1/49. Deterioration in parkinsonism with low-dose pergolide.The administration of dopamine agonists can have a role early in the course of Parkinson's disease, in an attempt to reduce the frequency of long-term motor complications associated with the use of levodopa. After treatment with dopamine agonists has begun, gradual dose escalation is recommended to reduce the incidence of side effects; at low doses, parkinsonian symptoms significantly decline in some patients, only to improve as the dose increases. We report a number of such patients and discuss the possible pathogenesis of this motor deterioration.- - - - - - - - - - ranking = 1keywords = parkinsonism, parkinsonian (Clic here for more details about this article) |
2/49. Intracranial dural arteriovenous fistula (DAVF) presenting progressive dementia and parkinsonism.We studied three patients with dural arteriovenous fistula (DAVF). Major symptoms were progressive dementia and parkinsonism, both of which progressed in step-wise fashion. Two of the three patients showed diffuse cerebral white matter lesions on brain CT and MRI. Progressive dementia and parkinsonism in our patients could be caused by diffuse cerebral parenchymal disturbance: impaired cerebral circulation due to severe venous hypertension. DAVF is important for the differential diagnosis in patients with progressive dementia and parkinsonism.- - - - - - - - - - ranking = 1.6591673638783keywords = parkinsonism (Clic here for more details about this article) |
3/49. Migratory basal ganglia lesions in subacute sclerosing panencephalitis (SSPE): clinical implications of axonal spread.We report a boy with subacute sclerosing panencephalitis (SSPE) who exhibited parkinsonian symptoms four months after onset. The symptoms improved after administration of levodopa. One year after onset, bilateral symmetric lesions appeared in the substantia nigra and the putamen, as observed using magnetic resonance imaging. After a one-year interval, the lesions migrated to the bilateral caudate and the cerebellar dentate nuclei. The series of migratory legions, each of which was connected by axonal pathways originating from the substantia nigra, suggests axonal spread of the sspe virus.- - - - - - - - - - ranking = 0.051904363498114keywords = parkinsonian (Clic here for more details about this article) |
4/49. FTDP-17: an early-onset phenotype with parkinsonism and epileptic seizures caused by a novel mutation.Recently, mutations in the tau gene on chromosome 17 were found causative for autosomal dominantly inherited frontotemporal dementia and parkinsonism (FTDP-17). We describe a family carrying a missense mutation at nucleotide 1137 C --> T, resulting in the amino acid substitution P301S. methods of investigations include clinical, electrophysiological, and imaging techniques. This kindred presents with a novel phenotype characterized by an early onset of rapidly progressive frontotemporal dementia and parkinsonism in combination with epileptic seizures. We define the dopaminergic deficits as being predominantly presynaptic by the use of single-photon emission computed tomography with a dopamine transporter ligand. The association of this early-onset phenotype with P301S mutation is not entirely consistent with current criteria for the diagnosis of frontotemporal dementias and may encourage the search for tau mutations in diseases similar but not identical to FTDP-17. Also, the change from proline to serine suggests that this mutation might contribute to tau hyperphosphorylation.- - - - - - - - - - ranking = 1.4221434547528keywords = parkinsonism (Clic here for more details about this article) |
5/49. Delayed onset of ascending paralysis after thoracic aortic stent graft deployment.Delayed spinal cord ischemia after thoracic aortic aneurysm repair is an infrequent but devastating complication. The use of stent grafts to exclude aortic aneurysms is thought to decrease the incidence of the neurologic deficit because there is no period of significant aortic occlusion. We report a case of paraplegia that progressed to quadriplegia occurring 48 hours after the apparently successful deployment of a thoracic aortic stent graft.- - - - - - - - - - ranking = 0.0025880118067068keywords = paralysis (Clic here for more details about this article) |
6/49. Juvenile parkinsonism: a heterogeneous entity.We studied the clinical features, laboratory investigation, management and natural history of a cohort of patients with Juvenile Parkinsonism (JP), seen at a tertiary referral centre. JP was defined as Parkinsonism with onset at age 20 years or less. Six patients (five male, one female) entered the study. The mean age at onset of Parkinsonism was 12.5 years (range 7-19) and the mean follow-up time was 49.3 months (range 40-57). Bradykinesia, rigidity, and postural instability were observed in all patients and five subjects had tremor. dystonia was present in four subjects. Other clinical features were dementia (five subjects), supranuclear ophthalmoparesis (five subjects), seizures (three subjects), multifocal myoclonus (one subject), decreased deep reflexes (one subject), pyramidal signs (one subject). family history of Parkinson's disease (PD) was positive in one subject. work-up for Wilson's disease was negative in all patients. neuroimaging studies showed cortical atrophy in two subjects and mild brainstem atrophy in two others. Sea-blue histiocytes were found in one subject. L-dopa improved the Parkinsonism in all subjects but four rapidly developed fluctuations and dyskinesias, requiring, in one, stereotaxic surgery. After a mean disease duration of 6.5 years, five subjects require assistance for performance of all daily activities. JP is a heterogeneous clinical entity. In the majority of patients, no underlying cause is identified. The unusual clinical features suggest most subjects have a CNS degenerative disease distinct from PD. There is, however, evidence suggesting that PD may rarely cause JP. Gangliosidosis is another cause of L-dopa-responsive JP. Regardless of the cause, in the present study JP displays an aggressive and rapidly progressive course in most patients.- - - - - - - - - - ranking = 0.94809563650189keywords = parkinsonism (Clic here for more details about this article) |
