1/133. Biofeedback training to overcome poststroke foot-drop.The technique has exciting potential for elderly hemiplegic patients, even those who are confined to a nursing home. There are limiting factors, of course; the method is time-consuming and the initial outlay for equipment is high. But the rewards can be well worth the time, effort, and cost involved. The recovery potential of some geriatric patients after a cerebrovascular accident may seem bleak because of multiple disabilities, e.g., paralysis, delayed reflexes, aphasia. Fortunately, these disorders do not necessarily decree failure of biofeedback training. Neither does advanced age. The strongest component in success is motivation. A case in point is the 82 year old woman described here who had been hemiparetic for seven years. With biofeedback training, she gained--and maintained--muscle strength.- - - - - - - - - - ranking = 1keywords = muscle (Clic here for more details about this article) |
2/133. Juvenile form of dihydropteridine reductase deficiency in 2 Tunisian patients.Two brothers are described who had juvenile-onset DHPR deficiency. Both were considered normal until six years of age when they developed a fluctuating and progressive encephalopathy combining mental retardation, epilepsy, pyramidal, cerebellar and extrapyramidal signs.- - - - - - - - - - ranking = 409.63324487088keywords = extrapyramidal (Clic here for more details about this article) |
3/133. Osmotic demyelination syndrome with two-phase movement disorders: case report.Osmotic demyelination syndrome (ODS) is characterized by regions of demyelination throughout the brain, which are most prominent in the pons. This demyelinating disease is associated with electrolyte disturbances and typically occurs in patients who are alcoholic or malnourished. movement disorders are not frequently recognized in patients with ODS. This report describes a 22-year-old woman with ODS after correction of profound hyponatremia. The main neurologic symptom was two-phase movement disorder. First, she had acute onset dystonia, then the movement disorder transformed to generalized rigidity and tremors in the delayed second phase. magnetic resonance imaging in the first phase revealed demyelinating lesions in the central pons, bilateral thalami and basal ganglia. In the second phase, the previous myelinolysis had been partially resolved. The clinical course of the two-phase movement disorder did not correlate with the resolving feature of neuroradiologic findings. During the second-phase movement disorder, the patient had a good response to propranolol and trihexyphenidyl.- - - - - - - - - - ranking = 1170.9592844585keywords = rigidity (Clic here for more details about this article) |
4/133. 18Fluoro-2-deoxyglucose (18FDG) PET scan of the brain in type IV 3-methylglutaconic aciduria: clinical and MRI correlations.The clinical, 18fluorodeoxyglucose positron emission tomography (18FDG PET) and the magnetic resonance imaging (MRI) brain scan characteristics of four patients diagnosed to have 3-methylglutaconic aciduria were reviewed retrospectively. The disease has a characteristic clinical pattern. The initial presentations were developmental delay, hypotonia, and severe failure to thrive. Later, progressive encephalopathy with rigidity and quadriparesis were observed, followed by severe dystonia and choreoathetosis. Finally, the patients became severely demented and bedridden. The 18FDG PET scans showed progressive disease, explaining the neurological status. It could be classified into three stages. Stage I: absent 18FDG uptake in the heads of the caudate, mild decreased thalamic and cerebellar metabolism. Stage II: absent uptake in the anterior half and posterior quarter of the putamina, mild-moderate decreased uptake in the cerebral cortex more prominently in the parieto-temporal lobes. Progressive decreased thalamic and cerebellar uptake. Stage III: absent uptake in the putamina and severe decreased cortical uptake consistent with brain atrophy and further decrease uptake in the cerebellum. The presence of both structural and functional changes in the brain, demonstrated by the combined use of MRI and 18FDG PET scan, with good clinical correlation, make the two techniques complementary in the imaging evaluation of 3-methylglutaconic aciduria.- - - - - - - - - - ranking = 1170.9592844585keywords = rigidity (Clic here for more details about this article) |
5/133. Neuronal activity in the basal ganglia in patients with generalized dystonia and hemiballismus.Microelectrode recording was performed in the basal ganglia of 3 patients with generalized dystonia and 1 patient with hemiballismus secondary to a brainstem hemorrhage. Neuronal activity was recorded from the internal and external segments of the globus pallidus and assessed for mean discharge rate and pattern of spontaneous activity. The responses of neurons in the internal segment of the globus pallidus to passive and active movements were also evaluated. Mean discharge rates of neurons in both segments of the pallidum in patients with dystonia and the patient with hemiballismus were considerably lower than those reported for patients with idiopathic Parkinson's disease. In addition, the pattern of spontaneous neuronal activity was highly irregular, occurring in intermittent grouped discharges separated by periods of pauses. Although receptive fields in the dystonia patients were widened and less specific than those reported in normal monkeys, neuronal responses to movement were uncommon in the hemiballismus patient. Before surgery, patients with dystonia experienced abnormal posturing and involuntary movements. Coactivation of agonist-antagonist muscle groups was observed both at rest and during the performance of simple movements. After pallidotomy there was a significant reduction in the involuntary movement associated with these disorders and a more normal pattern of electromyographic activity during rest and movement. Given the improvement in dystonic and hemiballistic movements in these patients after ablation of the sensorimotor portion of the internal segment of the globus pallidus, we suggest that pallidotomy can be an effective treatment for patients with dystonia and also for patients with medically intractable hemiballismus. Based on the finding of decreased neuronal discharge rates in pallidal neurons, we propose that physiologically dystonia most closely resembles a hyperkinetic movement disorder. A model for dystonia is proposed that incorporates the observed changes in the rate and pattern of neuronal activity in the pallidum with data from neuroimaging with positron emission tomography and 2-deoxyglucose studies.- - - - - - - - - - ranking = 1keywords = muscle (Clic here for more details about this article) |
