Case Report Funicular Myelosis in a Butcher: It Was the Cream Cans

Background. Funicular myelosis is a known consequence of exposure to nitrous oxide. Nevertheless, there are only a few clinical trials assessing its long-term effects and there is no literature about the role of nutritional vitamin B12 supplementation in the context of nitrous oxide abuse. Case Descriptions. We diagnosed funicular myelosis in a young butcher, who consumed high amounts of meat regularly. Since the diagnostic process did not reveal any metabolic causes, reinterrogation of the patient uncovered recreational abuse of nitrous oxide out of whipped cream can gas cartridges. After stopping abuse and supplementation of vitamin B12, the patient recovered almost completely. Conclusions. In our case, even high nutritional vitamin B12 uptake could not compensate the noxious effects of nitrous oxide. Since there are emerging reports of increasing misuse, this should be considered in the diagnostic and therapeutic care of patients with nitrous oxide abuse. Furthermore, our case emphasizes that patients with vitamin B12 deficiency should be assessed for nitrous oxide abuse. Case Presentation The 27-year-old patient, a butcher, presented with a 4-week history of ascending symmetric numbness in the limbs, as well as tingling in the feet and fingers. He felt clumsy while writing and unsecure during walking, with report of several dropping events. The neurological examination revealed a diminished position and vibration sense and hypalgesia and hypaesthesia of the upper and lower limbs, compatible with distal symmetric polyneuropathy. However, the Romberg test was negative and the remainder of the examination was normal; in particular, there were no weaknesses or neuropsychological symptoms. The MRI of the brain was normal. The spinal MRI showed T2-hyperintense lesions in the dorsal columns of the cervical spine (C1-C6) without contrast enhancement as typical in patients with funicular myelosis [1] Discussion Vitamin B12 deficiency, subsequent hyperhomocysteinaemia, and funicular myelosis have been observed in patients after exposure to nitrous oxide, and the underlying biochemical pathomechanism has been revealed. It indicates an irreversible inhibition of the active cobalt centre of vitamin B12, leading to decreased activity of 5-methyltetrahydrofolatehomocysteine methyltransferase (MTR), a vitamin B12 dependent enzyme converting homocysteine to methionine In addition to several case reports, a small study revealed that long-term nitrous oxide exposure in operating theatres may lead to decreased vitamin B12 serum levels Besides this abuse of nitrous oxide and the resulting degenerative effects (myelopathy and peripheral neuropathy), Cousaert et al. We consider this report to be worth publishing, as we found a unique constellation, where even high nutritional Case Reports in Neurological Medicine 3 vitamin B12 uptake with a diet rich in meat was not able to compensate the noxious effects of N 2 O. To our knowledge, there are no controlled studies available that concern the influence of nutritional supply of vitamin B12; therefore, the present case suggests that alimentary increased vitamin B12 uptake may not prevent onset of funicular myelosis upon nitrous oxide exposure. This may be of clinical importance when assessing risks for patients with abuse of or other reasons of long-term exposure to nitrous oxide. Furthermore, the case emphasizes that patients (and their next kin) should be asked for nitrous oxide exposure or abuse in cases of vitamin B12 deficiency of unknown origin. Consent The authors have received permission to cite any personal communications. Disclosure All data were obtained in the course of the diagnostic process in the mentioned institutions. No financial disclosure was reported by any of the authors. Conflict of Interests The authors declare that there is no conflict of interests regarding the publication of this paper. Authors' Contribution Fabian Wolpert and Roland Renzel were in charge for the diagnostic and therapeutic care during the hospitalization. Krisztina Baráth provided MRI diagnostics and figures. Andreas R. Gantenbein was the clinical supervisor during the hospitalization phase and is the senior author who reviewed the paper during the writing process. Michael Linnebank was providing fundamental and clinical advice during the diagnostic process and while reviewing the paper. Janis Brakowski contributed his expertise as experienced psychiatric clinician through reviewing the paper

Persistent generalized periodic discharges: A specific marker of fatal outcome in cerebral hypoxia

OBJECTIVES Electroencephalography (EEG) is one of the methods used in predicting the outcome after cerebral hypoxia. In this study we aim to evaluate the significance of generalized periodic discharges (GPD) as a prognostic marker. METHODS We retrospectively analyzed the medical histories of patients, who underwent an EEG after cardiac arrest during the time period from 2005 to 2013 at the University Hospital Zurich. All EEGs were re-interpreted using the 2012 American Clinical Neurophysiology Society (ACNS) classification for intensive care unit (ICU) EEGs. RESULTS Out of 131 patients, in which an EEG was recorded after cardiopulmonary resuscitation, 119 were included in our study. The average interval between cardiac arrest and EEG-recording was 3.8±3.0days (range: 0-14days). Persistent GPDs (i.e. GPDs more than 24h after the event) were found in thirty-two (26.9%) of the patients initial EEGs. The appearance of persistent GPDs preceded fatal outcome in 100% of all cases (vs. 69.0% in the non-GPD-group, p<0.0001). CONCLUSION Among other encephalopathic markers in EEG persistent GPDs are a highly specific prognostic marker of fatal outcome in patients with hypoxic encephalopathy. SIGNIFICANCE Using standardized EEG interpretation, this study identified persistent GPDs as a specific prognostic marker in post cardiac arrest syndrome

EEG after sleep deprivation is a sensitive tool in the first diagnosis of idiopathic generalized but not focal epilepsy

