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Sample records for subacute necrotizing encephalopathy

  1. [Leigh's encephalopathy (subacute necrotizing encephalopathy). Documentation of its evolution through neuroimaging].

    Science.gov (United States)

    Pena, J A; González-Ferrer, S; Martínez, C; Prieto-Carrasquero, M; Delgado, W; Mora La Cruz, E

    1996-09-01

    A 30 months-old boy developed bilateral nistagmus, tremor, gait disturbance, hypotonia and disartria. The diagnose of Leigh encephalopathy was suggested on the basis of clinical, neuroimaging and laboratory findings. Computed tomography and magnetic resonance imaging (MRI) at an early stage revealed bilateral and symmetric lesions in the putamen, appearing as hyperintense signal on T2-weighted images. Twelve months later a relatively large hypertense area in the posterior brainstem was observed. At this stage, the patient exhibited marked deterioration, dystonic manifestations, rigidity and respiratory disturbances. He died 6 months later for respiratory arrest during bronconeumonic infection. We believe MRI is a valuable means to allow assessment of the evolution of the disease.

  2. MR findings of subacute necrotizing myelopathy: case report

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    Na, Dong Gyu; Chang, Kee Hyun; Han, Moon Hee; Kim, Hyun Jip; Kim, Chong Jai; Chi, Je G. [Seoul National University College of Medicine, Seoul (Korea, Republic of)

    1994-04-15

    Subacute necrotizing myelopathy(SNM) is a rare non-tumorous disease of spinal cord characterized by subacute clinical course of progressive neurological deterioration. We report MR findings of a patient with pathologically proved SNM. 1 case of pathologically proved subacute necrotizing myelopathy. The patients was a 56-year-old man with progressive motor weakness and sensory loss of the lower extremities, and urinary and fecal incontinence for 11 months. Spine MRI revealed diffuse enlargement of the thoracic spinal cord from T2 to T7 level. Signal intensity of the expanded spinal cord was isointense relative to normal cord on T1-weighted image and hyperintense on proton-density and T2-weighted images. On contrast enhanced T1-weighted image, there was diffuse homogeneous enhancement in the expanded cord lesion. MR demonstration of stable persistence of spinal cord lesion or atrophy over months or years with clinical findings of gradual progressive neurologic deterioration may be helpful in the diagnosis of SNM.

  3. Successful treatment of encephalopathy and myoclonus with levetiracetam in a case of subacute sclerosing panencephalitis.

    Science.gov (United States)

    Becker, Daniel; Patel, Anup; Abou-Khalil, Bassel W; Pina-Garza, Jesus E

    2009-06-01

    Subacute sclerosing panencephalitis is a devastating progressive degenerative disease of the nervous system presumably caused by a persistent measles virus. Patients commonly present with myoclonia or encephalopathy. There are currently no known curative therapeutic options or effective symptomatic therapy. We treated a 12-year-old boy with subacute sclerosing panencephalitis who presented with acute encephalopathy and myoclonus. Electroencephalogram showed characteristic generalized periodic discharges. Levetiracetam produced dramatic improvement in both myoclonus and encephalopathy. The improvement was clear within 4 days. The electroencephalogram pattern showed improvement as well. Levetiracetam is a promising symptomatic therapy in subacute sclerosing panencephalitis for both the myoclonus and the encephalopathy. In this patient, it also appeared to improve the electroencephalographic pattern. We suggest that the generalized periodic discharges associated with the myoclonus contributed to the patient's encephalopathy.

  4. An Atypical Presentation of Subacute Encephalopathy with Seizures in Chronic Alcoholism Syndrome

    OpenAIRE

    Kim, Tae-Kyoung; Jung, Eui Sung; Park, Jong-Moo; Kang, Kyusik; Lee, Woong-Woo; Lee, Jung-Ju

    2016-01-01

    Subacute encephalopathy with seizures in chronic alcoholism syndrome is a rare clinical manifestation in patients with chronic alcohol abuse. We report the case of a patient with chronic alcoholism who presented with partial nonconvulsive status epilepticus associated with a thalamic lesion.

  5. Moyamoya disease in a child with previous acute necrotizing encephalopathy

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    Kim, Taik-Kun; Cha, Sang Hoon; Chung, Kyoo Byung; Kim, Jung Hyuck; Kim, Baek Hyun; Chung, Hwan Hoon [Department of Diagnostic Radiology, Korea University College of Medicine, Ansan Hospital, 516 Kojan-Dong, Ansan City, Kyungki-Do 425-020 (Korea); Eun, Baik-Lin [Department of Pediatrics, Korea University College of Medicine, Seoul (Korea)

    2003-09-01

    A previously healthy 24-day-old boy presented with a 2-day history of fever and had a convulsion on the day of admission. MRI showed abnormal signal in the thalami, caudate nuclei and central white matter. Acute necrotising encephalopathy was diagnosed, other causes having been excluded after biochemical and haematological analysis of blood, urine and CSF. He recovered, but with spastic quadriparesis. At the age of 28 months, he suffered sudden deterioration of consciousness and motor weakness of his right limbs. MRI was consistent with an acute cerebrovascular accident. Angiography showed bilateral middle cerebral artery stenosis or frank occlusion with numerous lenticulostriate collateral vessels consistent with moyamoya disease. (orig.)

  6. Acute necrotizing encephalopathy of childhood: typical findings in an atypical disease

    Energy Technology Data Exchange (ETDEWEB)

    Skelton, Brandon W.; Phillips, C.D. [University of Virginia Health System, Department of Neuroradiology, Charlottesville, VA (United States); Hollingshead, Michael C.; Castillo, Mauricio [University of North Carolina School of Medicine, Neuroradiology Section, Chapel Hill, NC (United States); Sledd, Andrew T. [University of Virginia Health System, Department of Pediatrics, Charlottesville, VA (United States)

    2008-07-15

    Acute necrotizing encephalopathy of childhood (ANEC) is a disease entity seen nearly exclusively in East Asian children that is characterized by multifocal, symmetric lesions involving the thalami, brainstem, cerebellum, and white matter. We present a child who developed dramatic neurologic symptoms following a viral prodrome. Serial MRI examinations demonstrated characteristic lesions of ANEC, while laboratory analyses revealed evidence of acute infection with human herpesvirus-6 (HHV-6). We highlight the MRI findings in both the acute and convalescent phases of ANEC, discuss the implications of neuroimaging on the child's clinical course, and emphasize the integral role of the radiologist in correctly diagnosing this rare disease. (orig.)

  7. Novel Influenza A (H1N1)-Associated Acute Necrotizing Encephalopathy: A Case Report

    OpenAIRE

    Kim, Ki Jung; Park, Eun Sook; Chang, Hyun Jung; Suh, Miri; Rha, Dong-Wook

    2013-01-01

    Several cases of acute necrotizing encephalopathy (ANE) with influenza A (H1N1) have been reported to date. The prognosis of ANE associated with H1N1 is variable; some cases resulted in severe neurologic complication, whereas other cases were fatal. Reports mostly focused on the diagnosis of ANE with H1N1 infection, rather than functional recovery. We report a case of ANE with H1N1 infection in a 4-year-old Korean girl who rapidly developed fever, seizure, and altered mentality, as well as ha...

  8. Novel Influenza A (H1N1)-Associated Acute Necrotizing Encephalopathy: A Case Report

    Science.gov (United States)

    Kim, Ki Jung; Park, Eun Sook; Chang, Hyun Jung; Suh, Miri

    2013-01-01

    Several cases of acute necrotizing encephalopathy (ANE) with influenza A (H1N1) have been reported to date. The prognosis of ANE associated with H1N1 is variable; some cases resulted in severe neurologic complication, whereas other cases were fatal. Reports mostly focused on the diagnosis of ANE with H1N1 infection, rather than functional recovery. We report a case of ANE with H1N1 infection in a 4-year-old Korean girl who rapidly developed fever, seizure, and altered mentality, as well as had neurologic sequelae of ataxia, intentional tremor, strabismus, and dysarthria. Brain magnetic resonance imaging showed lesions in the bilateral thalami, pons, and left basal ganglia. To our knowledge, this is the first report of ANE caused by H1N1 infection and its long-term functional recovery in Korea. PMID:23705127

  9. Necrotizing Enteritis and Hyperammonemic Encephalopathy Associated With Equine Coronavirus Infection in Equids.

    Science.gov (United States)

    Giannitti, F; Diab, S; Mete, A; Stanton, J B; Fielding, L; Crossley, B; Sverlow, K; Fish, S; Mapes, S; Scott, L; Pusterla, N

    2015-11-01

    Equine coronavirus (ECoV) is a Betacoronavirus recently associated clinically and epidemiologically with emerging outbreaks of pyrogenic, enteric, and/or neurologic disease in horses in the United States, Japan, and Europe. We describe the pathologic, immunohistochemical, ultrastructural, and molecular findings in 2 horses and 1 donkey that succumbed to natural infection with ECoV. One horse and the donkey (case Nos. 1, 3) had severe diffuse necrotizing enteritis with marked villous attenuation, epithelial cell necrosis at the tips of the villi, neutrophilic and fibrinous extravasation into the small intestinal lumen (pseudomembrane formation), as well as crypt necrosis, microthrombosis, and hemorrhage. The other horse (case No. 2) had hyperammonemic encephalopathy with Alzheimer type II astrocytosis throughout the cerebral cortex. ECoV was detected by quantitative polymerase chain reaction in small intestinal tissue, contents, and/or feces, and coronavirus antigen was detected by immunohistochemistry in the small intestine in all cases. Coronavirus-like particles characterized by spherical, moderately electron lucent, enveloped virions with distinct peplomer-like structures projecting from the surface were detected by negatively stained transmission electron microscopy in small intestine in case No. 1, and transmission electron microscopy of fixed small intestinal tissue from the same case revealed similar 85- to 100-nm intracytoplasmic particles located in vacuoles and free in the cytoplasm of unidentified (presumably epithelial) cells. Sequence comparison showed 97.9% to 99.0% sequence identity with the ECoV-NC99 and Tokachi09 strains. All together, these results indicate that ECoV is associated with necrotizing enteritis and hyperammonemic encephalopathy in equids. © The Author(s) 2015.

  10. Infection-triggered familial or recurrent cases of acute necrotizing encephalopathy caused by mutations in a component of the nuclear pore, RANBP2

    DEFF Research Database (Denmark)

    Neilson, Derek E; Adams, Mark D; Orr, Caitlin M D

    2009-01-01

    Acute necrotizing encephalopathy (ANE) is a rapidly progressive encephalopathy that can occur in otherwise healthy children after common viral infections such as influenza and parainfluenza. Most ANE is sporadic and nonrecurrent (isolated ANE). However, we identified a 7 Mb interval containing a ...

  11. Encephalopathy

    Science.gov (United States)

    ... drugs, radiation, paints, industrial chemicals, and certain metals), chronic progressive trauma, poor nutrition, or lack of oxygen or blood flow to the brain. The hallmark of encephalopathy is an altered mental state. Depending on the ...

  12. Optic neuritis and rapidly progressive necrotizing retinitis as the initial signs of subacute sclerosing panencephalitis: a case report with clinical and histopathologic findings.

    Science.gov (United States)

    Oray, Merih; Tuncer, Samuray; Kir, Nur; Karacorlu, Murat; Tugal-Tutkun, Ilknur

    2014-08-01

    We report a case of subacute sclerosing panencephalitis (SSPE) presenting first with optic neuritis and rapidly progressive necrotizing retinitis at the posterior pole. We reviewed the clinical, laboratory, photographic, angiographic, and histopathologic records of a patient with SSPE. A 15-year-old girl was referred after rapid loss of vision due to optic neuritis and macular necrosis in the right eye. She had a history of cardiac valve surgery, but had no systemic symptoms and extensive work-up was unrewarding. Contralateral involvement with rapidly progressive optic neuritis and macular necrotizing retinitis prompted retinochoroidal biopsy of the right eye, which revealed necrosis of inner retinal layers and perivascular lymphoplasmocytic infiltration with intact choroid and outer retina without any findings of inclusion bodies, microorganisms, or atypical cells. The diagnosis was based on histopathologic findings consistent with SSPE, and detection of elevated measles antibody titers in cerebrospinal fluid and serum. It was further confirmed by development of typical electroencephalography pattern at 6 months and neurological symptoms at 4-year follow-up. Clinicians need to be aware that optic neuritis and necrotizing retinitis at the posterior pole may be the presenting features of SSPE.

  13. A 29-year-old pregnant woman with worsening left hemiparesis, encephalopathy, and hemodynamic instability: a case report of subacute sclerosing panencephalitis.

    Science.gov (United States)

    Reis, Gerald F; Ritter, Jana M; Bellini, William J; Rota, Paul A; Bollen, Andrew W

    2015-01-01

    A 29-year-old pregnant woman developed progressively worsening encephalopathy, left hemiparesis, and hemodynamic instability over a 6-week period. Initial brain MRI and work-up for infectious and autoimmune causes were normal, although elevated IgG and oligoclonal bands were seen on analysis of the cerebrospinal fluid (CSF). After uncomplicated spontaneous delivery of a preterm healthy infant, her condition worsened. Repeat brain MRI demonstrated generalized volume loss and evidence of corticospinal tract degeneration. She underwent a brain biopsy, which showed characteristic viral inclusions of the type seen in subacute sclerosing panencephalitis (SSPE). The diagnosis was confirmed by immunohistochemistry and electron microscopy, and additional CSF analysis also showed markedly elevated IgG titer for measles. Sequence analysis of the nucleoprotein gene N-450 demonstrated a close relationship to the sequences of viruses in genotype D7. This case documents an ~ 6-month progression to death of SSPE in a pregnant woman.

  14. Acute Necrotizing Encephalopathy

    Directory of Open Access Journals (Sweden)

    J Gordon Millichap

    2003-07-01

    Full Text Available A 14-month-old girl who presented with sudden onset of unreponsiveness following a fever for 2 days developed decerebrate posturing within 9 hours after admission to Children’s Memorial Hospital, Chicago, IL.

  15. Granulomatous herpes simplex encephalitis in an infant with multicystic encephalopathy: a distinct clinicopathologic entity?

    Science.gov (United States)

    Schutz, Peter W; Fauth, Clarissa T; Al-Rawahi, Ghada N; Pugash, Denise; White, Valerie A; Stockler, Sylvia; Dunham, Christopher P

    2014-04-01

    Herpes simplex virus encephalitis can manifest as a range of clinical presentations including classic adult, neonatal, and biphasic chronic-granulomatous herpes encephalitis. We report an infant with granulomatous herpes simplex virus type 2 encephalitis with a subacute course and multicystic encephalopathy. A 2-month-old girl presented with lethargy and hypothermia. Computed tomography scan of the head showed multicystic encephalopathy and calcifications. Cerebrospinal fluid analysis by polymerase chain reaction testing for herpes simplex virus 1 and 2, enterovirus, and cytomegalovirus was negative. Normal cerebrospinal fluid interferon-α levels argued against Aicardi-Goutières syndrome. The patient died 2 weeks after presentation. At autopsy, multicystic encephalopathy was confirmed with bilateral gliosis, granulomatous inflammation with multinucleated giant cells, and calcifications. Bilateral healing necrotizing retinitis suggested a viral etiology, but retina and brain were free of viral inclusions and immunohistochemically negative for herpes simplex virus-2 and cytomegalovirus. However, polymerase chain reaction analysis showed herpes simplex virus-2 DNA in four cerebral paraffin blocks. Subsequent repeat testing of the initial cerebrospinal fluid sample using a different polymerase chain reaction assay was weakly positive for herpes simplex virus-2 DNA. Granulomatous herpes simplex virus encephalitis in infants can present with subacute course and result in multicystic encephalopathy with mineralization and minimal cerebrospinal fluid herpes simplex virus DNA load. Infectious etiologies should be carefully investigated in the differential diagnosis of multicystic encephalopathy with mineralization, in particular if multinucleated giant cells are present. Copyright © 2014 Elsevier Inc. All rights reserved.

  16. Encefalopatia necrotizante aguda: paciente com evolução recidivante e letal Acute necrotizing encephalopathy: patient with a relapsing and lethal evolution

    Directory of Open Access Journals (Sweden)

    Erasmo B. Casella

    2007-06-01

    Full Text Available A encefalopatia necrotizante aguda foi descrita inicialmente em crianças japonesas e se caracteriza por rápida evolução e lesões simétricas no tronco encefálico, cerebelo e especialmente nos tálamos. Avaliamos uma menina de 7 meses de idade, que apresentou dois episódios de depressão da consciência de rápida instalação e paresias, sem alterações metabólicas. Houve uma rápida melhora na primeira crise, porém o segundo episódio foi fulminante, tendo evoluído para estado de morte encefálica em dois dias. Os estudos de ressonância magnética mostraram lesões simétricas nos tálamos e acometimento também do tronco encefálico e cerebelo.Acute necrotizing encephalopathy was initially reported in Japanese children. The rapid evolution and symmetrical brain lesions seen in the brainstem, cerebellum and specially in the thalamus characterize the disease. We studied a 7-month-old-girl, who presented with two episodes of rapid loss of consciousness and paresis without metabolic disturbances. At the first time she had a rapid improvement, but at the second episode the course was fulminant and in two days she lapsed into a clinical state of brain death. The magnetic resonance studies showed symmetrical lesions in the thalamus and additional lesions involving the brainstem and the cerebellum.

