Neurological Sequalae Research Articles

A218 LISTERIA MONOCYTOGENES MENINGITIS IN A NEWLY DIAGNOSED PATIENT WITH INFLAMMATORY BOWEL DISEASE: A CASE REPORT

Abstract Background Anti-Tumor Necrosis Factor (TNF) is a frequently utilized therapy in the treatment of Inflammatory Bowel Disease (IBD). Opportunistic infections are a known but an infrequently encountered complication. Listeria monocytogenes (LM) is an aerobic gram-positive intracellular bacillus. Clinical presentation of infection is host dependent, ranging from self-limited illnesses in immunocompetent individuals to life-threatening sepsis and meningitis in the immunocompromised. TNF plays a crucial role in host’s defense against LM. Although published in adults, few case reports have documented invasive LM in children receiving infliximab (IFX), an anti-TNF agent used in IBD therapy. Aims Describe an adolescent with IBD-unclassified (IBD-U) in whom LM sepsis and meningitis was diagnosed after induction therapy with intravenous (IV) IFX. Methods Case report and literature review. Results A 15-year-old girl presented with 2-week history of progressive abdominal pain, bloody diarrhea, urgency, nocturnal stooling, tenesmus, and weight loss. She had been afebrile, with no history of exposures. Examination revealed pallor and tenderness in the right and left lower quadrants. Investigations showed elevated white cell count (WBC), platelets, inflammatory markers, and low albumin. Abdominal ultrasound showed thickening of the descending and sigmoid colon. Stool multiplex PCR was negative. Colonoscopy showed Mayo 3 pancolitis. The terminal ileum was not intubated and endoscopy was normal. She was diagnosed with IBD-U. Due to poor response to high dose IV steroids, IV IFX (after documenting normal vaccination titers) was given with good clinical response and no adverse effects; she was discharged on tapering prednisone. She re-presented 3 days later with fever, severe headaches, photophobia, and neck stiffness. Her IBD remained quiescent. She had consumed a cold meat sandwich 10 days prior. Antibiotics were started as investigations showed leukocytosis and very high inflammatory markers. Brain MRI showed pus in the lateral ventricles. Cerebrospinal fluid (CSF) analysis showed WBC 1832 x106/L, low glucose, and high protein. Blood and CSF cultures detected LM. She defervesced within 24 hours and completed a 21-day course of Ampicillin monotherapy. 2-month follow up showed IBD in continuous remission on IFX with no neurological sequalae, and a normal brain MRI. Conclusions This is the youngest patient with IBD reported with invasive listeriosis secondary to IFX and adds to 3 cases in children. It highlights the importance of vigilance when evaluating IBD patients with fever during IFX-based therapy. Physicians should be reminded of such patients’ immunocompromised state and their high risk of acquiring opportunistic infections. It is unclear if listeriosis avoidance precautions, currently recommended in pregnant women, should be adopted in patients receiving anti-TNF therapy. Funding Agencies None

A Review of the Current State of Management and Post-Recovery Rehabilitation of COVID-19

In all countries, COVID-19 has had a profound impact on society in general and on healthcare systems in particular. Its route of transmission is similar to that of SARS-CoV and MERS-CoV, and it spreads through respiratory droplets and direct physical contact. It displays greater infectivity rates than the two recent viral outbreaks (SARS and MERS), and early epidemiological studies reveal lower fatality rates. Elderly and multimorbid persons are at the highest risk of developing complications and death. Although men and women are thought to be at equal risk for COVID-19, more men than women are dying, presumably due to sex-based immunological differences and lifestyle factors such as smoking. N95 respirators are thought to be more effective in preventing viral transmission than surgical masks, but the evidence is limited and inconsistent. The current clinical management strategy of COVID-19 is the same as acute respiratory distress syndrome (ARDS). Novel treatments and therapies such as the use of plasmapheresis, chloroquine, hydroxychloroquine, and remdesivir have shown promising results in clinical trials for treating COVID-19. The roles of physical rehabilitation and medicine are expected to increase because postrecovery COVID-19 patients are experiencing cardiovascular, neurological, psychological, and cognitive sequalae. Innovative management strategies are needed to limit the spread of disease and to protect vulnerable persons. Significant evidence indicates that the use of telerehabilitation reduces hospitalizations and cardiac events compared to regular care. Actions are needed to prevent deconditioning in rehabilitation patients, and respiratory therapy practices will require close attention to reduce the dispersion of air droplets.

