Multiple Neurological Deficits Research Articles

Clinical presentation of severe COVID‑19 with heart failure: A single‑center retrospective study.

The coronavirus disease-19 (COVID-19) pandemic has led to a global transformation in public health interventions. The present study aimed to evaluate the clinical features as well as the outcomes of severe heart failure (HF) among patients with severe COVID-19. A single-center observational study was carried out at The 904th Hospital of Joint Logistic Support Force (Wuxi, China) from November 2022 to April 2023, and a total of 210 patients diagnosed with severe HF were included. Among these patients, 128 patients had COVID-19 whereas the remaining patients were not diagnosed with COVID-19. The analysis entailed investigated pre-existing medical records, that is, admission and discharge, laboratory values, neuroimaging, length of hospitalization, mortality and costs incurred by patients throughout the COVID-19 pandemic from the records. All the 210 incorporated patients accomplished the follow-up and it was established that there was no significant differences in baseline characteristics between HF combined with COVID-19 and HF without COVID-19 were affirmed (P>0.05). HF coupled with COVID-19 infection demonstrated an increased risk of 30-day mortality (28.91 vs. 14.63%; P=0.017), extended length of hospital stays (22.54±6.73 vs. 19.35±5.69; P<0.001) and higher expenses for hospitalization (P<0.001). Complications related to hospitalization, including pneumonia (76.56 vs. 35.37%; P=1.0x10-4), respiratory failure (47.66 vs. 24.39%; P=0.001), pulmonary embolism (8.59 vs. 2.44%; P=0.031), deep vein thrombosis (30.47 vs. 14.63%; P=0.009), 7 days delirium (60.16 vs. 45.12%; P=0.033), multiple organ dysfunction syndrome (32.81 vs. 18.29%; P=0.021) and neurological deficits (30.47% vs. 17.07%, P=0.029) increased significantly. In conclusion, HF combined with COVID-19, treatment and prognosis are getting worse. Enhancing preparedness for future COVID-19 and other similar pandemics necessitates the comprehension of this to refine care provided to patients with HF (registration no. THH-IPR-20221101 on 01 November 2022).

Surgical management of Rathke cleft cysts in pediatric patients: a single institution experience

ObjectiveRathke cleft cysts (RCCs) are benign, epithelial-lined sellar lesions that arise from remnants of the craniopharyngeal duct. Due to their rarity in the pediatric population, data are limited regarding the natural history and optimal management of growing or symptomatic RCCs. We present our institutional experience with the surgical management of RCCs.MethodsWe performed a retrospective study of consecutive RCC patients ≤ 18 years old treated surgically at our institution between 2006 and 2022.ResultsOverall, 567 patients with a diagnosis of pituitary mass or cyst were identified. Of these, 31 had a histopathological diagnosis of RCC, 58% female and 42% male. The mean age was 13.2 ± 4.2 years. Presenting symptoms included headache (58%), visual changes (32%), and endocrinopathies or growth delay (26%); 13% were identified incidentally and subsequently demonstrated growth on serial imaging. Six percent presented with symptomatic intralesional hemorrhage. Surgical approach was transsphenoidal for 90% of patients and orbitozygomatic for 10%. Preoperative headaches resolved in 61% of patients and preoperative visual deficits improvement in 55% after surgery. New pituitary axis deficits were seen in 9.7% of patients. Only two complications occurred from a first-time surgery: one cerebrospinal fluid leak requiring lumbar drain placement, and one case of epistaxis requiring cauterization. No patients experienced new visual or neurological deficits. Patients were followed postoperatively with serial imaging at a mean follow-up was 62.9 ± 58.4 months. Recurrence requiring reoperation occurred in 32% of patients. Five-year progression-free survival was 47.9%. Except for one patient with multiple neurological deficits from a concurrent tectal glioma, all patients had a modified Rankin Scale score of 0 or 1 (good outcome) at last follow-up.ConclusionDue to their secretory epithelium, pediatric RCCs may demonstrate rapid growth and can cause symptoms due to local mass effect. Surgical management of symptomatic or growing pediatric RCCs via cyst fenestration or partial resection of the cyst wall can be performed safely, with good neurologic outcomes. There is a nontrivial risk of endocrinologic injury, and long-term follow up is needed due to high recurrence rates.

