Stereotypy: Literature review

Stereotypies have been defined in a variety of ways, but are generally accepted to be involuntary movements that occur repeatedly in the same patterned. These movements are seen in a variety of neurological and psychological conditions but are perhaps less well recognised than other types of movement disorder. Various aspects of stereotypies including pathophysiology, differential diagnosis and treatment have been reviewed

24 hour-ambulatory blood pressure and heart rate profiles in diagnosing orthostatic hypotension in Parkinson’s disease and Multiple System Atrophy

Background:24-hour ambulatory blood pressure and heart rate monitoring (24hr-ABPM) can provide vital information on circadian blood pressure (BP) profiles, which are commonly abnormal in Parkinson’s disease with and without autonomic failure (PD+AF and PD) and multiple system atrophy (MSA). 24hr-ABPM has not been directly compared between these disorders regarding cardiovascular autonomic function. We aim to determine the usefulness of 24hr-ABPM with diary compared to Head-up Tilting (HUT) in diagnosing orthostatic hypotension (OH) in these patients. Methods: 74 patients (23 MSA,18 PD+AF,33 PD) underwent cardiovascular autonomic screening followed by 24hr-ABPM with diary. Standing tests were included during 24hr-ABPM. The sensitivity and specificity in detecting OH from the 24hr-ABPM standing test were compared with HUT. Results: There was no difference in OH during HUT between MSA and PD+AF (p>0.05). MSA and PD+AF had a higher proportion of abnormal BP circadian rhythms compared to PD (p0.05). Patients were divided into groups with (OH+) and without OH (OH-) on HUT. Using the standing test during 24hr-ABPM, a SBP fall of >20 mmHg showed a sensitivity and specificity of 82% and 100 % (AUC 0.91, 95% CI 0.84-0.98) in differentiating OH+ from OH-, respectively. Conclusions:PD+AF and MSA patients had similar circadian BP patterns suggesting that autonomic dysfunction influences abnormal BP circadian patterns similarly in these disorders. The higher sensitivity and specificity in detecting OH using a SBP fall of >20 mmHg compared to a DBP fall of >10 mmHg during standing test supports its usefulness to assess autonomic function in MSA and PD

Non-dipping nocturnal blood pressure and psychosis parameters in Parkinson disease

Conclusion: These results suggest that, among PD patients, a non-dipping circadian rhythm is associated with more severe symptoms of psychosis than is a dipping circadian rhythm. This association warrants further investigation

Lower urinary tract dysfunction in Parkinsonian syndromes

Purpose of review: The aim of this review is to outline the clinical presentation, pathophysiology and evaluation of lower urinary tract (LUT) dysfunction in Parkinson’s disease and other parkinsonian syndromes including multiple system atrophy, dementia with Lewy bodies, progressive supranuclear palsy and corticobasal degeneration. // Recent findings: LUT dysfunction commonly occurs in neurological disorders, including patients with parkinsonian syndromes. The pattern of LUT dysfunction and its severity are variable, depending upon the site of lesion within the neural pathways. Parkinsonian syndromes are broadly divided into Parkinson’s disease (PD) and a typical parkinsonian syndromes such as multiple system atrophy (MSA), dementia with Lewy bodies (DLB), progressive supranuclear palsy (PSP) and corticobasal degeneration (CBD). Different parkinsonian syndromes have distinct clinical features (e.g. dysautonomia, early dementia, supranuclear gaze palsy, higher cortical signs), and the pattern of LUT dysfunction and its severity can differ. // Conclusions: LUT dysfunction is a common feature in patients with parkinsonian syndromes. Recognising the pattern of LUT dysfunction during the assessment of these patients can help management and possibly facilitate an earlier diagnosis

Sudomotor and cardiovascular dysfunction in patients with early untreated Parkinson's disease.

BACKGROUND: According to Braak staging of Parkinson's disease (PD), detection of autonomic dysfunction would help with early diagnosis of PD. OBJECTIVE: To determine whether the autonomic nervous system is involved in the early stage of PD, we evaluated cardiovascular and sudomotor function in early untreated PD patients. METHODS: Orthostatic blood pressure regulation, heart rate variability, skin vasomotor function, and palmar sympathetic sweat responses were examined in 50 early untreated PD patients and 20 healthy control subjects. RESULTS: The mean decrease in systolic blood pressure during head-up tilt in PD patients was mildly but significantly larger than in controls (p = 0.0001). There were no differences between the 2 groups in heart rate variability, with analysis of low frequency (LF; mediated by baroreflex feedback), and high frequency (HF; mainly reflecting parasympathetic vagal) modulation. However, LF/HF, an index of sympatho-parasympathetic balance, was lower in the PD group than in controls (p = 0.02). Amplitudes of palmar sweat responses to deep inspiration (p = 0.004), mental arithmetic (p = 0.01), and exercise (p = 0.01) in PD patients were lower than in controls, with negative correlations with motor severity. Amplitudes of palmar skin vasomotor reflexes in PD patients did not differ from controls. CONCLUSIONS: Our study indicates impairment of sympathetic cardiovascular and sudomotor function with orthostatic dysregulation of blood pressure control, reduced LF/HF and reduction in palm sweat responses even in early untreated PD patients