  17. Acute necrotizing encephalopathy secondary to diphtheria, tetanus toxoid and whole-cell pertussis vaccination: diffusion-weighted imaging and proton MR spectroscopy findings

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    Aydin, Hale; Ozgul, Esra; Agildere, Ahmet Muhtesem [Baskent University Hospital, Department of Radiology, Ankara (Turkey)

    2010-07-15

    We present a previously healthy 6-month-old boy who was admitted to our hospital with lethargy, hypotonia and focal clonic seizures 6 days following diptheria, tetanus toxoid and whole-cell pertussis vaccination. A diagnosis of acute necrotising encephalopathy was made with the aid of MRI, including diffusion-weighted imaging and proton MR spectroscopy. (orig.)

  18. Hepatic Encephalopathy

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    Full Text Available ... Stages of Hepatic Encephalopathy? What Triggers or Can Cause HE to Get Worse? How is HE Diagnosed? ... portosystemic encephalopathy or PSE, is a condition that causes temporary worsening of brain function in people with ...

  19. Hashimoto's Encephalopathy

    National Research Council Canada - National Science Library

    Schiess, Nicoline; Pardo, Carlos A

    2008-01-01

    Hashimoto's encephalopathy (HE) is a controversial neurological disorder that comprises a heterogenous group of neurological symptoms that manifest in patients with high titers of antithyroid antibodies...

  20. Posterior reversible encephalopathy syndrome.

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    Fischer, Marlene; Schmutzhard, Erich

    2017-08-01

    The posterior reversible encephalopathy syndrome (PRES) is a neurological disorder of (sub)acute onset characterized by varied neurological symptoms, which may include headache, impaired visual acuity or visual field deficits, disorders of consciousness, confusion, seizures, and focal neurological deficits. In a majority of patients the clinical presentation includes elevated arterial blood pressure up to hypertensive emergencies. Neuroimaging, in particular magnetic resonance imaging, frequently shows a distinctive parieto-occipital pattern with a symmetric distribution of changes reflecting vasogenic edema. PRES frequently develops in the context of cytotoxic medication, (pre)eclampsia, sepsis, renal disease or autoimmune disorders. The treatment is symptomatic and is determined by the underlying condition. The overall prognosis is favorable, since clinical symptoms as well as imaging lesions are reversible in most patients. However, neurological sequelae including long-term epilepsy may persist in individual cases.

  1. Hepatic Encephalopathy

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    Full Text Available ... the Stages of Hepatic Encephalopathy? What Triggers or Can Cause HE to Get Worse? How is HE ... liver disease. When your liver is damaged it can no longer remove toxic substances from your blood. ...

  2. Hepatic Encephalopathy

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    Full Text Available ... Hepatic Encephalopathy so you can tell your doctor right away if you think you may have it. ... American Liver Foundation © 2018 American Liver Foundation. All rights reserved. Funding for the HE123 - Diagnosis, Treatment and ...

  3. Hepatic Encephalopathy

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    Full Text Available ... Get Worse? How is HE Diagnosed? Prior to Treatment Who treats HE? Preparing for your Medical Appointment Hepatic Encephalopathy Treatment Options Treatment Basics Treatment Medications Importance of Adhering ...

  4. Hepatic Encephalopathy

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    Full Text Available ... Hepatic Encephalopathy so you can tell your doctor right away if you think you may have it. ... American Liver Foundation © 2017 American Liver Foundation. All rights reserved. Funding for the HE123 - Diagnosis, Treatment and ...

  5. Hepatic Encephalopathy

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    Full Text Available ... Encephalopathy often starts slowly, and at first you may not be aware you have it. The stages ... your doctor right away if you think you may have it. Prompt identification and treatment of HE ...

  6. Hepatic Encephalopathy

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    Full Text Available ... to Treatment Who treats HE? Preparing for your Medical Appointment Hepatic Encephalopathy Treatment Options Treatment Basics Treatment ... treatment. Being a fully-informed participant in your medical care is an important factor in staying as ...

  7. Hepatic Encephalopathy

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    Full Text Available ... Symptoms to look for Caregiver Support Caregiver Stories Home › What is Hepatic Encephalopathy? Why Your Liver is ... questions about HE, one step at a time. Home About Us Ways to Give Contact Us Privacy ...

  8. Hepatic Encephalopathy

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    Full Text Available ... Hepatic Encephalopathy Treatment Options Treatment Basics Treatment Medications Importance of Adhering to Your Treatment Plan Long-Term ... disease is. It’s important for you and your family to become familiar with the signs of Hepatic ...

  9. Hepatic Encephalopathy

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    Full Text Available ... Reading Webinars Caregivers The Role of a Caregiver Signs and Symptoms to look for Caregiver Support Caregiver ... and your family to become familiar with the signs of Hepatic Encephalopathy so you can tell your ...

  10. Hepatic Encephalopathy

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    Full Text Available ... Are the Symptoms of HE? What Are the Stages of Hepatic Encephalopathy? What Triggers or Can Cause ... may not be aware you have it. The stages of HE span from mild to severe and ...

  11. Hepatic Encephalopathy

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    Full Text Available ... Cirrhosis of the Liver & Symptoms Why it’s Important to Treat HE Symptoms of Liver Failure Glossary of ... Hepatic Encephalopathy? What Triggers or Can Cause HE to Get Worse? How is HE Diagnosed? Prior to ...

  12. Unique optical coherence tomography findings in a case of macular retinitis caused by subacute sclerosing panencephalitis.

    Science.gov (United States)

    Hata, Masayuki; Oishi, Akio; Kurimoto, Yasuo

    2012-01-01

    Subacute sclerosing panencephalitis is a rare neurodegenerative disease. The ophthalmologic findings, typically necrotizing retinitis, often precede other neurologic signs and symptoms. Ocular findings are helpful for early diagnosis, but the findings of optical coherence tomography (OCT) images in an active phase of retinitis have never been reported. We report unique findings of OCT images in a case of subacute sclerosing panencephalitis. A 13-year-old boy was referred to our hospital complaining of visual loss and other neurologic symptoms. The fundus examination showed necrotizing retinitis. The OCT images showed empty spaces involving all layers of retina except the inner limiting membrane and hyperreflective lesion in inner retina. The diagnosis of subacute sclerosing panencephalitis was made from the blood and the spinal cerebral fluid test. This is the first report on OCT images of necrotizing retinitis in an active stage of subacute sclerosing panencephalitis case. The unique findings of OCT images as in this case help to diagnose the rare devastating disease.

  13. Hepatic Encephalopathy

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    Full Text Available ... toxic substances from your blood. These toxins build up and can travel through your body until they reach your brain, causing mental and physical symptoms of HE. Hepatic Encephalopathy often starts slowly, and at first you may not be ...

  14. Hepatic Encephalopathy

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    Full Text Available ... Triggers or Can Cause HE to Get Worse? How is HE Diagnosed? Prior to Treatment Who treats HE? Preparing for your Medical ... mild to severe and symptoms vary depending on how bad your liver disease is. It’s important for you and your family to become familiar with the signs of Hepatic Encephalopathy ...

  15. Hepatic Encephalopathy

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    Full Text Available ... Caregiver Signs and Symptoms to look for Caregiver Support Caregiver Stories Home › What is Hepatic Encephalopathy? Why Your Liver is ... questions about HE, one step at a time. Home About Us Ways to ... Funding for the HE123 - Diagnosis, Treatment and Support program is provided by Salix Pharmaceuticals

  16. Hashimoto's encephalopathy

    DEFF Research Database (Denmark)

    Montagna, Giacomo; Imperiali, Mauro; Agazzi, Pamela

    2016-01-01

    diseases and the most common feature is the presence of anti-thyroperoxidase antibodies (TPOAb). Patients are usually euthyroid or mildly hypothyroid at presentation. All age groups can be affected. The pathophysiology is still unclear, especially the link between elevated serum TPOAb...... and the encephalopathy. Most reported cases occurred in women and girls. Unspecific symptoms, non-pathognomonic laboratory neurophysiology and neuroimaging features make its diagnosis a real challenge for clinicians.The case of a 16 year old boy, with a clinical picture of HE associated with hypothyroidism...

  17. Necrotizing fasciitis: contribution and limitations of diagnostic imaging.

    Science.gov (United States)

    Malghem, Jacques; Lecouvet, Frédéric E; Omoumi, Patrick; Maldague, Baudouin E; Vande Berg, Bruno C

    2013-03-01

    Necrotizing fasciitis is a rare, rapidly spreading, deep-seated infection causing thrombosis of the blood vessels located in the fascia. Necrotizing fasciitis is a surgical emergency. The diagnosis typically relies on clinical findings of severe sepsis and intense pain, although subacute forms may be difficult to recognize. Imaging studies can help to differentiate necrotizing fasciitis from infections located more superficially (dermohypodermitis). The presence of gas within the necrotized fasciae is characteristic but may be lacking. The main finding is thickening of the deep fasciae due to fluid accumulation and reactive hyperemia, which can be visualized using computed tomography and, above all, magnetic resonance imaging (high signal on contrast-enhanced T1 images and T2 images, best seen with fat saturation). These findings lack specificity, as they can be seen in non-necrotizing fasciitis and even in non-inflammatory conditions. Signs that support a diagnosis of necrotizing fasciitis include extensive involvement of the deep intermuscular fascias (high sensitivity but low specificity), thickening to more than 3mm, and partial or complete absence on post-gadolinium images of signal enhancement of the thickened fasciae (fairly high sensitivity and specificity). Ultrasonography is not recommended in adults, as the infiltration of the hypodermis blocks ultrasound transmission. Thus, imaging studies in patients with necrotizing fasciitis may be challenging to interpret. Although imaging may help to confirm deep tissue involvement and to evaluate lesion spread, it should never delay emergency surgical treatment in patients with established necrotizing fasciitis. Copyright © 2012. Published by Elsevier SAS.

  18. Wernicke Encephalopathy.

    Science.gov (United States)

    Jenkins, Patricia F

    2015-01-01

    This paper reviews the complaints and associated symptoms/consequences of lacking essential nutrients and vitamins in our central and peripheral nervous systems. This has become important as there has been a rise in malnutrition following the increasing incidence of bariatric surgery for obesity. A case report example involving review of the clinical presentation and treatment. A 30-year-old Caucasian woman who had gastric sleeve surgery did not take the recommended capsules as they were too large to swallow. She noted diplopia and oscillopsia 2 months later, which led her to have full orthoptic and neuro-ophthalmic evaluations. After being treated with chewable vitamins with thiamine, she noted a tremendous improvement in her symptoms. Wernicke encephalopathy is a disease that was seen more in the 1940s and 1950s, following war times and mostly in underdeveloped countries. However, with the increasing utilization of bariatric surgery for obesity, neurological offices are seeing more patients with neurological impairments. We recommend inquiring about any obesity surgery in one's history and including Wernicke encephalopathy in possible differential diagnoses in those patients who have a recent onset of strabismus or nystagmus, altered mental status, and/or gait ataxia. © 2015 Board of regents of the University of Wisconsin System, American Orthoptic Journal, Volume 65, 2015, ISSN 0065-955X, E-ISSN 1553-4448.

  19. Clinical manifestations and treatment response of steroid in pediatric Hashimoto encephalopathy.

    Science.gov (United States)

    Yu, Hee Joon; Lee, Jeehun; Seo, Dae Won; Lee, Munhyang

    2014-07-01

    Hashimoto encephalopathy is a steroid-responsive encephalopathy associated with elevated titers of antithyroid antibodies. Clinical symptoms are characterized by behavioral and cognitive changes, speech disturbance, seizures, myoclonus, psychosis, hallucination, involuntary movements, cerebellar signs, and coma. The standard treatment is the use of corticosteroids along with the treatment of any concurrent dysthyroidism. Other options are immunoglobulins and plasmapheresis. We described symptoms and outcomes on 3 teenage girls with Hashimoto encephalopathy. Presenting symptoms were seizure or altered mental status. One patient took levothyroxine due to hypothyroidism before presentation of Hashimoto encephalopathy. After confirmation of elevated antithyroid antibodies, all patients were treated with steroids. One patient needed plasmapheresis because of the lack of response to steroids and immunoglobulins. Hashimoto encephalopathy should be considered in any patient presenting with acute or subacute unexplained encephalopathy and seizures. Even though the use of steroids is the first line of treatment, plasmapheresis can rescue steroid-resistant patients. © The Author(s) 2013.

  20. Autoimmune encephalopathies

    Science.gov (United States)

    Leypoldt, Frank; Armangue, Thaís; Dalmau, Josep

    2014-01-01

    Over the last 10 years the continual discovery of novel forms of encephalitis associated with antibodies to cell-surface or synaptic proteins has changed the paradigms for diagnosing and treating disorders that were previously unknown or mischaracterized. We review here the process of discovery, the symptoms, and the target antigens of twelve autoimmune encephatilic disorders, grouped by syndromes and approached from a clinical perspective. Anti-NMDAR encephalitis, several subtypes of limbic encephalitis, stiff-person spectrum disorders, and other autoimmune encephalitides that result in psychosis, seizures, or abnormal movements are described in detail. We include a novel encephalopathy with prominent sleep dysfunction that provides an intriguing link between chronic neurodegeneration and cell-surface autoimmunity (IgLON5). Some of the caveats of limited serum testing are outlined. In addition, we review the underlying cellular and synaptic mechanisms that for some disorders confirm the antibody pathogenicity. The multidisciplinary impact of autoimmune encephalitis has been expanded recently by the discovery that herpes simplex encephalitis is a robust trigger of synaptic autoimmunity, and that some patients may develop overlapping syndromes, including anti-NMDAR encephalitis and neuromyelitis optica or other demyelinating diseases. PMID:25315420

  1. Steroid-Responsive Encephalopathy Associated with Autoimmune Thyroiditis Presenting as Confusion, Dysphasia, and Myoclonus

    Directory of Open Access Journals (Sweden)

    S. A. Ryan

    2012-01-01

    Full Text Available Steroid response encephalopathy associated with autoimmune thyroiditis (SREAT, or Hashimoto’s encephalopathy, is a rare disorder believed to be immune-mediated. It is most often characterized by a subacute onset of confusion with altered level of consciousness, seizures, and myoclonus. We describe the case of a 48-year-old gentleman who presented with confusion and dysphasia. Specific clinical features and laboratory results led to a diagnosis of Hashimoto’s encephalopathy. This case highlights the core features of this condition and the potential for complete response to steroid therapy.

  2. Pathogenesis of Hepatic Encephalopathy

    Directory of Open Access Journals (Sweden)

    Irena Ciećko-Michalska

    2012-01-01

    Full Text Available Hepatic encephalopathy can be a serious complication of acute liver failure and chronic liver diseases, predominantly liver cirrhosis. Hyperammonemia plays the most important role in the pathogenesis of hepatic encephalopathy. The brain-blood barrier disturbances, changes in neurotransmission, neuroinflammation, oxidative stress, GABA-ergic or benzodiazepine pathway abnormalities, manganese neurotoxicity, brain energetic disturbances, and brain blood flow abnormalities are considered to be involved in the development of hepatic encephalopathy. The influence of small intestine bacterial overgrowth (SIBO on the induction of minimal hepatic encephalopathy is recently emphasized. The aim of this paper is to present the current views on the pathogenesis of hepatic encephalopathy.

  3. Pathogenesis of Hepatic Encephalopathy

    Science.gov (United States)

    Ciećko-Michalska, Irena; Szczepanek, Małgorzata; Słowik, Agnieszka; Mach, Tomasz

    2012-01-01

    Hepatic encephalopathy can be a serious complication of acute liver failure and chronic liver diseases, predominantly liver cirrhosis. Hyperammonemia plays the most important role in the pathogenesis of hepatic encephalopathy. The brain-blood barrier disturbances, changes in neurotransmission, neuroinflammation, oxidative stress, GABA-ergic or benzodiazepine pathway abnormalities, manganese neurotoxicity, brain energetic disturbances, and brain blood flow abnormalities are considered to be involved in the development of hepatic encephalopathy. The influence of small intestine bacterial overgrowth (SIBO) on the induction of minimal hepatic encephalopathy is recently emphasized. The aim of this paper is to present the current views on the pathogenesis of hepatic encephalopathy. PMID:23316223

  4. Fulminant adult-onset subacute sclerosing panencephalitis: a case report

    Science.gov (United States)

    Faivre, Anthony; Souraud, Jean-Baptiste; McGonigal, Aileen; Alla, Philippe; Grapperon, Jacques; Valance, Jacques

    2009-01-01

    We present the case of a young adult who developed acute encephalopathy with severe status epilepticus and rapid deterioration to vegetative state and death within 6 weeks. Although the clinical picture, MRI and EEG findings were atypical, the hypothesis of subacute sclerosing panencephalitis (SSPE) was suggested by markedly increased intrathecal IgG synthesis in the cerebrospinal fluid, and diagnosis was confirmed by the presence of high antimeasles antibodies in cerebrospinal fluid and brain biopsy findings. Acute SSPE is an exceptionally rare and little-known form of SSPE with protean symptomatology, and this case is to our knowledge the first observation of SSPE presenting with status epilepticus in adults. Our case reinforces the need to include, even in developed countries, SSPE as a diagnostic possibility in unexplained acute encephalopathies. PMID:21686559

  5. Necrotizing Skin Infections

    Science.gov (United States)

    ... Summer Camp Tips for Kids With Asthma, Allergies Antioxidants: The Good Health Helpers As Stroke 'Liquefies' Brain ... Video) Skin Cancer Additional Content Medical News Necrotizing Skin Infections By A. Damian Dhar, MD, JD, Private ...