Evaluation of the Western Blot Densitometry for the Diagnosis of Congenital Toxoplasmosis

BACKGROUND: Congenital toxoplasmosis can cause severe ocular and neurological sequels such hydrocephaly, seizures, and chorioretinitis, leading to permanent visual deficit and multiple neurological deficits, if left untreated. An early diagnosis is crucial to prevent the development of these consequences and western blot is the most important diagnostic tool for this disease in newborns, because can distinguish between antibodies transferred from the mother or those produced by the newborn. However, the reading of the results still being done by subjective analysis by expert clinical laboratory specialists, reaching a maximum of 73% of sensitivity. The digitalization of the image of the results of western blot and its analysis by a software quantifying the densitometry of the bands obtained by western blot assay can potentially improve the diagnostic performance of the technique. METHODS: Was evaluated the sensitivity and specificity by digitalizing pictures of western blot results from the laboratory of Biomedical Research at the University of Quindío and published in the scientific literature. For the research, GelAnalyzer 19.1 software was used to digitalize the bands from western blot membranes from mother and child serum and the molecular weight of protein bands was calculated. The images were from 15 cases of newborns during the first month of life (six from our laboratory and nine from literature), and five negative controls (three from the laboratory and two from literature). Definition of true cases were the persistence of IgM/IgA after day 10 of life, mother with IgG and IgM antibodies positive and newborn with positive IgG plus symptoms, or no decrease in IgG in follow-up. The true negative cases were all newborn whose become negative for IgG anti Toxoplasma before the month 10 of life in absence of treatment, indicating that were maternal passively transferred antibodies. RESULTS: When comparing the immunological profiles of the mothers and their children for IgG by densitometry, congenital infections were detected in 80% (12 out of 15) of the cases (sensitivity) in serum samples obtained during the first month of life. All controls were negative (100% specificity). There was a percentage of 53% (8 out of 15) of children not identifiable with the visual analysis performed by two undergraduate researchers, likewise, the specificity with observation was 57% (3 out of 5) contrasted with the present study. Finally, three bands were identified as the most common in newborns with congenital infection: 49 KDa, 98-97 KDa and 72 KDa proteins (in order of frequency). CONCLUSION: Digitalization of western blot images increase sensitivity for the diagnosis of newborn with congenital toxoplasmosis during the first month of life.

Acute disseminated encephalomyelitis and not-associated active viral infection

Introduction: Acute disseminated encephalomielitis (ADEM) is an autoinmune disorder of the central nervous system, which causes a broad inflammatory process in the brain and spinal cord. It is characterized by the association of acute encephalopathy and multiple neurological deficits, 1 or 2 weeks after an infectious process that will not always be evident or after an immunization. Case report: A 52- year- old male patient presented with intense headache associated with disorder of consciousness, motor deficit, aphasia and active infection with herpes simplex virus type 1. Magnetic resonance imaging of the simple skull is performed showing hyperintense images of a demyelinating nature. Conclusion: The diagnosis of a case of ADEM with an active acute infection truly constitutes an exceptional event and of great interest, considering that the etiology of this entity is 75 percent postinfectious. Identifying in time whether they are related pathologies or not determines the choice of treatment and evolution of the patient.