30.3 A MODEL OF CARE FOR ADDRESSING ATTENTIONAL ISSUES IN CHILDREN AND ADOLESCENTS WITH A HISTORY OF PRENATAL ALCOHOL AND DRUG EXPOSURE

Children exposed to alcohol and drugs in utero often present with a multitude of mental health symptoms, including ADHD-like symptoms of inattention, hyperactivity, and impulsivity. As a clinician trying to sort through caregiver and teacher reports, it can be challenging to understand whether symptoms are consistent with the neurologic sequalae of in utero exposure alone or whether they are truly within the range of ADHD symptomatology. It can also be challenging to understand to what extent symptoms are improving or worsening with medication adjustments. With the time pressures that some psychiatrists face for medication management visits and when family systems are in chaos, the information provided to psychiatrists can be confusing and at times contradictory and can lead to decisions about medication that may not be in the best interest of the child. It is therefore helpful to have additional, objective measures to help guide medication decisions.

Clinical Spectrum of West Nile Virus Neuroinvasive Disease.

West Nile virus (WNV) is the most common arbovirus infection in the United States. The diagnosis requires consideration of not only a broad spectrum of presenting symptoms, ranging from a mild febrile illness to severe encephalitis and acute flaccid paralysis, but also public health risk factors and seasonality. There is no approved targeted therapy for WNV, so treatment relies on supportive care, management of neurologic sequelae and airway, treatment of other systems including the eye, and aggressive rehabilitation. Here, we describe a series of 3 cases of WNV encountered in September 2018 at one institution. First, we describe a case of WNV encephalitis with worsened dyskinesias and a relatively good recovery. Second, we describe a severe WNV encephalitis with overlying motor neuron involvement with a poor outcome. Finally, we describe a case of a WNV meningitis with significant bilateral chorioretinitis, an underappreciated complication of WNV infections. Through these cases, we review the epidemiology of WNV, risk factors for infection, the neurologic sequalae and long-term outcomes, and the importance of recognizing ocular involvement to prevent ophthalmologic complications.

Lazarus in asystole: a case report of autoresuscitation after prolonged cardiac arrest.

BackgroundThird-degree atrioventricular (AV) block can result in sudden cardiac death if no reliable escape rhythm is present. Here, we report a case of an 86-year-old female patient who developed a third-degree AV block leading to cardiac arrest. Surprisingly, sinus rhythm returned after 4 min of asystole, and she showed complete neurological recovery.Case summaryEmergency services were contacted by the husband of an 86-year-old woman after she was found unconscious. Ambulance personnel diagnosed a third-degree AV block without an escape rhythm and transcutaneous pacing was started. At arrival on the emergency ward, pacing was inadequate, resulting in absence of circulation for ∼10 min. After consultation with the family, the patient turned out to have signed a ‘do not resuscitate’ order. Given the impression that the considerable delay deemed favourable neurological recovery unlikely, it was decided together with the family to stop the resuscitation. Subsequently, she had an intermittent junctional escape rhythm but eventually developed a documented asystole of more than 4 min. Against all expectations, she regained sinus rhythm and fully recovered. Eventually, a pacemaker was implanted and she was discharged home without neurological sequalae of the cardiac arrest.DiscussionAutoresuscitation, also known as the Lazarus syndrome, is the spontaneous return of circulation after cardiac arrest and is incidentally seen after failed cardiopulmonary resuscitation (CPR). Autoresuscitation in the absence of CPR is highly unusual, but could, in this case, be due to the total AV block as the cause of the cardiac arrest.