6/133. Bilateral ballism in a patient with overlapping Fisher's and Guillain-Barre syndromes.A 29 year old woman developed diplopia and ataxic gait. Neurological examination showed total ophthalmoplegia, cerebellar ataxia, and areflexia. Moreover, there was muscle weakness in all four limbs. An overlap of Fisher's and Guillain-Barre syndromes was dignosed. On day 5 she suddenly developed involuntary flinging movements that affected the face and four limbs. Surface EMG showed 1.5-2 Hz rhythmic grouping discharges. The involuntary movements were considered ballism. This is the first report of a patient with guillain-barre syndrome and a related disorder who showed ballism.- - - - - - - - - - ranking = 1keywords = muscle (Clic here for more details about this article) |
7/133. motor neuron disease-inclusion dementia presenting as cortical-basal ganglionic degeneration.The frontotemporal dementias are a group of relatively new and evolving clinical and pathologic entities. The predominant frontal-temporal atrophy causes a variety of clinical syndromes, usually dominated by disturbances in behavior, mood, and speech. The motor neuron disease-inclusion dementia (MNDID) subtype is characterized by the accumulation of specific intraneuronal ubiquitin-immunoreactive inclusions with the complete absence of tau immunoreactivity. We present a patient with the clinical and neuroimaging characteristics of a highly asymmetric neurodegenerative condition distinguished by limb rigidity, bradykinesia, dystonia with an alien limb phenomenon, cortical sensory findings, and limb apraxia. His premorbid diagnosis was cortical-basal ganglionic degeneration but he had the typical histologic features of a frontotemporal dementia of the MNDID subtype.- - - - - - - - - - ranking = 1170.9592844585keywords = rigidity (Clic here for more details about this article) |
8/133. Primary central nervous system lymphoma presenting with a parkinsonian syndrome of pure akinesia.The incidence of primary central nervous system lymphoma (PCNSL), once a rare tumour, has risen significantly in both immunocompetent and immunosuppressed patients. Although infiltration of the basal ganglia is not uncommon in PCNSL, extrapyramidal movement disorders are generally not recognised as a mode of clinical presentation of this type of cerebral tumour. We present the unusual case of a 75-year-old man who developed a parkinsonian syndrome of "pure akinesia" due to autopsy-confirmed PCNSL primarily involving the globus pallidus bilaterally. Parkinsonism due to bilateral pallidal lesions is known but rare, and such cases help in the understanding of basal ganglia function with regard to akinesia and freezing.- - - - - - - - - - ranking = 409.63324487088keywords = extrapyramidal (Clic here for more details about this article) |
9/133. Treating severe bruxism with botulinum toxin.BACKGROUND: Locally administered botulinum toxin, or BTX, is an effective treatment for various movement disorders. Its usefulness in treating bruxism, however, has not been systematically evaluated. SUBJECTS AND methods: The authors studied 18 subjects with severe bruxism and whose mean duration of symptoms was 14.8 /- 10.0 years (range three-40 years). These subjects audibly ground their teeth and experienced tooth wear and difficulty speaking, swallowing or chewing. Medical or dental procedures had failed to alleviate their symptoms. The authors administered a total of 241 injections of BTX type A, or BTX A, in the subjects' masseter muscles during 123 treatment visits. The mean dose of the BTX A was 61.7 /- 11.1 mouse units, or MU (range 25-100 MU), per side for the masseter muscles. RESULTS: The mean total duration of response was 19.1 /- 17.0 weeks (range six-78 weeks), and the mean peak effect on a scale of 0 to 4, in which 4 is equal to total abolishment of grinding, was 3.4 /- 0.9. Only one subject (5.6 percent) reported having experienced dysphagia with BTX A. CONCLUSION: The results of this study suggest that BTX administered by skilled practitioners is a safe and effective treatment for people with severe bruxism, particularly those with associated movement disorders. It should be considered only for those patients refractory to conventional therapy. Future placebo-controlled studies may be useful in further evaluating the potential of BTX in the treatment of bruxism.- - - - - - - - - - ranking = 2keywords = muscle (Clic here for more details about this article) |
10/133. Patterns of motor control reorganization in a patient with mirror movements.OBJECTIVE: To explore motor control reorganization in a 40-year-old, left-handed patient with perinatally acquired mirror movements. methods: We performed simultaneous bilateral recordings of motor evoked potentials (MEPs) following focal transcranial magnetic stimulation (fTMS) and of central silent period (cSP) during unilateral voluntary contraction in abductor pollicis brevis (APB) and abductor digiti minimi (ADM) muscles. RESULTS: For both muscles the MEP study showed bilateral fast-conducting corticospinal projections from the right undamaged hemisphere, and residual contralateral projections from the left hemisphere. The cSP findings differed in the two muscles: the mirror phenomenon was bilateral in the ADM, but present only on the right side in the APB muscles; the mirror activity of right ADM and APB muscles was inhibited only by fTMS of the ipsilateral right motor cortex; the mirror phenomenon in the left ADM muscle was inhibited only by fTMS of the contralateral right motor cortex. CONCLUSIONS: Mirror movements of right APB and ADM muscles were sustained by the ipsilateral connections from the undamaged motor cortex, while the mirror phenomenon in the left ADM muscle could be explained by hypothesizing a bilateral activation of motor cortices. This previously unreported electrophysiological picture demonstrates that different patterns of motor control may realize after perinatal cerebral lesions, even in different distal muscles of the same patient.- - - - - - - - - - ranking = 9keywords = muscle (Clic here for more details about this article) |
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