  6. Malnutrition in subacute care

    OpenAIRE

    Collins, Jorja Claire

    2017-01-01

    Malnutrition is a significant problem across all healthcare settings because of its high prevalence and association with adverse outcomes for patients and increased healthcare costs. There is a paucity of research considering subacute patients’ nutritional status and how this changes throughout inpatient stay. Additionally, a stronger evidence base for strategies to prevent and treat malnutrition specifically in this setting is required. This thesis aimed to address these research gaps to con...

  7. Hashimoto encephalopathy: Neurological and psychiatric perspective

    Directory of Open Access Journals (Sweden)

    Pavlović D.M.

    2009-01-01

    Full Text Available Hashimoto encephalopathy (HE is an autoimmune disease with neurological and neuropsychiatric manifestations and elevated titers of antithyroid antibodies in serum and cerebrospinal fluid. Patients are mostly women. Age varies from 8 to 86 years. Prevalence of HE is estimated to be 2.1/100,000. Neurological and/or psychiatric symptoms and signs constitute the clinical picture. The disease responds well to corticosteroid therapy, but sometimes other immunomodulatory therapies must be applied. Autoimmune mechanisms with antibodies against antigens in the brain cortex are suspected. The course of the disease can be acute, subacute, chronic, or relapsing/remitting. Some patients improve spontaneously, but a few died in spite of adequate therapy.

  8. [Necrotizing fasciitis after varicella].

    Science.gov (United States)

    Gonçalves, E; Furtado, F; Estrada, J; Vale, M C; Pinto, M; Santos, M; Moura, G; Vasconcelos, C

    2001-01-01

    Necrotizing fasciitis is a rare and severe infection characterised by extremely rapid progressive involvement of the superficial fascias and deep dermal layers of the skin, with resultant vasculitis and necrosis. The authors present three clinical cases of necrotizing fasciitis; all three patients previously had varicella rash, rapid progressive spreading erythema with severe pain and toxic shock syndrome. Two patients had positive cultures of b-haemolytic streptococcus. Early stage differential diagnosis with celulitis, aggressive antibiotic treatment and pediatric intensive care support are essential. However, the main therapy is early extensive surgical approach involving all indurate areas, down to and including the muscle fascia.

  9. [Rota virus encephalopathy].

    Science.gov (United States)

    Kashiwagi, Yasuyo; Kawashima, Hisashi; Suzuki, Shunsuke

    2011-03-01

    Rotavirus is the most common cause of severe gastroenteritis in young children, but the pathogenesis and immunity of this disease are not completely understood. Less well recognized is the association of rotavirus-induced central nervous system (CNS) involvement, which has been associated with seizure, encephalopathy and death etc. The term 'rotavirus encephalopathy' has been used for cases of rotavirus gastroenteritis with CNS involvement as evidenced by clinical features of encephalopathy with or without CSF pleocytosis. Here, we review the recent advances regarding its causative agent, prognosis, pathogenesis, and treatment.

  10. Chronic Traumatic Encephalopathy

    Science.gov (United States)

    ... com/home. Accessed Jan. 29, 2016. Concussion: Mayo's multidisciplinary approach. Mayo Clinic Neuroscience Update. 2013;10:2. ... al. Clinical appraisal of chronic traumatic encephalopathy: Current perspectives and future directions. Current Opinion in Neurology. 2011; ...

  11. Subacute and acute fasciolosis in sheep in southern Brazil.

    Science.gov (United States)

    Fiss, Leticia; de Lourdes Adrien, Maria; Marcolongo-Pereira, Clairton; Assis-Brasil, Nathalia D; Sallis, Eliza S V; Riet-Correa, Franklin; Ruas, Jerônimo L; Schild, Ana Lucia

    2013-02-01

    Eight outbreaks of acute and subacute fasciolosis are reported in sheep in the municipality of Santa Vitória do Palmar, southern Brazil, in areas used for irrigated rice cultivation, which are subject to frequent flooding. Two outbreaks occurred mid-winter during July and August, and six occurred during spring and early summer. Morbidity ranged from 3 to 66.7 %, and mortality ranged from 3 to 50 %. Clinical signs included weight loss, mucosal pallor, apathy, depression, muscle tremors, and drooling; these were followed by death within approximately 24 h. Some sheep were found dead. Gross lesions were characterized by an irregular liver capsule with fibrin deposition and hemorrhages. The liver parenchyma contained hemorrhagic tracts or irregular clear areas alternated with dark hemorrhagic areas. Histologically, hemorrhagic dark red tracts of necrotic liver parenchyma and an inflammatory infiltrate were noted; these lesions coincided with the presence of immature flukes. Langhans-type giant cells, fibrous tissue, and bile duct cell proliferation were observed in subacute cases. Chronic fasciolosis is a well-known disease to farmers in southern Brazil; nevertheless, acute and subacute fasciolosis, which are more difficult to diagnose and treat, may cause important economic losses. Efficient control of fasciolosis requires integration of measures to treat infections in the definitive host, to reduce the population of snails of the genus Lymnaea, and to avoid contact between the parasite and host by appropriate pasture management.

  12. Necrotizing ulcerative gingivitis

    OpenAIRE

    Rayhana Malek; Amina Gharibi; Nadia Khlil; Jamila Kissa

    2017-01-01

    Necrotizing ulcerative gingivitis (NUG) is a typical form of periodontal diseases. It has an acute clinical presentation with the distinctive characteristics of rapid onset of interdental gingival necrosis, gingival pain, bleeding, and halitosis. Systemic symptoms such as lymphadenopathy and malaise could be also found. There are various predisposing factors such as stress, nutritional deficiencies, and immune system dysfunctions, especially, HIV infection that seems to play a major role in t...

  13. Neonatal Necrotizing Enterocolitis

    Directory of Open Access Journals (Sweden)

    Saad Lahmiti

    2011-01-01

    Full Text Available Necrotizing enterocolitis (NEC is a gastrointestinal disease that mostly affects premature infants. It involves infection and inflammation that causes destruction of the bowel. Although it affects only 1 in 2,000 to 4,000 births, or between 1 and 5% of neonatal intensive care unit admissions, NEC is the most common and serious gastrointestinal disorder among hospitalized preterm infants. We present a very representative abdominal X-ray of this disease.

  14. Current pathogenetic aspects of hepatic encephalopathy and noncirrhotic hyperammonemic encephalopathy

    OpenAIRE

    Cichoż-Lach, Halina; Michalak, Agata

    2013-01-01

    Hepatic encephalopathy is a medical phenomenon that is described as a neuropsychiatric manifestation of chronic or acute liver disease that is characterized by psychomotor, intellectual and cognitive abnormalities with emotional/affective and behavioral disturbances. This article focuses on the underlying mechanisms of the condition and the differences between hepatic encephalopathy and noncirrhotic hyperammonemic encephalopathy. Hepatic encephalopathy is a serious condition that can cause ne...

  15. Surge of subacute sclerosing panencephalitis

    National Research Council Canada - National Science Library

    Lisel V Richter-Joubert; Donovan Jacobs; Tracy Kilborn

    2017-01-01

    Subacute sclerosing panencephalitis (SSPE) is a poorly understood complication of measles infection, presenting in up to 1 in 2500 infected children, resulting in devastating neurocognitive outcomes and a high mortality rate...

  16. Genetics Home Reference: ethylmalonic encephalopathy

    Science.gov (United States)

    ... have been identified worldwide, mostly in Mediterranean and Arab populations. Although ethylmalonic encephalopathy appears to be very ... sulfide (H(2)S) metabolism in ethylmalonic encephalopathy. Cold Spring Harb Perspect Biol. 2013 Jan 1;5(1): ...

  17. Isotretinoin-induced encephalopathy.

    Science.gov (United States)

    Wong, Adrian; Williams, Matthew; Gibb, William

    2010-11-01

    A 16-year-old male started on isotretinoin 80 mg daily for acne developed persistent headache 3 weeks later, with myoclonus and confusion 10 weeks later. During initial hospital assessment his Glasgow Coma Scale score fell acutely to 8 and he required ventilation. Brain imaging and cerebrospinal fluid (CSF) analysis were normal and an electroencephalogram (EEG) showed features of encephalopathy. No cause was found. He was extubated after 24 hours and made a full recovery. This is the first report of a generalized encephalopathy thought likely to be due to isotretinoin.

  18. GRIN2B encephalopathy

    DEFF Research Database (Denmark)

    Platzer, Konrad; Yuan, Hongjie; Schuetz, Hannah

    2017-01-01

    BACKGROUND: We aimed for a comprehensive delineation of genetic, functional and phenotypic aspects of GRIN2B encephalopathy and explored potential prospects of personalised medicine. METHODS: Data of 48 individuals with de novo GRIN2B variants were collected from several diagnostic and research c...

  19. Management of Hepatic Encephalopathy

    Directory of Open Access Journals (Sweden)

    G. Wright

    2011-01-01

    Full Text Available Hepatic encephalopathy (HE, the neuropsychiatric presentation of liver disease, is associated with high morbidity and mortality. Reduction of plasma ammonia remains the central therapeutic strategy, but there is a need for newer novel therapies. We discuss current evidence supporting the use of interventions for both the general management of chronic HE and that necessary for more acute and advanced disease.

  20. Subacute combined degeneration of the cord, dementia and parkinsonism due to an inborn error of folate metabolism.

    OpenAIRE

    Clayton, P.T.; Smith, I; Harding, B; Hyland, K; Leonard, J V; Leeming, R. J.

    1986-01-01

    A 2-year-old girl with 5,10-methylenetetrahydrofolate reductase deficiency developed subacute combined degeneration of the cord and a leuco-encephalopathy which was confirmed at necropsy. Total folate concentrations in serum, red cells and CSF were markedly reduced whereas vitamin B12 concentrations were normal. In addition the patient had Parkinsonism and reduced concentrations of homovanillic acid, 5-hydroxyindoleacetic acid and total biopterins in cerebrospinal fluid. Folic acid administra...

  1. Subacute sclerosing panencephalitis in pregnancy.

    Science.gov (United States)

    Chiu, Michael H; Meatherall, Bonnie; Nikolic, Ana; Cannon, Kristine; Fonseca, Kevin; Joseph, Jeffrey T; MacDonald, Judy; Pabbaraju, Kanti; Tellier, Raymond; Wong, Sallene; Koch, Marcus W

    2016-03-01

    We present a case of subacute sclerosing panencephalitis that developed in a previously healthy 29-year-old pregnant woman who had returned from a trip to rural India shortly before the onset of symptoms. She was admitted to hospital at 27 weeks' gestation with a history of cognitive decline and difficulty completing simple tasks. She had no clinical signs of infection. The working diagnosis was autoimmune encephalitis, although extensive investigations did not lead to a final classifying diagnosis. The patient became comatose and developed hypertension, and an emergency caesarean section was done at 31 weeks to deliver the child, who seemed healthy. The patient died about 6 weeks after the onset of symptoms. The patient was found to have had subacute sclerosing panencephalitis at autopsy. In this Grand Round, we review the clinical features and treatment of subacute sclerosing panencephalitis, and the epidemiological and public health aspects of the case. Copyright © 2016 Elsevier Ltd. All rights reserved.

  2. Role of Carbamazepine in the Symptomatic Treatment of Subacute Sclerosing Panencephalitis: A Case Report and Review of the Literature

    Directory of Open Access Journals (Sweden)

    Sandhya Ravikumar

    2013-01-01

    Full Text Available We describe the clinical presentation and clinical course of subacute sclerosing panencephalitis in a 13-year-old previously healthy boy who recently immigrated to the United States from Iraq. He presented with macular retinopathy, followed by progressive myoclonus and encephalopathy. After extensive workup, a diagnosis of subacute sclerosing panencephalitis was suspected by the presence of period epileptiform discharges on electroencephalogram and confirmed by elevated measles titers in the cerebrospinal fluid. Combination immunomodulatory therapy with isoprinosine, ribavirin, and intra-Ommaya interferon alpha did not result in clinical improvement. Within days following the administration of carbamazepine, there was remarkable improvement in the myoclonus and he was able to ambulate independently for a period of 4 months at which time he unfortunately progressed to a vegetative state. This case highlights the importance of carbamazepine as a potential first line symptomatic treatment of subacute sclerosing panencephalitis and provides a review of the literature on the subject.

  3. Role of carbamazepine in the symptomatic treatment of subacute sclerosing panencephalitis: a case report and review of the literature.

    Science.gov (United States)

    Ravikumar, Sandhya; Crawford, John Ross

    2013-01-01

    We describe the clinical presentation and clinical course of subacute sclerosing panencephalitis in a 13-year-old previously healthy boy who recently immigrated to the United States from Iraq. He presented with macular retinopathy, followed by progressive myoclonus and encephalopathy. After extensive workup, a diagnosis of subacute sclerosing panencephalitis was suspected by the presence of period epileptiform discharges on electroencephalogram and confirmed by elevated measles titers in the cerebrospinal fluid. Combination immunomodulatory therapy with isoprinosine, ribavirin, and intra-Ommaya interferon alpha did not result in clinical improvement. Within days following the administration of carbamazepine, there was remarkable improvement in the myoclonus and he was able to ambulate independently for a period of 4 months at which time he unfortunately progressed to a vegetative state. This case highlights the importance of carbamazepine as a potential first line symptomatic treatment of subacute sclerosing panencephalitis and provides a review of the literature on the subject.

  4. Lead encephalopathy in adults

    Directory of Open Access Journals (Sweden)

    Janapareddy Vijaya Bhaskara Rao

    2014-01-01

    Full Text Available Lead poisoning is a common occupational health hazard in developing countries. We report the varied clinical presentation, diagnostic and management issues in two adult patients with lead encephalopathy. Both patients worked in a battery manufacturing unit. Both patients presented with seizures and one patient also complained of abdominal colic and vomiting. Both were anemic and a lead line was present. Blood lead level in both the patients was greater than 25 µg/dl. Magnetic resonance imaging of brain revealed bilateral symmetric involvement of the thalamus, lentiform nucleus in both patients and also the external capsules, sub-cortical white matter in one patient. All these changes, seen as hyperintensities in T2-weighted images suggested demyelination. They were advised avoidance of further exposure to lead and were treated with anti-epileptics; one patient also received D-penicillamine. They improved well on follow-up. Lead encephalopathy is an uncommon but important manifestation of lead toxicity in adults.

  5. Preterm Hypoxic Ischemic Encephalopathy

    Directory of Open Access Journals (Sweden)

    Krishna G Gopagondanahalli

    2016-10-01

    Full Text Available Hypoxic ischemic encephalopathy (HIE is a recognizable and defined clinical syndrome in term infants that results from a severe or prolonged hypoxic ischemic episode before or during birth. However, in the preterm infant, defining hypoxic ischemic injury, its clinical course, monitoring and outcomes remains complex. Few studies examine preterm HIE, and these are heterogeneous, with variable inclusion criteria and outcomes reported. We examine the available evidence that implies that the incidence of hypoxic ischemic insult in preterm infants is probably higher than recognized, and follows a more complex clinical course, with higher rates of adverse neurological outcomes, compared to term infants. This review aims to elucidate the causes and consequences of preterm hypoxia ischemia, the subsequent clinical encephalopathy syndrome, diagnostic tools and outcomes. Finally, we suggest a uniform definition for preterm HIE that may help in identifying infants most at risk of adverse outcomes and amenable to neuroprotective therapies.

  6. GRIN2B encephalopathy

    DEFF Research Database (Denmark)

    Platzer, Konrad; Yuan, Hongjie; Schütz, Hannah

    2017-01-01

    presented with neurodevelopmental disorders and a spectrum of hypotonia, movement disorder, cortical visual impairment, cerebral volume loss and epilepsy. Six patients presented with a consistent malformation of cortical development (MCD) intermediate between tubulinopathies and polymicrogyria. Missense...... treatment response in the respective patients still remains to be demonstrated. CONCLUSIONS: In addition to previously known features of intellectual disability, epilepsy and autism, we found evidence that GRIN2B encephalopathy is also frequently associated with movement disorder, cortical visual impairment...

  7. Hashimoto encephalopathy: literature review.

    Science.gov (United States)

    Zhou, J Y; Xu, B; Lopes, J; Blamoun, J; Li, L

    2017-03-01

    Hashimoto encephalopathy (HE) presents as an encephalopathy without central nervous system infection or tumor. HE is associated with autoimmune thyroiditis and is thus considered to be an autoimmune disorder. The prevalence of HE is low, but death and status epilepticus have been reported. HE manifests with a wide range of symptoms that include behavioral changes and confusion. Elevated thyroid antibodies are present in the majority of cases and are required for the diagnosis of HE. Normal brain MRI findings are found in the majority of patients diagnosed with HE. The most consistent CSF abnormality noted in HE patients is the presence of elevated protein. Most HE patients respond well to steroid therapy. Clinical improvements are also observed with IV immunoglobulin and plasmapheresis. In conclusion, it is now generally accepted that the diagnosis of HE must include encephalopathy characterized by cognitive impairment associated with psychiatric features, such as hallucinations, delusions, and paranoia. Autoimmune encephalitis and prion disease should be considered in the differential diagnosis due to the similarity of the clinical features of these conditions to those of HE. © 2016 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  8. Necrotizing ulcerative gingivitis

    Directory of Open Access Journals (Sweden)

    Rayhana Malek

    2017-01-01

    Full Text Available Necrotizing ulcerative gingivitis (NUG is a typical form of periodontal diseases. It has an acute clinical presentation with the distinctive characteristics of rapid onset of interdental gingival necrosis, gingival pain, bleeding, and halitosis. Systemic symptoms such as lymphadenopathy and malaise could be also found. There are various predisposing factors such as stress, nutritional deficiencies, and immune system dysfunctions, especially, HIV infection that seems to play a major role in the pathogenesis of NUG. The treatment of NUG is organized in successive stages: first, the treatment of the acute phase that should be provided immediately to stop disease progression and to control patient's feeling of discomfort and pain; second, the treatment of the preexisting condition such as chronic gingivitis; then, the surgical correction of the disease sequelae like craters. Moreover, finally, maintenance phase that allows stable outcomes. This case report describes the diagnosis approach and the conservative management with a good outcome of NUG in a 21-year-old male patient with no systemic disease and probable mechanism of pathogenesis of two predisposing factors involved.