PS1296 ERYTROCYTAPHERESIS IN ACERULOPLASMINEMIA PREVENTS PROGRESSION OF CEREBRAL IRON ACCUMULATION AFTER CHELATOR‐INDUCED NORMALIZATION OF IRON STORES

Background:Hereditary aceruloplasminemia (HA) is a rare autosomal recessive disorder. It comprises mutations in the ceruloplasmin genes leading to the absence of ceruloplasmin, a protein required for iron oxidaztion that enables incorporation into transferrin. Due to diminished iron export from storage cells and a usually mild anemic state due to ineffective iron incorporation, iron absorption is increased resulting in the accumulating of iron in parenchymal cells like hepaocytes, insulin producing pancreatic cells and neurons in the brain that may resultin diabetes, anaemia, retinal degradation and neurological disorders (symptomatic penetrance is high but not 100%)..Several case reports illustrate that iron chelating theray (ICT), when appropriately dosed, may result in normaliztaion of live iron content and iron storage, but a decrease in cerebral iron content has not been observed. The follow‐up of most studies is also short (1 year or less). Only two case reports describe that the iron content in the brain remainded stable for 4 and 13 year during treatment with ICT without any neurological symptoms.Aims:We hypothesized that progressive accumulation of iron in the brain may also be prevented by applying erythrocytapheresis (EA) after normalization of physiological iron stores by ICT. In this way, a continuous increased demand for maximal erythropoiesis by erythrocytapheresis (EA) is induced that may result in the immediately utilization of every absorbed iron atom by the bone marrow and preventingparenchymal accumuation.Methods:A HA patient with a low normal haemoglobin, insulin‐dependent diabetes and no neurological symptoms, is treated with EA for six years now, after normalizing ferritin levels and liver iron with ICT; cerebral iron depostits were not depleted by ICT.Results:This controlled removal of erythrocytes created an enduring asymptomatic low haemoglobin level (around 10 g/dl,), ferritin level within the normal range (mean ferritin of 199 ± 60.9 μg/L before every EA). The liver iron content measured by MRI remained at the same normal level as reached after ICT, and the cerebral iron content, which was not diminished after iron chelation, remained stable. No neurological symptoms have appeared so far.Summary/Conclusion:EA appears to be an elegant therapy to prevent iron accumulation in the brain after inducing normal iron stores by ICT. The advantage of this approach is that prolonged exposure to ICT and its possible occurring medical and definite financial side effects can be avoided. The application of EA allows for controlled haemoglobin reduction preventing symptoms from anaemia. It remains unsure if this strategy prevented the development of neurological or cognitive symptoms, because the prevalence of these is not 100%. On the other hand does this therapy not harm the patient and may be effective. It may be worthwhile to attemnpt this approach in similar cases and even in those that already developed neurological and/or cognitive sequalae.

MON-333 Failure of Medical Therapy to Control Marked Hypercortisolemia by a Metastatic ACTH Producing Neuroendocrine Tumor

