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Abstract: Syncope associated to orthostatic hypotension (OH), urinary incontinence and constipation is common symptoms in demented patients, mainly in dementia with Lewy bodies (DLB) and in Parkinson''s disease dementia (PDD). Alzheimer''s disease (AD) and fronto temporal lobar degeneration (FTLD) show less autonomic dysfunction. Urinary symptoms are a prominent component of normal pressure hydrocephalus (NPH). There are non invasive tests including standard cardiovascular tests, 123 I-metaiodobenzylguanide (MIBG) cardiac scintigraphy, urodynamic tests, gastrointestinal motility studies, sweating reflexes and pupillary responses that assess autonomic dysfunction in these patients. The study of autonomic symptoms and abnormal tests in patients with dementia is useful to prevent morbidity due falls, severe constipation and to avoid side effects of drugs that interfere with autonomic function. [Copyright &y& Elsevier]

Encephalitis due to leucine-rich glioma inactivated 1 (Lgi1) antibodies, a subtype of VGKC-complex encephalitis, results in characteristic faciobrachial dystonic seizures (FBDS) [1]. Provided the circumscribed set of reactive and inflammatory pathologies in the limbic region and amygdala, the main entities in the differential diagnosis are more common forms of limbic encephalitis and viral encephalitis in particular. In cases of possible limbic encephalitis without a premortem diagnosis (i.e. the question is raised by neuropathologic findings), sera and/or cerebrospinal fluid collected at autopsy can be submitted for an autoimmune encephalopathy panel to clarify the final diagnosis. [Extracted from the article]

During 1991 to 1993, an epidemic of neurologic disease in Cuba affected 50,862 patients with optic neuropathy, sensorineural deafness, predominantly sensory peripheral neuropathy, and dorsolateral myelopathy. The clinical syndromes were identical to those of prisoners of war subjected to nutritional restriction in tropical prison camps during World War II (Strachan's disease). A dietary deficiency of group B vitamins and sulfur-containing amino acids appears to have been the primary cause of the epidemic. This was a consequence of economic and political events in Cuba linked to the collapse of the Soviet Union and socialist countries. The recently toughened 30-year-old US economic embargo on Cuba contributed to these problems and hampered the investigation, treatment, and prevention of the epidemic. A plea is made to the neurologic community to request the lifting of the trade blockade on a humanitarian basis. [ABSTRACT FROM AUTHOR]

Discusses the effect of the United States trade embargo against Cuba on the health condition of the nervous system of local people. Clinical features in the course of the epidemic; Factors leading to the outbreak of the nervous system epidemic diseases; Recommendations for the lifting of the trade embargo against Cuba on grounds of humanitarian reasons.

A cross-sectional prevalence study of Parkinson's disease in China was undertaken in 1997–98. We assessed 29454 residents aged ≥55 years from 79 rural and 58 urban communities in Beijing, Xian, and Shanghai, who were selected through stratified, multistage, cluster sampling. With a standardised diagnostic protocol, we identified 277 people with the disease. Prevalence of Parkinson's disease for those aged ≥65 years was 1.7% (95% CI 1.5–1.9). We estimate that 1.7 million people in China have the disease (aged ≥55 years). Our findings suggest that prevalence of Parkinson's disease in China is similar to that in developed countries. [ABSTRACT FROM AUTHOR]

Trial registration: ClinicalTrials.gov Identifier: NCT05821153, Registered April 20 2023, Retrospectively registered, https://classic.clinicaltrials.gov/ct2/show/NCT05821153 [ABSTRACT FROM AUTHOR]

A letter to the editor is presented in response to the article related to lack of diagnostic criteria for the paucity of epidemiologic studies of vascular dementia by Ingmar Skoog and colleagues in the January 21, 2007 issue.

Transactive response (TAR) DNA-binding protein 43 kDa (TDP-43) pathology is a hallmark of limbic-predominant age-related TDP-43 encephalopathy (LATE). The amygdala is affected early in the evolution of LATE neuropathologic change (LATE-NC), and heterogeneity of LATE-NC in amygdala has previously been observed. However, much remains to be learned about how LATE-NC originates and progresses in the brain. To address this, we assessed TDP-43 and other pathologies in the amygdala region of 184 autopsied subjects (median age = 85 years), blinded to clinical diagnoses, other neuropathologic diagnoses, and risk genotype information. As previously described, LATE-NC was associated with older age at death, cognitive impairment, and the TMEM106B risk allele. Pathologically, LATE-NC was associated with comorbid hippocampal sclerosis (HS), myelin loss, and vascular disease in white matter (WM). Unbiased hierarchical clustering of TDP-43 inclusion morphologies revealed discernable subtypes of LATE-NC with distinct clinical, genetic, and pathologic associations. The most common patterns were: Pattern 1, with lamina II TDP-43 + processes and preinclusion pathology in cortices of the amygdala region, and frequent LATE-NC Stage 3 with HS; Pattern 2, previously described as type-β, with neurofibrillary tangle-like TDP-43 neuronal cytoplasmic inclusions (NCIs), high Alzheimer's disease neuropathologic change (ADNC), frequent APOE ε4, and usually LATE-NC Stage 2; Pattern 3, with round NCIs and thick neurites in amygdala, younger age at death, and often comorbid Lewy body disease; and Pattern 4 (the most common pattern), with tortuous TDP-43 processes in subpial and WM regions, low ADNC, rare HS, and lower dementia probability. TDP-43 pathology with features of patterns 1 and 2 were often comorbid in the same brains. Early and mild TDP-43 pathology was often best described to be localized in the "amygdala region" rather than the amygdala proper. There were also important shared attributes across patterns. For example, all four patterns were associated with the TMEM106B risk allele. Each pattern also demonstrated the potential to progress to higher LATE-NC stages with confluent anatomical and pathological patterns, and to contribute to dementia. Although LATE-NC showed distinct patterns of initiation in amygdala region, there was also apparent shared genetic risk and convergent pathways of clinico-pathological evolution. [ABSTRACT FROM AUTHOR]