  9. Necrotizing Ulcerative Gingivitis.

    Science.gov (United States)

    Malek, Rayhana; Gharibi, Amina; Khlil, Nadia; Kissa, Jamila

    2017-01-01

    Necrotizing ulcerative gingivitis (NUG) is a typical form of periodontal diseases. It has an acute clinical presentation with the distinctive characteristics of rapid onset of interdental gingival necrosis, gingival pain, bleeding, and halitosis. Systemic symptoms such as lymphadenopathy and malaise could be also found. There are various predisposing factors such as stress, nutritional deficiencies, and immune system dysfunctions, especially, HIV infection that seems to play a major role in the pathogenesis of NUG. The treatment of NUG is organized in successive stages: first, the treatment of the acute phase that should be provided immediately to stop disease progression and to control patient's feeling of discomfort and pain; second, the treatment of the preexisting condition such as chronic gingivitis; then, the surgical correction of the disease sequelae like craters. Moreover, finally, maintenance phase that allows stable outcomes. This case report describes the diagnosis approach and the conservative management with a good outcome of NUG in a 21-year-old male patient with no systemic disease and probable mechanism of pathogenesis of two predisposing factors involved.

  10. Probiotics and necrotizing enterocolitis.

    Science.gov (United States)

    Fleming, Paul; Hall, Nigel J; Eaton, Simon

    2015-12-01

    Probiotics for the prevention of necrotizing enterocolitis have attracted a huge interest. Combined data from heterogeneous randomised controlled trials suggest that probiotics may decrease the incidence of NEC. However, the individual studies use a variety of probiotic products, and the group at greatest risk of NEC, i.e., those with a birth weight of less than 1000 g, is relatively under-represented in these trials so we do not have adequate evidence of either efficacy or safety to recommend universal prophylactic administration of probiotics to premature infants. These problems have polarized neonatologists, with some taking the view that it is unethical not to universally administer probiotics to premature infants, whereas others regard the meta-analyses as flawed and that there is insufficient evidence to recommend routine probiotic administration. Another problem is that the mechanism by which probiotics might act is not clear, although some experimental evidence is starting to accumulate. This may allow development of surrogate endpoints of effectiveness, refinement of probiotic regimes, or even development of pharmacological agents that may act through the same mechanism. Hence, although routine probiotic administration is controversial, studies of probiotic effects may ultimately lead us to effective means to prevent this devastating disease.

  11. Approach to Clinical Syndrome of Jaundice and Encephalopathy in Tropics

    Science.gov (United States)

    Anand, Anil C.; Garg, Hitendra K.

    2015-01-01

    A large number of patients present with jaundice and encephalopathy in tropical country like India and acute liver failure is the usual cause. Clinical presentation like ALF is also a complication of many tropical infections, and these conditions may mimic ALF but may have subtle differences from ALF. Moreover, what hepatologists see as acute liver failure in tropics is different from what is commonly described in Western Textbooks. Paracetamol overdose, which is possibly the commonest cause of ALF in UK and USA, is hardly ever seen in India. Most common etiology here is viral hepatitis (hepatitis E > hepatitis B> hepatitis A). Apart from ALF, one may also come across subacute hepatic failure (SAHF) as well as acute-on-chronic liver failure (ACLF) due to viral hepatitis. Interestingly, a host of other conditions can mimic ALF because clinical presentation in these conditions can be dominated by jaundice and encephalopathy. Malarial hepatopathy is possibly the best-known condition out of these and is not an uncommon manifestation of severe malaria. A similar presentation can also be seen in other common infections in tropics such as dengue fever, typhoid fever, leptospirosis, scrub typhus, amoebic liver abscesses, tuberculosis and other bacterial and fungal infections with or without human immunodeficiency virus (HIV) related disease. In many of these conditions, liver failure may not be underlying pathophysiology. Some pregnancy related liver diseases could also present with jaundice and encephalopathy. This review summarizes the commonly seen presentations in tropical country like India, where jaundice and encephalopathy dominate the clinical picture. PMID:26041951

  12. DNM1 encephalopathy

    DEFF Research Database (Denmark)

    von Spiczak, Sarah; Helbig, Katherine L; Shinde, Deepali N

    2017-01-01

    evolving into Lennox-Gastaut syndrome. Two patients had profound global developmental delay without seizures. In addition, we describe a single patient with normal development before the onset of a catastrophic epilepsy, consistent with febrile infection-related epilepsy syndrome at 4 years. All mutations...... cluster within the GTPase or middle domains, and structural modeling and existing functional data suggest a dominant-negative effect on DMN1 function. CONCLUSIONS: The phenotypic spectrum of DNM1-related encephalopathy is relatively homogeneous, in contrast to many other genetic epilepsies. Up to one...

  13. Subacute thyroiditis--61 cases review.

    Science.gov (United States)

    Hwang, S C; Jap, T S; Ho, L T; Ching, K N

    1989-02-01

    To examine the hospitalized patients with available data suggesting subacute thyroiditis, we have reviewed 80 hospital charts over the last 11 years and the data demonstrated 61 patients (48 females, 13 males, Aged 22-75 years) had evidence of subacute thyroiditis clinically or pathologically. Fifty seven of those patients had the clinical manifestation of severe tenderness over the neck, and others were painless. The thyroid function test were abnormal in 53% (30/57) of cases. All had rapid sedimentation rate (46/46). The thyroid antibodies were positive in 26% (10/39) of cases. Sixteen patients received unnecessary thyroid operation and complicated with hypothyroidism with or without hypoparathyroidism in two cases. In conclusion, all patients with painful or painless goiter should be appreciated carefully with additional study such as aspiration cytology to avoid misdiagnosis and inappropriate management.

  14. Subacute sclerosing panencephalitis: A clinical appraisal

    National Research Council Canada - National Science Library

    Jagtap, Sujit Abajirao; Nair, M D; Kambale, Harsha J

    2013-01-01

    Subacute sclerosing panencephalitis (SSPE) is a rare chronic, progressive encephalitis affecting primarily children and young adults, caused by a persistent infection of immune resistant measles virus...

  15. Transmissible spongiform encephalopathies.

    Science.gov (United States)

    Liemann, S; Glockshuber, R

    1998-09-18

    Scrapie, bovine spongiform encephalopathy (BSE), and the Creutzfeldt-Jakob disease (CJD) belong to a group of lethal neurodegenerative disorders in mammals. Prion diseases or transmissible spongiform encephalopathies (TSEs) are characterized by the accumulation of an abnormal isoform (PrPSc) of the host-encoded cellular prion protein (PrPC) in the brain. The infectious agent, the 'prion,' is believed to be devoid of informational nucleic acid and to consist largely, if not entirely, of PrPSc. The PrP isoforms contain identical amino acid sequences yet differ in their overall secondary structure with the PrPSc isoform possessing a higher beta-sheet and lower alpha-helix content than PrPC. Elucidation of the three-dimensional structure of PrPC has provided important clues on the molecular basis of inherited human TSEs and on the species barrier phenomenon of TSEs. Nevertheless, the molecular mechanism of the conformational rearrangement of PrPC into PrPSc is still unknown, mainly due to the lack of detailed structural information on PrPSc. Within the framework of the 'protein only' hypothesis, two plausible models for the self-replication of prions have been suggested, the conformational model and the nucleation-dependent polymerization model.

  16. [Prevention of hepatic encephalopathy].

    Science.gov (United States)

    Morillas, Rosa M; Sala, Marga; Planas, Ramon

    2014-06-06

    Hepatic encephalopathy (HE) is a frequent complication of cirrhosis which, in addition to producing a great social impact, deteriorates the quality of life of patients and is considered a sign of advanced liver disease and therefore a clinical indication for liver transplant evaluation. Patients who have had episodes of HE have a high risk of recurrence. Thus, after the HE episode resolves, it is recommended: control and prevention of precipitating factors (gastrointestinal bleeding, spontaneous bacterial peritonitis, use of diuretics with caution, avoid nervous system depressant medications), continued administration of non-absorbable disaccharides such as lactulose or lactitol, few or non-absorbable antibiotics such as rifaximin and assess the need for a liver transplant as the presence of a HE episode carries a poor prognosis in cirrhosis. Copyright © 2013 Elsevier España, S.L. All rights reserved.

  17. Diabetic encephalopathy: a cerebrovascular disorder?

    NARCIS (Netherlands)

    Manschot, S.M.

    2006-01-01

    Animal study: The aim was to investigate the role of vascular disturbances in the development of experimental diabetic encephalopathy. We describe the effects of treatment with the Angiotensin Converting Enzyme(ACE)-inhibitor enalapril (treatment aimed at the

  18. Dopamine agents for hepatic encephalopathy

    DEFF Research Database (Denmark)

    Junker, Anders Ellekær; Als-Nielsen, Bodil; Gluud, Christian

    2014-01-01

    BACKGROUND: Patients with hepatic encephalopathy may present with extrapyramidal symptoms and changes in basal ganglia. These changes are similar to those seen in patients with Parkinson's disease. Dopamine agents (such as bromocriptine and levodopa, used for patients with Parkinson's disease) have...... therefore been assessed as a potential treatment for patients with hepatic encephalopathy. OBJECTIVES: To evaluate the beneficial and harmful effects of dopamine agents versus placebo or no intervention for patients with hepatic encephalopathy. SEARCH METHODS: Trials were identified through the Cochrane...... of the trials followed participants after the end of treatment. Only one trial reported adequate bias control; the remaining four trials were considered to have high risk of bias. Random-effects model meta-analyses showed that dopamine agents had no beneficial or detrimental effect on hepatic encephalopathy...

  19. Fatal Necrotizing Fasciitis following Episiotomy

    Directory of Open Access Journals (Sweden)

    Faris Almarzouqi

    2015-01-01

    Full Text Available Introduction. Necrotizing fasciitis is an uncommon condition in general practice but one that provokes serious morbidity. It is characterized by widespread fascial necrosis with relative sparing of skin and underlying muscle. Herein, we report a fatal case of necrotizing fasciitis in a young healthy woman after episiotomy. Case Report. A 17-year-old primigravida underwent a vaginal delivery with mediolateral episiotomy. Necrotizing fasciitis was diagnosed on the 5th postpartum day, when the patient was referred to our tertiary care medical center. Surgical debridement was initiated together with antibiotics and followed by hyperbaric oxygen therapy. The patient died due to septic shock after 16 hours from the referral. Conclusion. Delay of diagnosis and consequently the surgical debridement were most likely the reasons for maternal death. In puerperal period, a physician must consider necrotizing fasciitis as a possible diagnosis in any local sings of infection especially when accompanied by fever and/or tenderness. Early diagnosis is the key for low mortality and morbidity.

  20. EBV driven natural killer cell disease of the central nervous system presenting as subacute cognitive decline.

    Science.gov (United States)

    Brett, Francesca M; Flavin, Richard; Chen, Daphne; Loftus, Teresa; Looby, Seamus; McCarthy, Allan; de Gascun, Cillian; Jaffe, Elaine S; Nor, Nurul; Javadpour, Mohsen; McCabe, Dominick

    2017-11-01

    Brain biopsy in patients presenting with subacute encephalopathyis never straightforward and only undertaken when a 'treatable condition' is a realistic possibility. This 63 year old right handed, immunocompetent Caucasian woman presented with an 8 month history of rapidly progressive right-sided hearing impairment, a 4 month history of intermittent headaches, tinnitus, 'dizziness', dysphagia, nausea and vomiting, with the subsequent evolution of progressive gait ataxia and a subacute global encephalopathy. The possibility of CJD was raised. Brain biopsy was carried out. Western blot for prion protein was negative. She died 9 days later and autopsy brain examination confirmed widespread subacute infarction due to an EBV positive atypical NK/T-cell infiltrate with positivity for CD3, CD56, granzyme B, perforin and EBER with absence of CD4, CD5 and CD8 expression. Molecular studies for T-cell clonality were attempted but failed due to insufficient DNA quality. Serology was consistent with past EBV infection (EBV VCA and EBNA IgG Positive). There was no evidence of disease outside the CNS. Primary central nervous system NK/T-cell lymphoma is extremely rare. The rare reported cases all present with a discrete intracranial mass, unlike the diffuse infiltrative pattern in this case. Whilst the diffuse interstitial pattern is reminiscent of chronic active EBV infection (CAEBV) seen in other organ systems such as the liver and bone marrow, the clinical presentation and epidemiologic profile are not typical for CAEBV.

  1. Subacute sclerosing panencephalitis presenting as acute cerebellar ataxia and brain stem hyperintensities.

    Science.gov (United States)

    Saini, Arushi Gahlot; Sankhyan, Naveen; Padmanabh, Hansashree; Sahu, Jitendra Kumar; Vyas, Sameer; Singhi, Pratibha

    2016-05-01

    Subacute sclerosing panencephalitis is a devastating neurodegenerative disease with a characteristic clinical course. Atypical presentations may be seen in 10% of the cases. To describe the atypical clinical and radiological features of SSPE in a child form endemic country. A 5-year-old boy presented with acute-onset cerebellar ataxia without associated encephalopathy, focal motor deficits, seizures or cognitive decline. He had varicella-like illness with vesicular, itchy truncal rash erupting one month prior to the onset of these symptoms. He underwent detailed neurological assessment, relevant laboratory and radiological investigations. Neuroimaging revealed peculiar brain stem lesions involving the pons and cerebellum suggestive of demyelination. With a presumptive diagnosis of clinically isolated syndrome of demyelination, he was administered pulse methylprednisolone (30 mg/kg/day for 5 days). Four weeks later he developed myoclonic jerks. Electroencephalogram showed characteristic periodic complexes time-locked with myoclonus. CSF and serum anti-measles antibody titres were elevated (1:625). Our report highlights that subacute sclerosing panencephalitis can present atypically as isolated acute cerebellar ataxia and peculiar involvement of longitudinal and sparing of transverse pontine fibres. The predominant brainstem abnormalities in the clinical setting may mimick acute demyelinating syndrome. Hence, it is important to recognize these features of subacute sclerosing panencephalitis in children, especially in the endemic countries. Copyright © 2016 European Paediatric Neurology Society. Published by Elsevier Ltd. All rights reserved.

  2. Chronic necrotizing pulmonary aspergillosis presenting as bilateral pleural effusion: a case report

    Directory of Open Access Journals (Sweden)

    Rajalingham Sakthiswary

    2012-02-01

    Full Text Available Abstract Introduction Chronic necrotizing pulmonary aspergillosis is an uncommon subacute form of Aspergillus infection. It typically occurs in immunocompromised individuals and in those with underlying lung disease. This interesting case highlights the occurrence of this entity of aspergillosis in an immunocompetent middle-aged woman with atypical radiological findings. To the best of our knowledge this is the first case report of chronic necrotizing pulmonary aspergillosis presenting with pleural effusion. Case presentation Our patient was a 64-year-old Malay woman with a background history of epilepsy but no other comorbidities. She was a lifelong non-smoker. She presented to our facility with a six-month history of productive cough and three episodes of hemoptysis. An initial chest radiograph showed bilateral pleural effusion with bibasal consolidation. Bronchoscopy revealed a white-coated endobronchial tree and bronchoalveolar lavage culture grew Aspergillus niger. A diagnosis of chronic necrotizing pulmonary aspergillosis was made based on the clinical presentation and microbiological results. She responded well to treatment with oral itraconazole. Conclusions The radiological findings in chronic necrotizing pulmonary aspergillosis can be very diverse. This case illustrates that this condition can be a rare cause of bilateral pleural effusion.

