1/25. Parietal cheiro-oral syndrome.Cheiro-oral syndrome due to a parietal lesion has been reported in conjuction with a brain tumor, infarction and migraine. Only six reports of cheiro-oral syndrome due to a parietal infarction have been reported to date. We treated a 45-year-old woman with cheiro-oral syndrome due to a parietal infarction. Her sensory disturbance was characterized by paresthesia in the lower face and hand on the left side, and severe involvement of stereognosis and graphesthesia in the left hand. The pathogenesis of parietal cheiro-oral syndrome is discussed.- - - - - - - - - - ranking = 1keywords = hand (Clic here for more details about this article) |
2/25. Subcortical anarthria: a case report.A 56-year-old right-handed male with a history of hypertension and diabetes presented two episodes of stroke: The first affected territory was the left anterior coroidal artery (capsular and paracapsular infarcts at the level of the genu and posterior arm of the internal capsule) and the second was the right thalamus, due to a hematoma. Following the first stroke, the patient developed severe dysarthria and after the second stroke remained anarthric. The pathophysiology of the disorder is discussed, and the role of the left and right thalamus as far as speech is concerned is reviewed.- - - - - - - - - - ranking = 0.5keywords = hand (Clic here for more details about this article) |
3/25. Pure anarthria with predominantly sequencing errors in phoneme articulation: a case report.A 77-year-old left-handed man presented with pure anarthria following cerebral infarction. The lesion was restricted to the right precentral gyrus extending to the immediately underlying subcortical white matter and the frontal part of the insular cortex. Qualitative analysis of anarthria revealed that half of the phonemic-articulatory errors in spontaneous speech were sequencing ones. Sequential errors were detected at the phoneme level in both consonants and vowels, and at the syllable level. Most of the sequential errors were pre-positioning. Sequential errors were observed both within and across words. In clear contrast with anarthria, writing and comprehension was preserved, which suggested the problem was limited to oral expression. Our findings provide further support that the precentral gyrus and/or the insular cortex of the language dominant hemisphere is responsible for the temporal sequencing of the articulatory programming.- - - - - - - - - - ranking = 0.5keywords = hand (Clic here for more details about this article) |
4/25. A 15-year-old boy with central nervous system vasculopathy presenting with dysarthria-clumsy hand syndrome.dysarthria-clumsy hand stroke syndrome has been described frequently in adults but not in children. We report a 15-year-old right-handed boy with sudden onset of dysarthria, dysphagia, right facial weakness, and mild right-hand clumsiness. Computed tomographic scan and magnetic resonance imaging demonstrated infarction in the genu and posterior limb of the left internal capsule. magnetic resonance angiography and conventional angiography demonstrated stenosis of the supraclinoid portion of the left internal carotid artery and the origin of the left ophthalmic artery. Lacunar infarction in an older adult is not the only mechanism leading to dysarthria-clumsy hand syndrome.- - - - - - - - - - ranking = 36316.153338615keywords = hand syndrome, hand (Clic here for more details about this article) |
5/25. Choreoathetosis related to lithium intoxication.A 76-year old woman was admitted because of severe gait ataxia and dysarthric speech that had worsened over the last 24 h. The patient was initially suspected of having repeated transitory ischemic attacks (TIAs) as her daughter reported a similar episode that had happened 3 weeks prior to admission. The onset of spontaneous twisting, choreoathetotic movements of both hands and arms and worsening of symptoms one hour after admission together with a history of lithium therapy lead to the correct diagnosis and appropriate treatment.- - - - - - - - - - ranking = 0.5keywords = hand (Clic here for more details about this article) |
6/25. Cerebellar agenesis: clinical, neuropsychological and MR findings.Cases of cerebellar agenesis are rare. The degree of motor impairment is a matter of discussion. It has been claimed that normal motor function can be observed. Detailed descriptions of neurological findings, however, are lacking. Neuropsychological testing in cerebellar agenesis is of additional interest based on recent findings of impaired non-motor functions in cerebellar disease. The case of an elderly woman with cerebellar agenesis is presented. 3D-MR imaging was used to confirm the diagnosis. Neurological and neuropsychological examination was performed including video documentation (see the authors' own website). To assess deficits of motor learning eyeblink conditioning was investigated. Neurological examination revealed mild to moderate signs of cerebellar dysarthria, upper and lower limb ataxia and ataxia of stance and gait. Motor learning was affected as shown by inability to acquire conditioned eyeblink responses. In addition, neuropsychological testing disclosed mild to moderate deficits in IQ, planning behavior, visuospatial abilities, memory and attention. cerebellar ataxia, although clearly present, was less than one would expect in almost complete absence of the cerebellum. Neuropsychological deficits, on the other hand, appeared to be more marked than one would expect in cerebellar disease. No conclusion, however, could be drawn whether impaired cognitive development and neuropsychological test performance were directly related to lack of cerebellar function, or caused by impaired motor development and performance.- - - - - - - - - - ranking = 0.5keywords = hand (Clic here for more details about this article) |
