Association of Circumscribed Subcortical Gray and White Matter Lesions With Apraxic Deficits in Patients With Left Hemisphere Stroke
The findings provide new insights into the subcortical anatomy of apraxia after LH stroke, suggesting a specific contribution of caudate nucleus lesions to apraxic deficits.
KEY FINDINGS: These findings provide new insights into the subcortical anatomy of apraxia after LH stroke, suggesting a specific contribution of caudate nucleus lesions to apraxic deficits.
BACKGROUND: Apraxia is commonly attributed to left hemisphere (LH) lesions of the cortical fronto-temporo-parietal praxis networks or white matter lesions causing disconnections between cortical nodes. By contrast, the contribution of lesions to the subcortical gray matter, that is, basal ganglia or thalamus, to apraxic deficits remains controversial. Here, we investigate whether damage to these subcortical gray matter structures (i.e., caudate nucleus, putamen, globus pallidus, and thalamus) or the adjacent white matter tracts was associated with apraxic deficits.
DETAILS: Identified patients with distinct subcortical lesions with and without apraxia from a large retrospective sample of subacute LH ischemic stroke patients (n = 194). To test which subcortical structures (caudate nucleus, putamen, globus pallidus, thalamus, and adjacent white matter tracts), when lesioned, contributed to apraxic deficits, we statistically compared the proportion of lesioned voxels within subcortical gray and white matter structures between the apraxic and nonapraxic patients. Of the 194 stroke patients screened, 39 (median age = 65 years, range 30-82 years; median time poststroke at the apraxia assessment = 7 days, range 1-44 days) had lesions confined to subcortical regions (gray and white matter). Eleven patients showed apraxic deficits when imitating gestures or pantomiming object use. Region-wise statistical lesion comparison (controlled for lesion size) revealed a more significant proportion of damage ('lesion load') in the caudate nucleus in apraxic stroke patients (mean difference = 6.9%, 95% CI 0.4-13.3, p = 0.038, ηp2 = 0.11). By contrast, apraxic patients had lower lesion load in the globus pallidus (mean difference = 9.9%, 95% CI 0.1-19.8, p = 0.048, ηp2 = 0.10), whereas the lesion load in other subcortical structures (putamen, thalamus, and adjacent white matter tracts) did not differ significantly between the apraxic and nonapraxic patients.
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Source: Schmidt, C. C., Achilles, E. I. S., Bolte, K., et al. (2023). Association of Circumscribed Subcortical Gray and White Matter Lesions With Apraxic Deficits in Patients With Left Hemisphere Stroke. American Academy of Neurology. 2023; 101 (11): Published: September 12, 2023. DOI: 10.1212/WNL.0000000000207598.
Developmental Trajectories of Autism
Most autistic individuals show improved communication and social functioning as they age, but not all do. Trajectory group membership is correlated with socioeconomic status. Future research should investigate what drives these correlations.
KEY FINDINGS: Most autistic individuals show improved communication and social functioning as they age, but not all do. Trajectory group membership is correlated with socioeconomic status. Future research should investigate what drives these correlations.
BACKGROUND: The goal of this study was to describe the typical, longitudinal, developmental trajectories of communication and social functioning in individuals with autism spectrum disorder from childhood through adulthood and to determine the correlates of these trajectories.
DETAILS: Children with autism spectrum disorder who were born in California from 1992 through 2016 and enrolled with the California Department of Developmental Services were identified. Subjects with <4 evaluations in the database were excluded, resulting in a sample of 71,285 individuals. Score sequences were constructed based on evaluative items for communication and social functioning. Typical trajectories were identified using group-based latent trajectory modeling, and logistic regression was used to determine the odds of classification into a social adolescent decline trajectory by individual-, family-, and zip code-level factors. Six typical patterns of communication functioning and 7 typical patterns of social functioning were identified. Whereas the majority of autistic individuals exhibit improved communication functioning as they age, the majority of individuals exhibit steady social functioning. A small group of individuals (5.0%) exhibits high social functioning in childhood that declines in adolescence. Membership in this adolescent decline group is associated with maternal non-Hispanic white race and ethnicity, female sex, moderate levels of maternal education, lower zip code-level median home values and population density, and higher zip code-level inequality.
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Source: Fountain, C., Winter, A. S., Cheslack-Postava, K., et al. (2023). Developmental Trajectories of Autism . XXXXXXXX. 2023; 152(3): e2022058674. Published: September, 2023. DOI: 10.1542/peds.2022-058674.
Nitrous Oxide-Induced Myeloneuropathy
Preventable neurological harm from N2O abuse is increasingly seen worldwide. Ease of access to canisters and larger cylinders of N2O has led to an apparent rise in cases of N2O-myeloneuropathy in several areas of the UK.
