Journal of neurology, neurosurgery, and psychiatry
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J. Neurol. Neurosurg. Psychiatr. · Dec 2001
Case Reports"Fou rire prodromique" as the presentation of pontine ischaemia secondary to vertebrobasilar stenosis.
"Fou rire prodromique" (prodrome of crazy laughter) is a rare form of pathological laughter of uncertain pathophysiology. A patient is presented with pathological laughter as the first manifestation of pontine ischaemia due to vertebrobasilar stenosis. A 65 year old man developed uncontrollable and unemotional laughter for almost an hour followed by transient right facial-brachial paresis. ⋯ Necropsy confirmed the extensive brain stem infarction. Pathological laughter can be the very first presenting manifestation of ischaemia of the ventrotegmental junction of the upper pons. It is hypothesised that the pathological laughter in this patient was secondary to ischaemic ephaptic stimulation of the descending corticopontine/ bulbar pathways.
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J. Neurol. Neurosurg. Psychiatr. · Dec 2001
Case ReportsA large family with hereditary spastic paraparesis due to a frame shift mutation of the spastin (SPG4) gene: association with multiple sclerosis in two affected siblings and epilepsy in other affected family members.
Hereditary spastic paraparesis (HSP) is a clinically and genetically heterogeneous neurodegenerative disorder characterised by progressive lower limb spasticity and weakness. Some forms have been associated with white matter lesions and complex phenotypes. This study was prompted by the diagnosis of multiple sclerosis (MS) in two sisters from a large pedigree with hereditary spastic paraparesis. ⋯ Genetic analysis of the family has shown linkage to chromosome 2p and sequencing of the spastin gene has identified a 1406delT frameshift mutation in exon 10. This kindred demonstrates the clinical heterogeneity of HSP associated with spastin mutations. The possible relevance of the concurrence of HSP and MS in the sib pair is discussed.
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J. Neurol. Neurosurg. Psychiatr. · Dec 2001
Prevention of spinal cord injury with time-frequency analysis of evoked potentials: an experimental study.
To verify the applicability and validity of time-frequency analysis (TFA) of evoked potential (EP) signals in detecting the integrity of spinal cord function and preventing spinal cord injury. ⋯ It was found that TFA of EPs provided an earlier and more sensitive indication of injury than time domain monitoring alone. It is suggested that TFA of EP signals should therefore be useful in preventing spinal cord injury during surgery.
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J. Neurol. Neurosurg. Psychiatr. · Dec 2001
Biography Historical ArticleThe subthalamic nucleus and Jules Bernard Luys (1828-97).