Behavioural Neurology

Objective. This study is aimed at investigating the association between the twenty-four-hour ambulatory blood pressure variability monitoring (ABPM) and cerebral small vessel disease (cSVD) burden in acute ischemic stroke (AIS) patients. Methods. 115 AIS patients with demographics, vascular risk factors, 24 h ABPM, and brain magnetic resonance imaging (MRI) were retrospectively enrolled. 3.0 T MRI was used to assess cSVD burden by combining four MRI markers including white matter hyperintensities (WMHs), cerebral microbleeds (CMBs), perivascular spaces (PVS), and lacunes. Correlation analysis was conducted to detect whether ABPM was associated with cSVD burden in AIS patients. Results. 115 AIS patients with mean age years and 75.7% male were enrolled in this study. 112 AIS patients (97.4%) had at least one cSVD marker. Spearman correlation analysis indicated that hypertension was positively correlated with cSVD burden (, ). High-density lipoprotein (HDL) was negatively correlated with cSVD burden (, ). Blood pressure variability such as 24 h mean SBP (, ), day mean SBP (, ), and night mean SBP (, ) was positively correlated with higher cSVD burden. Ordinal logistic regression analysis demonstrated that higher 24 h SBP SD and day mean SBP were independent risk factors for cSVD after controlling for other confounders. Conclusions. Higher BPV was significantly related to total cSVD burden in AIS patients. 24 h SBP SD and day mean SBP were independent risk factors for cSVD burden in AIS patients but not DBP or DBP variability.

Aromatic L-amino acid decarboxylase (AADC) deficiency is a rare congenital autosomal recessive metabolic disorder caused by pathogenic homozygous or compound heterozygous variants in the dopa decarboxylase (DDC) gene. Adeno-associated viral vector-mediated gene transfer of the human AADC gene into the putamina has become available. This systematic review on PubMed, Scopus databases, and other sources is aimed at describing the AADC whole phenotypic spectrum in order to facilitate its early diagnosis. Literature reviews, original articles, retrospective and comparative studies, large case series, case reports, and short communications were considered. A database was set up using Microsoft Excel to collect clinical, molecular, biochemical, and therapeutic data. By analysing 261 patients from 41 papers with molecular and/or biochemical diagnosis of AADC deficiency for which individuality could be determined with certainty, we found symptom onset to occur in the first 6 months of life in 93% of cases. Hypotonia and developmental delay are cardinal signs, reported as present in 73.9% and 72% of cases, respectively. Oculogyric crises were seen in 67% of patients while hypokinesia in 42% and ptosis in 26%. Dysautonomic features have been revealed in 53% and gastrointestinal symptoms in 19% of cases. With 37% and 30% of patients reported being affected by sleep and behavioural disorders, it seems to be commoner than previously acknowledged. Although reporting bias cannot be excluded, there is still a need for comprehensive clinical descriptions of symptoms at onset and during follow-up. In fact, our review suggests that most of the neurological and extraneurological symptoms and signs reported, although quite frequent in this condition, are not pathognomonic, and therefore, ADCC deficiency can remain an underdiscovered disorder.

The purpose of the study is to investigate how much of variance in Parkinson’s Disease (PD) individuals’ speech intelligibility could be predicted by seven speech fluency indicators (i.e., repetition, omission, distortion, correction, unfilled pauses, filled pauses, and speaking rate). Speech data were retrieved from a database containing a reading task produced by a group of 16 English-speaking individuals with PD (Jaeger, Trivedi & Stadtchnitzer, 2019). The results from a multiple regression indicated that an addition of 54% of variance in the speech intelligibility scores among individuals with PD could be accounted for after the speakers’ PD severity level measured based on Hoehn and Yahr’s (1967) disease stage was included as a covariate. In addition, omission and correction were the two fluency indicators that contributed to the general intelligibility score in a statistically significant way. Specifically, for every one-unit gain in the number of correction and omission, speech intelligibility scores would decline by 0.687 and 0.131 point (out of a 7-point scale), respectively. The current study hence supported Magee, Copland, and Vogel’s (2019) view that the language production abilities and quantified dysarthria measures among individuals with PD should be explored together. Additionally, the clinical implications based on the current findings were discussed.