Two rare cases of myelin oligodendrocyte glycoprotein antibody-associated disorder in children with leukodystrophy-like imaging findings

BackgroundChildren with acquired demyelinating syndromes (ADS) whose sera are positive for myelin oligodendrocyte glycoprotein (MOG) immunoglobulin (IgG) can be diagnosed with MOG-IgG associated disorder (MOGAD). Cases with leukodystrophy-like imaging findings with recurrent MOGAD have rarely been reported.Case presentationTwo children with MOGAD, whose onset age was 6 months and 3 years, respectively, were admitted to the hospital due to fever and altered consciousness. In both children, MOG-IgG was detected in the serum using live cell-based assay. Brain magnetic resonance imaging (MRI) revealed leukodystrophy-like lesions with diffuse bilateral white matter. Cerebrospinal fluid (CSF) analysis showed mild pleocytosis with normal or slightly increased protein levels and no oligoclonal bands. Metabolic and inflammatory blood/CSF markers were all negative. Full exon gene testing revealed normal results, and nuclear and mitochondrial DNA were normal. Despite regular immunotherapy and reduction of lesions based on brain MRI results, the patients repeatedly relapsed and had residual neurological dysfunction at 3–4 years of follow-up.ConclusionsAlthough MOGAD is a monophasic and benign condition, certain MOGAD patients can experience multiple relapses and residual neurologic deficits. The spectrum of clinical manifestations in MOGAD is wider in children than in previously reported cases, including cases with leukodystrophy-like imaging findings. Such imaging findings along with MOG-IgG may occur recurrently and result in severe neurological prognosis. Patients with extensive and confluent white matter lesions should undergo early testing of MOG-IgG to ensure early therapy. In refractory cases, MOGAD treatment may need to be escalated beyond the current therapy, which means second-line immunotherapy should be performed as early as possible and hormone levels should not be rapidly reduced. Early diagnosis and appropriate treatment may improve the prognosis of children with MOGAD.

Unravelling the Clinical Co-Morbidity and Risk Factors Associated with Agenesis of the Corpus Callosum.

Agenesis of the Corpus Callosum (ACC) can result in multiple neurological deficits including social and behavioural issues. However, the underlying aetiology, clinical co-morbidity and the contributing risk factors remain elusive, resulting in inaccurate prognosis and delayed therapy. The main objective of this study was to comprehensively describe the epidemiology and clinical co-morbidity associated with patients diagnosed with ACC. The secondary objective was to identify the factors that contribute towards increased risk for ACC. For this, we analysed 22 years (1998-2020) of clinical data across the whole of Wales, UK collected through the Congenital Anomaly Register & Information Service (CARIS) and Public Health Wales (PHW). Our results demonstrate that complete ACC (84.1%) was the prevalent subtype, in comparison to partial ACC. Further, ventriculomegaly/hydrocephalus (26.37%) and ventricular septal defect (21.92%) were identified to be the most prevalent neural malformation (NM) and congenital heart disorder (CHD) in our cohort. Although 12.7% of subjects with ACC had both an NM and CHD, we found no significant association between them (χ2 (1, n = 220) = 3.84, p = 0.33). We found socioeconomic deprivation and increased maternal age contributed towards an increased risk for ACC. To the best of our knowledge, this study for the first time defines the clinical phenotypes and the factors that contribute to ACC within the Welsh population. These findings will be of value to both patients and healthcare professionals, who may take preventative or remedial measures.

