Uncovering the clinicopathological spectrum in neuropathy associated with anti-MAG antibodies

  Neuropathy associated with anti-myelin-associated glycoprotein (anti-MAG) IgM antibodies typically presents as a distal variant of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) called distal acquired demyelinating symmetric (DADS) neuropathy. Clinically, it is characterized by chronic distal symmetric sensory or sensorimotor deficits, associated with sensory ataxia and postural tremor. However, patients with positive anti-MAG antibodies range from […]

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A window into the pathoanatomy of ALS: visualising disease spread using DTI

The critical involvement of central pathways in the spread of amyotrophic lateral sclerosis (ALS) is well-recognised both clinically and neuropathologically, and post-mortem studies have classified distinct pathological disease stages.  Recently, neuroimaging studies have proposed four main anatomical stages of disease spread: the first stage involving the corticospinal tract (CST), stage 2 involving corticorubral and corticopontine […]

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Chitotriosidase, neuroinflammation and ALS

  In the last few years, the role of neuroinflammation has been established as a relevant pathophysiological mechanism in multiple neurodegenerative diseases. Specifically, in ALS, activated microglia contribute to the non-cell autonomous neurodegeneration of the motor neurons. Moreover, there is no doubt that biomarkers for ALS diagnosis, prognosis and clinical trial stratification are needed. In […]

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Atrial fibrillation and white matter hyperintensities: more than just coincidence

  Cerebral small vessel disease (SVD) is quite a common finding on computer tomography (CT) and magnetic resonance imaging (MRI) scans of older people. SVD includes white matter lesions (WML) and lacunar infarcts, which are frequently associated to cardiovascular factors such as hypertension, diabetes mellitus and atrial fibrillation (AF). Interestingly, patients with WML present an […]

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On track for better outcomes: in vivo analysis of white matter pathways to enhance neuro-oncological practice

Starting from the early 19th Century, white matter fibre pathways have been increasingly recognised as integral to brain function. Since this time, the development of non-invasive advanced MRI techniques such as diffusion tractography (DT) has allowed the intricate architecture of these networks to be further unravelled and the complexity that these pathways contribute to human […]

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Deep brain stimulation in dystonia: is it a long-term effective and safe treatment?

  In the November issue of JNNP, Meoni and colleagues have published an interesting observational retrospective study exploring the long-term effect of pallidal deep brain stimulation (DBS) on motor and functional disability in patients with idiopathic, inherited and acquired dystonia. DBS is a neuromodulatory strategy base on a brain implanted electronic device used to modulate […]

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Cognitive impairment in Parkinson’s disease: is there finally an accessible biomarker?

In the October issue of JNNP, Lin and colleagues explore plasma levels of a-synuclein as a biomarker in patients with varying stages of Parkinson’s disease (PD), across both motor and non-motor domains.The extensive evaluation of biofluids in the hunt for diagnostic and prognostic biomarkers in PD has been disappointing and largely inconclusive to date. Studies […]

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The cerebellum in neurodegeneration: more than just balance control

  In the current issue of JNNP, Gellersen and colleagues have published the first meta-analysis designed to assess patterns of cerebellar atrophy across different neurodegenerative diseases. Typically, neurodegenerative diseases have been linked to impairment in cortical and basal ganglia structures, however increasing evidence suggests that degeneration in the cerebellum also plays a role in these […]

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Cognitive profiling in bvFTD and ALS-bvFTD: is there a difference?

In the August issue of JNNP, Saxon and colleagues analyse the cognitive characteristics of pure behavioural variant FTD (bvFTD) and compare this to the behavioural and cognitive symptoms that develop in a proportion of amyotrophic lateral sclerosis (ALS) patients (ALS-bvFTD). The occurrence of cognitive impairment is now well recognised in ALS, and the clinical and […]

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Anti-neurofascin-155 antibodies: dissecting peripheral nerve abnormalities in CIDP

  In the June JNNP issue, Koike and colleagues have reported the morphological changes in sural nerve biopsies of patients with chronic inflammatory demyelinating polyneuropathy (CIDP) who have autoantibodies against paranodal structures. CIDP is an immune-mediated neuropathy that develops over 8 week, which manifests with progressive proximal and distal weakness and is associated with sensory […]

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