Objective: We present a young woman with rapidly progressive Cushing’s syndrome due to ectopic ACTH production from a pancreatic NET. We describe severe neurological and immunosuppressive sequalae of marked hypercortisolemia associated with chemotherapy and reconsider our therapeutic strategy. Case: A previously active and healthy 28 year old woman was admitted with weakness and leg swelling. Two months prior she noticed a petechial rash on her face, back and arms. One week prior she was found to have DM and elevated liver chemistries prompting an abdominal US showing abnormal liver architecture. An outpatient CT showed a 3.7 cm pancreatic mass with liver lesions. Pertinent findings: BP 118/74, rounded facies, acneiform rash and leg edema, WBC 9400/ul, Glucose 173mg/dl, A1C 8.1%, K 2.3meql/L, random cortisol 114ug/dl, ACTH 830pg/ml, Calcitonin 1135 pg/mL, Chromogranin A 1482 ng/mL and Glucagon 276 pg/mL. After a 2 mg DST, 8 AM cortisol was 123 mcg/dL. We planned to reduce her ectopic ACTH production and hypercortisolemia with ketoconazole and chemotherapy followed by bilateral adrenalectomy. FOLFOX protocol reduced ACTH to 76 pg/mL and Cortisol to 73 mcg/dL but she clinically deteriorated, delaying adrenal surgery. She began having muscle weakness, aphasia, and other polyneuropathies. Blood cultures were positive for K. pneuomoniae and she received antibiotics. Cultures were negative for fungemia. Brain MRI showed extensive subcortical changes suggestive of PRES. Cortisol levels increased to 635 mcg/dL! In addition to ketoconazole and spironolactone, mifepristone was started with improvements in her hypokalemia and glucose. Mental status did not improve and repeat MRI showed findings suggestive of multiple abscesses. She was no longer a candidate for further chemotherapy or surgery and died within 40 days of admission. Discussion: Autopsy showed NET with hepatic metastases and multiple organs with fungal hyphae indicating Aspergillus. Both hypercortisolemia and chemotherapy predisposes to opportunistic infections. This patient’s remarkably elevated cortisol levels raised the consideration of urgent adrenalectomy prior to chemotherapy to limit steroid related complications and the polypharmacy required to control this. Both cortisol and chemotherapy impair cell mediated immunity and down regulate pro-inflammatory cytokines providing the opportunity for fungal and other uncommon infections to develop. This patient’s outcome could not have been worse if we elected adrenalectomy as the first therapeutic option to preclude the marked immunosuppressant effects of persisting hypercortisolemia together with chemotherapy. Otherwise, in a patient so profoundly immunosuppressed by endogenous steroids, a multitude of fungi and other atypical infections which are difficult to detect, need be considered when sepsis develops in association with chemotherapy.

Cerebral Vascular Function in a Murine Model of Experimental Cerebral Malaria

Cerebral Malaria (CM) is among the most severe complications of malarial infection, with a mortality rate of 15–20%. Many survivors of CM develop lasting neurological sequalae, cognitive and behavioral impairments, or epilepsy. However, the etiology of CM is poorly defined, and treatment strategies are limited.Studies in the murine model of experimental cerebral malaria (ECM) have demonstrated cerebral blood flow (CBF) impairments that are associated with decreased nitric oxide (NO) bioavailability, blood brain barrier disruption, endothelial activation, impaired pial vessel reactivity, and cerebral ischemia. While these complications have been observed in vivo, cerebral artery function has not specifically been evaluated in ECM. Therefore, the aim of this study was to interrogate vasoreactivity and NO bioavailability in cerebral arteries (CAs) isolated from ECM mice, with the hypothesis that arterial vasodilatory function would be impaired by ECM.ECM was induced by Plasmodium berghei ANKA (PbA) infection in 10–12 week old C57Bl/6J mice and confirmed by murine coma score. On day 6 post‐infection, CBF was measured by laser speckle imaging. Cerebral arteries (CAs) were dissected from the brain into a modified Krebs buffer. Cerebral vasoreactivity was assessed by ex vivo pressure myography in middle cerebral arteries (MCA) obtained from control (n=4) and ECM (n=7) mice. NO production from dissected vessels was measured in an ex vivo Daf‐2 fluorescence assay (n=6, n=6).As expected, ECM mice had a significant, 25% reduction in CBF. However, ex vivo MCAs from ECM mice maintained robust endothelium‐dependent vasodilation to methacholine (MCh), which was inhibited by L‐NAME. ECM MCAs also exhibited normal dose responses to serotonin‐induced vasoconstriction and nitroprusside‐induced vasodilation, suggesting normal vasoreactivity of ECM cerebral arteries. Similarly, the results of the ex vivo Daf‐2 assay showed that the MCh‐induced NO production from ECM arteries was comparable to controls. To test whether ECM plasma would induce vascular dysfunction in ex vivo CAs, we incubated CAs obtained from ECM (n=2) and control (n=6) mice in plasma collected from ECM or control animals. Surprisingly, we found that plasma from ECM mice increased NO production of both ECM and control CAs.These unexpected results indicate that NO bioavailability and vasoreactivity are preserved in cerebral vessels in ECM, and that circulating factors in the plasma during PbA infection actively stimulate NO production. Our next focus will be on reconciling decreased CBF with preserved vascular function, as well as identifying the NO‐stimulating mediator(s) present within ECM plasma.Support or Funding InformationResearch and funding support from NIAID Division of Intramural Research.This abstract is from the Experimental Biology 2019 Meeting. There is no full text article associated with this abstract published in The FASEB Journal.