347 Management of Rathke Cleft Cysts in Children: The Rady Children’s Hospital Approach

INTRODUCTION: Rathke cleft cysts (RCCs) are benign, epithelial-lined sellar lesions that arise from remnants of the craniopharyngeal duct. Due to their rarity in the pediatric population, data are limited regarding the natural history and management of growing or symptomatic RCCs. METHODS: We performed a retrospective study of RCC patients age ≤18 years treated between 2006-2022 at our institution. RESULTS: 567 patients with a diagnosis of pituitary mass or cyst were identified. Of these, 31 underwent surgery and had a histopathological diagnosis of RCC, 58% female and 42% male. Mean age was 13.2 +/- 4.2 years. Presenting symptoms included headache (58%), visual changes (35%), and endocrinopathies or growth delay (26%); 13% were identified incidentally and grew significantly on serial imaging. Six percent presented with intralesional hemorrhage. Surgical approach was transsphenoidal for 90% of patients and orbitozygomatic for 10%. Preoperative headaches resolved in 61% of patients after surgery. New pituitary axis deficits were seen in 9.7%. Only two complications occurred from a first-time surgery: one cerebrospinal fluid leak requiring lumbar drain placement, and one case of epistaxis requiring cauterization. No patients experienced new visual or neurological deficits. Patients were followed postoperatively with serial imaging. Mean follow-up was 62.9 +/- 58.4 months. Recurrence requiring reoperation occurred in 32% of patients (transsphenoidal n = 8, pterional n = 2). Five-year progression-free survival was 47.9%. Except for one patient with multiple neurological deficits from a concurrent tectal glioma, all patients had a modified Rankin Scale score of 0 or 1 (good outcome) at last follow-up. CONCLUSIONS: Due to their secretory epithelium, RCCs may demonstrate rapid growth and recurrence. Incidental RCCs can be managed conservatively, whereas symptomatic or growing lesions may be fenestrated or resected. Due to their dynamic and variable nature, careful patient selection and close postoperative surveillance are critical to optimizing patient outcomes.

Abstract 12896: Case of Probable Takutsubo's Cardiomyopathy Post Intravenous Immunoglobulin Infusion for Neuromyopathy Syndrome

Introduction: Takotsubo’s Cardiomyopathy (TCM) is a diagnosis of exclusion recognized by regional left ventricle (LV) dysfunction and absent coronary artery disease. There are isolated reports of patients with autoimmune conditions developing TCM post intravenous immunoglobulin (IVIG). IVIG is a plasma product which inactivates microbial antigens and downregulates certain cytokines, but its cardiac effects are varied. Our case describes a female with progressive neuromyopathy who developed cardiomyopathy post IVIG infusion. Case: A 68 year old female, with type 2 diabetes, hypertension, stroke, and neuromyopathy presented with worsening dysarthria and gait issues for 2 years. Examination revealed multiple neurological deficits, and vital signs showed pulse of 105 with blood pressure of 101/75 mmHg. Initial electrocardiogram (EKG) showed sinus tachycardia and CT angiogram of head was unremarkable. She had a negative cardiac catheterization 2 years ago and a normal echocardiogram 6 months ago. IVIG 400 mg/kg daily for 5 days was started for neuromyopathy flare. She had chest pain on infusion day 4. EKG showed ST elevation in leads V2-V4, and labs revealed troponin I of 0.96 ng/ml with proBNP of 7583 pg/mL. Echo showed LV akinesis with preserved basal segmental function suggestive of TCM. Carvedilol and losartan were started. Further ischemic work up was not done as she died from respiratory failure. Discussion: IVIG is linked to thromboembolic events including strokes and myocardial infarction (MI), but TCM is rare. Risk factors include high dose therapy (&gt;1g/kg), coronary artery disease, prior MI, smoking, diabetes, hypertension, autoimmune conditions, or hyperviscosity states. Our patient had multiple risk factors for MI post infusion including age, gender, and comorbidities. Her assessment is limited by incomplete ischemic workup. IVIG may have negative cardiac effects secondary to vasoconstrictive cytokines, arterial spasm, or increased blood viscosity, but is confounded by other factors in this case. Conclusions: IVIG has poorly described cardiac effects including possible induction of TCM. Further trials monitoring cardiac outcomes post IVIG stratified by dosage and risk would improve patient selection and education.

Antiseizure medications (antiepileptic drugs) in adults: starting, monitoring and stopping.

