Anti-NMDA-receptor (anti-NMDAR) encephalitis is often associated with ovarian teratoma (OT). The best management of anti-NMDAR encephalitis patients with normal imaging studies (pelvic ...ultrasound/MRI) but clinically high risk of OT (e.g., female, adult, black) is unclear. We report on the surprising diagnostic quest in a young black woman with anti-NMDAR encephalitis, in whom invasive procedures could finally disclose two OTs that were hidden from the initial non-invasive diagnostics.
The patient presented with a one-week history of psychotic symptoms, developing oro-facial dyskinesia, seizures and coma, eventually requiring mechanical ventilation. NMDA-receptor antibodies were positive in serum and cerebrospinal fluid. Pelvic MRI and transabdominal ultrasound were normal. Exploratory laparoscopy was also unremarkable at first, but due to a suspicious echogenic mass (15 mm) in the right ovary on perioperative transvaginal ultrasound, an ovarian incision was performed which led to the detection of a first OT and its removal via ovarian-preserving cystectomy. Following a severe therapy-refractory clinical course despite aggressive immunotherapy and tumor removal, 6 months later bilateral oophorectomy was performed as ultima ratio, disclosing a second micro-OT (6 mm) in the left ovary. Unfortunately, the patient has not improved clinically yet.
In therapy-refractory anti-NMDAR encephalitis with high risk of OT, small and bilateral OTs hidden from primary non-invasive diagnostics should be considered, which may trigger further invasive diagnostic procedures.
Abstract
Background
Recently, antibodies against the alpha isoform of the glial-fibrillary-acidic-protein (GFAPα) were identified in a small series of patients with encephalomyelitis. Coexisting ...autoantibodies (NMDA receptor, GAD65 antibodies) have been described in a few of these patients. We describe a patient with rapidly progressive encephalomyeloradiculitis and a combination of anti-ITPR1, anti-GFAP and anti-MOG antibodies.
Case presentation and literature review
A 44-year old caucasian woman with a flu-like prodrome presented with meningism, progressive cerebellar signs and autonomic symptoms, areflexia, quadriplegia and respiratory insufficiency. MRI showed diffuse bilateral T2w-hyperintense brain lesions in the cortex, white matter, the corpus callosum as well as a longitudinal lesion of the medulla oblongata and the entire spinal cord. Anti-ITPR1, anti-GFAP and anti-MOG antibodies were detected in cerebrospinal fluid along with lymphocytic pleocytosis. Borderline tumor of the ovary was diagnosed. Thus, the disease of the patient was deemed to be paraneoplastic. The patient was treated by surgical removal of tumor, steroids, immunoglobulins, plasma exchange and rituximab. Four months after presentation, the patient was still tetraplegic, reacted with mimic expressions to pain or touch and could phonate solitary vowels. An extensive literature research was performed.
Conclusion
Our case and the literature review illustrate that multiple glial and neuronal autoantibodies can co-occur, that points to a paraneoplastic etiology, above all ovarian teratoma or thymoma. Clinical manifestation can be a mixture of typically associated syndromes, e.g. ataxia associated with anti-ITPR1 antibodies, encephalomyelitis with anti-GFAPα antibodies and longitudinal extensive myelitis with anti-MOG antibodies.
Around 91% of migraine patients use over-the-counter medicines to treat attacks, often without further treatment or medical consultation. This therapeutic principle is established in most countries, ...regardless of how the healthcare system is otherwise structured or financed. Using Germany as an example, the basis for an expansion of attack therapy with rizatriptan as an over-the-counter triptan is described. To achieve the best possible tolerability and safety in the context of self-medication, the lowest possible dose should be selected to provide the most favourable tolerability and safety profile in the context of self-medication through low dosages. The lowest approved dose of rizatriptan is 5 mg. This was investigated in three randomized controlled trials with 752 patients. The results show that rizatriptan at a dose of 5 mg is more effective than the triptans naratriptan 2.5 mg, almotriptan 12.5 mg and sumatriptan 50 mg, which were previously available for self-medication in Germany. There was no significant difference in the frequency of adverse events with rizatriptan 5 mg compared to placebo. Rizatriptan 5 mg does not have a higher side effect potential than sumatriptan 50 mg, which is already exempt from the prescription requirement. The reasons given show that rizatriptan in a dose of 5 mg for the treatment of acute migraine attacks fulfils the requirements for a transfer from prescription to pharmacy-only status at least as well as sumatriptan 50 mg, naratriptan 2.5 mg and almotriptan 12.5 mg. From a clinical care perspective, it is desirable for affected patients to have other options available for self-medication. Non-responders to other substances also have a further treatment option with rizatriptan 5 mg, with the same or even better risk–benefit profile, to treat migraine attacks safely, effectively and in a tolerable manner as part of self-medication.