Multi-modal Biomarkers Quantify Recovery in Autoimmune Autonomic Ganglionopathy

Objective: To evaluate patients with ganglionic acetylcholine receptor antibody (gAChR‐Ab) positive autoimmune autonomic ganglionopathy using a multi‐modal testing protocol to characterise their full clinical phenotype and explore biomarkers to quantify immunotherapy response. Methods: Cohort study of thirteen individuals (seven female; 21–69 years) with autonomic failure and gAChR‐Ab>100pM identified between 2005–2019. From 2018, all patients were longitudinally assessed with cardiovascular, pupillary, urinary, sudomotor, lacrimal and salivary testing, and COMPASS‐31 autonomic symptom questionnaires. The orthostatic intolerance ratio was calculated by dividing change in systolic blood pressure over time tolerated on head‐up tilt. Eleven patients received immunotherapy. Results: At first assessment, all 13 patients had cardiovascular and pupillary impairments, 7/8 had post‐ganglionic sudomotor dysfunction, 9/11 had urinary retention and xeropthalmia, and 6/8 had xerostomia. After immunotherapy, there were significant improvements in orthostatic intolerance ratio (33.3[17.8–61.3] to 5.2[1.4–8.2], P = .007), heart rate response to deep breathing (1.5[0.0–3.3] to 4.5[3.0–6.3], P = .02), pupillary constriction to light (12.0[5.5–18.0] to 19.0[10.6–23.8]%, P = .02), saliva production (0.01[0.01–0.05] to 0.08[0.02–0.20]g/min, P = .03) and COMPASS‐31 scores (52 to 17, P = .03). Orthostatic intolerance ratio correlated with autonomic symptoms at baseline (r = 0.841, P = .01) and following immunotherapy (r = 0.889, P = .02). Immunofluorescence analyses of skin samples from a patient 32 years after disease onset showed loss of nerve fibres supplying the dermal autonomic adnexa and epidermis, with clear improvements following immunotherapy. Interpretation: Patients with autoimmune autonomic ganglionopathy demonstrated objective evidence of widespread sympathetic and parasympathetic autonomic failure, with significant improvements after immunotherapy. Quantitative autonomic biomarkers should be used to define initial deficits, guide therapeutic decisions, and document treatment response

Effects of Onion (Allium cepa L.) Extract Administration on Intestinal α-Glucosidases Activities and Spikes in Postprandial Blood Glucose Levels in SD Rats Model

Diets high in calories and sweetened foods with disaccharides frequently lead to exaggerated postprandial spikes in blood glucose. This state induces immediate oxidant stress and free radicals which trigger oxidative stress-linked diabetic complications. One of the therapeutic approaches for decreasing postprandial hyperglycemia is to retard absorption of glucose by the inhibition of carbohydrate hydrolyzing enzymes, α-amylase and α-glucosidases, in the digestive organs. Therefore, the inhibitory activity of Korean onion (Allium cepa L.) extract against rat intestinal α-glucosidases, such as sucrase, maltase, and porcine pancreatic α-amylase were investigated in vitro and in vivo. The content of quercetin in ethyl alcohol extract of onion skin (EOS) was 6.04 g/100 g dried weight of onion skin. The in vitro half-maximal inhibitory concentrations (IC50) of EOS and quercetin, a major phenolic in onion, on rat intestinal sucrase were 0.40 and 0.11 mg/mL, respectively. The postprandial blood glucose lowering effects of EOS and quercetin were compared to a known type 2 diabetes drug (Acarbose), a strong α-glucosidase inhibitor in the Sprague-Dawley (SD) rat model. In rats fed on sucrose, EOS significantly reduced the blood glucose spike after sucrose loading. The area under the blood glucose-time curve (AUClast) in EOS-treated SD rats (0.5 g-EOS/kg) was significantly lower than in untreated SD rats (259.6 ± 5.1 vs. 283.1 ± 19.2 h·mg/dL). The AUClast in quercetin-treated SD rats (0.5 g-quercetin/kg) was similar to in EOS-treated group (256.1 ± 3.2 vs. 259.6 ± 5.1 h·mg/dL). Results from this study indicates that although quercetin does have blood glucose lowering potential via α-glucosidase inhibition, there are other bioactive compounds present in onion skin. Furthermore, the effects of two weeks administration of EOS in a high carbohydrate-dietary mixture (Pico 5053) on sucrase and maltase activities in intestine were evaluated in SD rat model. Compared to the upper and middle parts of intestine, the activities of sucrase in the lower parts of intestine remained significantly higher after two weeks of EOS treatment. These results indicate that EOS may improve exaggerated postprandial spikes in blood glucose and glucose homeostasis since it inhibits intestinal sucrase and thus delays carbohydrate absorption, although clinical trials are needed

