concurrent dementia is the greatest determinant of motor disability.- - - - - - - - - - ranking = 1keywords = basal ganglion, ganglion (Clic here for more details about this article) |
2/16. Neuropathology of Joubert syndrome.Very little documentation of the neuropathologic changes in Joubert syndrome exists. This paper presents a detailed postmortem neuropathologic study of a clinically and radiographically well-documented case of Joubert syndrome. In addition to aplasia of the cerebellar vermis and fragmentation of the dentate nuclei, there was marked dysplasia of structures at the pontomesencephalic junction and caudal medulla. There was abnormal decussation of the superior cerebellar peduncles and an enlarged iter (rostral 4th ventricle) with elongated tegmental nuclei (including the locus coeruleus). neurons of the basis pontis and reticular formation appeared reduced. Extensive malformations of the medulla included hypoplasia of the inferior olivary nuclei, solitary nuclei and tracts, and the nucleus and spinal tracts of trigeminal nerve (cranial nerve V). Even more striking was dysplasia of the caudal medulla at the cervicomedullary junction, which was characterized by the absence of a posterior median sulcus, neuronal swelling and axonal spheroids in the region of malformed nuclei gracilis and cuneatus, and absence of pyramidal decussation. This study suggests that, in addition to vermal agenesis, Joubert syndrome is characterized by malformation of multiple brainstem structures. The latter could explain certain clinical features of the syndrome, including episodic hyperpnea and oculomotor apraxia.- - - - - - - - - - ranking = 0.088576717285528keywords = nucleus (Clic here for more details about this article) |
3/16. Expressive language disorder after infarction of left lentiform nucleus.A 53 year old bilingual woman presented with apraxia of speech and writing in English and German after ischaemic infarction of the left posterior lentiform nucleus. Detailed language assessment revealed impairments of articulation, verbal fluency, auditory repetition, interpretation of complex semantic relationships, formulation of definitions and verbal short-term memory. The case illustrates the role of the basal ganglia in speech planning, word retrieval and verbal short-term memory.- - - - - - - - - - ranking = 0.44288358642764keywords = nucleus (Clic here for more details about this article) |
4/16. Cortical-basal ganglionic degeneration: a clinical, functional and cognitive evaluation (1-year follow-up).We decided to evaluate a patient who was diagnosed with cortical-basal ganglionic degeneration from a clinical, instrumental and neuropsychological perspective. Our aim was to employ a new instrumental tool, functional magnetic resonance, in order to evaluate his cortical damage. We then followed the pathological course for 1 year and tested the patient again: we discuss the results of our evaluation, having an overview of the literature on the topic. In particular, we focused our attention on his apraxia, trying to suggest a dynamic and anatomical model to guarantee a possible explanation of his behavior.- - - - - - - - - - ranking = 5keywords = basal ganglion, ganglion (Clic here for more details about this article) |
5/16. Transient crossed aphasia and persistent amnesia after right thalamic haemorrhage.A 45-year-old right-handed woman suffered transient aphasia and persistent amnesia after a right thalamic haemorrhage. This patient appeared to have crossed aphasia, although it disappeared within 8 weeks. It is noteworthy that the patient had a unilateral right thalamic lesion but exhibited both verbal and non-verbal memory impairment. Computed tomography and magnetic resonance imaging revealed cerebral haemorrhage in the right thalamus involving the ventral anterior nucleus, medioventral nucleus, mamillothalamic tract, internal medullary lamina, and mediodorsal nucleus. An amytal test was performed and suggested that the right hemisphere was dominant for language functions and the left hemisphere was dominant for visuospatial functions. Single photon emission CT revealed a low perfusion area only in the right thalamus. These findings suggest that the right hemisphere might be dominant for both verbal and non-verbal memory function in this patient, although visuospatial function was lateralized in the left hemisphere.- - - - - - - - - - ranking = 0.26573015185659keywords = nucleus (Clic here for more details about this article) |
6/16. Cortico-basal ganglionic degeneration. A case report.The case of a Brazilian patient with cortico-basal ganglionic degeneration (CBGD) is presented. Since three years ago, a 71-year old male displays asymmetric ideomotor apraxia, gait apraxia, cortical sensory impairment, myoclonus, limp dystonia and rigidity. His mental status is spared. There is neither consanguinity nor similar cases in his family. The differential diagnosis of CBGD is discussed. A brief review of the literature is made stressing the clinical and pathological features of CBGD. This disease is poorly known and probably underdiagnosed. Its diagnosis can be safely made based on clinical grounds.- - - - - - - - - - ranking = 5keywords = basal ganglion, ganglion (Clic here for more details about this article) |