Up to 10% of people living to 80years of age have one or more seizures; and many will not require anti-seizure medication (ASMs). In 85% of patients, the diagnosis comes from the history of the index event. One-third of patients with an apparent "first seizure" have previous events, changing their diagnosis to epilepsy. Targeted investigations are important for classification and risk prediction. Patients with a low risk of seizure recurrence are not usually offered ASM treatment. High-risk patients have multiple seizures, neurological deficits, intellectual disability and/or relevant abnormal investigations; and are offered ASMs. Individual factors modulate this decision-making. Future integrated technologies offer the game-changing potential for seizure monitoring and prediction, but are not yet robust, convenient or affordable. Therapeutic drug monitoring in patients taking ASMs may confirm ASM toxicity, or when non-adherence, malabsorption, or rapid metabolism are suspected causes of breakthrough seizures. They are less useful when these factors are intermittent or irregular. Current evidence does not favour routine monitoring of serum levels, as it neither reliably predicts control, relapse, or adverse effects. The decision to discontinue ASM should follow a full discussion with the patient of risks and benefits. Along with population risk factors for seizure recurrence, the patient's lifestyle and preferences must be considered. ASM are usually discontinued in a slow step-wise fashion, one at a time, after at least two years of remission. Seizure recurrence risk plateaus only after 2years following ASM discontinuation, and patients need access to specialist follow-up over that period.

BET protein inhibition promotes non-myeloid cell mediated neuroprotection after rodent spinal cord contusion

Spinal cord injuries (SCI) often lead to multiple neurological deficits as a result from the initial trauma and also the secondary damage that follows. Despite abundant preclinical data proposing anti-inflammatory therapies to minimize secondary injury and improve functional recovery, the field still lacks an effective neuroprotective treatment. Epigenetic proteins, such as bromodomain and extraterminal domain (BET) proteins, are emerging as new targets to regulate inflammation. More importantly, pharmacological inhibition of BET proteins suppresses pro-inflammatory gene transcription after SCI. In this study, we tested the therapeutic potential of inhibiting BET proteins after SCI with clinically relevant compounds, and investigated the role of the BET protein BRD4 in macrophages during progression of SCI pathology. Systemic inhibition of BET proteins with I-BET762 significantly reduced lesion size 8 weeks after a contusion injury in rats. However, we observed no histological or locomotor improvements after SCI when we deleted Brd4 in macrophages through the use of myeloid-specific Brd4 knockout mice or after macrophage-targeted pharmacological BET inhibition. Taken together, our data indicate that systemic I-BET762 treatment is neuroprotective, and the histopathological improvement observed is likely to be a result of effects on non-macrophage targets. Expanding our understanding on the role of BET proteins after SCI is necessary to identify novel therapeutic targets that can effectively promote repair after SCI.

A Calcified Hydatid Cyst Transmitted from the Brain to the Liver

This is a case report of an 80-year-old female patient who came to the emergency room complaining of severe headache, vomiting, multiple neurological deficits, increased intracranial pressure, and breathing difficulties due to presence of two calcified cysts in the brain and in the liver. This case will show the relation between the cyst in the liver and the cyst in the brain. The patient own many goats and this report will explain and discuss this medical issue in details about it's relation with animals. The aim of this paper is to discuss this rare condition and provide image documentation of the findings.

MRI diagnosis of spontaneous intraventricular tension-pneumocephalus in a 10-month-old male Saarloos Wolfdog.

A 10-month-old male Saarloos Wolfdog was presented with a history of multiple neurologic deficits that had acutely progressed. Neurologic examination findings localized signs to the cerebrum and brainstem. Magnetic resonance imaging revealed markedly enlarged and gas-filled lateral ventricles with a mass effect leading to cerebellar herniation. A right-sided defect of the cribriform plate with a dysplastic ethmoturbinate was identified as the inlet of air and origin of the intraventricular tension pneumocephalus. Surgical findings were consistent with a ruptured, congenital, nasal meningocele.