KEY FINDINGS: Preventable neurological harm from N2O abuse is increasingly seen worldwide. Ease of access to canisters and larger cylinders of N2O has led to an apparent rise in cases of N2O-myeloneuropathy in several areas of the UK. The results highlight the range of clinical manifestations in a large group of patients to improve awareness of risk, aid early recognition, and promote timely treatment.
BACKGROUND: Nitrous oxide (N2O) is the second most common recreational drug used by 16- to 24-year-olds in the UK. Neurological symptoms can occur in some people that use N2O recreationally, but most information comes from small case series.
DETAILS: Authors describe 119 patients with N2O-myeloneuropathy seen at NHS teaching hospitals in three of the UK's largest cities: London, Birmingham and Manchester. This work summarises the clinical and investigative findings in the largest case series to date. Paraesthesia was the presenting complaint in 85% of cases, with the lower limbs more commonly affected than the upper limbs. Gait ataxia was common, and bladder and bowel disturbance were frequent additional symptoms. The mid-cervical region of the spinal cord (C3-C5) was most often affected on MRI T2-weighted imaging. The number of N2O canisters consumed per week correlated with methylmalonic acid levels in the blood as a measure of functional B12 deficiency (rho (ρ)=0.44, p=0.04).
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Source: Mair, D., Paris, A., Zaloum, S. A., et al. (2023). Nitrous Oxide-Induced Myeloneuropathy: A Case Series. Journal of Neurology, Neurosurgery & Psychiatry. 2023; 94(9): 681-688. Published: September, 2023. DOI: 10.1136/jnnp-2023-331131.
Longitudinal Analysis of Respiratory Function of Different Types of Limb Girdle Muscular Dystrophies Reveals Independent Trajectories
Respiratory dysfunction is a frequent complication of patients with LGMDs that needs to be carefully studied and has direct implications in the care offered in daily clinics. Respiratory dysfunction is associated with disease progression because it is especially seen in patients who are full-time wheelchair users, being more frequent in patients with mutations in the Fkrp and sarcoglycan genes.
KEY FINDINGS: Respiratory dysfunction is a frequent complication of patients with LGMDs that needs to be carefully studied and has direct implications in the care offered in daily clinics. Respiratory dysfunction is associated with disease progression because it is especially seen in patients who are full-time wheelchair users, being more frequent in patients with mutations in the Fkrp and sarcoglycan genes.
BACKGROUND: The prevalence and progression of respiratory muscle dysfunction in patients with limb girdle muscular dystrophies (LGMDs) has been only partially described to date. Most reports include cross-sectional data on a limited number of patients making it difficult to gain a wider perspective on respiratory involvement throughout the course of the disease and to compare the most prevalent LGMD subtypes.
DETAILS: Reviewed the results of spirometry studies collected longitudinally in our cohort of patients in routine clinical visits from 2002 to 2020 along with additional clinical and genetic data. A linear mixed model was used to investigate the factors associated with the progression of respiratory dysfunction. Followed up 156 patients with 5 different forms of LGMDs for a median of 8 years (range 1–25 years). Of them, 53 patients had pathogenic variants in the Capn3 gene, 47 patients in the Dysf gene, 24 patients in the Fkrp gene, 19 in the Ano5 gene, and 13 in one of the sarcoglycan genes (SCG). At baseline, 58 patients (37.1%) had a forced vital capacity percentage predicted (FVCpp) below 80%, while 14 patients (8.9%) had peak cough flow (PCF) values below 270 L/min. As a subgroup, FKRP was the group with a higher number of patients having FVC <80% and/or PCF <270 L/min at initial assessment (66%). We observed a progressive decline in FVCpp and PCF measurements over time, being age, use of wheelchair, and LGMD subtype independent factors associated with this decline. Fkrp and sarcoglycan patients had a quicker decline in their FVC (Kaplan–Meier curve, F test, p < 0.001 and p = 0.02, respectively). Only 7 of the 58 patients with low FVCpp values reported symptoms of respiratory dysfunction, which are commonly reported by patients with FVCpp below 50%–60%. The number of patients ventilated increased from 2 to 8 during follow-up.
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Source: Muni-Lofra, R., Juanola-Mayos, E., Schiava, M., et al. (2023). Longitudinal Analysis of Respiratory Function of Different Types of Limb Girdle Muscular Dystrophies Reveals Independent Trajectories. Neuro Genetics. 2023; 9(4): e200084. Published: August, 2023. DOI: 10.1212/NXG.0000000000200084.
