Journal of neurology, neurosurgery, and psychiatry
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J. Neurol. Neurosurg. Psychiatr. · Jun 2018
Friedreich and dominant ataxias: quantitative differences in cerebellar dysfunction measurements.
Sensitive outcome measures for clinical trials on cerebellar ataxias are lacking. Most cerebellar ataxias progress very slowly and quantitative measurements are required to evaluate cerebellar dysfunction. ⋯ Cerebellar dysfunction, as measured with the CCFS and SARA scales, was more severe in FRDA than in patients with SCA, but with lower progression indexes, within the limits of these types of indexes. Ceiling effects may occur at late stages, for both scales. The CCFS scale is rater-independent and could be used in a multicentre context, as it is simple, rapid and fully automated.
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J. Neurol. Neurosurg. Psychiatr. · Jun 2018
ReviewAutoimmune nodo-paranodopathies of peripheral nerve: the concept is gaining ground.
Peripheral neuropathies are classified as primarily demyelinating or axonal. Microstructural alterations of the nodal region are the key to understand the pathophysiology of neuropathies with antibodies to gangliosides and the new category of nodo-paranodopathy has been proposed to better characterise these disorders and overcome some inadequacies of the dichotomous classification. Recently, the research in autoimmune neuropathies has been boosted by reports of patients carrying immunoglobulin G4 antibodies against paranodal axo-glial proteins with distinct phenotypes and showing loss of transverse bands, terminal myelin loop detachment, nodal widening and axonal loss. ⋯ Evidence from nerve conductions and electron microscopy studies in patients and mutant animal models can reconcile the apparent contrast between the electrophysiological 'demyelinating' features, explainable just by the paranodal involvement and the axonal pathology. These patients broaden the autoimmune nodo-paranodopathy category and re-emphasise the usage of the term that pointing to the site of nerve injury reminds specific pathophysiological mechanisms, reconciles contrasting electrophysiological and pathological findings, and avoids misdiagnosis and taxonomic confusion. In our opinion, the nodo-paranodopathy term more adequately classifies the peripheral nerve disorders due to an autoimmune attack directed and limited to the nodal region integrating the traditional classification of peripheral neuropathies.
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J. Neurol. Neurosurg. Psychiatr. · Jun 2018
Incidence and disease burden of chemotherapy-induced peripheral neuropathy in a population-based cohort.
To assess disease burden of chemotherapy-induced peripheral neuropathy (CIPN), which is a common dose-limiting side effect of neurotoxic chemotherapy. Late effects of CIPN may increase with improved cancer survival. ⋯ Results from our population-based study are consistent with previous reports of high incidence of CIPN in the first 2 years following incident exposure to neurotoxic chemotherapeutic agents, and its association with significant pain symptomatology and accompanied long-term opioid use. Increased survival following exposure to neurotoxic chemotherapy and its long-term disease burden necessitates further study among survivors.
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J. Neurol. Neurosurg. Psychiatr. · Jun 2018
Prevalence of hippocampal enlarged perivascular spaces in a sample of patients with hypertension and their relation with vascular risk factors and cognitive function.
The clinical importance of hippocampal enlarged perivascular spaces (H-EPVS) remains uncertain. We aimed to study their association with vascular risk factors, cognitive function and mild cognitive impairment (MCI). ⋯ H-EPVS are a frequent finding in patients with hypertension and are associated with ageing and poor hypertension treatment compliance. Besides, H-EPVS are associated with worse verbal reasoning function.
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J. Neurol. Neurosurg. Psychiatr. · Jun 2018
Auras and the risk of seizures with impaired consciousness following epilepsy surgery: implications for driving.
To calculate the chance of a seizure in the next year (COSY) for seizures with impaired awareness in those experiencing auras only, those with no seizures and those with continuing seizures. Epilepsy surgery is an effective treatment for refractory focal epilepsy. Driving is an important factor affecting quality of life. In the UK, driving is not permitted if focal seizures with no impairment of awareness (auras, simple partial seizures) continue, if there is a prior history of seizures with impaired awareness, as will invariably be the case in those having epilepsy surgery. Current UK driving regulations allow driving if COSY is less than 20%. ⋯ Individuals with auras only after epilepsy surgery had a higher COSY than those who were seizure-free. If a COSY of below 20% is regarded as an acceptable risk, it may be suggested that those with auras only in a given year be allowed to drive. The relative risk of these patients causing accidents is lower than population groups such as those aged <25 or >75 years, who are permitted to drive.