Delayed treatment with the immunotherapeutic LNFPIII ameliorates multiple neurological deficits in a pesticide-nerve agent prophylactic mouse model of Gulf War Illness

Residual effects of the 1990–1991 Gulf War (GW) still plague veterans 30 years later as Gulf War Illness (GWI). Thought to stem mostly from deployment-related chemical overexposures, GWI is a disease with multiple neurological symptoms with likely immunological underpinnings. Currently, GWI remains untreatable, and the long-term neurological disease manifestation is not characterized fully. The present study sought to expand and evaluate the long-term implications of prior GW chemicals exposure on neurological function 6–8 months post GWI-like symptomatology induction. Additionally, the beneficial effects of delayed treatment with the glycan immunotherapeutic lacto-N-fucopentaose III (LNFPIII) were evaluated. Male C57BL/6J mice underwent a 10-day combinational exposure (i.p.) to GW chemicals, the nerve agent prophylactic pyridostigmine bromide (PB) and the insecticide permethrin (PM; 0.7 and 200 mg/kg, respectively). Beginning 4 months after PB/PM exposure, a subset of the mice were treated twice a week until study completion with LNFPIII. Evaluation of cognition/memory, motor function, and mood was performed beginning 1 month after LNFPIII treatment initiation. Prior exposure to PB/PM produced multiple locomotor, neuromuscular, and sensorimotor deficits across several motor tests. Subtle anxiety-like behavior was also present in PB/PM mice in mood tests. Further, PB/PM-exposed mice learned at a slower rate, mostly during early phases of the learning and memory tests employed. LNFPIII treatment restored or improved many of these behaviors, particularly in motor and cognition/memory domains. Electrophysiology data collected from hippocampal slices 8 months post PB/PM exposure revealed modest aberrations in basal synaptic transmission and long-term potentiation in the dorsal or ventral hippocampus that were improved by LNFPIII treatment. Immunohistochemical analysis of tyrosine hydroxylase (TH), a dopaminergic marker, did not detect major PB/PM effects along the nigrostriatal pathway, but LNFPIII increased striatal TH. Additionally, neuroinflammatory cells were increased in PB/PM mice, an effect reduced by LNFPIII. Collectively, long-term neurobehavioral and neurobiological dysfunction associated with prior PB/PM exposure was characterized; delayed LNFPIII treatment provided multiple behavioral and biological beneficial effects in the context of GWI, highlighting its potential as a GWI therapeutic.

S1995 Dysphagia Secondary to Acute Stroke Masquerading as Food Impaction

INTRODUCTION: Dysphagia is a commonly encountered symptom in gastroenterology, and esophageal food impaction is a common cause of acute onset dysphagia presenting in the emergency room. As such, gastroenterologists are often among the first to evaluate patients with a chief complaint of dysphagia. Here, we present a case of acute onset dysphagia concerning for food impaction, where the patient was then noted to have multiple neurologic deficits suggestive of acute stroke. CASE DESCRIPTION/METHODS: The patient is a 54 year-old male with history of hypertension who presented with acute onset dysphagia for two hours. His symptoms began with lightheadedness, and subsequently developed inability to swallow liquids. His symptoms did not develop acutely after ingestion of any solid foods. He then noted discomfort in his neck, prompting presentation to the emergency department. On arrival, he was hemodynamically stable. Initial labs were unremarkable. Physical exam at time of arrival notable for normal cardiovascular and neurologic exams. Oropharyngeal exam was abbreviated as he was wearing a mask due to the COVID-19 pandemic. GI was consulted for concern for food impaction. While plans were being made for urgent endoscopy, he developed a left facial droop, nystagmus and gait ataxia concerning for acute posterior circulation stroke. He underwent CTA which was notable for occlusion of right vertebral and proximal basilar arteries. He promptly received tPA and was admitted to the stroke service. His neurologic exam improved over the course of his hospitalization and tolerated pureed diet by time of discharge, though with some residual dysphagia. DISCUSSION: The differential diagnosis for dysphagia is commonly differentiated into oropharyngeal and esophageal pathologies. Within each of these categories, there are both neuromuscular and structural processes which can manifest with symptoms of dysphagia. As gastroenterologists, we are frequently exposed to common esophageal pathologies. However, maintaining a broad differential and avoiding anchoring bias is key for timely recognition of a multitude of diagnoses. Urgent diagnoses which can present with acute onset dysphagia include oropharyngeal infections, acute stroke, and food and/or foreign body impaction. This case highlights the importance of the gastroenterologist, who may be the first person to evaluate a patient with acute onset dysphagia, in maintaining a broad and appropriate differential diagnosis to ensure appropriately and timely treatment of the patient.