The constant internal monitoring of speech is a crucial feature to ensure the fairly error-free process of speech production. It has been argued that internal speech monitoring takes place through ...detection of conflict between different response options or “speech plans”. Speech errors are thought to occur because two (or more) competing speech plans become activated, and the speaker is unable to inhibit the erroneous plan(s) prior to vocalization. A prime example for a speech plan that has to be suppressed is the involuntary utterance of a taboo word. The present study seeks to examine the suppression of involuntary taboo word utterances. We used the “Spoonerisms of Laboratory Induced Predisposition” (SLIP) paradigm to elicit two competing speech plans, one being correct and one embodying either a taboo word or a non-taboo word spoonerism. Behavioral data showed that inadequate speech plans generally were effectively suppressed, although more effectively in the taboo word spoonerism condition. Event-related potential (ERP) analysis revealed a broad medial frontal negativity (MFN) after the target word pair presentation, interpreted as reflecting conflict detection and resolution to suppress the inadequate speech plan. The MFN was found to be more pronounced in the taboo word spoonerism compared to the neutral word spoonerism condition, indicative of a higher level of conflict when subjects suppressed the involuntary utterance of taboo words.
Cigarette smoking and other addictive behaviors are among the main preventable risk factors for several severe and potentially fatal diseases. It has been argued that addictive behavior is controlled ...by an automatic-implicit cognitive system and by a reflective-explicit cognitive system, that operate in parallel to jointly drive human behavior. The present study addresses the formation of implicit attitudes towards smoking in both smokers and non-smokers, using a Go/NoGo association task (GNAT), and behavioral and electroencephalographic (EEG) measures. The GNAT assesses,
quantifying participants' reaction times, the strength of association between a target category and either pole of an evaluative dimension (positive or negative). EEG analysis is performed to determine the temporal course of the event-related potential (ERP) components underlying Go/NoGo decisions and implicit attitude formation. Both smokers and non-smokers showed prolonged reaction times to smoking-related pictures when the pictures were coupled with positive evaluative words ("incongruent condition"). This indicates negative implicit attitudes towards smoking in both groups alike at the time point of the behavioral response (600-700 ms post-stimulus). However, only the non-smokers, not the smokers, were found to show a delay of the N200 component in the incongruent condition. This is interpreted as reflecting ambivalent or even positive implicit attitudes towards smoking in the smoker group at the time point of the N200 (300-400 ms post-stimulus). Our study thus provides evidence for the hypothesis that implicit attitudes are subject to changes within several hundred milliseconds after stimulus presentation, and can be altered in the course of their formation.