Neurofilament Light and Its Association With CNS Involvement in Patients With Classic Infantile Pompe Disease
Found significant differences in NfL levels between patients and controls and a good association between NfL and cognition. NfL deserves further exploration as a biomarker for CNS involvement in patients with classic infantile Pompe disease.
KEY FINDINGS: Found significant differences in NfL levels between patients and controls and a good association between NfL and cognition. NfL deserves further exploration as a biomarker for CNS involvement in patients with classic infantile Pompe disease.
BACKGROUND: Enzyme replacement therapy (ERT) has substantially improved the outcome of classic infantile Pompe disease, an inheritable muscle disease previously fatal at infancy. However, under treatment, patients develop white matter abnormalities and neurocognitive problems. Therefore, upcoming therapies also target the brain. Currently, biomarkers reflecting CNS involvement are lacking. We aimed to study the association of neurofilament light (NfL) and CNS involvement.
DETAILS: To investigate the potential of NfL, we analyzed serum samples of patients with classic infantile Pompe disease who were treated with ERT. The samples were collected at ages of <1, 5, and 10 years, as well as around MRI scans. We compared the outcomes with levels in age- and sex-matched peers. Control samples were originally collected as part of routine blood work in children who underwent small surgeries and stored in the biobank of the Erasmus MC/Sophia Children's Hospital. The authors analyzed 74 serum samples of 17 patients collected at ages ranging from 22 days to 21.2 years (1-8 samples per patient) and compared these with outcomes of 71 matched peers. In the first year of age, NfL levels in patients and controls were similar (10.3 vs 11.0 pg/mL), but mixed linear model analysis showed a yearly increase of NfL of 6.0% in patients, compared with a decrease of 8.8% in controls (p < 0.001). Higher NfL was associated with lower IQ scores (p = 0.009) and lower processing speed scores (p = 0.001).
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Source: Mackenbach, M. J., Willemse, E. A. J., van den Dorpel, J. J. A., et al. (2023). Neurofilament Light and Its Association With CNS Involvement in Patients With Classic Infantile Pompe Disease. American Academy of Neurology. 2023; 101(6): e594-e601. Published: August, 2023. DOI: 10.1212/WNL.0000000000207482.
Ischemic Stroke Management: Posthospitalization and Transition of Care
Poststroke problems with mobility, balance, cognition, dysphagia, and depression are common. Rehabilitation involves a multidisciplinary, multimodal approach that includes physical therapy, speech therapy, and treatment of chronic pain and poststroke depression.
KEY FINDINGS: Poststroke problems with mobility, balance, cognition, dysphagia, and depression are common. Rehabilitation involves a multidisciplinary, multimodal approach that includes physical therapy, speech therapy, and treatment of chronic pain and poststroke depression.
BACKGROUND: Ischemic stroke is the result of a sudden blockage of blood flow to the central nervous system due to thrombotic or embolic phenomena, and it is a major cause of morbidity and mortality worldwide. In 2019, stroke was the fifth leading cause of death in the United States. Although stroke can be hemorrhagic or ischemic, approximately 87% of strokes in the United States are ischemic. Almost 800,000 strokes occur annually in the United States, often leading to long-term disability.
DETAILS: Ischemic stroke is a major cause of morbidity and mortality worldwide. Ischemic stroke and transient ischemic attack exist on a continuum of the same disease process. Ischemic stroke is common, and more than 85% of stroke risk is attributed to modifiable risk factors. The initial management of acute stroke is usually performed in the emergency department and hospital settings. Family physicians have a key role in follow-up, ensuring that a complete diagnostic evaluation has been performed, addressing modifiable risk factors, facilitating rehabilitation, and managing chronic sequelae. Secondary prevention of ischemic stroke includes optimization of chronic disease management (e.g., hypertension, type 2 diabetes mellitus, dyslipidemia), nonpharmacologic lifestyle interventions (e.g., diet changes, exercise, substance use counseling), and pharmacologic interventions. Dual antiplatelet therapy with aspirin and clopidogrel is generally indicated for minor noncardioembolic ischemic strokes and high-risk transient ischemic attacks and should be converted to single antiplatelet therapy after 21 to 90 days. Secondary prevention of cardioembolic stroke requires long-term anticoagulation. Direct oral anticoagulants are preferred over warfarin for patients with nonvalvular atrial fibrillation.
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Source: Larson, S. T., Ray, B. E., and Wlbur, J., (2023). Ischemic Stroke Management: Posthospitalization and Transition of Care. Am Fam Physician.. 2023; 108(1):70-77. Published: July, 2023. DOI: XXXXXXXX.
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