Praxis of a new tool to sharpen neurodegenerative testing

  Clinicopathological correlation in dementia subtypes remains challenging, with similar clinical syndromes often caused by different pathologies. With the diagnosis of dementia relying primarily on clinical evaluation, this ambiguity clearly impacts clinical practice and has accelerated the search for biomarkers to help facilitate diagnosis and treatment. In this regard, apraxia profiling has recently gained momentum […]

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Sporadic Creutzfeldt-Jakob disease: beyond central nervous system dysfunction

  Sporadic Creutzfeldt-Jacob disease (sCJD) is a complex neurodegenerative disorder characterized by the accumulation of misfolded human prion protein (PrPSc). Classically, sCJD presents with neurological symptoms and signs of central nervous system (CNS) dysfunction, including cognitive impairment, cerebellar dysfunction, myoclonus, visual impairment and pyramidal signs, amongst others. Moreover, clinical diagnostic criteria requires the presence of […]

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Neurochemical changes in the ALS brain: further insights into disease heterogeneity

In a 1-year longitudinal study recently published in JNNP, ‘Neurochemical correlates of functional decline in amyotrophic lateral sclerosis’, ultra-high field 1H-MRS scans were used in 19 ALS patients by Cheong and colleagues to explore the relationship between neurochemical brain changes and functional decline. Specifically, the authors determined the regional levels of (i) N-acetylaspartate:myo-inositol (tNAA:mIns) from […]

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Primary lateral sclerosis and progressive muscular atrophy as a multidomain brain disorder

  From the diagnostic classification of amyotrophic lateral sclerosis (ALS) as defined in the El Escorial criteria, primary lateral sclerosis (PLS) and progressive muscular atrophy (PMA) have been considered as restrictive phenotypes, meaning that in PLS neurodegeneration is just restricted to the motor cortex and in PMA the loss of motor neurons occurs exclusively in […]

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Inflammatory neuropathies with paranodal antibodies: more than just IgG

  In the last five years exciting discoveries have been made regarding the role of different antibodies in inflammatory neuropathies. Specifically, patients with IgG-autoantibodies against paranodal proteins (e.g. contactin-1 or neurofascin-155) have a specific clinical phenotype characterized by a subacute motor predominant neuropathy, which tend to be refractory to intravenous immunoglobulins (IVIG) but may respond […]

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Epilepsy and psychiatry: a bidirectional response that needs further evaluation

The meta-analysis presented in the article ‘Cognitive-behavioural therapy does not meaningfully reduce depression in most people with epilepsy’ by Nobel and colleagues in this month’s October edition of JNNP explored the clinical impact of psychological treatment (cognitive-behavioural therapy, CBT) in patients with epilepsy. Specifically, findings from 5 randomised controlled studies that used CBT to treat […]

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“No evidence of disease activity” does not mean no evidence of brain deterioration

  Tremendous advances have been achieved in the therapeutic armament of disease modifying therapies (DMT) in relapsing-remitting multiple sclerosis (RRMS), leading to the concept of “no evidence of disease activity” (NEDA). This concept measures the success of treatment determined by the absence of clinical worsening (EDSS increase) and radiological activity (new/enlarging T2 lesions or gadolinium-enhancing […]

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Symptomatic intracranial haemorrhage after tPA: identifying the risks

‘Time is brain’ is an increasingly iterated mantra in stroke care, underlying the push for swifter treatment for one of the leading causes morbidity and mortality in the world. The use of intravenous tissue plasminogen activator (tPA) has undeniably remodelled expectations of stroke care, and the recent introduction of the First Mobile Stroke Unit in […]

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Novel motor mapping techniques of the spinal cord: why does it matter?

  Intraoperative neurophysiological monitoring (IONM) is generally used during complex surgeries that can compromise central and peripheral nervous system structures, providing real-time information about the functional integrity of these pathways and detecting reversible neurophysiological changes. This is particularly relevant in the surgery of intramedullary spinal cord (SC) tumors, which is associated with a high risk […]

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Functional neurological disorders: deconstructing “the crisis for neurology”

Functional neurological disorders are increasingly prevalent, but a limited understanding of their pathophysiology continues to make this one of the most challenging disorders seen in neurological practice. Its chequered history is reflected in outdated terms such as ‘hysteria’, ‘psychogenic’ and ‘non-organic’, and for the present time, the term ‘functional’ has been adopted for use in […]

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