Background Being critical for brain development and neurocognitive function thyroid hormones may have an effect on behaviour and brain structure. Our exploratory study aimed to delineate the ...influence of mutations in the thyroid hormone receptor (TR) ss gene on brain structure. Methods High-resolution 3D T1-weighted images were acquired in 21 patients with a resistance to thyroid hormone ss (RTHss) in comparison to 21 healthy matched-controls. Changes in grey and white matter, as well as cortical thickness were evaluated using voxel-based morphometry (VBM) and diffusion tensor imaging (DTI). Results RTHss patients showed elevated circulating fT4 & fT3 with normal TSH concentrations, whereas controls showed normal thyroid hormone levels. RTHss patients revealed significantly higher scores in a self-rating questionnaire for attention deficit hyperactivity disorder (ADHD). Imaging revealed alterations of the corticospinal tract, increased cortical thickness in bilateral superior parietal cortex and decreased grey matter volume in bilateral inferior temporal cortex and thalamus. Conclusion RTHb patients exhibited structural changes in multiple brain areas. Whether these structural changes are causally linked to the abnormal behavioral profile of RTHss which is similar to ADHD, remains to be determined. Keywords: Resistance to thyroid hormone, voxel based morphometry, Diffusion tensor imaging, Thyroid hormone receptor beta
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IZUM, KILJ, NUK, PILJ, PNG, SAZU, UL, UM, UPUK
Background Thyroid hormone action is mediated by two forms of thyroid hormone receptors (α, β) with differential tissue distribution. Thyroid hormone receptor β (TRβ) mutations lead to resistance to ...thyroid hormone action in tissues predominantly expressing the β form of the receptor (pituitary, liver). This study seeks to identify the effects of mutant TRβ on pituitary size. Methods High-resolution 3D T1-weighted magnetic resonance images were acquired in 19 patients with RTHβ in comparison to 19 healthy matched controls. Volumetric measurements of the pituitary gland were performed independently and blinded by four different raters (two neuroradiologists, one neurologist, one neuroscientist). Results Patients with mutant TRβ (resistance to thyroid hormone β, RTHβ) showed elevated free tri-iodothyronine/thyroxine levels with normal thyroid-stimulating hormone levels, whereas healthy controls showed normal thyroid hormone levels. Imaging revealed smaller pituitary size in RTHβ patients in comparison to healthy controls (F(1,35) = 7.05, P = 0.012, partial η2 = 0.17). Conclusion RTHβ subjects have impaired sensitivity to thyroid hormones, along with decreased size of the pituitary gland.
The mismatch negativity and the P3a of the event-related EEG potential reflect the electrocortical response to a deviant stimulus in a series of stimuli. Although both components have been ...investigated in various paradigms, these paradigms usually incorporate many repetitions of the same deviant, thus leaving open whether both components vary as a function of the deviant's position in a series of deviant stimuli-i.e. whether they are subject to qualitative/quantitative habituation from one instantiation of a deviant to the next. This is so because the detection of mismatch negativity/P3a in the event-related EEG potential requires an averaging over dozens or hundreds of stimuli, i.e. over many instantiations of the deviant per participant. The present study addresses this research gap. We used a two-tone oddball paradigm implementing only a small number of (deviant) stimuli per participant, but applying it to a large number of participants (n > 230). Our data show that the mismatch negativity amplitude exhibits no decrease as a function of the deviant's position in a series of (standard and) deviant stimuli. Importantly, only after the very first deviant stimulus, a distinct P3a could be detected, indicative of an orienting reaction and an attention shift, and thus documenting a dissociation of mismatch negativity and P3a.
Implicit social cognition refers to attitudes and stereotypes that may reside outside conscious awareness and control but that still affect human behavior. In particular, the implicit favoritism of ...an ingroup, to which an individual belongs, as opposed to an outgroup, to which the individual does not belong, characterized as ingroup bias, is of interest and investigated here.
We used a Go/NoGo association task (GNAT) and behavioral and electroencephalographic (event-related EEG potential ERP analysis) measures to investigate the implicit bias toward cities in East Germany, West Germany, and Europe, in 16 individuals each from West and East Germany (mixed gender, Mage = 24). The GNAT assesses an individual's Go and NoGo responses for a given association between a target category and either pole (positive or negative) of an evaluative dimension.
Behavioral measures revealed slightly faster reaction times to the combination of European city names and negative, as compared with positive, evaluative words in both groups. ERP analysis showed an increased negativity at 400-800 ms poststimulus in the incongruent conditions of East German city/positive word pairings (in West Germans) and West German city/positive word pairings (in East Germans).
An implicitly moderately negative evaluation of Europe by both groups was exhibited based on the behavioral data, and an increased level of conflict arising from the "incongruent" pairings (ie, as manifestation of an implicitly negative attitude toward East Germany in West Germans, and toward West Germany in East Germans) was exhibited based on the electrophysiological data.
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