1/16. "Apraxia of eyelid opening" induced by levodopa therapy and apomorphine in atypical parkinsonism (possible progressive supranuclear palsy): a case report.We report a female patient in whom so-called apraxia of eyelid opening (AEO) developed after the onset of possible progressive supranuclear palsy (national institute of neurological disorders and stroke criteria) and the introduction of antiparkinsonian medications including levodopa. Although parkinsonian symptoms responded poorly to levodopa, AEO worsened after increasing levodopa dosage and disappeared when levodopa was discontinued. Later, a dose of apomorphine widely accepted for acute tests had no significant effect on limb motor activity but induced AEO. overall, these observations are grounds for thinking that AEO developing in the course of parkinsonism may be either disease- or drug-related. The possibility of manipulating dopaminergic treatment should always be considered when dealing with AEO associated with parkinsonism.- - - - - - - - - - ranking = 1keywords = progressive supranuclear, supranuclear, supranuclear palsy, palsy (Clic here for more details about this article) |
2/16. Apraxia of eyelid closure in Huntington's disease.We report a patient with genetically confirmed Huntington's disease (HD) presenting apraxia of eyelid closure (AEC). She was unable to close her eyes at command but was able to blink. chorea and AEC ameliorated significantly during treatment with olanzapine and riluzole, an inhibitor of glutamate release. AEC is reported in progressive supranuclear palsy, Creutzfeldt-Jakob's disease, amyotrophic lateral sclerosis, and as post-stroke AEC. No report on HD is available so far, although oculomotor disturbances are quite common in this disease.- - - - - - - - - - ranking = 0.2keywords = progressive supranuclear, supranuclear, supranuclear palsy, palsy (Clic here for more details about this article) |
3/16. Impaired volitional closure of the left eyelid after right anterior cerebral artery infarction: apraxia due to interhemispheric disconnection?BACKGROUND: The inability of volitional unilateral eyelid closure is an uncommon symptom of a central nervous system disorder. When it occurs as the result of a localized brain lesion, it is debated to be a form of supranuclear facial palsy or an apraxic phenomenon. OBJECTIVES: To report and discuss a unilateral (left-sided) higher-order movement disorder of the facial periocular musculature bearing apraxic features. SETTING: University neurology department. PATIENT: A 78-year-old right-handed man was admitted to the hospital with a left-sided brachiofacial hemiparesis of sudden onset. After thrombolysis with intravenous recombinant tissue-type plasminogen activator, the hemiparesis, including the left-sided facial weakness, disappeared. Serial computed tomographic scans showed that the patient was left with a stroke in the right anterior cerebral artery territory, affecting the frontal commissural fibers of the corpus callosum. There were no signs of upper motor neuron facial paresis on the left side when gesturing in a natural context. Eyelid closure was complete during sleep. However, left eyelid closure and elevation of the left eyebrow were not possible on verbal command. In contrast, voluntary innervation of the perioral facial musculature was performed properly. CONCLUSIONS: The voluntary-automatic dissociation of our patient's eyelid closure was suggestive of an apraxic disorder. Disconnection from a praxis center caused by callosal damage may be assumed to be the underlying cause. The unilaterality of the symptom might imply that in a bilaterally organized corticonuclear system such as upper face innervation, it is the crossing fibers that are primarily involved in praxis tasks.- - - - - - - - - - ranking = 0.077868499147995keywords = supranuclear, palsy (Clic here for more details about this article) |
4/16. Atypical progressive supranuclear palsy underlying progressive apraxia of speech and nonfluent aphasia.Progressive supranuclear palsy (PSP) is a clinicopathological entity typically presenting as an akinetic rigid syndrome with early falls, axial rigidity, vertical supranuclear gaze palsy and levodopa resistance. Pathological features consist of tau deposition in neuronal and glial cells located mainly in subcortical and brainstem structures. Rare cases with the pathological diagnosis of atypical PSP have been described in which neocortical tau deposition is more widespread than what is usually seen in typical PSP. Progressive nonfluent aphasia (PNFA) is a syndrome characterized by spontaneous nonfluent speech and early preserved comprehension of language. Apraxia of speech (AOS) is a motor speech disorder that may be a feature of PNFA. We report the clinical and pathological findings of four cases that presented with features most consistent with PNFA predominated by AOS. Pathological features in these four cases included the typical features of PSP subcortically and in brainstem structures, but combined with tau-positive neuronal and glial pathology in the neocortex.Comprehensive semiquantitative analyses of tau burden including neurofibrillary tangles and pretangles, coiled bodies, tufted astrocytes and threads were undertaken in the four cases of atypical PSP and compared to 10 cases of typical PSP. Semiquantitative analysis demonstrated that in atypical PSP, the pathology shifts from subcortical grey and brainstem regions, commonly affected in typical PSP, towards neocortical regions. This shift in pathology accounts for the presentation of PNFA and AOS observed in our patients, as well as the lack of classic features of PSP. These cases demonstrate that atypical PSP can present as AOS and PNFA without the classic features of PSP.- - - - - - - - - - ranking = 0.96996592178507keywords = progressive supranuclear, supranuclear, supranuclear palsy, palsy (Clic here for more details about this article) |
