1/19. magnetic resonance imaging findings in corticobasal degeneration.Two women (patient 1, 77 years old, and patient 2, 63 years old) with strong clinical evidence for corticobasal degeneration (CBD) are presented. Patient 2 was in an early stage of the disease with only a mild disability of her left hand. In addition to the clinical characteristics, both patients presented the typical cortical reflex myoclonus. magnetic resonance imaging studies for both patients revealed nearly identical hyperintense lesions somatotopic from the left-hand primary motor cortex (M1), extending to the midline and possibly supplementary motor area (SMA) in patient 2. To our knowledge, this has not been previously described in patients with CBD. These lesions may play a role in the etiology and the development of CBD with involvement of the M1 and may correspond to the underlying pathology of demyelination or gliosis.- - - - - - - - - - ranking = 1keywords = hand (Clic here for more details about this article) |
2/19. Problems and pitfalls in the diagnosis of ALS.Although misdiagnosis of amyotrophic lateral sclerosis (ALS) is rare, it may be more difficult to make a diagnosis in some groups of patients than in others. If a patient presents in the later stages of the disease, only a small number of alternative diagnoses need to be considered. These include spinal muscular atrophies of adult onset, inclusion body myositis and motor neuropathies with conduction block. The latter group in particular may present a serious diagnostic problem, as several groups have recently reported patients suffering from lower motor neuron syndrome without detectable conduction block, who responded unexpectedly to treatment with immunoglobulins. As recent laboratory results suggest that a lengthy pre-clinical period may precede clinical ALS, there is increased pressure for clinicians to make an early diagnosis so that the maximum effect can be achieved from neuroprotective drugs. Thus, diseases such as distal motor amyotrophies, pressure palsies of motor branches of hand nerves, and cervical myelopathies, which can be differentiated mainly by their time-course, may be relevant in the differential diagnosis of ALS in some patients. During recent years, a few patients have been seen in our clinic who presented with pure motor deficits but later developed a more complex pattern of vulnerability suggestive of multisystem degeneration. The existence of patients with a disease that borders the spectrum of motor neuron diseases cannot be disputed. These patients include those carrying the Huntington mutation and those suffering from guam and new guinea disease ('ALS/PD'). From our experience, however, these 'difficult' diagnoses represent less than 10% of the patients seen in our clinic.- - - - - - - - - - ranking = 0.5keywords = hand (Clic here for more details about this article) |
3/19. Pallido-Luysio-Nigral atrophy revealed by rapidly progressive hemidystonia: a clinical, radiologic, functional, and neuropathologic study.Pallido-luysio-nigral atrophy (PLNA) is a rare neurodegenerative disease in which the clinical and radiologic correlates have not yet been clearly established. A 62-year-old man insidiously developed dystonic postures, choreoathetoid movements, slowness, and stiffness, which initially affected the right hand and foot and progressively spread to the entire right side. T2-weighted magnetic resonance imaging showed increased signal intensity in both left and right medial pallida and in the left substantia nigra. Tests using HMPAO-SPECT and FDG-PET demonstrated left cortical hyperperfusion and hypermetabolism, whereas the left lenticular nucleus was slightly hypometabolic. At age 65, abnormal movements and postures involved all four limbs and the axis causing major gait disturbances, and facial and bulbar muscles atrophied resulting in dysarthria, dysphagia, and impaired breathing. Diffuse amyotrophy and fasciculations also appeared. death occurred at age 66, 4 years after onset. At autopsy, severe bilateral neuronal loss and gliosis restricted to the pallidum, the subthalamic nucleus, the substantia nigra, and the hypoglossal nucleus were noted, accounting for the diagnosis of PLNA with lower motor neuron involvement. Progressive hemidystonia with adult onset represents an unusual clinical presentation for this disorder. Moreover, this observation indicates that a diagnosis of PLNA should be considered for specific magnetic resonance imaging, SPECT, and/or PET data, and suggests that in PLNA, pallidal dysfunction might play a key role in the dystonic presentation.- - - - - - - - - - ranking = 0.5keywords = hand (Clic here for more details about this article) |
4/19. Progressive infantile neurodegeneration caused by 2-methyl-3-hydroxybutyryl-CoA dehydrogenase deficiency: a novel inborn error of branched-chain fatty acid and isoleucine metabolism.We report a novel inborn error of metabolism identified in a child with an unusual neurodegenerative disease. The male patient was born at term and recovered well from a postnatal episode of metabolic decompensation and lactic acidosis. Psychomotor development in the first year of life was only moderately delayed. After 14 mo of age, there was progressive loss of mental and motor skills; at 2 years of age, he was severely retarded with marked restlessness, choreoathetoid movements, absence of directed hand movements, marked hypotonia and little reaction to external stimuli. Notable laboratory findings included marked elevations of urinary 2-methyl-3-hydroxybutyrate and tiglylglycine without elevation of 2-methylacetoacetate, mild elevations of lactate in CSF and blood, and a slightly abnormal acylcarnitine profile. These abnormalities became more apparent after isoleucine challenge. Enzyme studies showed absent activity of 2-methyl-3-hydroxybutyryl-CoA dehydrogenase (MHBD) in the mitochondrial oxidation of 2-methyl branched-chain fatty acids and isoleucine. Under dietary isoleucine restriction, neurologic symptoms stabilized over the next 7 months.- - - - - - - - - - ranking = 0.5keywords = hand (Clic here for more details about this article) |
