ABSTRACT
Background: The phenotypic diversity of functional movement disorders (FMD) is considered a reflection of its many etiological subtypes. Ehlers-Danlos syndrome (EDS), a joint hypermobility syndrome, also has variable phenotypes, which may include functional symptoms. Objectives: To determine the prevalence of combined diagnoses of FMD and EDS. Methods: We searched our Electronic Medical Records for patients carrying diagnostic codes for EDS and FMD. Further data extraction was done through chart review. Results: Of 11,621 patients evaluated from January 1, 2016 to May 1, 2022, 16 carried a diagnosis of EDS, of which 9 (56.3%) were also diagnosed with FMD. Conversely, a diagnosis of FMD was documented in 190 (1.6%), of whom 16 (8.4%) were diagnosed with EDS. In all EDS-FMD cases, the diagnosis of EDS preceded the onset and diagnosis of FMD. Conclusions: The co-occurrence of FMD and EDS is beyond chance, suggesting association. EDS may represent a prodromal subtype of, and share common pathophysiologic features with, FMD.
ABSTRACT
Guillain-Barré syndrome (GBS) is an inflammatory polyneuropathy that classically presents with low back pain, sensory paresthesias, and rapidly progressive weakness. Patients with GBS can develop dysautonomia, and Takotsubo cardiomyopathy (TCM) is a rare potential manifestation of this dysautonomia. This association has been reported only 12 times in the literature so far, which we review here. We present two cases of GBS associated with TCM, to increase awareness with regard to this comorbid relationship, which would encourage prompt initiation of proper supportive care to avoid morbidity and mortality. We report the case of two patients - a 58-year-old man and a 79-year-old woman - who developed TCM in the setting of axonal variants of GBS. Electrodiagnostic results, cerebrospinal fluid profiles, and echocardiogram findings were consistent with these diagnoses. Both patients were treated with intravenous immunoglobulin (IVIG) in an intensive care unit (ICU) setting. Echocardiogram findings were reversible. TCM should be recognized as a potential complication of GBS in patients with dysautonomia. This case series adds to the sparse body of literature describing the association between these two conditions. It is not clear if patients with axonal variants of GBS are more predisposed to developing TCM; further, larger case series in the future may help identify the risk factors associated with it. We hope to shed more light on this possible association to expedite the diagnosis and management of this condition.
ABSTRACT
UNLABELLED: Recent studies have demonstrated that long-term developmental effects of neonatal anesthesia were more prominent in males. We tested whether steroids, in general, and sex steroids, in particular, are involved in the mediation of sevoflurane-caused paradoxical cortical seizures during the early postnatal period. METHODS: Cortical electroencephalograms, hippocampal synaptic activity, serum levels of steroids and the loss of the righting reflex (LORR), a marker of anesthetic effect, were measured on postnatal days 4-6 in Sprague Dawley rats of both genders exposed to 2.1% sevoflurane. RESULTS: Episodes of seizures, persistent spikes in electroencephalograms and increases in serum corticosterone were similar in both genders. In the order of increasing potency, the corticosteroid receptor antagonist RU 28318, the estradiol receptor antagonist ICI 182780 and the estradiol synthesis inhibitor formestane decreased sevoflurane-induced seizures. Exogenous estradiol increased sevoflurane-caused seizures, spikes and serum levels of corticosterone. These estradiol-enhanced seizures and spikes were depressed by ICI 182780 and the NKCC1 inhibitor, bumetanide, while RU 28318 decreased seizures only. In hippocampal CA1 neurons, estradiol increased the amplitude, rise time and area under the curve of gamma-aminobutyric acid type A receptor (GABAAR)-mediated miniature postsynaptic currents. Exogenous estradiol shortened, while ICI 182780 and formestane lengthened the time needed for sevoflurane to induce LORR. CONCLUSION: These findings provide evidence for gender-independent acute electroencephalographic effects of sevoflurane at this age. Corticosterone and estradiol are involved in the mediation of sevoflurane-induced seizures. Estradiol, but not corticosterone, also contributes to sevoflurane-caused spikes, by enhancing GABAAR-mediated excitation in the cortex. By increasing GABAAR-mediated inhibition in more mature caudal regions of the brain, estradiol contributes to sevoflurane-induced LORR.