Zika virus replication and cytopathic effects in liver cells.

Zika virus (ZIKV) has emerged globally as an important pathogen, since it has been recognized as a cause of microcephaly and other neurologic processes and sequalae in newborns. The virus shares homology with Hepaciviruses and therefore may be a cause of hepatitis. We sought to characterize ZIKV replication in hepatocyte-derived cell lines. Huh7.5 and HepG2 cells were infected with ZIKV and replication potential was evaluated by multiple methods including plaque assay, qRT-PCR, negative-strand ZIKV RNA production, and ZIKV NS1 protein production. Growth curves in cells and supernatant were compared to replicative capacity in Vero cells. Overall, viral replication in both hepatocyte lines approximated that observed in the Vero cells. Cell cytopathology was observed after 3 days of infection and apoptosis markers increased. Transmission electron microscopy revealed evidence of viral capsids in cells and negative staining revealed ZIKV particles in the supernatant. Conclusions: Hepatocyte-derived cell lines are permissive for ZIKV replication and produce an overt cytopathic effect consistent with development of an acute viral hepatitis. Further evaluation of replication and injury is warranted.

Paradoxical Reactions in Tuberculosis : Clinicians Burnout

We want to make our observations regarding worsening of clinical and radiologic features in a patient of potts spine after institution of antitubercular therapy. The index case is 34 year old male presented with pain in lower back and fever for past many months. Clinical examination revealed febrile, weak and anxious person with normal clinical examination. His investigations revealed Hb 11.8 mg/dl, TLC, DLC, platelet count normal. ESR was 52 mm at 1st hour and Mantoux was 12 mm at 72 hrs. CXR, USG abdomen were normal. Brucella agglutination titre was not suggestive for endemic area. Retroviral card test and serology was negative. MRI lumbosacral spine revealed spondodiscitis L2 and L3 vertebrae with large paraspinal collection. Patient underwent decompression surgery with removal of necrotic collection. Histocytology of the specimens revealed langerhan type gaint cells and caseous necrotic material. Patient was put on antitubercular treatment. Patient started with subjective worsening after 2 weeks of treatment when repeat imaging revealed accumulation of paraspinal collection with pressure on lower end of spinal cord and cauda equina. Patient antitubercular treatment were continued with addition of dexamethasone. The clinical scenario started improving after 5 days of institution of steroids. The steroids were continued for 4 weeks and then tapered over next 2 weeks. Patient started to show marked clinical improvement at 2 months. The intensive phase of antitubercular therapy was continued for additional one month and maintenance phase was given for 9 months. After one year of institution of treatment patient had no neurologic sequalae and complete radiologic resolution. JMS 2018: 21 (2):127-128

Lateral temporal lobe meningoencephalocele: Transmastoid repair with surgicel and temporalis graft

Temporal lobe meningoencephalocele is a rare entity and the acquired etiology is commoner especially from iatrogenic injury to the tegmen during mastoidectomy. Non-specificity of its clinical presentations often lead to delay in diagnosis and its neurological sequalae can be devastating. Hence, both clinical suspicion and imaging are both complementary. Various approaches have been described in meningoencephalocele repair. Here, we described a successful repair of the disease via transmastoid method for a huge tegmen defect, with simple materials, included surgicel and temporalis fascia graft.

Clinico-epidemiological features of Japanese encephalitis patients hospitalized in a tertiary care center

Background: Japanese encephalitis (JE) is of particular interest as it has a high morbidity and mortality. Neurological sequale is the most dreaded damage caused by JE. It is a preventable disease with specific interventions. The objective of the study was to study the demography, clinical profile and outcome of patients with Japanese Encephalitis admitted to the wards of Internal Medicine and Pediatrics at Jorhat Medical College Hospital.Methods: Hospital based observational study for one year in Jorhat Medical College, Jorhat, Assam.Results: The mean age for JE was 32.25±27 years for male, 27.47±22 years for female and 29.94±24 years overall. Assessment of clinical signs and symptoms showed that fever and change in mental status were present in 100% of JE cases followed by neck rigidity in 79.3% and headache in 68.9%. 44.8% of JE cases had history of seizure, 37.9% had vomiting, 34.5% had irritability, 13.8% were unconscious. The peak of JE incidence occurred in the month of July (77.6%). Complete recovery was seen in 39.2%, followed by death in 32.6% and recovery with neurological sequalae in 28.2% at the time of discharge.Conclusions: Vigorous awareness activities should be carried out to sensitize people on prevention of JE.

