Journal of neurology, neurosurgery, and psychiatry
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J. Neurol. Neurosurg. Psychiatr. · Jul 2013
Review Case ReportsDysfunction of the locus coeruleus-norepinephrine system and related circuitry in Parkinson's disease-related dementia.
Although resting tremor, cogwheel rigidity, hypokinesia/bradykinesia and postural instability usually dominate the clinical picture of sporadic Parkinson's disease (PD), both clinical and epidemiological data reveal that a wide variety of additional symptoms impair patients' quality of life considerably, parallel to the chronic progressive neurodegenerative movement disorder. Autopsy based retrospective studies have shown that α-synuclein immunoreactive Lewy pathology (LP) develops in the locus coeruleus (LC) of patients with neuropathologically confirmed sporadic PD, as well as in individuals with incidental (prodromal or premotor) Lewy body disease but not in age and gender matched controls. ⋯ The contribution of noradrenergic deficit to cognitive dysfunction in PD has been underappreciated. Noradrenergic therapeutic interventions might not only alleviate depressive symptoms and anxiety but also delay the onset of cognitive decline.
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J. Neurol. Neurosurg. Psychiatr. · Jul 2013
Case ReportsPathophysiology of HNPP explored using axonal excitability.
Hereditary liability to pressure palsies (HNPP) is an autosomal dominant disorder of myelination resulting in susceptibility to pressure palsies from compression or stretching of peripheral nerves. ⋯ Structural abnormalities at the node of Ranvier are sufficient to explain the changes in axonal excitability in HNPP, and these abnormalities would predispose the nerves to conduction block when subjected to pressure or stretch.
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J. Neurol. Neurosurg. Psychiatr. · Jul 2013
ReviewTherapeutic strategies for tau mediated neurodegeneration.
Based on the amyloid hypothesis, controlling β-amyloid protein (Aβ) accumulation is supposed to suppress downstream pathological events, tau accumulation, neurodegeneration and cognitive decline. However, in recent clinical trials, Aβ removal or reducing Aβ production has shown limited efficacy. Moreover, while active immunisation with Aβ resulted in the clearance of Aβ, it did not prevent tau pathology or neurodegeneration. ⋯ Therefore, it is timely and very important to develop tau directed therapies. The pathomechanisms of tau mediated neurodegeneration are unclear but hyperphosphorylation, oligomerisation, fibrillisation and propagation of tau pathology have been proposed as the likely pathological processes that induce loss of function or gain of toxic function of tau, causing neurodegeneration. Here we review the strategies for tau directed treatments based on recent progress in research on tau and our understanding of the pathomechanisms of tau mediated neurodegeneration.
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J. Neurol. Neurosurg. Psychiatr. · Jul 2013
What drives the increasing utilisation of hemicraniectomy in acute ischaemic stroke?
Survival after malignant middle cerebral artery infarcts is dismal. In 2007, a pooled analysis of randomised trials in Europe demonstrated a substantial survival benefit from decompressive hemicraniectomy, with a number needed to treat of 2 for survival. Our objective was to review factors driving the nationwide utilisation of this potentially lifesaving procedure in the USA. ⋯ Utilisation of hemicraniectomy in the USA has increased significantly, in line with compelling results from European clinical trials. Early transfer of patients with malignant infarctions to urban teaching centres could potentially extend the survival benefit to a larger population.
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J. Neurol. Neurosurg. Psychiatr. · Jul 2013
Grey matter correlates of clinical variables in amyotrophic lateral sclerosis (ALS): a neuroimaging study of ALS motor phenotype heterogeneity and cortical focality.
Body region of onset and functional disability are key components of disease heterogeneity in amyotrophic lateral sclerosis (ALS). ⋯ Focal grey matter atrophy within the motor homunculus corresponds with functional disability in ALS. The findings support the existing concepts of cortical focality and motor phenotype heterogeneity in ALS.