The purpose of this study was to discuss the effect of voluntary wheel running on striatal dopamine levels and anxiety-like behavior in rats with global cerebral ischemia. The male Sprague-Dawley rats were signed on in this study and randomly divided into following 4 groups: Control group (C group), Sham group (S group), ischemia group (I group), and 3 weeks physical exercise before ischemia group (3RI group). The rats in the 3RI group were placed in a voluntary running wheel for three weeks to exercise. Then, the rats in I and 3RI groups received bilateral carotid artery ligation (2-VO) operation. The C and S group did not perform voluntary running exercise and the bilateral common carotid arteries of S group were exposed without ligation. In vivo microdialysis was used in conjunction with high performance liquid chromatography (HPLC) and electrochemical detection to ascertain the level of dopamine in the striatum. Elevated plus maze (EPM) and open field (OF) were used to test anxiety status at 24 hours and 7days after 2-VO cerebral ischemia. Meanwhile, gait and motor coordination evaluations were carried out to eliminate the influence of non-specific motor problems. The results indicated that cerebral ischemia instigate the increase of striatal dopamine in I group rats during acute cerebral ischemia. A 3-week voluntary wheel running significantly enhances the striatal dopamine before ischemia and obstructs a further increase of dopamine during acute cerebral ischemia in 3RI group rats. At 24 hours after ischemia, striatal dopamine returned to pre-ischemic levels in 3RI group. Striatal dopamine in I group were less than pre-ischemic levels at 7 days. Behavioral data indicated that 3-week voluntary wheel running promoted recovery of anxiety-like behavior and gait were not affected by 2-VO cerebral ischemia at 24 hours post-ischemia rats. Therefore, it can be concluded that 3-week physical exercise significantly increased the striatal dopamine and improved anxiety-like behavior by inhibiting the increase of dopamine during acute cerebral ischemia and suppressing the decrease of dopamine after 24 hours and 7 days cerebral ischemia.

Acceptance of illness is regarded as an indicator of functioning and predictor of quality of life. However, quality of life of patients with epilepsy in sub-Saharan countries worsen because of low medication adherence, increased morbidity and mortality, and the stigmatization associated with the disease. This research is aimed at assessing the level of acceptance of illness of patients with epilepsy and associated quality of life in North-East Ethiopia. Methods. A cross-sectional study was conducted from January to June 2021 at the Debre Berhan Referral Hospital, North-East Ethiopia. A total of 78 patients with epilepsy aged more than 18 years were randomly selected and assessed using Quality of Life in Epilepsy Inventory 31 and acceptance of illness scale. In addition, authors owned questionnaire were used to evaluate the sociodemographic and clinical characteristics of the patients. value < 0.05 at 95% confidence level was considered to be statistically significant in all the analysis. Result. The study participants’ age varied between 18 and 67 years with the mean age of 28.9 years. Phenobarbital was the most used (73.9%) antiepileptic drug, and 68.7% () of the patients seizure was controlled. 72.9% () of the patients had medium acceptance of illness (scored 20-30), while 17.7% () had low illness acceptance level (scored 8-19), and 9.4% () had high acceptance of illness (scored 31-40). The mean of overall acceptance of illness among epileptic patients was . The overall score of QOLIE-31 was , and the highest mean score was for cognitive (), while the lowest mean score was that of medication effect (). Five of the seven QOLIE-31 components correlated significantly with level of acceptance of illness. Cognitive domain (, ) demonstrated the highest correlation followed by overall quality of life (, ), seizure worry (, ), energy/fatigue (, ), and emotional well-being (, ). Conclusion. Patients with epilepsy in the study area had medium acceptance of illness, and nearly half of them had mean and more than the mean quality of life. The patients’ acceptance of illness was significantly associated with overall quality of life, seizure worry, emotional well-being, and cognitive domain of the patients.

Introduction. Primary progressive aphasia (PPA) is a clinically variable syndrome manifesting as slow progressive loss of speech and language with multiple underlying neurodegenerative pathologies. Materials and Methods. We included data from nine PPA patients with available autopsies. We then retrospectively reviewed all available medical records, neuropsychology, and MRI results to confirm the corresponding subtypes of PPA and compared them with postmortem neuropathological results. Results. Clinical presentations corresponded to the nonfluent/agrammatic variant in six cases, the semantic variant in one case, the logopenic variant in one case, and the mixed variant (concomitant nonfluent/agrammatic plus semantic variant) in one case. Patients with a broader clinical presentation, i.e., combining manifestations of one PPA subtype and symptoms of another PPA variant, had autopsy comorbidities showing multiple neurodegenerative disorders. Of the nine subjects enrolled in the study, Alzheimer’s disease (AD) was found in eight cases; however, in only one case, AD was detected as an isolated neuropathological substrate of PPA. In eight brain samples, different comorbid neuropathologies were detected: three cases with comorbid AD and dementia with Lewy bodies, two cases with comorbid AD and TDP-43 pathology, one case with comorbid AD and complex tauopathies, and one case with comorbid AD with both tau and TDP-43 deposits. Finally, one case had comorbid tau and TDP-43 pathology but without comorbid AD pathology. Conclusions. Our observation suggests that PPA cases could be more heterogeneous in their etiology than previously thought and underlying neurodegenerative comorbidities should be considered in routine practice, especially if the clinical presentation of PPA is atypical.