5/19. Involuntary hand levitation associated with parietal damage: another alien hand syndrome.The alien hand syndrome (AHS) usually consists of an autonomous motor activity perceived as an involuntary and purposeful movement, with a feeling of foreignness of the involved limb, commonly associated with a failure to recognise ownership of the limb in the absence of visual clues. It has been described in association to lesions of the frontal lobes and corpus callosum. However, parietal damage can promote an involuntary, but purposeless, hand levitation, which, sometimes, resembles AHS. In the present study, four patients (cortico-basal ganglionic degeneration - n=2; Alzheimer's disease - n=1 and parietal stroke - n=1) who developed alien hand motor behaviour and whose CT, MRI and/or SPECT have disclosed a major contralateral parietal damage or dysfunction are described. These results reinforce the idea that parietal lobe lesions may also play a role in some patients with purposeless involuntary limb levitation, which is different from the classic forms of AHS.- - - - - - - - - - ranking = 3424.322986079keywords = alien hand, alien, hand (Clic here for more details about this article) |
6/19. A walking stick for a pure akinesia patient.We describe a 63-year-old female patient with pure akinesia whose gait was facilitated by a handmade converted walking stick. A posterior ventral pallidotomy had been performed, but it did not alleviate symptoms. Her husband made a walking stick with a wire loop at the bottom, perpendicular to the walking direction. When the patient stepped over the loop, the frozen gait was improved. This converted walking stick is easily made and inexpensive. Although the walking stick did not improve the patient's gait radically, use of the converted walking stick effectively improved the patient's daily life because successful treatment of pure akinesia cannot be established.- - - - - - - - - - ranking = 0.5keywords = hand (Clic here for more details about this article) |
7/19. Manual approach during hand gesture imitation.BACKGROUND: patients' tendency to draw near or into the target when copying figures, a phenomenon termed closing-in, has been previously described. That the closing-in could occur when copying hand gestures has also been noted. OBJECTIVES: To study a patient with corticobasal degeneration to quantify his manual approach behavior and to test a possible working memory hypothesis. methods: The subject of this study is a patient with severe ideomotor apraxia from probable corticobasal degeneration. fluorine 18-labeled deoxyglucose-positron emission tomographic findings revealed a hypometabolism involving the bilateral parietotemporal and the right frontal lobes. When asked to copy an examiner's (J.C.K.) hand gesture, the patient approached, touched, or grasped the examiner's hand, a behavior mostly consistent with the closing-in behavior previously proposed. To investigate the frequency and severity of closing-in, the patient was asked to copy 20 meaningless hand gestures (10 simple and 10 complex). Copying the 20 hand gestures was performed with either the left or the right hand while the patient was seated opposite the examiner (across condition) or on the same side of the examiner (lateral condition). RESULTS: Of the 80 trials, closing-in occurred in 43 (53.8%) (35 with approaching, 6 with touching, and 2 with grasping). The closing-in was more frequent and more severe when gesturing with the left than the right hand, but it did not differ between the lateral and across conditions and between simple and complex gestures. CONCLUSIONS: Corticobasal degeneration might be associated with aberrant manual approach behavior. Although our results do not support the working memory hypothesis, frontal dysfunction might have led to a loss of voluntary control of ontologically primitive propensity to move the forelimb in the direction to which one attends.- - - - - - - - - - ranking = 5.5keywords = hand (Clic here for more details about this article) |
8/19. 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.- - - - - - - - - - ranking = 243.63290921795keywords = alien (Clic here for more details about this article) |
9/19. The youngest reported case of corticobasal degeneration.Corticobasal degeneration (CBD) is a movement disorder characterized by early apraxia and asymmetric parkinsonism that responds poorly to anti-Parkinson medications. There are clinical manifestations reflecting dysfunction in both the cerebral cortex and the basal ganglia. patients typically present between the sixth and seventh decades. Previously, the youngest clinically diagnosed individual was 40 years old. Here we describe a 34-year-old woman who meets the clinical diagnostic criteria for CBD with onset of symptoms at age 28. In this patient, the first symptom was an 'uncooperative' right hand. This was soon followed by right hand dystonia. Symptoms progressed rapidly, and she developed generalized bradykinesia, rigidity, and corticospinal tract signs with preservation of the initial asymmetry. Her symptoms did not respond to a daily dose of 1200 mg of immediate release levodopa. Extensive laboratory workup and brain imaging were normal. Neuropsychological evaluation revealed mild deficits consistent with frontal-subcortical dysfunction. The chronic, progressive course, asymmetric limb rigidity, apraxia, focal dystonia, and lack of response to levodopa suggest CBD. To our knowledge, this is the youngest reported case of clinically probable CBD.- - - - - - - - - - ranking = 1keywords = hand (Clic here for more details about this article) |
10/19. A compensatory mechanism in unilateral akinetic-rigid syndrome: an fMRI study.The motor mechanisms of a patient with unilateral hand clumsiness in the early stages of akinetic-rigid syndrome were assessed by functional magnetic resonance imaging (fMRI). Movements of the unaffected hand produced activation in the contralateral sensorimotor cortex (SMC) and ipsilateral SMC and superior parietal lobule (SPL). The affected hand activated the bilateral SMCs, supplementary motor areas, and SPLs. We speculated that the bilateral activation indicated recruitment of a pre-existing bilaterally organized large-scale neural network to perform the motor task.- - - - - - - - - - ranking = 1.5keywords = hand (Clic here for more details about this article) |
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