Journal of neurology, neurosurgery, and psychiatry
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J. Neurol. Neurosurg. Psychiatr. · Apr 2018
ReviewApathy: a neurocircuitry model based on frontotemporal dementia.
Apathy is a symptom shared among many neurological and psychiatric disorders. However, the underlying neurocircuitry remains incompletely understood. Apathy is one of the core features of behavioural variant frontotemporal dementia (bvFTD), a neurodegenerative disease presenting with heterogeneous combinations of socioaffective symptoms and executive dysfunction. ⋯ Integrating findings across studies, we revise a neurocircuitry model of apathy divided along three subcomponents (cognition/planning, initiation, emotional-affective/motivation) with specific neuroanatomical and cognitive substrates. To increase consistency in clinical practice, a recommendation is made to modify the bvFTD diagnostic criteria of apathy/inertia. More generally, we argue that bvFTD constitutes a disease model to study the neurocircuitry of complex behaviours as a 'lesion-based' approach to neuropsychiatric symptoms observed across diagnostic categories.
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J. Neurol. Neurosurg. Psychiatr. · Apr 2018
Imaging the pathoanatomy of amyotrophic lateral sclerosis in vivo: targeting a propagation-based biological marker.
Neuropathological studies in amyotrophic lateral sclerosis (ALS) have shown a dissemination in a regional sequence in four anatomically defined patterns. The aim of this retrospective study was to see whether longitudinal diffusion tensor imaging (DTI) data support the pathological findings. ⋯ The DTI-based protocol was able to image the disease patterns of ALS in vivo cross-sectionally and longitudinally, in support of DTI as a technical marker to image ALS stages.
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J. Neurol. Neurosurg. Psychiatr. · Apr 2018
Comparison of elevated phosphorylated neurofilament heavy chains in serum and cerebrospinal fluid of patients with amyotrophic lateral sclerosis.
Phosphorylated neurofilament heavy chain (pNfH) levels are elevated in cerebrospinal fluid (CSF) of patients with amyotrophic lateral sclerosis (ALS). Instead of CSF, we explored blood as an alternative source to measure pNfH in patients with ALS. ⋯ CSF and serum pNfH concentrations are elevated in patients with ALS and correlate with the disease progression rate. Moreover, CSF pNfH correlates with the burden of motor neuron dysfunction. Our findings encourage further pursuit of CSF and serum pNfH concentrations in the diagnostic pathway of patients suspected to have ALS.
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J. Neurol. Neurosurg. Psychiatr. · Apr 2018
Prevalence of sleep apnoea and capnographic detection of nocturnal hypoventilation in amyotrophic lateral sclerosis.
This retrospective study aimed to investigate whether overnight oxymetry and early morning blood gas analysis predict nocturnal hypoventilation (NH) as reflected by night-time hypercapnia in patients with amyotrophic lateral sclerosis (ALS). In addition, prevalence and clinical determinants of sleep apnoea in ALS were evaluated. ⋯ In ALS, sleep apnoea is common and often accompanies NH. It is mainly obstructive, and central apnoea appears to be clinically irrelevant. Polygraphy or oxymetry alone are not sufficient to uncover NH. Combination of EMBE and t<90% may increase sensitivity, but transcutaneous capnography is strongly recommended for reliable detection of NH in patients with ALS.