European journal of neurology : the official journal of the European Federation of Neurological Societies
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Amyotrophic lateral sclerosis (ALS) is a motor neuron disorder, although extra-motor degeneration is well recognized, especially in frontotemporal regions manifested as ALS with frontotemporal dementia (ALS-FTD). Previous neuroimaging studies of the brains of ALS-FTD patients have measured abnormalities of either grey matter (GM) or white matter (WM) structures but not of both together. Therefore, the aim was to evaluate both GM and WM in the same ALS-FTD patient using functional and structural neuroimaging. By doing so, insights could be gained into whether neurodegeneration in ALS-FTD is primarily a neuronopathy or axonopathy. ⋯ Abnormalities in significantly fewer WM networks underlying the affected cortical regions suggest that neurodegeneration in brains of ALS-FTD patients is primarily a 'neuronopathy' rather than an 'axonopathy.'
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Idiopathic normal pressure hydrocephalus (iNPH) is a clinical entity without established pathological hallmarks. Previous autopsy studies reported that patients with an antemortem diagnosis of iNPH had a different postmortem diagnosis, commonly progressive supranuclear palsy (PSP). Disproportionately enlarged subarachnoid space hydrocephalus (DESH) has been reported as a characteristic feature of iNPH on magnetic resonance imaging (MRI). In addition, periventricular white matter hyperintensities (PVHs) are noted in most patients with iNPH; these PVHs are supposed to reflect transependymal movement of ventricular cerebrospinal fluid. It is hypothesized that PSP develops more iNPH-like MRI features than other neurodegenerative disorders. ⋯ This study demonstrated that some PSP patients develop iNPH-like MRI features, suggesting the presence of iNPH-like features in the clinical spectrum of PSP. A clinical phenotype of PSP with hydrocephalus is proposed, which should be further investigated in future studies.