  3. Wernicke encephalopathy: MR findings and clinical presentation.

    Science.gov (United States)

    Weidauer, Stefan; Nichtweiss, Michael; Lanfermann, Heinrich; Zanella, Friedhelm E

    2003-05-01

    Wernicke encephalopathy (WE) is a severe neurological disorder caused by vitamin B1 deficiency. The aim of the study was to analyse MRI findings typical for this disease and to evaluate the significance of their correlations with clinical symptoms. Magnetic resonance images and clinical features of 12 patients with WE were analysed. The patients underwent MR imaging within 3-14 days after onset of clinical symptoms. In 7 of 12 patients MR imaging showed symmetrical diencephalic and midbrain lesions. Postcontrast T1-weighted images from 5 of 9 patients examined during the initial 6 days of acute WE showed a subtle enhancement of the mamillary bodies, the tectal plate, the periaqueductal area and the periventricular region of the third ventricle including the paramedian thalamic nuclei. In addition, T2-weighted and fluid-attenuated inversion recovery (FLAIR) images revealed hyperintense signals in these regions (except for 2 patients where the mamillary bodies were normal). Hyperintense lesions on T2-weighted images without any enhancement on postcontrast T1-weighted images were detected in 2 patients by MR imaging performed 11 or 14 days after onset of WE. Patients with hyperintensities on T2-weighted images of the periventricular region of the third ventricle and the paramedian thalamic nuclei had poor recovery from their mental dysfunction. The MR examination in case of WE shows a typical pattern of lesions in 58% of cases. Enhancement of the mamillary bodies, the periventricular region of the third ventricle including the paramedian thalamic nuclei, and the periaqueductal area on postcontrast T1-weighted images can be observed in the initial period after clinical onset of symptoms and are characteristic signs of the acute stage of WE. Hyperintense lesions in the periventricular region and the paramedian thalamic nuclei on T2-weighted and FLAIR images in the subacute stage of WE and enhancement on postcontrast T1-weighted images of the mamillary bodies and the

  4. Wernicke encephalopathy: MR findings and clinical presentation

    Energy Technology Data Exchange (ETDEWEB)

    Weidauer, Stefan; Lanfermann, Heinrich; Zanella, Friedhelm E. [Institute of Neuroradiology, University of Frankfurt, Frankfurt (Germany); Nichtweiss, Michael [Department of Neurology, Municipal Hospital of Wismar, Wismar (Germany)

    2003-05-01

    Wernicke encephalopathy (WE) is a severe neurological disorder caused by vitamin B1 deficiency. The aim of the study was to analyse MRI findings typical for this disease and to evaluate the significance of their correlations with clinical symptoms. Magnetic resonance images and clinical features of 12 patients with WE were analysed. The patients underwent MR imaging within 3-14 days after onset of clinical symptoms. In 7 of 12 patients MR imaging showed symmetrical diencephalic and midbrain lesions. Postcontrast T1-weighted images from 5 of 9 patients examined during the initial 6 days of acute WE showed a subtle enhancement of the mamillary bodies, the tectal plate, the periaqueductal area and the periventricular region of the third ventricle including the paramedian thalamic nuclei. In addition, T2-weighted and fluid-attenuated inversion recovery (FLAIR) images revealed hyperintense signals in these regions (except for 2 patients where the mamillary bodies were normal). Hyperintense lesions on T2-weighted images without any enhancement on postcontrast T1-weighted images were detected in 2 patients by MR imaging performed 11 or 14 days after onset of WE. Patients with hyperintensities on T2-weighted images of the periventricular region of the third ventricle and the paramedian thalamic nuclei had poor recovery from their mental dysfunction. The MR examination in case of WE shows a typical pattern of lesions in 58% of cases. Enhancement of the mamillary bodies, the periventricular region of the third ventricle including the paramedian thalamic nuclei, and the periaqueductal area on postcontrast T1-weighted images can be observed in the initial period after clinical onset of symptoms and are characteristic signs of the acute stage of WE. Hyperintense lesions in the periventricular region and the paramedian thalamic nuclei on T2-weighted and FLAIR images in the subacute stage of WE and enhancement on postcontrast T1-weighted images of the mamillary bodies and the

  5. [Posterior reversible encephalopathy syndrome].

    Science.gov (United States)

    Fischer, M; Schmutzhard, E

    2016-06-01

    Posterior reversible encephalopathy syndrome refers to a neurological disorder characterized by headache, disorders of consciousness, visual disturbances, epileptic seizures, and subcortical vasogenic edema. About two thirds of patients develop neurological symptoms, which are associated with blood pressure fluctuations. One hypothesis is that hypertensive episodes cause autoregulatory failure, and values above the upper limit of cerebral autoregulation result in a breakthrough followed by hyperperfusion and blood-brain barrier dysfunction. In another hypothesis, endothelial dysfunction triggered by numerous factors including preeclampsia, immunosuppressive agents, chemotherapeutics, sepsis, or autoimmune disorders is thought to be the key pathomechanism. Endo- or exogenic toxic agents including pharmacological substances, cytokines, or bacterial toxins are supposed to trigger endothelial activation and dysfunction resulting in the release of vasoconstrictors, pro-inflammatory mediators, and vascular leakage. Diagnosis is usually based on clinical and neuroimaging findings that frequently show a bilateral, symmetric, and parietooccipital pattern. However, the diagnosis can often only be confirmed during the course of disease after excluding important differential diagnoses. Currently, there is no specific treatment available. Lowering of arterial blood pressure and eliminating the underlying cause usually leads to an improvement of clinical and neuroradiological findings. Admission to a critical care unit is required in about 40 % of patients due to complicating conditions including status epilepticus, cerebral vasoconstriction, ischemia, or intracerebral hemorrhage. Prognosis is favorable; in the majority of patients neurological deficits and imaging findings resolve completely.

  6. Survey of subacute sclerosing panencephalitis in Japan.

    Science.gov (United States)

    Abe, Yusaku; Hashimoto, Koichi; Iinuma, Kazuie; Ohtsuka, Yoko; Ichiyama, Takashi; Kusuhara, Koichi; Nomura, Keiko; Mizuguchi, Masashi; Aiba, Hideo; Suzuki, Yasuhiro; Mizusawa, Hidehiro; Hosoya, Mitsuaki

    2012-12-01

    Investigators conducted a retrospective epidemiological study of subacute sclerosing panencephalitis, a fatal disease caused by measles infection, over the past few years in Japan. Data on 118 cases obtained from a questionnaire sent to attending physicians were analyzed. The annual incidence of subacute sclerosing panencephalitis was approximately 0.03 cases per million from 2001 to 2005. Children infected with measles at a young age (panencephalitis, and those infected before 6 months of age showed earlier onset. Because a positive correlation was found between the prevalence of measles and the onset of subacute sclerosing panencephalitis, particularly among children infected at an early age, it is vital to eradicate measles infection by vaccination.

  7. MRI finding of ethylmalonic encephalopathy: case report

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Jin Yong; Lee, Shi Kyung; Han, Chun Hwan; Rho, Eun Jin [Kangnam General Hospital Public Corporation, Seoul (Korea, Republic of)

    2002-12-01

    Ethylmalonic encephalopathy is a rare syndrom characterized by developmental delay, acrocyanosis, petechiae, chronic diarrhea, and ethylmalonic, lactic, and methylsuccinic aciduria. We report the MRI finding of ethylmalonic encephalopathy including previously unreported intracranial hematoma.

  8. Necrotizing meningoencephalitis in a cow.

    Science.gov (United States)

    Rissi, D R; Barros, C S L

    2013-09-01

    An 18-month-old Charolais cow developed depression and drooling and was submitted for necropsy after euthanasia. The cow was 1 of 50 moved between 2 farms approximately 5 days before the onset of clinical disease. Gross findings included swollen and hemorrhagic areas of malacia in the frontal, temporal, and parietal lobes of the cerebral cortex. Microscopically there was a necrotizing meningoencephalitis with intranuclear astrocytic and neuronal eosinophilic viral inclusions in the frontal, temporal, and parietal cerebral cortex as well as in the basal nuclei and thalamus. The gross and microscopic findings were consistent with necrotizing meningoencephalitis caused by bovine herpesvirus (BHV-1 or BHV-5), and the diagnosis was confirmed by detection of bovine herpesviral antigen on fresh samples of brain via fluorescent antibody test using a monoclonal antibody against BHV-1 glycoprotein C.

  9. Necrotizing fasciitis of the perineum.

    Science.gov (United States)

    Akın, Yasemin; Celayir, Ayşenur Cerrah; Aköz, Tayfun; Civan, Hasret Ayyıldız; Kurt, Gökmen; Ağzıkuru, Turgut; Sahin, Ceyhan

    2013-05-01

    Necrotizing fasciitis is a severe soft tissue infection characterized by rapidly progressing necrosis, involving subcutaneous tissues. This rare condition carries a high mortality rate and requires prompt diagnosis and urgent treatment with radical debridement and antibiotics; but early diagnosis, which is essential to successful treatment, remains a challenge. Physical examination findings, preoperative and operative findings, histopathological results of the structure, and follow-up results of the patient are discussed with related reports. A 15-month old girl had a history of trivial perineal dermatitis after treatment of anemia and pneumonia. Perineal dermatitis progressed fastly as necrotizing fasciitis which was successfully managed with intensive medical treatment, surgical debridement and reconstructive surgery. Lack of cutaneous findings early in the disease makes the diagnosis challenging, so a high suspicion is essential. Recovery of the patient from this life-threatening condition needs a multi-disciplinary approach involving pediatrics, pediatric surgery, and plastic and reconstructive surgery.

  10. Recent advances in hepatic encephalopathy

    Science.gov (United States)

    DeMorrow, Sharon

    2017-01-01

    Hepatic encephalopathy describes the array of neurological alterations that occur during acute liver failure or chronic liver injury. While key players in the pathogenesis of hepatic encephalopathy, such as increases in brain ammonia, alterations in neurosteroid levels, and neuroinflammation, have been identified, there is still a paucity in our knowledge of the precise pathogenic mechanism. This review gives a brief overview of our understanding of the pathogenesis of hepatic encephalopathy and then summarizes the significant recent advances made in clinical and basic research contributing to our understanding, diagnosis, and possible treatment of hepatic encephalopathy. A literature search using the PubMed database was conducted in May 2017 using “hepatic encephalopathy” as a keyword, and selected manuscripts were limited to those research articles published since May 2014. While the authors acknowledge that many significant advances have been made in the understanding of hepatic encephalopathy prior to May 2014, we have limited the scope of this review to the previous three years only. PMID:29026534

  11. Benzodiazepine receptor antagonists for hepatic encephalopathy

    DEFF Research Database (Denmark)

    Als-Nielsen, B; Gluud, L L; Gluud, C

    2004-01-01

    Hepatic encephalopathy may be associated with accumulation of substances that bind to a receptor-complex in the brain resulting in neural inhibition. Benzodiazepine receptor antagonists may have a beneficial effect on patients with hepatic encephalopathy.......Hepatic encephalopathy may be associated with accumulation of substances that bind to a receptor-complex in the brain resulting in neural inhibition. Benzodiazepine receptor antagonists may have a beneficial effect on patients with hepatic encephalopathy....

  12. Necrotizing fasciitis: an urgent diagnosis

    Energy Technology Data Exchange (ETDEWEB)

    Paz Maya, Silvia; Dualde Beltran, Delfina [Hospital Clinico Universitario de Valencia, Valencia (Spain); Lemercier, Pierre; Leiva-Salinas, Carlos [Hospital Politecnico y Universitario La Fe, Valencia (Spain)

    2014-05-15

    Necrotizing fasciitis (NF) is a rare, life-threatening soft-tissue infection and a medical and surgical emergency, with increasing incidence in the last few years. It is characterized by a rapidly spreading, progressive necrosis of the deep fascia and subcutaneous tissue. Necrotizing fasciitis is often underestimated because of the lack of specific clinical findings in the initial stages of the disease. Many adjuncts such as laboratory findings, bedside tests - e.g., the ''finger test'' or biopsy - and imaging tests have been described as being helpful in the early recognition of the disease. Imaging is very useful to confirm the diagnosis, but also to assess the extent of the disorder, the potential surgical planning, and the detection of underlying etiologies. The presence of gas within the necrotized fasciae is characteristic, but may be lacking. The main finding is thickening of the deep fasciae due to fluid accumulation and reactive hyperemia, best seen on magnetic resonance imaging. (orig.)

  13. Metronidazole-Induced Encephalopathy in Chronic Diarrhoea.

    Science.gov (United States)

    Haridas, Ashwathy; Trivedi, Trupti H; Moulick, Nivedita D; Joshi, Anagha R

    2015-06-01

    Metronidazole-induced encephalopathy (MIE) is a rare cause of drug-induced toxic encephalopathy. We report the clinical and neuroimaging findings of a patient with chronic diarrhoea who developed metronidazole-induced encephalopathy. After the drug was discontinued there was complete reversal of the condition.

  14. Investigation of metabolic encephalopathy | van der Watt ...

    African Journals Online (AJOL)

    Encephalopathy may be a presenting sign in a wide range of medical conditions. This review focuses only on the diagnosis and initial management of those inherited metabolic diseases (IMDs) prevalent in South Africa that may present with encephalopathy in childhood. Metabolic encephalopathy is a medical emergency, ...

  15. MRI in subacute sclerosing panencephalitis

    Energy Technology Data Exchange (ETDEWEB)

    Tuncay, R. [Department of Neurology, Istanbul Medical Faculty, University of Istanbul (Turkey); Akman-Demir, G. [Department of Neurology, Istanbul Medical Faculty, University of Istanbul (Turkey); Goekyigit, A. [Department of Neurology, Istanbul Medical Faculty, University of Istanbul (Turkey); Eraksoy, M. [Department of Neurology, Istanbul Medical Faculty, University of Istanbul (Turkey); Barlas, M. [Department of Neurology, Istanbul Medical Faculty, University of Istanbul (Turkey); Tolun, R. [Department of Neurology, Istanbul Medical Faculty, University of Istanbul (Turkey); Guersoy, G. [Department of Neurology, Istanbul Medical Faculty, University of Istanbul (Turkey)

    1996-10-01

    Subacute sclerosing panencephalitis (SSPE) is a progressive, slow virus infection of the brain, caused by the measles virus, attacking children and young adults. We investigated 15 patients with SSPE by MRI, with 5 normal and 10 pathological results. In the early period, lesions were in the grey matter and subcortical white matter. They were asymmetrical and had a predilection for the posterior parts of the hemispheres. Later, high-signal changes in deep white matter and severe cerebral atrophy were observed. Parenchymal lesions significantly correlated with the duration of disease. A significant relationship between MRI findings and clinical stage was observed in the 1st year of the disease. (orig.). With 4 figs., 1 tab.

  16. Acute Necrotizing Ulcerative Gingivitis (ANUG

    Directory of Open Access Journals (Sweden)

    Nicholas E. Kman

    2017-04-01

    Full Text Available History of Present Illness: A 34-year-old HIV positive female presented to the emergency department with a three-week history of swollen, painful gums. She had difficulty eating and chewing, along with aches and general malaise. The patient was an everyday smoker and was not taking any antiretroviral medication. Significant findings: Physical examination revealed inflamed gingiva, ulceration, and soft tissue necrosis (Image 1 along with mandibular lymphadenopathy (not shown. Given her symptoms, poor oral care, and her immunocompromised state, she was given a diagnosis of Acute Necrotizing Ulcerative Gingivitis (ANUG or Vincent’s Angina. Discussion: Acute Necrotizing Ulcerative Gingivitis (ANUG, Vincent’s Angina, or Trench Mouth is the only periodontal disease in which bacteria invade non-necrotic tissue. The etiology is usually secondary to fusobacteria and spirochete overgrowth of bacteria which is normally present in the oral cavity. HIV infection, previous necrotizing gingivitis, poor oral hygiene, malnutrition, smoking, and stress are predisposing factors. Antibiotics and improved nutrition have significantly decreased the incidence of ANUG. The prevalence of ANUG among HIV infected patients varies from 4.3% to 16.0%. ANUG is 20.8 times more likely to be seen in AIDS patients with CD4 counts less than 200 cells/mm3. In developing countries, like those in Sub-Saharan Africa, incidence of ANUG is increasing among children with a prevalence as high as 23% in children under 10 years of age.1 Treatment for ANUG is multifactorial. Patients need good debridement under anesthesia so dental referral is imperative. Pain control with Ibuprofen or low dose opioids is indicated. Oral hygiene instructions include Chlorhexidine 0.12% twice daily, proper nutrition, appropriate fluid intake, and smoking cessation. For signs of systemic involvement, the recommended antibiotics are Amoxicillin and Metronidazole.2 If left untreated, ANUG may lead to rapid

  17. Inflammation in Epileptic Encephalopathies.

    Science.gov (United States)

    Shandra, Oleksii; Moshé, Solomon L; Galanopoulou, Aristea S

    2017-01-01

    West syndrome (WS) is an infantile epileptic encephalopathy that manifests with infantile spasms (IS), hypsarrhythmia (in ~60% of infants), and poor neurodevelopmental outcomes. The etiologies of WS can be structural-metabolic pathologies (~60%), genetic (12%-15%), or of unknown origin. The current treatment options include hormonal treatment (adrenocorticotropic hormone and high-dose steroids) and the GABA aminotransferase inhibitor vigabatrin, while ketogenic diet can be given as add-on treatment in refractory IS. There is a need to identify new therapeutic targets and more effective treatments for WS. Theories about the role of inflammatory pathways in the pathogenesis and treatment of WS have emerged, being supported by both clinical and preclinical data from animal models of WS. Ongoing advances in genetics have revealed numerous genes involved in the pathogenesis of WS, including genes directly or indirectly involved in inflammation. Inflammatory pathways also interact with other signaling pathways implicated in WS, such as the neuroendocrine pathway. Furthermore, seizures may also activate proinflammatory pathways raising the possibility that inflammation can be a consequence of seizures and epileptogenic processes. With this targeted review, we plan to discuss the evidence pro and against the following key questions. Does activation of inflammatory pathways in the brain cause epilepsy in WS and does it contribute to the associated comorbidities and progression? Can activation of certain inflammatory pathways be a compensatory or protective event? Are there interactions between inflammation and the neuroendocrine system that contribute to the pathogenesis of WS? Does activation of brain inflammatory signaling pathways contribute to the transition of WS to Lennox-Gastaut syndrome? Are there any lead candidates or unexplored targets for future therapy development for WS targeting inflammation? © 2017 Elsevier Inc. All rights reserved.