Inhibition of Sar1b, the Gene Implicated in Chylomicron Retention Disease, Impairs Migration and Morphogenesis of Developing Cortical Neurons

Chylomicron Retention Disease (CMRD) is a rare inherited lipid malabsorption syndrome that exhibits a recessive hypocholesterolemia in infants. CMRD has been associated with genetic mutations of SAR1B—a member of the Arf GTPase family involved in the secretory pathway from the endoplasmic reticulum to the Golgi. CMRD patients suffer from multiple neurological deficits, the etiologies of which remain unclear. In this study, we found that Sar1b protein is expressed in developing mouse neocortex. The knockdown of Sar1b does not affect the proliferation and mitotic exit of the neural progenitors but inhibits the radial migration of the newborn cortical neurons. At postnatal day 3, the neurons stalled in the white matter fail to develop axons across the midline of the corpus callosum, resulting in the loss of the neurons later on. hSAR1B(D137N), a CMRD-associated mutant of SAR1B, also impairs the positioning of the cortical neurons in the mouse brain, suggesting a dominant-negative effect by the human heterozygous mutant. The results indicate that SAR1B is crucial to radial migration and axon morphogenesis of the cortical neurons. Our study reveals a cell-autonomous action of Sar1b, which is unrelated to lipid absorption from the gut, on the development of the cerebral cortex.

Network diffusion modeling predicts neurodegeneration in traumatic brain injury.

ObjectiveTraumatic brain injury (TBI) is a heterogeneous disease with multiple neurological deficits that evolve over time. It is also associated with an increased incidence of neurodegenerative diseases. Accordingly, clinicians need better tools to predict a patient’s long‐term prognosis.MethodsDiffusion‐weighted and anatomical MRI data were collected from 17 adolescents (mean age = 15y8mo) with moderate‐to‐severe TBI and 19 healthy controls. Using a network diffusion model (NDM), we examined the effect of progressive deafferentation and gray matter thinning in young TBI patients. Moreover, using a novel automated inference method, we identified several injury epicenters in order to determine the neural degenerative patterns in each TBI patient.ResultsWe were able to identify the subject‐specific patterns of degeneration in each patient. In particular, the hippocampus, temporal cortices, and striatum were frequently found to be the epicenters of degeneration across the TBI patients. Orthogonal transformation of the predicted degeneration, using principal component analysis, identified distinct spatial components in the temporal–hippocampal network and the cortico‐striatal network, confirming the vulnerability of these networks to injury. The NDM model, best predictive of the degeneration, was significantly correlated with time since injury, indicating that NDM can potentially capture the pathological progression in the chronic phase of TBI.InterpretationThese findings suggest that network spread may help explain patterns of distant gray matter thinning, which would be consistent with Wallerian degeneration of the white matter connections (i.e., “diaschisis”) from diffuse axonal injuries and multifocal contusive injuries, and the neurodegenerative patterns of abnormal protein aggregation and transmission, which are hallmarks of brain changes in TBI. NDM approaches could provide highly subject‐specific biomarkers relevant for disease monitoring and personalized therapies in TBI.

Age-Dependency of Levetiracetam Effects on Exocytotic GABA Release from Nerve Terminals in the Hippocampus and Cortex in Norm and After Perinatal Hypoxia.