Autonomic function and non-motor symptoms in primary chronic autonomic failure disorders

The autonomic nervous system innervates and influences every organ in the body through two major efferent pathways; the sympathetic and parasympathetic nervous systems. Autonomic dysfunction, especially orthostatic hypotension (OH) and olfactory dysfunction, are commonly present in a variety of neurological disorders, particularly, multiple system atrophy (MSA) and allied disorders, such as Parkinson’s disease (PD) and Pure Autonomic Failure (PAF) and can significantly impact quality of life and cause significant morbidity. Similarly, non-motor symptoms have been increasingly recognized in PD. The overlapping autonomic features of PD, PAF and MSA, e.g., OH, can sometimes make it difficult to differentiate a diagnosis between these conditions. The further evaluation of autonomic function, e.g., cardiovascular, olfactory and gastrointestinal function, in patients with primary chronic autonomic failure disorders could offer better diagnostic accuracy, improve the understanding of disease progression and inform the development of treatments. Cardiovascular autonomic function screening tests are commonly used to confirm a diagnosis of autonomic failure, e.g., orthostatic hypotension, but OH is not often reliable for distinguishing between PD and MSA. Novel indices of cardiovascular autonomic function in patients with chronic autonomic failure have therefore been evaluated as well as the severity of olfactory dysfunction and other non-motor (e.g., daily activities and depression) symptoms in MSA, PD and PAF. Results demonstrated that baroreflex sensitivity and blood pressure recovery time (BRPT) in response to the Valsalva Manoeuvre are useful for differentiating MSA from PD with autonomic failure (PD+AF). BPRT was also significantly prolonged in PD patients compared to healthy controls. In addition, an association of BPRT and disease duration in PD also suggests that this index may be useful for monitoring disease progression in PD. Other findings indicated that assessing olfactory function is also helpful for distinguishing between PD, MSA and PAF. A greater degree of depression and impairment of daily activities in MSA relative to PD and PAF were also evident. In order to further investigate the presenting symptoms and features of PAF, a time when diagnosis is often still unclear, and other disorders, such as MSA, can be suspected, the clinical characteristics and laboratory investigations, in a large cohort of PAF patients were examined. Results indicated that abnormal white matter lesions are prevalent in PAF. Furthermore, gastrointestinal symptoms were also evident in PAF and can also occur in other autonomic disorders, e.g., MSA. Using electrogastrography, impaired indexes of gastric motility were also evident in PAF.Open Acces

ORAL HEALTH CONDITIONS AND BEHAVIORS AMONG HEARING IMPAIRED AND NORMAL HEARING COLLEGE STUDENTS AT RATCHASUDA COLLEGE, NAKHON PATHOM, THAILAND

Abstract. This study aimed to explore oral health and oral health related behaviors among hearing impaired and normal hearing students at Rachasuda College. The association between socioeconomic factors, hearing status, oral health behaviors, oral hygiene levels and dental caries status were also examined. The students filled out a self-administered questionnaire with assistance of a sign language video to obtain personal and behavior information. A total of 180 students, 83 normal hearing and 97 hearing impaired students completed the questionnaire and underwent an oral examination. The prevalences of caries were 53.6% and 50.6% among students with hearing impairment and normal hearing, respectively (p=0.354). After age stratification, the hearing impaired students aged 18-21 years had significantly less filled teeth (p=0.012), and those older than 21 years had less missing teeth due to caries than normal-hearing students (p=0.023). Poor oral hygiene was found in 51.8% and 42.2% of normal and hearing-impaired students, respectively (p=0.365). Caries status was significantly associated with maternal education level (OR 3.56; 95% CI: 1.52-8.32) and oral hygiene (OR 3.26; 95% CI: 1.64-6.45). The high prevalence of dental caries and poor oral hygiene among college students is alarming. Hearing impairment did not appear to affect the prevalences of these conditions compared to those with normal hearing. Oral health education tools need to be developed and utilized for both normal hearing and hearing impaired college students in Thailand