  18. Familial subacute sclerosing panencephalitis associated with short latency.

    Science.gov (United States)

    Sharma, Vinod; Gupta, Vineet B; Eisenhut, Michael

    2008-03-01

    The familial recurrence of subacute sclerosing panencephalitis is rare. The study of such cases and a comparison of intrafamilial with sporadic cases of subacute sclerosing panencephalitis may shed light on important pathogenetic factors. We report on the occurrence of subacute sclerosing panencephalitis in two brothers from rural India, who contracted measles infection simultaneously at ages 3 and 11 years. They developed subacute sclerosing panencephalitis 21 and 37 months later, respectively. A diagnosis of subacute sclerosing panencephalitis was based on history, electroencephalographic changes, and significantly raised levels of cerebrospinal-fluid anti-measles virus immunoglobulin G. A comparison of intrafamilial with sporadic cases of subacute sclerosing panencephalitis revealed that latency in familial subacute sclerosing panencephalitis involved a median of 6.4 years (range, 1.0-10.9), significantly (P panencephalitis from the Indian subcontinent. We confirmed a more rapid manifestation of subacute sclerosing panencephalitis after measles virus infection in intrafamilial compared with sporadic subacute sclerosing panencephalitis.

  19. Blindness, dancing extremities, and corpus callosum and brain stem involvement: an unusual presentation of fulminant subacute sclerosing panencephalitis.

    Science.gov (United States)

    Singhi, Pratibha; Saini, Arushi Gahlot; Sankhyan, Naveen; Gupta, Pankaj; Vyas, Sameer

    2015-01-01

    A 4-year-old girl presented with acute visual loss followed 2 weeks later with loss of speech and audition, fulminant neuroregression, and choreo-athetoid movements of extremities. Fundus showed bilateral chorioretinitis. Electroencephalography showed periodic complexes. Measles antibody titers were elevated in both serum and cerebrospinal fluid, consistent with subacute sclerosing panencephalitis. Neuroimaging showed discontiguous involvement of splenium of the corpus callosum and ventral pons with sparing of cortical white matter. Our case highlights the atypical clinical and radiologic presentations of subacute sclerosing panencephalitis. Pediatricians need to be aware that necrotizing chorioretinitis in a child and/or atypical brain stem changes could be the heralding feature of this condition in endemic countries. © The Author(s) 2014.

  20. Pseudotumor cerebri secondary to subacute sclerosing panencephalitis.

    Science.gov (United States)

    Ayçiçek, Ali; Işcan, Akin; Ceçe, Hasan

    2009-05-01

    Unusual presentations are not rare in subacute sclerosing panencephalitis. Five patients initially diagnosed with pseudotumor cerebri were ultimately determined to have pseudotumor cerebri secondary to subacute sclerosing panencephalitis. The present study retrospectively reviewed 56 cases history, neurologic symptoms, and clinical and laboratory data, as well as the outcomes. On admission, five patients (group 1) presenting with pseudotumor cerebri exhibited bilateral papilledema, and in each of them cranial magnetic resonance imaging revealed small lateral ventricles, effacement of the subarachnoid space, and no mass lesion. On admission, 51 patients (group 2) had no pseudotumor cerebri findings. The year of original measles infection, the interval between measles and onset of subacute sclerosing panencephalitis, and initial neurologic symptoms were similar, but length of symptoms before diagnosis of subacute sclerosing panencephalitis was shorter in group 1, and the clinical stage of subacute sclerosing panencephalitis on admission was more advanced in group 2. Cerebrospinal fluid mean open pressure was 378 +/- 22 H(2)O in group 1 and 146 +/- 28 H(2)O in group 2; cerebrospinal fluid antibody was 2038 +/- 768 U/L in group 1 and was 664 +/- 214 U/L in group 2. Only three of the five patients with pseudotumor cerebri had typical periodic discharges on electroencephalographic examination. These findings suggest that subacute sclerosing panencephalitis can cause pseudotumor cerebri.

  1. Magnetic resonance imaging findings of postresuscitation encephalopathy. Sequential change and correlation with clinical outcome

    Energy Technology Data Exchange (ETDEWEB)

    Goto, Yasunobu; Wataya, Takafumi; Arakawa, Yoshiki; Hojo, Masato; Chin, Masaki; Yamagata, Sen; Kikuta, Kenichiro; Ishii, Akira [Kurashiki Central Hospital, Okayama (Japan)

    2001-06-01

    The purpose of this study was to describe the findings of sequential magnetic resonance imaging (MRI) in postresuscitation encephalopathy. Although its outcome is known to be overwhelming, but its acute findings by variable imaging methods are subtle and show only limited values. The correlation of the findings of MRI with clinical outcome were also analyzed. Twelve patients with global cerebral anoxia who underwent MRI with conventional and diffusion-weighted imaging were enrolled in this study. Compared with normal MRI images, abnormal signal regions were checked and described in cortex, basal ganglia and white matter. Also medical records were carefully reviewed to study the cause, the time necessary for resuscitation and long term clinical outcome. The earliest finding was obtained by diffusion-weighted image less than 24 hours (acute period) in bilateral cerebral cortex as bright high signal intensity regions. Similar abnormality of bright high signal area in FLAIR and T2 was followed according to the time elapsed in early subacute period (1-13 days). Succeedingly, white matter was involved and laminar necrosis in cortical area was observed in late subacute period (14-20 days). Finally, diffuse brain atrophy and obtundation of gray-white matter junction were seen in chronic stage (after 21 days). These MR findings were coincided well with histopathological findings reported in literatures. The poor outcome was closely and significantly correlated with abnormality in MR images. MRI was a useful diagnostic modality to diagnose the whole brain ischemic encephalopathy and to predict the prognosis. (author)

  2. CT and MR in non-neonatal hypoxic-ischemic encephalopathy: radiological findings with pathophysiological correlations

    Energy Technology Data Exchange (ETDEWEB)

    Gutierrez, Leonardo Guilhermino; Portela, Luiz Antonio Pezzi [Hospital Alemao Oswaldo Cruz and Hospital do Coracao, Diagnostic Imaging Division, Sao Paulo (Brazil); Rovira, Alex [University Hospital Vall d' Hebron, MR Unit, Department of Radiology, Barcelona (Spain); Costa Leite, Claudia da [Clinics Hospital of the University of Sao Paulo, School of Medicine, Department of Radiology, Sao Paulo (Brazil); Lucato, Leandro Tavares [Hospital Alemao Oswaldo Cruz and Hospital do Coracao, Diagnostic Imaging Division, Sao Paulo (Brazil); Clinics Hospital of the University of Sao Paulo, School of Medicine, Department of Radiology, Sao Paulo (Brazil)

    2010-11-15

    Non-neonatal hypoxic-ischemic encephalopathy is a clinical condition often related to cardiopulmonary arrest that demands critical management and treatment decisions. Management depends mainly on the degree of neurological impairment and prognostic considerations. Computed tomography (CT) is often used to exclude associated or mimicking pathology. If any, only nonspecific signs such as cerebral edema, sulci effacement, and decreased gray matter (GM)/white matter (WM) differentiation are evident. Pseudosubarachnoid hemorrhage, a GM/WM attenuation ratio <1.18, and inverted GM attenuation are associated with a poor prognosis. Magnetic resonance (MR) imaging is more sensitive than CT in assessing brain damage in hypoxic-ischemic encephalopathy. Some MR findings have similarities to those seen pathologically, based on spatial distribution and time scale, such as lesions distributed in watershed regions and selective injury to GM structures. In the acute phase, lesions are better depicted using diffusion-weighted imaging (DWI) because of the presence of cytotoxic edema, which, on T2-weighted images, only become apparent later in the early subacute phase. In the late subacute phase, postanoxic leukoencephalopathy and contrast enhancement could be observed. In the chronic phase, atrophic changes predominate over tissue signal changes. MR can be useful for estimating prognosis when other tests are inconclusive. Some findings, such as the extent of lesions on DWI and presence of a lactate peak and depleted N-acetyl aspartate peak on MR spectroscopy, seem to have prognostic value. (orig.)

  3. Current status of transmissible spongiform encephalopathies in ruminants

    Directory of Open Access Journals (Sweden)

    Penders J.

    2004-01-01

    Full Text Available Transmissible spongiform encephalopathies (TSE encompass subacute neurological degenerative diseases for which the prototypes are scrapie in sheep and some forms of Creutzfeldt-Jakob disease in man. The emergence of a new form of TSE in cattle in United Kingdom (UK since 1986, namely bovine spongiform encephalopathy (BSE, sharply increased the interest for these diseases, especially because of the epidemic nature of BSE in UK, its subsequent spread in continental Europe and the later discovery of its zoonotic character. The number of measures of veterinary public health taken to control the disease and to prevent its spread to animals and human beings increased in time and culminated by the total feed ban. Indeed, since the beginning of 2001, feed containing proteins of animal origin is prohibited for the feeding of production animals, including ruminants and monogastric species. The effect of this total ban of mammalian meat and bone meal needs to be evaluated. The incidence of BSE has a trend to decrease in UK and in most of the other European member states. However, as BSE is a rare event distributed in a large bovine population, it is difficult to state unambiguously whether this trend is significant. Furthermore, the evaluation of this measure will be only effective at least five years after its introduction, since this period is the mean incubation time of BSE. The main concern is currently the eradication of BSE in the infected countries. Additionally, the control of scrapie is also carried out due to the possible contamination of sheep with the BSE agent. These actions must take into account several new facts: the recent discovery of BSE cases in countries with a low geographical BSE risk level as Japan, Canada and the United States of America (USA; the growing incidence of chronic wasting disease, a spongiform encephalopathy observed in deer in USA; the characterization of a new pattern of bovine amyloidotic spongiform encephalopathy in

  4. Psychopathology and Hepatic Encephalopathy

    Directory of Open Access Journals (Sweden)

    João Gama Marques

    2013-12-01

    Full Text Available Since Hippocrates that neuropsychiatric illness secondary to liver disease fascinates physicians, but only in the XIX century Marcel Nencki and Ivan Pavlov suggested the relation between high concentrations of ammonia and Hepatic Encephalopathy (HE. The reaction of ammonia and glutamate (origins glutamine, “the Trojan Horse of neurotoxicity of ammonia continues to be the main responsible for the neurologic lesions, recently confirmed by neurochemistry and neuroimagiology studies. Glutamine starts the inflammatory reaction at the central nervous sys- tem but other important actors seem to be manganese and the neurotransmitters systems of GABA and endocanabinoids. Nowadays there are three different etiologic big groups for HE: type A associated with acute liver failure; type B associated with portosystemic bypass; and type C associated with cirrhosis of the liver. The staging of HE is still based on classic West Haven system, but a latent Grade 0 was introduced (the so called minimal HE; remaining the aggra- vating HE from Grade 1 (subtle changes at clinical examination to Grade 4 (coma. In this work a bibliographic review was made on 30 of the most pertinent and recent papers, focusing in psychopathology, physiopathology, etiology and staging of this clinical entity transversal to Psychiatry and Gastroenterology. Alterations are described in vigility and conscience like temporal, spatial and personal disorientation. Attention, concentration and memory are impaired very early, on latent phase and can be accessed through neuropsychological tests. Mood oscillates between euphoric and depressive. Personality changes begin obviously and abruptly or in a subtle and insidious way. There can be changes in perception like visual hallucinations or even of acoustic-verbal. The thought disorders can be of delusional type, paranoid, systematized or not, but also monothematic ala Capgras Syndrome. Speech can be accelerated, slowed down or completely in

  5. Bovine spongiform encephalopathy in sheep?

    NARCIS (Netherlands)

    Schreuder, B.E.C.; Somerville, R.A.

    2003-01-01

    Bovine spongiform encephalopathy (BSE) in sheep has not been identified under natural conditions at the time of writing and remains a hypothetical issue. However, rumours about the possible finding of a BSE-like isolate in sheep have led to great unrest within the sheep industry, among the general

  6. Subacute sclerosing panencephalitis: an update.

    Science.gov (United States)

    Gutierrez, Jose; Issacson, Richard S; Koppel, Barbara S

    2010-10-01

    Subacute sclerosing panencephalitis (SSPE) is a chronic encephalitis occurring after infection with measles virus. The prevalence of the disease varies depending on uptake of measles vaccination, with the virus disproportionally affecting regions with low vaccination rates. The physiopathology of the disease is not fully understood; however, there is evidence that it involves factors that favour humoral over cellular immune response against the virus. As a result, the virus is able to infect the neurons and to survive in a latent form for years. The clinical manifestations occur, on average, 6 years after measles virus infection. The onset of SSPE is insidious, and psychiatric manifestations are prominent. Subsequently, myoclonic seizures usually lead to a final stage of akinetic mutism. The diagnosis is clinical, supported by periodic complexes on electroencephalography, brain imaging suggestive of demyelination, and immunological evidence of measles infection. Management of the disease includes seizure control and avoidance of secondary complications associated with the progressive disability. Trials of treatment with interferon, ribavirin, and isoprinosine using different methodologies have reported beneficial results. However, the disease shows relentless progression; only 5% of individuals with SSPE undergo spontaneous remission, with the remaining 95% dying within 5 years of diagnosis.

  7. Proton pump inhibitor-induced subacute cutaneous lupus erythematosus

    DEFF Research Database (Denmark)

    Sandholdt, L H; Laurinaviciene, R; Bygum, Anette

    2014-01-01

    Drug-induced subacute cutaneous lupus erythematosus (SCLE) has been known in the literature since 1985 and is increasingly recognized.......Drug-induced subacute cutaneous lupus erythematosus (SCLE) has been known in the literature since 1985 and is increasingly recognized....

  8. Masticatory function in subacute TMD patients before and after treatment.

    NARCIS (Netherlands)

    Pereira, L.J.; Steenks, M.H.; Wijer, A. de; Speksnijder, C.M.; Bilt, A. van der

    2009-01-01

    Masticatory function can be impaired in temporomandibular disorders (TMDs) patients. We investigated whether treatment of subacute non-specific TMD patients may influence oral function and clinical outcome measures. Fifteen patients with subacute TMD participated in the study. We quantified

  9. Necrotizing Fasciitis Associated with Staphylococcus lugdunensis

    Directory of Open Access Journals (Sweden)

    Tony Hung

    2012-01-01

    Full Text Available Necrotizing fasciitis is a life-threatening soft tissue infection that results in rapid local tissue destruction. Type 1 necrotizing fasciitis is characterized by polymicrobial, synergistic infections that are caused by non-Group A streptococci, aerobic and anaerobic organisms. Type 2 necrotizing fasciitis involves Group A Streptococcus (GAS with or without a coexisting staphylococcal infection. Here we provide the first report of necrotizing fasciitis jointly associated with the microbes Group B Streptococcus and Staphylococcus lugdunensis. S. lugdunensis is a commensal human skin bacterium known to cause often painful and prolonged skin and soft tissue infections. To our knowledge, however, this is the first case of Staph. lugdunensis-associated necrotizing fasciitis to be reported in the literature.

  10. Metabolic encephalopathies in the critical care unit.

    Science.gov (United States)

    Frontera, Jennifer A

    2012-06-01

    This article summarizes the most common etiologies and approaches to management of metabolic encephalopathy. Metabolic encephalopathy is a frequent occurrence in the intensive care unit setting. Common etiologies include hepatic failure, renal failure, sepsis, electrolyte disarray, and Wernicke encephalopathy. Current treatment paradigms typically focus on supportive care and management of the underlying etiology. Directed therapies that target neurochemical and neurotransmitter pathways that mediate encephalopathy are not currently available and represent an important area for future research. Although commonly thought of as reversible neurologic insults, delirium and encephalopathy have been associated with increased mortality, prolonged length of stay and hospital complications, and worse long-term cognitive and functional outcomes. Recognition and treatment of encephalopathy is critical to improving outcomes in critically ill patients.

  11. Genetics Home Reference: STXBP1 encephalopathy with epilepsy

    Science.gov (United States)

    ... Conditions STXBP1 encephalopathy with epilepsy STXBP1 encephalopathy with epilepsy Printable PDF Open All Close All Enable Javascript ... the expand/collapse boxes. Description STXBP1 encephalopathy with epilepsy is a condition characterized by recurrent seizures (epilepsy), ...

  12. Micturitional disturbance in Wernicke's encephalopathy.

    Science.gov (United States)

    Sakakibara, R; Hattori, T; Yasuda, K; Yamanishi, T; Tojo, M; Mori, M

    1997-01-01

    A 24-year-old pregnant woman started to have hyperemesis gravidarum 6 weeks before admission. Four weeks later she had vertigo, diplopia, staggering gait, mild dyspnea, dysphagia, and incontinence of urine. On admission she presented with ophthalmoplegia, ptosis, ataxia, decreased tendon reflex, and memory disturbance. Brain magnetic resonance imaging revealed abnormal intensities in medial thalamic-hypothalamic regions and the periaqueductal area, and she was diagnosed with Wernicke's encephalopathy. Urodynamic studies revealed decreased bladder volume and detrusor hyperreflexia. Six weeks after the administration of 100 mg/day of thiamine, urge incontinence gradually recovered, together with neurological signs. Lesions of the medial thalamic-hypothalamic area and the periaqueductal gray matter seemed to be mainly responsible for micturitional disturbance in our patient with Wernicke's encephalopathy.

  13. Ketogenic Diet in Epileptic Encephalopathies

    Directory of Open Access Journals (Sweden)

    Suvasini Sharma

    2013-01-01

    Full Text Available The ketogenic diet is a medically supervised high-fat, low-carbohydrate diet that has been found useful in patients with refractory epilepsy. It has been shown to be effective in treating multiple seizure types and epilepsy syndromes. In this paper, we review the use of the ketogenic diet in epileptic encephalopathies such as Ohtahara syndrome, West syndrome, Dravet syndrome, epilepsy with myoclonic atonic seizures, and Lennox-Gastaut syndrome.

