Autonomic function testing in long-COVID syndrome patients with orthostatic intolerance.

The association between dysautonomia and long-COVID syndrome has gained considerable interest. This study retrospectively characterized the findings of autonomic reflex screen (ARS) in long-COVID patients presenting with orthostatic intolerance (OI). Fourteen patients were identified. All patients had normal cardiovagal function and 2 patients had abnormal sudomotor function. The head-up tilt table (HUTT) was significantly abnormal in 3 patients showing postural orthostatic tachycardia syndrome (POTS). CASS ranged from 0 to 2. The most common clinical scenario was symptoms of orthostatic intolerance without demonstrable HUTT orthostatic tachycardia or orthostatic hypotension (OH) (n = 8, 57 %). In our case series, most long-COVID patients presenting to our laboratory with OI had no significant HUTT abnormalities; only 3 patients met the criteria for POTS.

Prevalence and patterns of symptoms of dysautonomia in patients with long-COVID syndrome: A cross-sectional study.

BACKGROUND: The association between autonomic dysfunction and long-COVID syndrome is established. However, the prevalence and patterns of symptoms of dysautonomia in long-COVID syndrome in a large population are lacking. OBJECTIVE: To evaluate the prevalence and patterns of symptoms of dysautonomia in patients with long-COVID syndrome. METHODS: We administered the Composite Autonomic Symptom Score 31 (COMPASS-31) questionnaire to a sample of post-COVID-19 patients who were referred to post-COVID clinic in Assiut University Hospitals, Egypt for symptoms concerning for long-COVID syndrome. Participants were asked to complete the COMPASS-31 questionnaire referring to the period of more than 4 weeks after acute COVID-19. RESULTS: We included 320 patients (35.92 ± 11.92 years, 73% females). The median COMPASS-31 score was 26.29 (0-76.73). The most affected domains of dysautonomia were gastrointestinal, secretomotor, and orthostatic intolerance with 91.6%, 76.4%, and 73.6%, respectively. There was a positive correlation between COMPASS-31 score and long-COVID duration (p < 0.001) and a positive correlation between orthostatic intolerance domain score and post-COVID duration (p < 0.001). There was a positive correlation between orthostatic intolerance domain score and age of participants (p = 0.004). Two hundred forty-seven patients (76.7%) had a high score of COMPASS-31 >16.4. Patients with COMPASS-31 >16.4 had a longer duration of long-COVID syndrome than those with score <16.4 (46.2 vs. 26.8 weeks, p < 0.001). CONCLUSIONS: Symptoms of dysautonomia are common in long-COVID syndrome. The most common COMPASS-31 affected domains of dysautonomia are gastrointestinal, secretomotor, and orthostatic intolerance. There is a positive correlation between orthostatic intolerance domain score and patients' age.

Autonomic Reflex Screen Test Abnormalities in Cold-Induced Sweating Syndrome Type 1.

Cold-induced sweating syndrome (CISS) is a rare autosomal recessive disease due to mutation in the Cytokine receptor-like factor 1 (CRLF1). The characteristic symptom of CISS is the tendency to sweat profusely especially in the upper body and hands when the patient is exposed to cold temperature. We sought to first report the findings of autonomic reflex screen in a case of CISS type 1 with Cytokine receptor-like factor 1 mutation. Valsalva morphology, Valsalva ratio, and heart rate response to deep breathing were normal for the patient's age. Quantitative sudomotor axon reflex test showed nonlength dependent decrease in the sweat volume. Tilt table revealed evidence of reflex (vasovagal) "syncope," however, the patient was asymptomatic without loss of consciousness.

Histopathologic Findings in 5 Patients With Hypomyopathic Dermatomyositis: The Importance of MHC-1 Expression on Myofibers.

Muscle histopathologic findings in hypomyopathic dermatomyositis (HDM) have not been adequately characterized. We sought to determine the results of conventional and immunohistopathology in HDM. Light microscopic and immunohistochemical analysis was performed on muscle from 5 patients with HDM without muscle weakness. Ages ranged from 49 to 56 years. Creatine kinase level was normal. Electromyography showed mild "myopathic" changes in 2. The median duration of skin disease before biopsy was 18 months. Abnormal major histocompatibility (MHC) class I immunoreactivity was noted in myofibers in all specimens even when conventional histopathology was normal (1 patient) or only mildly abnormal (3 patients). One specimen had the characteristic findings of dermatomyositis. Patchy MHC-1 expression on myofibers was a consistent finding in HDM in the absence of other histopathologic abnormalities. The presence of MHC-1 expression could indicate a degree of endoplasmic reticulum stress even in the absence of clinical muscle weakness, muscle enzyme abnormalities, or significant inflammatory infiltrate.