Nervous System Injury and Neuroimaging of Zika Virus Infection.

In 2016, World Health Organization announced Zika virus infection and its neurological sequalae are a public health emergency of global scope. Preliminary studies have confirmed a relationship between Zika virus infection and certain neurological disorders, including microcephaly and Guillain–Barre syndrome (GBS). The neuroimaging features of microcephaly secondary to Zika virus infection include calcifications at the junction of gray–white matter and subcortical white matter with associated cortical abnormalities, diminution of white matter, large ventricles with or without hydrocephalus, cortical malformations, hypoplasia of cerebellum and brainstem, and enlargement of cerebellomedullary cistern. Contrast enhancement of the cauda equine nerve roots is the typical neuroimaging finding of GBS associated with Zika virus. This review describes the nervous system disorders and associated imaging findings seen in Zika virus infection, with the aim to improve the understanding of this disease. Imaging plays a key role on accurate diagnosis and prognostic evaluation of this disease.

Varicella meningitis : An atypical presentation in an immunocompetent individual with seizures managed with acyclovir

Viral meningitis, also known as aseptic meningitis, is the most common cause of meningitis and is responsible for between 26,000 and 42,000 hospitalizations yearly in the United States. Bacterial meningitis affects about 4,000 people each year, while viral meningitis affects about 10 people in every 100,000. The most commonly identified pathogens are enterovirus, herpes simplex virus and human immunodeficiency virus. Varicella, rabies and influenza are other viruses that are rarely responsible for viral meningitis. Varicella Zoster is a relatively common disorder in immunocompromised patients characterized by radicular pain and grouped vesicular eruptions. The typical findings in reported cases of VZV meningitis involve skin manifestations and classical signs of meningitis such as neck stiffness. However, only a few cases of meningitis caused by Varicella Zoster Virus (VZV) have been reported. The incidence of VZV meningitis in immunocompetent patients has been reported to be as low as 0.5%. We report an atypical presentation of VZV meningitis in an immunocompetent patient with headache, dizziness, sequalae of seizures without fever and skin manifestations.Our patient is a 59‐year‐ old African American male who presented with worsening dizziness, confusion, ataxia, lethargy and left‐sided frontal headache. Magnetic resonance imaging revealed mild to moderate burden of white matter abnormality, nonspecific but most likely due to chronic microvascular ischemic disease. No acute stroke. The next day sedation was weaned off and patient remained seizure free on continuous EEG and was extubated 3 days later the CSF came back positive for an elevated Varicella PCR of 42016. Patient was seen by infectious diseases consultant and was advised a course of acyclovir 10mg/kg intravenous every 8 hours for 14 days. All other antibiotics were stopped. Upon discharge, the patient had fully recovered from his symptoms with no neurologic sequalae.This unique case illustrates an atypical presentation of VZV meningoencephalitis in an immunocompetent patient that lacks many of the obvious features that usually distinguish the disease. Our patient lacked the vesicular rash, as well as other typical features of aseptic meningitis including nuchal rigidity, headache, photophobia, etc. Additionally, the patient's course was complicated by seizure, another relatively uncommon feature of VZV meningoencephalitis. VZV infection or reactivation is, rightfully, most commonly associated with the vesicular rash and prodromal pain that usually accompanies it.This abstract is from the Experimental Biology 2018 Meeting. There is no full text article associated with this abstract published in The FASEB Journal.

