Journal of neurology, neurosurgery, and psychiatry
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J. Neurol. Neurosurg. Psychiatr. · Sep 2013
Rapidly progressive scoliosis and respiratory deterioration in Ullrich congenital muscular dystrophy.
To characterise the natural history of Ullrich congenital muscular dystrophy (UCMD). ⋯ The natural history of scoliosis, respiratory function and walking ability in UCMD patients were characterised. Although the age of onset varied, scoliosis, as well as restrictive respiratory dysfunction, progressed rapidly within years, once they appeared.
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J. Neurol. Neurosurg. Psychiatr. · Sep 2013
Fasciculation potentials and earliest changes in motor unit physiology in ALS.
There is little information on the earliest changes in motor unit (MU) physiology in amyotrophic lateral sclerosis (ALS) and the development of the classical neurophysiological features of ALS over time. ⋯ FPs are a very early marker of ALS and anticipate MUP instability or reinnervation, consistent with a very early phase of increased axonal excitability. Later, widespread neuronal dysfunction causes widespread fibs-sw and loss of MUPs with compensatory reinnervation. Our results confirm the importance of FP morphology analysis in the differential diagnosis of ALS and other disorders, and indicate that benign FPs represent a different phenomenon.
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J. Neurol. Neurosurg. Psychiatr. · Sep 2013
Transcranial magnetic stimulation as an efficient treatment for psychogenic movement disorders.
Management of psychogenic movement disorders (PMDs) is challenging for neurologists and, to date, there is no consensus about their treatment. Recent studies suggested a possible therapeutic effect of repeated transcranial magnetic stimulation (TMS) in psychogenic paralysis and tremor. ⋯ TMS is a therapeutic option for PMDs, including chronic PMDs.
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J. Neurol. Neurosurg. Psychiatr. · Sep 2013
Lies tell the truth about cognitive dysfunction in essential tremor: an experimental deception study with the guilty knowledge task.
Research conducted in the past decade challenges the traditional view that essential tremor (ET) is characterised exclusively by movement disorder, and increasingly shows that these patients have deficits in cognitive and behavioural functioning. The available evidence suggests that this impairment might arise from dysfunction in either the fronto-subcortical or cortico-cerebellar circuits. Although abnormalities in the fronto-subcortical circuits could imply difficulty in lying, no study has investigated deception in patients with ET. ⋯ Besides confirming impaired deception in patients with PD, our results show a lie production deficit in patients with ET also. These findings suggest that difficulty in lying is an aspecific cognitive feature in movement disorders characterised by fronto-subcortical circuit dysfunction, such as PD and ET. Current knowledge along with our new findings in patients with ET--possibly arising from individually unrecognised extremely mild, cognitive difficulties--should help in designing specific rehabilitative programmes to improve cognitive and behavioural disturbances in patients.
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J. Neurol. Neurosurg. Psychiatr. · Sep 2013
Long-term subthalamic nucleus stimulation improves sensorimotor integration and proprioception.
Sensorimotor integration is impaired in patients with Parkinson's disease (PD). Short latency afferent inhibition (SAI) and long latency afferent inhibition (LAI) measured with transcranial magnetic stimulation (TMS) can be used to measure sensorimotor integration. Subthalamic nucleus (STN) deep brain stimulation (DBS) has been found to restore these abnormalities, but the time course of these changes is not known. We prospectively evaluated the short-term and long-term effects of STN DBS on SAI, LAI and proprioception. We hypothesised plasticity changes induced by chronic stimulation are necessary to normalise sensorimotor integration and proprioception. ⋯ Chronic STN DBS in PD normalises sensorimotor integration and proprioception, likely through long-term plastic changes in the basal ganglia thalamocortical circuit.