7/25. The human basis pontis: motor syndromes and topographic organization.Clinical-anatomic correlations were performed in 25 patients with focal infarcts in the basilar pons to determine whether pontine lacunar syndromes conform to discrete clinical entities, and whether there is topographic organization of the motor system within the human basis pontis. Twelve clinical signs were scored on a 6-point scale, neuroimaging lesions were mapped and defined with statistical certainty, and structure-function correlation was performed to develop a topographic map of motor function. Clinical findings ranged from major devastation following extensive lesions (pure motor hemiplegia) to incomplete basilar pontine syndrome and restricted deficits after small focal lesions (ataxic hemiparesis, dysarthria-clumsy hand syndrome, dysarthria-dysmetria and dysarthria-facial paresis). The syndromes are not absolutely discrete, and are distinguished from each other by the relative degree of involvement of each clinical feature. Structure-function correlations indicate that strength is conveyed by the corticofugal fibres destined for the spinal cord, whereas dysmetria results from lesions involving the neurons of the basilar pons that link the ipsilateral cerebral cortex with the contralateral cerebellar hemisphere. Facial movement and articulation are localized to rostral and medial basilar pons; hand coordination is medial and ventral in rostral and mid-pons; and arm function is represented ventral and lateral to the hand. Leg coordination is in the caudal half of the pons, with lateral predominance. Swallowing is dependent upon the integrity of a number of regions in the rostral pons. Gait is in medial and lateral locations throughout the rostral- caudal extent of the pons. Dysmetria ipsilateral to the lesion constitutes a disconnection syndrome, as it occurs when the hemipontine lesion is extensive and interrupts pontocerebellar fibres traversing from the opposite, intact side of the pons. The heterogeneity of manifestations reflects the well-organized topography of motor function in the human basis pontis, in agreement with the anatomic organization of the motor corticopontine projections in the monkey. Higher order impairments including motor neglect, paraphasic errors and pathological laughter result from rostral and medial pontine lesions, and may result from disruption of the pontine component of associative corticopontocerebellar circuits.- - - - - - - - - - ranking = 7263.9306677231keywords = hand syndrome, hand (Clic here for more details about this article) |
8/25. Bilateral subthalamic nucleus deep brain stimulation in a patient with cervical dystonia and essential tremor.The role of subthalamic nucleus (STN) deep brain stimulation (DBS) is well established in Parkinson's disease, but experience with STN DBS in other movement disorders is limited. We report on a patient with medically refractory cervical dystonia and essential tremor resulting in dystonic head tremor and action tremor of the hands who obtained complete tremor suppression and near resolution of her cervical dystonia with bilateral STN stimulation. The results in this case demonstrate that STN DBS can dramatically improve dystonia and tremor in nonparkinsonian movement disorders.- - - - - - - - - - ranking = 0.5keywords = hand (Clic here for more details about this article) |
9/25. 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.- - - - - - - - - - ranking = 1keywords = hand (Clic here for more details about this article) |
10/25. Spastic paraplegia, optic atrophy, and neuropathy is linked to chromosome 11q13.We report an autosomal recessive neurodegenerative disorder in 25 white members from a large inbred Brazilian family, 22 of whom were evaluated clinically. This condition is characterized by (1) subnormal vision secondary to apparently nonprogressive congenital optic atrophy; (2) onset of progressive spastic paraplegia in infancy; (3) onset of progressive motor and sensory axonal neuropathy in late childhood/early adolescence; (4) dysarthria starting in the third decade of life; (5) exacerbated acoustic startle response; and (6) progressive joint contractures and spine deformities. Motor handicap was severe, and all patients were wheelchair bound after 15 years old. We performed a genome-wide screen including 25 affected individuals and 49 of their unaffected relatives. Linkage was detected at 11q13 region with a maximum logarithm of odds score of 14.43, obtained with marker D11S1883. The candidate region, which lies between D11S1908 and D11S1889, encompasses approximately 4.8Mb and has more than 100 genes and expressed sequences. We propose the acronym SPOAN (spastic paraplegia, optic atrophy, and neuropathy) for this complex syndrome.- - - - - - - - - - ranking = 0.5keywords = hand (Clic here for more details about this article) |
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