  14. Ketogenic Diet in Epileptic Encephalopathies

    OpenAIRE

    Suvasini Sharma; Manjari Tripathi

    2013-01-01

    The ketogenic diet is a medically supervised high-fat, low-carbohydrate diet that has been found useful in patients with refractory epilepsy. It has been shown to be effective in treating multiple seizure types and epilepsy syndromes. In this paper, we review the use of the ketogenic diet in epileptic encephalopathies such as Ohtahara syndrome, West syndrome, Dravet syndrome, epilepsy with myoclonic atonic seizures, and Lennox-Gastaut syndrome.

  15. EBV driven natural killer cell disease of the central nervous system presenting as subacute cognitive decline

    Directory of Open Access Journals (Sweden)

    Francesca M. Brett

    2017-11-01

    This 63 year old right handed, immunocompetent Caucasian woman presented with an 8 month history of rapidly progressive right-sided hearing impairment, a 4 month history of intermittent headaches, tinnitus, ‘dizziness’, dysphagia, nausea and vomiting, with the subsequent evolution of progressive gait ataxia and a subacute global encephalopathy. The possibility of CJD was raised. Brain biopsy was carried out. Western blot for prion protein was negative. She died 9 days later and autopsy brain examination confirmed widespread subacute infarction due to an EBV positive atypical NK/T-cell infiltrate with positivity for CD3, CD56, granzyme B, perforin and EBER with absence of CD4, CD5 and CD8 expression. Molecular studies for T-cell clonality were attempted but failed due to insufficient DNA quality. Serology was consistent with past EBV infection (EBV VCA and EBNA IgG Positive. There was no evidence of disease outside the CNS. Primary central nervous system NK/T-cell lymphoma is extremely rare. The rare reported cases all present with a discrete intracranial mass, unlike the diffuse infiltrative pattern in this case. Whilst the diffuse interstitial pattern is reminiscent of chronic active EBV infection (CAEBV seen in other organ systems such as the liver and bone marrow, the clinical presentation and epidemiologic profile are not typical for CAEBV.

  16. Metabolic Causes of Epileptic Encephalopathy

    Directory of Open Access Journals (Sweden)

    Joe Yuezhou Yu

    2013-01-01

    Full Text Available Epileptic encephalopathy can be induced by inborn metabolic defects that may be rare individually but in aggregate represent a substantial clinical portion of child neurology. These may present with various epilepsy phenotypes including refractory neonatal seizures, early myoclonic encephalopathy, early infantile epileptic encephalopathy, infantile spasms, and generalized epilepsies which in particular include myoclonic seizures. There are varying degrees of treatability, but the outcome if untreated can often be catastrophic. The importance of early recognition cannot be overemphasized. This paper provides an overview of inborn metabolic errors associated with persistent brain disturbances due to highly active clinical or electrographic ictal activity. Selected diseases are organized by the defective molecule or mechanism and categorized as small molecule disorders (involving amino and organic acids, fatty acids, neurotransmitters, urea cycle, vitamers and cofactors, and mitochondria and large molecule disorders (including lysosomal storage disorders, peroxisomal disorders, glycosylation disorders, and leukodystrophies. Details including key clinical features, salient electrophysiological and neuroradiological findings, biochemical findings, and treatment options are summarized for prominent disorders in each category.

  17. Hypertensive Encephalopathy with Reversible Brainstem Edema

    National Research Council Canada - National Science Library

    Lee, Sungjoon; Cho, Byung-Kyu; Kim, Hoon

    2013-01-01

    .... The patient's condition was thus interpreted as hypertensive brainstem encephalopathy. While many consider this a vasogenic phenomenon, induced by sudden, severe hypertension, the precise mechanism remains unclear...

  18. Venlafaxine as single therapy associated with hypertensive encephalopathy

    National Research Council Canada - National Science Library

    Bengt Edvardsson

    2015-01-01

      Introduction Hypertensive encephalopathy with the clinicoradiological entity posterior reversible encephalopathy syndrome in the setting of venlafaxine as single therapy has not been reported earlier...

  19. Infection increases mortality in necrotizing pancreatitis

    DEFF Research Database (Denmark)

    Werge, Mikkel; Novovic, Srdjan; Schmidt, Palle N

    2016-01-01

    OBJECTIVES: To assess the influence of infection on mortality in necrotizing pancreatitis. METHODS: Eligible prospective and retrospective studies were identified through manual and electronic searches (August 2015). The risk of bias was assessed using the Newcastle-Ottawa Scale (NOS). Meta...... sterile necrosis and organ failure was associated with a mortality of 19.8%. If the patients had infected necrosis without organ failure the mortality was 1.4%. CONCLUSIONS: Patients with necrotizing pancreatitis are more than twice as likely to die if the necrosis becomes infected. Both organ failure...... and infected necrosis increase mortality in necrotizing pancreatitis....

  20. MRI findings of Wernicke encephalopathy revisited due to hunger strike.

    Science.gov (United States)

    Unlu, Ercument; Cakir, Bilge; Asil, Talip

    2006-01-01

    The purpose of this study was to determine the characteristic magnetic resonance imaging (MRI) findings among a group of patients who presented with Wernicke encephalopathy (WE) due to the neurological complications of a long-term hunger strike (HS). MRI studies also including the fluid-attenuated inversion recovery (FLAIR) sequence and diffusion-weighted imaging (DWI) of six male patients with WE aged from 25 to 38 years (mean age 31 years) were evaluated. In all subjects, T2-weighted sequences, FLAIR and DWI revealed a signal hyperintensity within the posteromedial thalami and surrounding the third ventricle. In particular, on coronal images, the hyperintense areas around the third ventricle showed a suggestive "double wing" configuration. We observed an increased signal on proton-density and T2-weighted images in the mamillary bodies of three patients. Four patients demonstrated additional hyperintensities within the periaqueductal region and/or the tectal plate. At least one lesion area in five of six patients demonstrated contrast enhancement. The consistent imaging findings of our study suggest that MRI is a reliable means of diagnosing WE. Acute WE is sometimes underdiagnosed, yet early diagnosis and treatment of WE is crucial in order to avoid persistent brain damage. MRI, including postcontrast T1-weighted imaging, DWI beneath standardized T2-weighted imaging, and FLAIR sequences may prove to be a valuable adjunct to clinical diagnosis and to provide additional information in acute and/or subacute WE.

  1. MRI findings of Wernicke encephalopathy revisited due to hunger strike

    Energy Technology Data Exchange (ETDEWEB)

    Unlu, Ercument [Department of Radiology, Trakya University School of Medicine, Mimar Sinan m, Muammer Aksoy c, Yorulmaz apt, No 50, D-1 22030 Edirne (Turkey)]. E-mail: drercument@yahoo.com; Cakir, Bilge [Department of Radiology, Trakya University School of Medicine, Mimar Sinan m, Muammer Aksoy c, Yorulmaz apt, No 50, D-1 22030 Edirne (Turkey); Asil, Talip [Department of Neurology, Trakya University School of Medicine, Edirne (Turkey)

    2006-01-15

    Background and Purpose: The purpose of this study was to determine the characteristic magnetic resonance imaging (MRI) findings among a group of patients who presented with Wernicke encephalopathy (WE) due to the neurological complications of a long-term hunger strike (HS). Methods: MRI studies also including the fluid-attenuated inversion recovery (FLAIR) sequence and diffusion-weighted imaging (DWI) of six male patients with WE aged from 25 to 38 years (mean age 31 years) were evaluated. Results: In all subjects, T2-weighted sequences, FLAIR and DWI revealed a signal hyperintensity within the posteromedial thalami and surrounding the third ventricle. In particular, on coronal images, the hyperintense areas around the third ventricle showed a suggestive 'double wing' configuration. We observed an increased signal on proton-density and T2-weighted images in the mamillary bodies of three patients. Four patients demonstrated additional hyperintensities within the periaqueductal region and/or the tectal plate. At least one lesion area in five of six patients demonstrated contrast enhancement. Conclusion: The consistent imaging findings of our study suggest that MRI is a reliable means of diagnosing WE. Acute WE is sometimes underdiagnosed, yet early diagnosis and treatment of WE is crucial in order to avoid persistent brain damage. MRI, including postcontrast T1-weighted imaging, DWI beneath standardized T2-weighted imaging, and FLAIR sequences may prove to be a valuable adjunct to clinical diagnosis and to provide additional information in acute and/or subacute WE.

  2. Subacute Sclerosing Panencephalitis: The Foothold in Undervaccination.

    Science.gov (United States)

    Holt, Rebecca L; Kann, Dylan; Rassbach, Caroline E; Schwenk, Hayden T; Ritter, Jana M; Rota, Paul A; Elbers, Jorina

    2016-12-01

    Subacute sclerosing panencephalitis (SSPE) is a fatal complication of measles infection. We present a case of a fully vaccinated 3-year-old boy who was diagnosed with and treated for autoimmune encephalitis before arriving at a diagnosis of SSPE. We discuss the challenges of diagnosing SSPE in developed countries. Copyright © 2016 Elsevier Inc. All rights reserved.

  3. Papulo Necrotic Tuberculid on Glans Penis

    Directory of Open Access Journals (Sweden)

    K Pavithran

    1982-01-01

    Full Text Available A case of papulo-necrotic tuberculids affecting only glans penis is described. A positive Mantoux test, characteristic histology and response to anti tuberculous drugs confirmed the diagnosis.

  4. Fatal encephalopathy complicating persistent vomiting in pregnancy ...

    African Journals Online (AJOL)

    care to a patient with persistent HEG resulted in a fatal metabolic encephalopathy with neurological signs probably in ... Fatal encephalopathy complicating persistent vomiting in pregnancy: Importance of clinical awareness on the .... Since assessment of serum thia mine levels is not routinely available, the diagnosis of WE ...

  5. Birth defects in children with newborn encephalopathy

    NARCIS (Netherlands)

    Felix, JF; Badawi, N; Kurinczuk, JJ; Bower, C; Keogh, JM; Pemberton, PJ

    2000-01-01

    This study was designed to investigate birth defects found in association with newborn encephalopathy. All possible birth defects were ascertained in a population-based study of 276 term infants with moderate or severe encephalopathy and 564 unmatched term control infants. A strong association

  6. Normalization of the Psychometric Hepatic Encephalopathy score ...

    African Journals Online (AJOL)

    This is an open access article distributed under the terms of the Creative Commons. Attribution-Non Commercial-Share ... encephalopathy score (PHES) and evaluate the prevalence of minimal hepatic encephalopathy (MHE) among .... that can affect cognitive function; (3) diabetes mellitus;. (4) significant comorbid illness ...

  7. Ganciclovir-induced ataxia and encephalopathy.

    Science.gov (United States)

    Möhlmann, M C; Stiksma, J; Kramer, M H H

    2016-12-01

    Ganciclovir can be used to treat a primary cytomegalovirus (CMV) infection, however it can cause side effects. We describe a 60-year-old immunocompromised woman with a primary CMV infection who was treated with ganciclovir. She developed an encephalopathy which resolved after discontinuation of ganciclovir. A reversible encephalopathy as a side effect of ganciclovir.

  8. Wernicke's Encephalopathy in a Nigerian with Schizophrenia

    African Journals Online (AJOL)

    ANNALS

    alcoholic populations at risk for thiamine deficiency and Wernicke's encephalopathy and carrying out a detailed neurological examination in such patients. References. 1. Loh Y, Watson WD, Verma A, Chang ST,. Stocker DJ, Labutta RJ. Acute Wernicke's encephalopathy following bariatric surgery: clinical course and MRI ...

  9. Management of descending necrotizing mediastinitis.

    Science.gov (United States)

    Fu, Y L; Fahn, H J; Shi, H S; Wu, Y C; Huang, M H; Wang, L S

    1998-09-01

    Descending necrotizing mediastinitis (DNM) is uncommon, and may be lethal if not treated adequately and promptly. Delayed diagnosis of the disease is sometimes encountered in clinical practice. Eight consecutive patients with acute DNM were identified between 1991 and 1995, including five men and three women. The mean age was 45.8 years (range, 22-71 years). The infectious sources consisted of six esophageal perforations, one cervical cutting injury and one tonsillitis. The clinical presentations were evaluated. Diagnostic procedures including chest radiograph, sonogram and computerized tomography scans of the chest and neck were examined. Diagnosis and treatment, including culture results from drained fluids and debrided tissues, and antibiotic and supportive therapies were reviewed. Six patients who underwent aggressive surgical treatment recovered well. Two patients who received supportive treatment died of sepsis alone. The cultured bacteria included: Klebsiella oxytoca, Staphylococcus aureus, Trichosporum and other mixed oral cavity flora. Early diagnosis and adequate antibiotic and support therapies are essential to achieve good patient outcomes in acute descending mediastinitis. Adequate drainage and debridement, appropriate antibiotic therapy, and sufficient nutritional and respiratory support are the main treatment elements.

  10. No evidence of ischemia in stroke-like lesions of mitochondrial POLG encephalopathy.

    Science.gov (United States)

    Tzoulis, Charalampos; Henriksen, Eilen; Miletic, Hrvoje; Bindoff, Laurence A

    2017-01-01

    Stroke-like lesions are characteristically associated with mitochondrial encephalopathies such as those caused by mutations of polymerase gamma (POLG) and the m.3243A>G mitochondrial DNA (mtDNA) mutation. The combination of acute clinical onset, MRI and pathological abnormalities, have led to the suggestion that these lesions are ischemic. Here, we sought to determine the role of ischemia in the pathogenesis of mitochondrial stroke-like lesions. We performed a systematic study of cerebral blood vessel morphology, density and distribution in post mortem brain tissue from nine patients with POLG-encephalopathy and seven neurologically healthy controls. We found that patients had significantly higher cerebral vascular density than controls: this was more pronounced in areas of chronic neurodegeneration, where vascular density correlated with the severity of neuronal loss, but was also seen in acute lesions. Further, blood vessels were patent and, in acute lesions, dilated suggesting increased perfusion. In contrast to what would be expected in ischemia, stroke-like lesions were not pan-necrotic and were highly vascularized. Our results suggest that ischemia does not contribute to the pathogenesis of either the chronic neurodegeneration or acute lesions in POLG encephalopathy. Neovascularization and vascular dilatation does occur and suggests a compensatory response. We suggested the acute lesions are more likely to reflect energy insufficiency and our earlier studies suggest that this is driven in large part by seizure activity. Copyright © 2016 Elsevier B.V. and Mitochondria Research Society. All rights reserved.

  11. MR findings of wernicke encephalopathy

    Energy Technology Data Exchange (ETDEWEB)

    Yoon, Hyun Ki; Chang, Kee Hyun; Lee, Goo; Han, Moon Hee [Seoul National University College of Medicine, Seoul (Korea, Republic of); Park, Sung Ho; Na, Duk Yull; Song, Chi Sung [Young-Deung-Po City Hospital, Seoul (Korea, Republic of)

    1991-07-15

    Seven patients (33 to 58 years old) with clinical diagnoses of Wernicke encephalopathy were examined with MR on either a 2.0T (5 cases) or a 0.5T scanner (2 cases) using spin-echo pulse sequences. In 2 patients, follow-up MR studies were performed 1 and 5 weeks after thiamine (vitamine B1) treatment. Five patients (4 chronic alcoholics and 1 with hyperemesis gravidarum) showed atrophy of both mamillary bodies, along with patchy lesions around the third ventricle, medial thalami, tectum of the midbrain, and periaqueductal gray matter. Another patient with hyperemesis of gravidrum demonstrated only slightly atrophic mamillary bodies, and the last patient with severe vomiting after gastrojejunostomy showed only diencephaic/mesencephalic lesions with apparently normal mamillary bodies. A follow-up MR showed a decrease in previously-noted diencephalic/-/mesencephalic lesions but no change in the size of the mamillary bodies. Diencephalic/mesencephalic lesions were well seen as a high-signal intensity on proton-and T2-weighted axial images, while atrophy of the mamillary bodies was seen best on T1-weighted sagittal images. MR imaging is very useful in demonstrating the characteristic lesions of Wernicke encephalopathy and in evaluating the result of treatment on follow-up study.

  12. Atypical unilateral posterior reversible encephalopathy syndrome mimicking a middle cerebral artery infarction

    Energy Technology Data Exchange (ETDEWEB)

    Camidag, Ilkay [Dept. of Radiology, Ondokuz Mayis University, Faculty of Medicine, Samsun (Turkmenistan); Cho, Yang Je; Park, Mina; Lee, Seung Koo [Yonsei University Severance Hospital, Seoul (Korea, Republic of)

    2015-10-15

    Posterior reversible encephalopathy syndrome (PRES) is usually a reversible clinical and radiological entity associated with typical features on brain MR or CT imaging. However, the not-so-uncommon atypical radiological presentations of the condition are also present and they may go unrecognised as they are confused with other conditions. Here, we report a very rare case of atypical, unilateral PRES in a 49-year-old uremic, post-transplant female patient who presented with seizures. Initial MRI showed high-grade occlusion of the left middle cerebral artery (MCA) and lesions suggestive of subacute infarction in the ipsilateral frontotemporoparietal lobe. Patient symptoms had resolved a day after the onset without any specific treatment but early follow-up CT findings suggested hemorrhagic transformation. Follow-up MRI performed 2 years later showed complete disappearence of the lesions and persisting MCA occlusion.