Giant subdural empyema in adult

Subdural empyema (SDE) is a fairly uncommon diagnosis, accounting for only 20% of intracranial infections, the usual source for SDE is direct extension from a contiguous source, such as acute sinusitis or otitis media, although in one-fourth of cases no source of infection is found. An 18-year-old man was admitted to our Institution because of dysarthria, lethargy and fever for 2 days. His medical history was marked by chronic otitis media badly treated by self-medication with several antibiotics. The first clinical examination revealed an increased body temperature of 37.6°C, heart rate of 82 beats per minute, blood pressure 140/74mmHg and respiratory rate of 18 breaths/minute. He was disorientated, his cranial nerves were intact and his pupils were reactive, round and equal. His white cell count was 6,620 per ¼L. His hemoglobin was 11.1g/dL and C-reactive protein was 80mg/L. The levels of his chemistries were normal. A computed tomography (CT) scan with contrast in emergency was performed and showed an extensive subdural fluid collection in the parietal site on the left side with peripheral enhancement, a mass effect was evident with displacement of the middle line structures toward the opposite side (A). An emergent craniotomy was done to evacuate the subdural empyema (B). A cultural examination was attempted with the fluid evacuation and showed no presence of bacteroides. Ceftriaxone and metronidazole were administered for 6 weeks. The patient was improved without neurological sequalae.

Cobinamide is effective for treatment of hydrogen sulfide-induced neurological sequelae in a mouse model.

Hydrogen sulfide (H2 S) is a highly neurotoxic gas. Acute exposure can lead to neurological sequelae among survivors. A drug for treating neurological sequelae in survivors of acute H2 S intoxication is needed. Using a novel mouse model we evaluated the efficacy of cobinamide (Cob) for increasing survival of, and reducing neurological sequalae in, mice exposed to sublethal doses of H2 S. There were two objectives: (1) to determine the dose-response efficacy of Cob and (2) to determine the effective therapeutic time window of Cob. To explore objective 1, mice were injected intramuscularly with Cob at 0, 50, or 100mg/kg at 2min after H2 S exposure. For objective 2, mice were injected intramuscularly with 100mg/kg Cob at 2, 15, and 30min after H2 S exposure. For both objectives, mice were exposed to 765ppm of H2 S gas. Cob significantly reduced H2 S-induced lethality in a dose-dependent manner (P<0.05). Cob-treated mice exhibited significantly fewer seizures and knockdowns compared with the H2 S-exposed group. Cob also reversed H2 S-induced weight loss, behavioral deficits, neurochemical changes, cytochrome c oxidase enzyme inhibition, and neurodegeneration in a dose- and time-dependent manner (P<0.01). Overall, these findings show that Cob increases survival and is neuroprotective in a mouse model of H2 S-induced neurological sequelae.

Cause of acute encephalitis/encephalopathy in Japanese children diagnosed by a rapid and comprehensive virological detection system and differences in their clinical presentations

BackgroundAcute encephalitis/encephalopathy (AE/E) is a rare and severe complication of common childhood infections; however, a treatment strategy based on clinical and pathological evidence has not been established. MethodsThe clinical data and aetiological results using a rapid and comprehensive virological detection system of 62 Japanese children diagnosed with AE/E from 2010 to 2014 were collected. We assessed clinical differences between causes and effectiveness of our multiplex PCR system to establish a pathogen-based treatment strategy for AE/E. ResultsSuspected causes were detected in 84% of patients, and our multiplex PCR system contributed to diagnosing 38% of the patients. Furthermore, a negative virus PCR might be important for inferring underlying disease. Most cases were triggered by human herpes virus (HHV) 6/7 (32%) and influenza virus (24%). The causes of AE/E depended on age (p=0.00089) but not on sex (p=0.94). The median age of HHV6/7-associated AE/E was 2.3years, which is lower than the median ages of AE/E associated with other viruses. Major initial treatments were pulse steroid therapy (83.9%) and acyclovir (71%). Most of the patients in this study had good prognoses: 77% recovered without neurological sequalae. ConclusionsOur virological detection system was useful for detecting the cause of AE/E, and may also contribute to construction of pathogen-based treatment strategies for AE/E. Our data indicated the possibility that early intervention with pulse steroid therapy could be effective for treating AE/E. Further investigation for selection of antiepileptic drugs and additional therapies might be required to prevent progression of AE/E.