OBJECTIVES: Electroencephalography (EEG) is an essential tool in the diagnosis of epilepsy. EEG after sleep deprivation might increase the likelihood of finding specific epileptiform abnormalities. However conflicting data exist concerning the sensitivity and specificity of this method. We aimed to evaluate the role of EEG after sleep deprivation in the first diagnosis of epilepsy. METHODS: We analyzed retrospectively the medical histories of patients who underwent at least one unspecific standard EEG and a subsequent EEG after sleep deprivation during the time period from 2001 to 2014 at the University Hospital Zurich because of suspected epilepsy. RESULTS: Out of 237 patients who fulfilled all inclusion criteria, 69 were finally diagnosed with epilepsy. Seventeen of them showed interictal epileptiform patterns in EEGs after sleep deprivation, giving this method an overall sensitivity of 25%. Sensitivity of EEG after sleep deprivation was superior in patients with primary generalized epilepsies compared to patients with focal epilepsies (64% vs. 17%, p=0.0011). Overall EEG after sleep deprivation was not more sensitive than a subsequent repeated standard EEG in a subgroup of 55 patients (22% vs. 9%; p=0.065). CONCLUSION: After an unspecific standard EEG, EEG after sleep deprivation is a useful tool to increase diagnostic sensitivity in patients with idiopathic generalized epilepsy but not in those with focal epilepsy. SIGNIFICANCE: This study provides further evidence about the usefulness of EEG after sleep deprivation as an additional diagnostic tool in epilepsy

St. John's Daily Star, 1920-06-19

The St. John's Daily Star was published daily except Sunday between 17 April 1915 - 23 July 1921

Funicular myelosis in a butcher: it was the cream cans

Background. Funicular myelosis is a known consequence of exposure to nitrous oxide. Nevertheless, there are only a few clinical trials assessing its long-term effects and there is no literature about the role of nutritional vitamin B12 supplementation in the context of nitrous oxide abuse. Case Descriptions. We diagnosed funicular myelosis in a young butcher, who consumed high amounts of meat regularly. Since the diagnostic process did not reveal any metabolic causes, reinterrogation of the patient uncovered recreational abuse of nitrous oxide out of whipped cream can gas cartridges. After stopping abuse and supplementation of vitamin B12, the patient recovered almost completely. Conclusions. In our case, even high nutritional vitamin B12 uptake could not compensate the noxious effects of nitrous oxide. Since there are emerging reports of increasing misuse, this should be considered in the diagnostic and therapeutic care of patients with nitrous oxide abuse. Furthermore, our case emphasizes that patients with vitamin B12 deficiency should be assessed for nitrous oxide abuse

Deep brain stimulation of the anterior nucleus of the thalamus increases slow wave activity in non-rapid eye movement sleep

OBJECTIVE Previous studies suggest that intermittent deep brain stimulation (DBS) of the anterior nucleus of the thalamus (ANT) affects physiological sleep architecture. Here, we investigated the impact of continuous ANT DBS on sleep in epilepsy patients in a multicenter crossover study in 10 patients. METHODS We assessed sleep stage distribution, delta power, delta energy, and total sleep time in standardized 10/20 polysomnographic investigations before and 12 months after DBS lead implantation. RESULTS In contrast to previous studies, we found no disruption of sleep architecture or alterations of sleep stage distribution under active ANT DBS (p = .76). On the contrary, we observed more consolidated and deeper slow wave sleep (SWS) under continuous high-frequency DBS as compared to baseline sleep prior to DBS lead implantation. In particular, biomarkers of deep sleep (delta power and delta energy) showed a significant increase post-DBS as compared to baseline (36.67 ± 13.68 μV$^{2}$ /Hz and 799.86 ± 407.56 μV$^{2}$ *s, p < .001). Furthermore, the observed increase in delta power was related to the location of the active stimulation contact within the ANT; we found higher delta power and higher delta energy in patients with active stimulation in more superior contacts as compared to inferior ANT stimulation. We also observed significantly fewer nocturnal electroencephalographic discharges in DBS ON condition. In conclusion, our findings suggest that continuous ANT DBS in the most cranial part of the target region leads to more consolidated SWS. SIGNIFICANCE From a clinical perspective, these findings suggest that patients with sleep disruption under cyclic ANT DBS could benefit from an adaptation of stimulation parameters to more superior contacts and continuous mode stimulation

Transcriptome reprogramming during developmental switching in Physarum polycephalum involves extensive remodeling of intracellular signaling networks

Abstract Activation of a phytochrome photoreceptor triggers a program of Physarum polycephalum plasmodial cell differentiation through which a mitotic multinucleate protoplasmic mass synchronously develops into haploid spores formed by meiosis and rearrangement of cellular components. We have performed a transcriptome-wide RNAseq study of cellular reprogramming and developmental switching. RNAseq analysis revealed extensive remodeling of intracellular signaling and regulation in switching the expression of sets of genes encoding transcription factors, kinases, phosphatases, signal transduction proteins, RNA-binding proteins, ubiquitin ligases, regulators of the mitotic and meiotic cell cycle etc. in conjunction with the regulation of genes encoding metabolic enzymes and cytoskeletal proteins. About 15% of the differentially expressed genes shared similarity with members of the evolutionary conserved set of core developmental genes of social amoebae. Differential expression of genes encoding regulators that act at the transcriptional, translational, and post-translational level indicates the establishment of a new state of cellular function and reveals evolutionary deeply conserved molecular changes involved in cellular reprogramming and differentiation in a prototypical eukaryote