5/16. Interactions between volitional and automatic breathing during respiratory apraxia.The sites and forms of interactions between voluntary breathing acts and automatic respiratory rhythm generation are the subject of considerable research interest. We report here observations of the control of breathing in a patient suffering from an advanced form of progressive supranuclear palsy (Steele-Richardson-Olszewski syndrome). This patient demonstrated a severely compromised ability to perform volitional respiratory acts upon command, despite exacerbated behavioural and automatic control of respiration. The presence of residual volitional control of breathing in this patient provided interesting insights concerning the interaction between the automatic and the voluntary control of respiration. We observed that (1) when the subjects was asked to inspire voluntarily he could at best mobilize a volume similar to spontaneous VT and only very slowly; (2) automatic breathing movements persisted, superimposed onto the active voluntary movements, with an amplitude that decreased when the inspiratory activity, albeit weak, reached its maximal level; (3) during breath holding both the amplitude and the frequency of the basal spontaneous rhythmic activity were depressed. This observation therefore supports the idea of a strong interaction between volitional and automatic breathing in the form of an inhibition of automatic activity during voluntary breathing. Although, the site of interaction (spinal versus supraspinal) could not be determined during volitional inspiration, the effect of breath holding on the frequency of the spontaneous breathing activity supports the view that a volitional breathing arrest has some inhibitory effects on the respiratory oscillator at the medullary level. Finally, in an attempt to reconcile the persistence of a rhythmic activity during voluntary inspiration and expiration with previous data from the literature, it is proposed that the normal suppression of the automatic activity during voluntary inspiration relies on cortical and sub-cortical structures involved in the planning, i.e. the praxic component, of a respiratory task rather than on projections originating from the primary motor cortex.- - - - - - - - - - ranking = 0.2keywords = progressive supranuclear, supranuclear, supranuclear palsy, palsy (Clic here for more details about this article) |
6/16. Progressive oculo-orofacial-speech apraxia (POOSA).A loss of speech can be related to disorders of the motor units (paresis), language deficits (aphasia), or speech programming deficits (apraxia of speech). Although apraxia of speech has been reported to be associated with degenerative diseases, we observed a patient with a unique constellation of signs that included apraxia of speech, oculo-orofacial apraxia and a supranuclear ophthalmoplegia in the absence of extrapyramidal (Parkinsonian) signs. Post-mortem examination revealed a loss of neurons in the frontal and temporal regions, but there was also a marked loss of neurons and astrogliosis in the caudate, claustrum, globus pallidus, substantia nigra, and loss of axons in the anterior cerebral peduncles. This patient's clinical presentation and the pathological correlates suggest that he might have suffered with a distinct disorder we call progressive oculo-orofacial-speech apraxia or POOSA.- - - - - - - - - - ranking = 0.077856798510949keywords = supranuclear (Clic here for more details about this article) |
7/16. Ocular motor apraxia following cardiac surgery.Ocular motor apraxia is characterized by a deficiency in the generation of voluntary saccadic eye movements despite the presence of spontaneous saccades. We report two cases of isolated ocular motor apraxia, both in children, that developed following cardiac surgery. Electro-oculography was done in one case and showed slow saccades and decreased pursuit gain. The authors review the neuroanatomic and neurophysiologic features relevant to supranuclear gaze mechanisms.- - - - - - - - - - ranking = 0.077856798510949keywords = supranuclear (Clic here for more details about this article) |
8/16. Corticobasal degeneration. A unique pattern of regional cortical oxygen hypometabolism and striatal fluorodopa uptake demonstrated by positron emission tomography.Corticobasal degeneration presents with an asymmetric akinetic-rigid syndrome, apraxia and combinations of supranuclear gaze palsy, myoclonus, and an alien limb. Six patients aged 59-77 yrs, diagnosed on clinical criteria as having corticobasal degeneration, have been studied with positron emission tomography using tracers of dopamine storage capacity and oxygen metabolism. Striatal 18F-6-fluorodopa uptake was reduced in an asymmetric pattern, caudate and putamen being involved in all cases. Uptake into medial frontal cortex was also impaired. Regional cortical oxygen metabolism was most significantly depressed in the superior and posterior temporal, inferior parietal, and occipital associated cortices. Within the frontal lobe, the hypometabolism was chiefly posterior. This unique combination of regional hypometabolism and disruption of the nigrostriatal system is discussed in relation to the clinical features of the disease and is compared with reported findings in other disorders of cognition and movement.- - - - - - - - - - ranking = 0.077868499147995keywords = supranuclear, palsy (Clic here for more details about this article) |
9/16. Parkinsonism and extraocular motor abnormalities with unusual neuropathological findings.Parkinsonian patients with ocular motility abnormalities are usually considered to have progressive supranuclear palsy. However, a number of other conditions have been noted to have the combination of parkinsonism and ocular problems. We report a case of rigid akinetic parkinsonism, oculomotor palsy, and eyelid apraxia with postmortem examination. Our findings are unusual in that there was marked gliosis of the substantia nigra with a large amount of free extracellular neuromelanin despite a 3-year clinical course. Only rare hyaline inclusion bodies and no neurofibrillary tangles were seen in the brainstem. Excessive calcification of the vessels of the globus pallidus were also noted. This case represents another example of the diversity of conditions producing parkinsonism with extraocular motor abnormalities.- - - - - - - - - - ranking = 0.20001170063705keywords = progressive supranuclear, supranuclear, supranuclear palsy, palsy (Clic here for more details about this article) |
10/16. Congenital vertical ocular motor apraxia.The case of a 4 1/2-year-old boy with congenital vertical ocular motor apraxia who was otherwise developmentally and neurologically normal is reported. The presence of perinatal hypoxia in this patient may have been etiologic. While the presence of a supranuclear vertical ocular motor abnormality usually suggests a serious, acquired neurologic or systemic disease it may rarely occur as an isolated congenital finding, as demonstrated in this case.- - - - - - - - - - ranking = 0.077856798510949keywords = supranuclear (Clic here for more details about this article) |
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