7/49. Asymmetrical temporal lobe atrophy with massive neuronal inclusions in multiple system atrophy.This report concerns a rare association of asymmetrical temporal lobe atrophy with multiple system atrophy (MSA). A 53-year-old Japanese woman developed cerebellar ataxia and parkinsonism and was diagnosed as olivopontocerebellar atrophy (OPCA). This patient showed forgetfulness and subsequent disorientation even in the early stage of the disease. She fell into a decorticate state at the age of 64, and died a year later. The autopsy showed MSA with asymmetrical atrophy of temporal lobes, intraneuronal globular inclusions mostly confined to the hippocampus, amygdaloid nucleus, and most abundant in the granule cells in the dentate fascia. These inclusions were intensely argyrophilic and expressed marked immunoreactivity to ubiquitin, but not to neurofilament (NF), tau and paired helical filaments (PHF). Ultrastructurally, they were composed of scattered short filamentous structures of 15 to 30 nm in diameter, ribosome-like granules, mitochondria and lipofuscin. The lack of immunoreactivity against tau, NF and PHF suggests that the inclusions are distinct from Pick bodies. To our knowledge, MSA in association with asymmetrical temporal lobe atrophy with the present neuronal inclusions has not been reported. This case is distinct from MSA combined with atypical Pick's disease in the distribution and immunohistochemical properties of neuronal inclusions, and may present a new variant of MSA since the neuronal inclusions are similar, in many respects, to those of neuronal inclusions reported in MSA. Globular inclusions are also discussed in variants of Pick's disease, amyotrophic lateral sclerosis and Alzheimer's disease.- - - - - - - - - - ranking = 0.23702390912547keywords = parkinsonism (Clic here for more details about this article) |
8/49. Acute thoracoabdominal aortic dissection presenting as painless, transient paralysis of the lower extremities: a case report.Acute dissection of the aorta can be one of the most dramatic of cardiovascular emergencies. Its symptoms can occur abruptly and progress rapidly. Prompt recognition and appropriate intervention is crucial. However, not all aortic dissections present with classic symptoms of abrupt chest, back, or abdominal pain, and the diagnosis may be missed. A 63-year-old woman presented with transient loss of lower extremity motor and sensory function as the only symptom of an acute thoracoabdominal aortic dissection. Aortic dissection presenting as a transient neurologic syndrome is quite unusual. The sudden onset of weakness and parasthesia can result from the interruption of blood flow to the spinal cord. These symptoms of acute spinal cord ischemia, suggestive of a serious cardiovascular event, must be recognized and thoroughly investigated.- - - - - - - - - - ranking = 0.0025880118067068keywords = paralysis (Clic here for more details about this article) |
9/49. Left vocal cord paralysis associated with long-standing patent ductus arteriosus.SUMMARY: Left vocal cord paralysis in association with patent ductus arteriosus is unusual. We report a patient with long-standing patent ductus arteriosus (PDA) in whom CT studies obtained before and after paralysis developed showed an interval increase in size of the pulmonary trunk. The pathogenesis of left vocal cord paralysis in association with long-standing PDA is discussed.- - - - - - - - - - ranking = 0.0045290206617369keywords = paralysis (Clic here for more details about this article) |
10/49. Phenotypic characterisation of autosomal recessive PARK6-linked parkinsonism in three unrelated Italian families.The clinical features of nine patients (three women and six men) affected by PARK6-linked parkinsonism, belonging to three unrelated Italian families, are reported. The occurrence of affected men and women within one generation suggested an autosomal recessive mode of inheritance in all three families. Mean age at disease onset was 36 /- 4.6 years; all cases except one presented with asymmetrical signs, consisting of tremor and akinesia of one upper limb or unilateral short step gait. Affected individuals had a mean age of 57 /- 8.5 years, and average disease duration was 21 /- 7.8 years. Parkinsonian features included benign course, early onset of drug-induced dyskinesias, and a good and persistent response to levodopa. There were no other associated features (i.e., pyramidal or cerebellar signs, dysautonomia, or diurnal fluctuations unrelated to drug treatment). cognition was unaffected. The clinical picture was remarkably similar in all patients; no relevant family-related differences were found. PARK6 disease is a new form of early-onset parkinsonism without other atypical clinical features.- - - - - - - - - - ranking = 1.4221434547528keywords = parkinsonism (Clic here for more details about this article) |
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