Perinatal hypoxia can lead to multiple chronic neurological deficits, e.g., mental retardation, behavioral abnormalities, and epilepsy. Levetiracetam (LEV), 2S-(2-oxo-1-pyrrolidiny1) butanamide, is an anticonvulsant drug with proven efficiency in treating patients with focal and generalized seizures. Rats were underwent hypoxia and seizures at the age of 10-12 postnatal days (pd). The ambient level and depolarization-induced exocytotic release of [3H]GABA (γ-aminobutyric acid) were analyzed in nerve terminals in the hippocampus and cortex during development at the age of pd 17-19 and pd 24-26 (infantile stage), pd 38-40 (puberty) and pd 66-73 (young adults) in norm and after perinatal hypoxia. LEV had no effects on the ambient [3H]GABA level. The latter increased during development and was further elevated after perinatal hypoxia in nerve terminals in the hippocampus during the whole period and in the cortex in young adults. Exocytotic [3H]GABA release from nerve terminals increased after perinatal hypoxia during development in the hippocampus and cortex, however this effect was preserved at all ages during blockage of GABA transporters by NO-711 in the hippocampus only. LEV realized its anticonvulsant effects at the presynaptic site through an increase in exocytotic release of GABA. LEV exerted more significant effect after perinatal hypoxia than in norm. Action of LEV was strongly age-dependent and can be registered in puberty and young adults, but the drug was inert at the infantile stage.

Multiple Neurologic Deficits and Cognitive Decline in a Young Woman.

We present the clinicopathologic conference of a 34-year-old lady with history of facial palsy 14 years ago who developed new deficits of mononeuritis multiplex, maculopapular rash, pancytopenia, splenomegaly, lung involvement and cognitive decline rapidly over three years. Investigations revealed pancytopenia, reversal of albumin globulin ratio, mediastinal adenopathy, ANA positivity, low C3 levels with the CSF being inflammatory and MRI showing extensive hemorrhagic lesions with mass effect. She had a rapidly progressive fatal course over three years with the disease being undiagnosed. This case was presented in the annual meeting of the Indian Academy of Neurology in September 2018.

Airway management in a patient with Goldenhar syndrome

Goldenhar syndrome involves multiple facial, cardiac, renal and neurological deficits which generally culminate into difficult ventilation and intubation. Various devices have been reported, in successfully securing the airway of these children. In our case we have preferred CMAC laryngoscope in securing the difficult paediatric airway and have found it effective, safe and less cumbersome than the traditional direct laryngoscopy.

Intracranial involvement of posttransplant lymphoproliferative disorder multiple myeloma

We report a 61-year-old man with intracranial multiple myeloma (MM) presenting as a posttransplant lymphoproliferative disorder (PTLD) following a kidney transplant. Two months after his transplant, the man developed acute rejection with Epstein–Barr virus (EBV) viremia, requiring aggressive immunosuppression. Twenty months following transplantation, the patient presented with multiple neurologic deficits. Imaging revealed numerous lytic lesions in the skull, most conspicuously a 4.1cm right frontal skull mass with prominent intracranial extension. Histologic sections of the frontal bone lesion showed sheets of atypical plasma cells that were positive for CD138 and kappa immunoglobulin light chains. Chromogenic in situ hybridization for EBV-encoded small RNA was also positive. Plasma cell neoplasms, either as MM or a plasmacytoma, are one of the least common forms of PTLD, and their rarity limits the possibility of major studies to detail their behavior. Most often seen after renal transplantation, the majority are EBV-driven, similarly to other PTLD. While studies have demonstrated several risk factors, behavior and optimal management of PTLD plasma cell neoplasms are unknown. Plasma cell neoplasms affect the nervous system in a variety of ways but rarely via intracranial disease. MM usually presents initially with several classic signs and symptoms, but our patient’s presentation was typical of a localized brain tumor with generalized and focal gross neurologic defects.