7/16. Improvement of apraxia of lid opening by STN-stimulation in a 70-year-old patient with Parkinson's disease. A case report.We report on a 70-year-old female patient with Parkinson's disease, who showed an improvement of a preexisting apraxia of lid opening on electrical impulses, so-called deep brain stimulation (DBS) delivered to the subthalamic nucleus (STN). This was not described by any other authors before. Up to now, the appearance of apraxia of lid opening was observed only as a side effect after deep brain stimulation in the nucleus subthalamicus. We suggest that these differences may be due to the region of the nucleus subthalamicus that is influenced by the stimulation.- - - - - - - - - - ranking = 0.26573015185659keywords = nucleus (Clic here for more details about this article) |
8/16. The metabolic landscape of cortico-basal ganglionic degeneration: regional asymmetries studied with positron emission tomography.Regional metabolic rate for glucose (rCMRGlc) was estimated using [18F]fluorodeoxyglucose (FDG) and positron emission tomography (PET) in five patients (four men, one woman; mean age 68; mean disease duration 2.4 years) with clinical findings consistent with the syndrome of cortico-basal ganglionic degeneration (CBGD). Left-right rCMRGlc asymmetry, (L-R)/(L R) x 100, was calculated for 13 grey matter regions and compared with regional metabolic data from 18 normal volunteers and nine patients with asymmetrical Parkinson's disease (PD). In the CBGD group mean metabolic asymmetry values in the thalamus, inferior parietal lobule and hippocampus were greater than those measured in normal control subjects and patients with asymmetrical PD (p less than 0.02). parietal lobe asymmetry of 5% or more was evident in all CBGD patients, whereas in PD patients and normal controls, all regional asymmetry measures were less than 5% in absolute value. Measures of frontal, parietal and hemispheric metabolic asymmetry were found to be positively correlated with asymmetries in thalamic rCMRGlc (p less than 0.05). The presence of cortico-thalamic metabolic asymmetry is consistent with the focal neuropathological changes reported in CBGD brains. Our findings suggest that metabolic asymmetries detected with FDG/PET may support a diagnosis of CBGD in life.- - - - - - - - - - ranking = 5keywords = basal ganglion, ganglion (Clic here for more details about this article) |
9/16. Cortical Lewy body dementia: clinical features and classification.Seven patients, aged 65-72 years, are described with dementia and cortical lewy bodies. In one patient a Parkinsonian syndrome was followed by dementia and motor neuron disease. In the remaining six patients dementia was accompanied by dysphasia, dyspraxia and agnosia. One developed a Parkinsonian syndrome before the dementia, in three cases a Parkinsonian syndrome occurred later, and in two cases not at all. All patients showed lewy bodies and cell loss in the substantia nigra, locus coeruleus and dorsal vagal nucleus, as in Parkinson's disease. The severity of cell loss in the nucleus basalis varied from mild to severe. lewy bodies were also present in the parahippocampus and cerebral cortex, but Alzheimer-type pathology was mild or absent, and insufficient for a diagnosis of Alzheimer's disease. patients with moderate or severe dementia, some with temporal or parietal features, may have cortical lewy body disease, Alzheimer's disease, or a combination of the two. Cortical lewy body disease may be associated with dementia in Parkinson's disease more often than realised, but is not necessarily associated with extensive Alzheimer pathology.- - - - - - - - - - ranking = 0.17715343457106keywords = nucleus (Clic here for more details about this article) |
10/16. aluminum accumulation in tangle-bearing neurons of Alzheimer's disease with Balint's syndrome in a long-term aluminum refiner.Progressive dementia had developed since the age of 55 in a male patient, who had worked as an aluminum refiner for 30 years. Balint's syndrome was observed from at 60 and he died of bronchopneumonia at 65. Neuropathological examination revealed characteristic features of Alzheimer's disease with marked atrophy of the occipito-temporal lobes and senile plaques of the cerebellum. Wavelength-dispersive X-ray microanalysis disclosed focal aluminum accumulation within the nucleus and cytoplasm of the tangle-bearing neurons. Microanalysis by the same method failed to disclose aluminum accumulation in the tangle-bearing neurons in a case of senile dementia of Alzheimer's type who had no possible inhalation of aluminum.- - - - - - - - - - ranking = 0.088576717285528keywords = nucleus (Clic here for more details about this article) |
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