A 64-year-old man with a history of traumatic brain injury 4 years previously presented with progressive cognitive decline and gait abnormality. MRI revealed diffusion restriction in the bilateral centrum semiovale and multiple serpiginous flow voids. Cerebral angiogram revealed a total of six intracranial dural arteriovenous fistulas with separate fistulas of the right and left sphenoid bones, left clival plexus, right transverse sinus, right sigmoid sinus, and superior sagittal sinus. A diffuse pseudophlebitic pattern of venous drainage indicating severe venous hypertension was also observed. The patient underwent a series of endovascular treatments over the next 10 months to achieve resolution of all arteriovenous shunting. Repeat MRI showed resolution of the diffusion restriction and marked reduction in T2 vascular flow voids. The patient's clinical status improved significantly over the course of treatment, paralleling the improvement in venous hypertension. [ABSTRACT FROM AUTHOR]

Background: Maternal thyroid status and autoimmunity during pregnancy have been associated with impaired development of the offspring in animal and human studies. Our objective was to examine whether elevated titers of maternal thyroid peroxidase antibodies (TPOAbs) in early pregnancy increased the risk of cognitive impairment and problem behavior in preschool children. Second, we aimed at exploring to what extent any effect on child behavior was mediated by maternal thyroid parameters during pregnancy. Methods: In the Generation R Study, a population-based cohort of 3139 children and their mothers, we measured maternal thyroid parameters (thyrotropin [TSH], free Thyroxine, and TPOAbs) at 13.5±1.8 weeks of gestation. Children's verbal and nonverbal cognitive functioning was measured at 2.5 years using the Language Development Survey and the Parent Report of Children Abilities. At 3 years, children's behavior was assessed using the Child Behavior Checklist. Results: Elevated titers of TPOAbs during pregnancy did not predict the verbal and nonverbal cognitive functioning of the children. However, elevated titers of TPOAbs in mothers were associated with externalizing problems in children (odds ratio [OR] =1.64, 95% confidence interval [CI]: 1.17-2.29, p=0.004). In particular, children of TPOAb-positive mothers were at a higher risk of attention deficit/hyperactivity problems (OR =1.77, 95% CI: 1.15-2.72, p=0.01). To explore whether the effect of maternal TPOAbs on child problem behavior was mediated by maternal thyroid parameters, we added maternal TSH to the model. After correcting for TSH, the effect of TPOAbs on externalizing problems was attenuated slightly but remained significant (OR =1.56, 95% CI: 1.14, 2.14, p=0.005). Conclusions: Our findings imply that the elevated titers of TPOAbs during pregnancy impact children's risk of problem behavior, in particular, attention deficit/hyperactivity. The observed effect is only partially explained by maternal TSH levels. These findings may point to a specific mechanism of Attention Deficit/Hyperactivity Disorder in children. Nevertheless, we can only speculate about public health implication of the study, as there is no specific treatment for TPOAb-positive pregnant women with normal thyroid function. Further investigation is needed to explore whether TPOAb-positive pregnant women and their children can benefit from close monitoring and early detection of developmental delay in populations at risk. [ABSTRACT FROM AUTHOR]

To investigate the relationship between metabolic markers of cobalamin deficiency and cognitive function in normal older adults. Cross-sectional study. Queen's University and St. Mary's of the Lake Hospital, Kingston, Ontario, Canada. Two hundred eighty-one cognitively normal, community-dwelling participants aged 65 and older. Serum cobalamin, red blood cell folate, methylcitric acid, homocysteine, and methylmalonic acid were determined. Cognitive instruments included the California Verbal Learning Test, Mattis Dementia Rating Scale, and the Stroop Neuropsychological Screening Inventory (Stroop). Serum levels of methylcitric acid had a significant negative correlation with recall, learning, and discriminability (factor 1) of the California Verbal Learning Test after adjusting for age and sex (β=−0.138, P=.019). Subjects with elevated methylcitric acid had significantly lower scores (factor 1) than subjects with normal methylcitric acid ( P<.01). Bivariate analysis showed significant correlations between levels of homocysteine and the Stroop score and between cobalamin, methylmalonic acid, and homocysteine and some scores of the California Verbal Learning Test, but these relationships did not remain significant after multivariate analysis. Subjects with high homocysteine (tHcy) had lower Stroop scores than subjects with normal tHcy ( P<.05). No biochemical parameters were associated with the Mattis Dementia Rating Scale scores. This study indicates that, in normal elderly subjects, some cognitive scores are related to serum methylcitric acid and possibly homocysteine. [ABSTRACT FROM AUTHOR]