Brain involvement in myotonic dystrophy type 1 (DM1) is characterized by heterogeneous cognitive, behavioral, and affective symptoms and imaging alterations indicative of widespread grey and white matter involvement. The aim of the present study was to systematically review the literature on brain pathology in DM1. We conducted a structured search in EMBASE (index period 1974–2017) and MEDLINE (index period 1887–2017) on December 11, 2017, using free text and index search terms related to myotonic dystrophy type 1 and brain structures or regions. Eligible studies were full‐text studies reporting on microscopic brain pathology of DM1 patients without potentially interfering comorbidity. We discussed the findings based on the anatomical region and the nature of the anomaly. Neuropathological findings in DM1 can be classified as follows: (1) protein and nucleotide deposits; (2) changes in neurons and glial cells; and (3) white matter alterations. Most findings are unspecific to DM1 and may occur with physiological aging, albeit to a lesser degree. There are similarities and contrasts with Alzheimer's disease; both show the appearance of neurofibrillary tangles in the limbic system without plaque occurrence. Likewise, there is myelin loss and gliosis, and there are dilated perivascular spaces in the white matter resemblant of cerebral small vessel disease. However, we did not find evidence of lacunar infarction or microbleeding. The various neuropathological findings in DM1 are reflective of the heterogeneous clinical and neuroimaging features of the disease. The strength of conclusions from this study's findings is bounded by limited numbers of participants in studies, methodological constraints, and lack of assessed associations between histopathology and clinical or neuroimaging findings.

Longitudinal changes in cerebral blood flow and their relation with cognitive decline in patients with dementia: Current knowledge and future directions.

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The aim of this study was to objectively assess and compare gait capacity and gait performance in rehabilitation inpatients with stroke or incomplete spinal cord injury (iSCI) using inertial measurement units (IMUs). We investigated how gait capacity (what someone can do) is related to gait performance (what someone does). Twenty-two inpatients (11 strokes, 11 iSCI) wore ankle positioned IMUs during the daytime to assess gait. Participants completed two circuits to assess gait capacity. These were videotaped to certify the validity of the IMU algorithm. Regression analyses were used to investigate if gait capacity was associated with gait performance (i.e., walking activity and spontaneous gait characteristics beyond therapy time). The ankle positioned IMUs validly assessed the number of steps, walking time, gait speed, and stride length (r ≥ 0.81). The walking activity was strongly (r ≥ 0.76) related to capacity-based gait speed. Maximum spontaneous gait speed and stride length were similar to gait capacity. However, the average spontaneous gait speed was half the capacity-based gait speed. Gait capacity can validly be assessed using IMUs and is strongly related to gait performance in rehabilitation inpatients with neurological disorders. Measuring gait performance with IMUs provides valuable additional information about walking activity and spontaneous gait characteristics to inform about functional recovery.

Research suggests an association between cerebrovascular health and cognitive decline, but previous work is limited by its cross-sectional nature or short (< 1–2 years) follow-up. Our aim was to examine, across 10 years of follow-up in healthy older adults, changes in cerebrovascular health and their relationship with subjective memory complaints as an early marker of cognitive decline. Between 2008 and 2010, twenty-eight healthy older adults (69 ± 4 years) underwent baseline blood pressure and transcranial Doppler measurements to assess middle cerebral artery blood velocity (MCAv), cerebrovascular resistance index (CVRi), and measures of cerebral autoregulation (CA). After 9–12 years of follow-up, these measurements were repeated, and presence of memory complaints was evaluated. Linear mixed-model analyses explored effects of aging on cerebrovascular parameters and whether memory complaints were associated with cerebrovascular changes. Across a median follow-up of 10.9 years, no changes in MCAv, CVRi, or CA were found. At baseline, these parameters were not different between subjects with (n = 15) versus without (n = 13) memory complaints. During follow-up, subjects with memory complaints showed larger decreases in MCAv (− 10% versus + 9%, P = 0.041) and increases in CVRi (+ 26% versus − 9%, P = 0.029) compared to other peers without memory complaints, but no distinct changes in CA parameters (P > 0.05). Although a decade of aging does not lead to deterioration in cerebral blood flow or autoregulation, our findings suggest that reductions in cerebral blood flow and increases in cerebrovascular resistance are associated with early subjective cognitive decline.

OBJECTIVE To examine whether perioperative changes in cerebral blood flow relate to postoperative neurological deficits in patients undergoing aortic arch surgery involving antegrade selective cerebral perfusion. METHODS We retrospectively analysed data from patients who underwent aortic arch surgery involving antegrade selective cerebral perfusion and perioperative transcranial Doppler assessments. Linear mixed-model analyses were performed to examine perioperative changes in mean bilateral blood velocity in the middle cerebral arteries, reflecting changes in cerebral blood flow, and their relation with neurological deficits, i.e. ischaemic stroke and/or delirium. Logistic regression analyses were performed to explore possible risk factors for postoperative neurological deficits. RESULTS In our study population (N = 102), intraoperative blood velocities were lower compared to preoperative levels, and lowest during antegrade selective cerebral perfusion. 36 (35%) patients with postoperative neurological deficits (ischaemic stroke, n = 9; delirium, n = 25; both, n = 2) had lower blood velocity during antegrade selective cerebral perfusion compared to patients without (25.4 vs 37.0 cm/s; P = 0.002). Logistic regression analyses revealed lower blood velocity during antegrade selective cerebral perfusion as an independent risk factor for postoperative neurological deficits (odds ratio = 0.959; 95% CI: 0.923, 0.997; P = 0.037). CONCLUSIONS Lower intraoperative cerebral blood flow during antegrade selective cerebral perfusion seems independently related to postoperative neurological deficits in patients undergoing aortic arch surgery. Because cerebral blood flow is a modifiable factor during antegrade selective cerebral perfusion, our observation has significant potential to improve clinical management and prevent neurological deficits.