Acute cerebellitis in children: A series of eight cases

Objective: Acute cerebellitis is a rare condition often characterized by severe headache, vomiting, fever, ataxia and loss of consciousness. Its course is usually self-limited but occasionally fulminant. Differentiation of acute infectious cerebellitis from postinfectious cerebellitis is usually based on the presence of fever and absence of a latent period following a non-specific infection. Methods: We present clinical and paraclinical findings, treatment and outcome data of eight children diagnosed with acute cerebellitis in our department between 2010-2016. Results: There were 5 girls and 3 boys aged 3-16.5 years, median 8 years. Main presenting symptoms were headache, fever, vomiting and ataxia. Most common MR imaging finding at presentation was bilateral, diffuse signal changes in cerebellar hemispheres. Two patients had hydrocephalus, one had herniation, and one, both hydrocephalus and herniation. Etiological work-up for infectious pathogens revealed mycoplasma pneumoniae and influenza virus in one case each. Two cases underwent lumbar puncture: one was normal, the other showed elevated protein and low glucose. Treatment included steroids in all cases, acyclovir in 7, antibiotics in 2, intravenous immunoglobulins in 1, oseltamivir in one case. No patients required surgery for hydrocephalus or herniation. Four patients had full recovery and four had residual neurologic deficits consisting in cerebellar tremor and ataxia (n=3) and cognitive deficit (n=2). Patients with neurological sequalae had cerebellar atrophic changes on follow-up MRI. Patients admitted >7 days after symptom onset recovered with sequalae while those admitted<3 days showed complete recovery. Conclusion: There were no fatalities in this series of acute cerebellitis, suggesting early diagnosis and treatment can increase the chance of uneventful recovery.

Medical management and perioperative issues in pediatric pheochromocytoma – A case report with review of literature

Pheochromocytoma is a tumor arising out of embryological neural crest, being a tumor of adrenal medulla secreting predominantly norepinephrine as well as epinephrine(about 20% cases) leading to frequent surges of vasopressor catecholamines leading to systemic hypertension, hypertensive emergencies and associated cardiac, neurological, and renal sequalae. Though somewhat rare in pediatric age group, it remains an important etiological factor to think about when there is intractable hypertension or rapid swings of blood pressures leading to headaches and palpitations with family history of hypertension or as a part of familial endocrine neoplasia. Treatment remains laproscopic or open surgical resection under general anesthesia, however perioperative care remains a challenge for the anesthesiologist, intensivist requiring adequate medical control of hypertension with alpha blockers such as phenoxybenza- mine before undertaking anesthetic induction and important monitoring measures instituted during intraoperative and postoperative period for close monitoring of blood pressure and intravascular volume status as well as hemodynamics. We review a case report with literature review regarding perioperative management of pheochromocytoma.

Giant subdural empyema following ventriculo-peritoneal shunt in a child.

Subdural empyema is an intracranial focal collection of purulent material located between the dura mater and the arachnoid mater. It developed from varied sources, but the paranasal sinuses, the ears, and the mastoids processes were predominantly affected. Giant subdural empyema secondary to cerebrospinal fluid shunt placement has been extremely unusual. A 9-years-old girl presented with prolonged low-grade fever, vomiting, local wound infection in frontal area and general weakness. She was previously hydrocephalic with large head size since birth. A ventriculo-peritoneal shunt was already inserted for 7 years. Neurological examination showed the patient fully conscious and left hemiparesis. Routine hematological investigation revealed leukocytosis and high C-reactive protein (CRP). A contrast-enhanced CT scan showed an hypodense fluid collection with peripheral ring enhancement on the right of the midline supporting a giant subdural empyema. Emergency surgery was performed to drainage the pus. Pus culture resulted no growth of bacteria. Ceftriaxone and metronidazole were administered for 6 weeks. Follow-up CT Scan showed reduction of mass effect. The patient was improved leaving neurological sequalae.