  13. Defining encephalopathy in acute disseminated encephalomyelitis.

    Science.gov (United States)

    Fridinger, S E; Alper, Gulay

    2014-06-01

    The International Pediatric Multiple Sclerosis Study Group requires the presence of encephalopathy to diagnose acute disseminated encephalomyelitis. Clinical characteristics of encephalopathy are inadequately delineated in the pediatric demyelinating literature. The authors' purpose was to better define encephalopathy in pediatric acute disseminated encephalomyelitis by describing the details of the mental status change. A retrospective chart review was conducted for 25 children diagnosed with acute disseminated encephalomyelitis according to the International Pediatric Multiple Sclerosis Study Group guidelines. Frequency of encephalopathy-defining features was determined. Clinical characteristics, cerebrospinal fluid findings, and electroencephalography (EEG) findings were compared between patients with different stages of encephalopathy. The authors found irritability (36%), sleepiness (52%), confusion (8%), obtundation (20%), and coma (16%) as encephalopathy-defining features in acute disseminated encephalomyelitis. Twenty-eight percent had seizures, and 65% demonstrated generalized slowing on EEG. Approximately half of the patients in this study were diagnosed with encephalopathy based on the presence of irritability and/or sleepiness only. Such features in young children are often subtle and transient and thus difficult to objectively determine. © The Author(s) 2013.

  14. Brainstem involvement in subacute sclerosing panencephalitis.

    Science.gov (United States)

    Sharma, Pawan; Singh, Dileep; Singh, Maneesh Kumar; Garg, Ravindra Kumar; Kohli, Neera

    2011-01-01

    The parieto-occipital region of the brain is most frequently and severely affected in subacute sclerosing panencephalitis (SSPE). The basal ganglia, cerebellum and corpus callosum are less commonly involved. Brainstem involvement is rarely described in SSPE, and usually there is involvement of other regions of the brain. We describe a patient with subacute sclerosing panencephalitis with brain magnetic resonance imaging showing extensive brainstem involvement without significant involvement of other cortical structures. Though rarely described in SSPE, one should be aware of such brainstem and cerebellum involvement, and SSPE should be kept in mind when brainstem signal changes are seen in brain MRI with or without involvement of other regions of brain to avoid erroneous reporting.

  15. Multiple Complications Due to Subacute Suppurative Otitis Media

    OpenAIRE

    Qirjazi, Brikena; Bardhyli, Dolores; Hoxhallari, Xhevair

    2012-01-01

    Subacute otitis media is a well-known pathology of ENT practice which is easily diagnosed and subsequently treated in the outpatient clinic. The rate of complications in acute otitis media is lower than in chronic otitis media. We present here the history of a young patient with subacute otitis media who developed both localized labyrinthitis and facial palsy requiring surgical treatment. We conclude that the treatment of subacute otitis media should be carefully monitored because complicatio...

  16. Subacute Sclerosing Panencephalitis in a Child with Recurrent Febrile Seizures

    OpenAIRE

    Ayşe Kartal; Ayşegül Neşe Çıtak Kurt; Tuğba Hirfanoğlu; Kürşad Aydın; Ayşe Serdaroğlu

    2015-01-01

    Subacute sclerosing panencephalitis (SSPE) is a devastating disease of the central nervous system (CNS) caused by persistent mutant measles virus infection. The diagnosis of SSPE is based on characteristic clinical and EEG findings and demonstration of elevated antibody titres against measles in cerebrospinal fluid. Subacute sclerosing panencephalitis can have atypical clinical features at the onset. Herein, we report an unusual case of subacute sclerosing panencephalitis in a child with recu...

  17. Subacute sclerosing panencephalitis in immunized Thai children.

    Science.gov (United States)

    Khusiwilai, Khanittha; Viravan, Sorawit

    2011-12-01

    Subacute sclerosing panencephalitis (SSPE) is a progressive neurodegenerative disease with high mortality and poor prognosis. This is caused by persistent defective measles virus infection. Clinical presentations are variable including behavioral-cognitive change, myoclonic seizure, visual problem, spasticity or abnormal movement. The authors report a case of 10 year-old boy, previously healthy with complete immunization, presenting with frequent myoclonic jerks, abnormal movements, spasticity and altered mental status. Electroencephalographic (EEG), magnetic resonance imaging (MRI), and laboratory findings are typical for SSPE.

  18. Subacute sclerosing panencephalitis presenting as mania

    Directory of Open Access Journals (Sweden)

    Aggarwal Ashish

    2011-01-01

    Full Text Available Subacute sclerosing panencephalitis (SSPE is a rare, invariably fatal degenerative disease of the central nervous system developing after measles infection. Besides neurological symptoms as initial presenting symptoms, rare reports of its presentation with pure psychiatric symptoms have been reported. We here report a case of 14 year old male who initially presented with manic symptoms and then subsequently diagnosed to be suffering from SSPE. Improtance of ruling our organic conditions is emphasized.

  19. Late Onset Subacute Sclerosing Panencephalitis: Presenting Psychosis

    Directory of Open Access Journals (Sweden)

    Yavuz Altunkaynak

    2013-04-01

    Full Text Available Subacute Sclerosing Panencephalitis (SSPE is the late complication of measles and is characterized by seizures, myoclonus, ataxia, behavioral and personality changes, extrapyramidal dysfunctions and vision problems. A 19 year old female patient with SSPE who was followed up at psychiatry clinic with the diagnosis of atypical psychotic disorder was presented. While psychiatric signs and symptoms were dominant, she was diagnosed as SSPE.

  20. Subacute sclerosing panencephalitis: serial electroencephalographic studies.

    Science.gov (United States)

    Wulff, C H

    1982-01-01

    A total of 42 EEGs from five patients with subacute sclerosing panencephalitis were studied. Periodic complexes were noticed in 35 (83%) of these. The interval between the complexes shortened in all patients with progression of the illness. The gradual EEG changes may reflect the increasing number of infected cells as well as an on-going accumulation of immature virus structures. The records without complexes were either from the early onset (one record) or terminal stage (six records). PMID:7086454

  1. Subacute sclerosing panencephalitis presenting as mania

    Science.gov (United States)

    Aggarwal, Ashish; Khandelwal, Ashish; Jain, Manish; Jiloha, R. C.

    2011-01-01

    Subacute sclerosing panencephalitis (SSPE) is a rare, invariably fatal degenerative disease of the central nervous system developing after measles infection. Besides neurological symptoms as initial presenting symptoms, rare reports of its presentation with pure psychiatric symptoms have been reported. We here report a case of 14 year old male who initially presented with manic symptoms and then subsequently diagnosed to be suffering from SSPE. Improtance of ruling our organic conditions is emphasized. PMID:21808475

  2. Multiple Complications Due to Subacute Suppurative Otitis Media

    Directory of Open Access Journals (Sweden)

    Xhevair Hoxhallari

    2012-06-01

    Full Text Available Subacute otitis media is a well-known pathology of ENT practice which is easily diagnosed and subsequently treated in the outpatient clinic. The rate of complications in acute otitis media is lower than in chronic otitis media. We present here the history of a young patient with subacute otitis media who developed both localized labyrinthitis and facial palsy requiring surgical treatment. We conclude that the treatment of subacute otitis media should be carefully monitored because complications may occur. Surgical treatment and pressure release can be efficient for the treatment of a peripheral facial palsy in the course of acute or subacute otitis media.

  3. Subacute sclerosing panencephalitis in a child with recurrent febrile seizures.

    Science.gov (United States)

    Kartal, Ayşe; Çıtak Kurt, Ayşegül Neşe; Hirfanoğlu, Tuğba; Aydın, Kürşad; Serdaroğlu, Ayşe

    2015-01-01

    Subacute sclerosing panencephalitis (SSPE) is a devastating disease of the central nervous system (CNS) caused by persistent mutant measles virus infection. The diagnosis of SSPE is based on characteristic clinical and EEG findings and demonstration of elevated antibody titres against measles in cerebrospinal fluid. Subacute sclerosing panencephalitis can have atypical clinical features at the onset. Herein, we report an unusual case of subacute sclerosing panencephalitis in a child with recurrent febrile seizures. The disease progressed with an appearance of myoclonic jerks, periodic high amplitude generalized complexes on EEG, and elevated titers of measles antibodies in cerebrospinal fluid leading to the final diagnosis of subacute sclerosing panencephalitis.

  4. Subacute Sclerosing Panencephalitis in a Child with Recurrent Febrile Seizures

    Directory of Open Access Journals (Sweden)

    Ayşe Kartal

    2015-01-01

    Full Text Available Subacute sclerosing panencephalitis (SSPE is a devastating disease of the central nervous system (CNS caused by persistent mutant measles virus infection. The diagnosis of SSPE is based on characteristic clinical and EEG findings and demonstration of elevated antibody titres against measles in cerebrospinal fluid. Subacute sclerosing panencephalitis can have atypical clinical features at the onset. Herein, we report an unusual case of subacute sclerosing panencephalitis in a child with recurrent febrile seizures. The disease progressed with an appearance of myoclonic jerks, periodic high amplitude generalized complexes on EEG, and elevated titers of measles antibodies in cerebrospinal fluid leading to the final diagnosis of subacute sclerosing panencephalitis.

  5. Fundus Findings in Wernicke Encephalopathy

    Directory of Open Access Journals (Sweden)

    Tal Serlin

    2017-07-01

    Full Text Available Wernicke encephalopathy (WE is an acute neuropsychiatric syndrome resulting from thiamine (vitamin B1 deficiency, classically characterized by the triad of ophthalmoplegia, confusion, and ataxia. While commonly associated with chronic alcoholism, WE may also occur in the setting of poor nutrition or absorption. We present a 37-year-old woman who underwent laparoscopic sleeve gastrectomy and presented with visual disturbance with bilateral horizontal nystagmus, confusion, and postural imbalance. Fundus examination revealed bilateral optic disc edema with a retinal hemorrhage in the left eye. Metabolic workup demonstrated thiamine deficiency. Her symptoms resolved after thiamine treatment. This case raises the awareness of the possibility of posterior segment findings in WE, which are underreported in WE.

  6. Suicide and Chronic Traumatic Encephalopathy.

    Science.gov (United States)

    Iverson, Grant L

    2016-01-01

    For nearly 80 years, suicidality was not considered to be a core clinical feature of chronic traumatic encephalopathy (CTE). In recent years, suicide has been widely cited as being associated with CTE, and now depression has been proposed to be one of three core diagnostic features alongside cognitive impairment and anger control problems. This evolution of the clinical features has been reinforced by thousands of media stories reporting a connection between mental health problems in former athletes and military veterans, repetitive neurotrauma, and CTE. At present, the science underlying the causal assumption between repetitive neurotrauma, depression, suicide, and the neuropathology believed to be unique to CTE is inconclusive. Epidemiological evidence indicates that former National Football League players, for example, are at lower, not greater, risk for suicide than men in the general population. This article aims to discuss the critical issues and literature relating to these possible relationships.

  7. Necrotizing soft-tissue infection: laboratory risk indicator for necrotizing soft tissue infections score.

    Science.gov (United States)

    Kulkarni, Madhuri; Vijay Kumar, Gs; Sowmya, Gs; Madhu, Cp; Ramya, Sr

    2014-01-01

    Necrotizing soft tissue infections (NSTI) can be rapidly progressive and polymicrobial in etiology. Establishing the element of necrotizing infection poses a clinical challenge. A 64-year-old diabetic patient presented to our hospital with a gangrenous patch on anterior abdominal wall, which progressed to an extensive necrotizing lesion within 1 week. Successive laboratory risk indicator for necrotizing softtissue infections (LRINEC) scores confirmed the necrotizing element. Cultures yielded Enterococci, Acinetobacter species and Apophysomyces elegans and the latter being considered as an emerging agent of Zygomycosis in immunocompromised hosts. Patient was managed with antibiotics, antifungal treatment and surgical debridement despite which he succumbed to the infection. NSTI's require an early and aggressive management and LRINEC score can be applied to establish the element of necrotizing pathology. Isolation of multiple organisms becomes confusing to establish the etiological role. Apophysomyces elegans, which was isolated in our patient is being increasingly reported in cases of necrotizing infections and may be responsible for high morbidity and mortality. This scoring has been proposed as an adjunct tool to Microbiological diagnosis when NSTI's need to be diagnosed early and managed promptly to decrease mortality and morbidity, which however may not come in handy in an immunocompromised host with polymicrobial aggressive infection.

  8. Chronic traumatic encephalopathy: The unknown disease.

    Science.gov (United States)

    Martínez-Pérez, R; Paredes, I; Munarriz, P M; Paredes, B; Alén, J F

    2017-04-01

    Chronic traumatic encephalopathy is a neurodegenerative disease produced by accumulated minor traumatic brain injuries; no definitive premortem diagnosis and no treatments are available for chronic traumatic encephalopathy. Risk factors associated with chronic traumatic encephalopathy include playing contact sports, presence of the apolipoprotein E4, and old age. Although it shares certain histopathological findings with Alzheimer disease, chronic traumatic encephalopathy has a more specific presentation (hyperphosphorylated tau protein deposited as neurofibrillary tangles, associated with neuropil threads and sometimes with beta-amyloid plaques). Its clinical presentation is insidious; patients show mild cognitive and emotional symptoms before progressing to parkinsonian motor signs and finally dementia. Results from new experimental diagnostic tools are promising, but these tools are not yet available. The mainstay of managing this disease is prevention and early detection of its first symptoms. Copyright © 2014 Sociedad Española de Neurología. Publicado por Elsevier España, S.L.U. All rights reserved.

  9. Outcome Factors in Hypoxic Ischemic Encephalopathy

    OpenAIRE

    J Gordon Millichap

    2002-01-01

    The predictive value of history, examination, Glasgow Coma Scale (GCS) scores, EEG and sensory evoked potentials (SEP) in the prognosis of children with acute hypoxic-ischemic encephalopathy (HIE) was evaluated at the University Hospital of Lille, France.

  10. Posterior reversible encephalopathy syndrome: Some novel ...

    African Journals Online (AJOL)

    transient and reversible neurological disorder clinically characterised by headache, seizures, blindness and altered consciousness associated with radiological ... presented with transient encephalopathy following blood transfusion but involving the anterior brain rather than the posterior part classically described in PRES.

  11. Ethylmalonic encephalopathy associated with crescentic glomerulonephritis.

    Science.gov (United States)

    Dweikat, Imad; Naser, Enas; Damsah, Nadera; Libdeh, Bassam Abu; Bakri, Izzeddin

    2012-12-01

    Ethylmalonic encephalopathy (EE) is a rare autosomal recessive disorder caused by mutations in the ETHE1 gene and characterized by chronic diarrhea, encephalopathy, relapsing petechiae and acrocyanosis. Nephrotic syndrome has been described in an infant with EE but the renal histology findings were not described in previous reports. We report a Palestinian girl with EE who presented with chronic diarrhea, encephalopathy, petechial rash and acrocyanosis. Subsequently, she developed progressive deterioration of renal function caused by rapidly progressive glomerulonephritis resulting in death within few days. This is, to our knowledge, the first reported occurrence of rapidly progressive glomerulonephritis in a child with ethylmalonic encephalopathy. Its presence is a serious complication associated with poor prognosis and may be explained by the diffuse vascular damage.

  12. Acute Necrotizing Esophagitis Followed by Duodenal Necrosis

    Science.gov (United States)

    del Hierro, Piedad Magdalena

    2011-01-01

    Acute Necrotizing Esophagitis is an uncommon pathology, characterized by endoscopic finding of diffuse black coloration in esophageal mucosa and histological presence of necrosis in patients with upper gastrointestinal bleeding. The first case of acute necrotizing esophagitis followed by duodenal necrosis, in 81 years old woman with a positive history of Type 2 Diabetes Mellitus, Hypertension, and usual intake of Nonsteroidal Anti-inflammatory drugs, is reported. Although its etiology remains unknown, the duodenal necrosis suggests that ischemia could be the main cause given that the branches off the celiac axis provide common blood supply to the distal esophageal and duodenal tissue. The massive gastroesophagic reflux and NSAID intake could be involved. PMID:27957030

  13. Cervicofacial necrotizing fasciitis following periodontal abscess.

    Science.gov (United States)

    Medeiros, Rui; Catunda, Ivson de Sousa; Queiroz, Isaac Vieira; de Morais, Hecio Henrique Araujo; Leao, Jair Carneiro; Gueiros, Luiz Alcino Monteiro

    2012-01-01

    Soft tissue infections are characterized by acute inflammation, diffuse edema, and suppuration, and are often associated with symptoms such as malaise, fever, tachycardia, and chills. Necrotizing fasciitis is a destructive bacterial infection affecting subcutaneous tissue and superficial fascia and is associated with high rates of mortality. It usually involves the abdomen and extremities, but it also can occur in the head and neck. Early diagnosis is critical and the most commonly accepted treatment includes radical surgical intervention and administration of broad-spectrum antibiotics. This article reports and discusses the case of a patient with odontogenic cervicofacial necrotizing fasciitis, and emphasizes the importance of early and effective treatment.

  14. Necrotizing fasciitis caused by group A streptococcus

    Directory of Open Access Journals (Sweden)

    Mikić Dragan

    2002-01-01

    Full Text Available The first case of the confirmed necrotizing fasciitis caused by Group A Streptococcus in Yugoslavia was presented. Male patient, aged 28, in good health, suddenly developed symptoms and signs of severe infective syndrome and intensive pain in the axillary region. Parenteral antibiotic, substitution and supportive therapy was conducted along with the radical surgical excision of the necrotizing tissue. The patient did not develop streptococcal toxic shock syndrome thanks to the early established diagnosis and timely applied aggressive treatment. He was released from the hospital as completely cured two months after the admission.

  15. Acute hepatic encephalopathy with diffuse cortical lesions

    Energy Technology Data Exchange (ETDEWEB)

    Arnold, S.M.; Spreer, J.; Schumacher, M. [Section of Neuroradiology, Univ. of Freiburg (Germany); Els, T. [Dept. of Neurology, University of Freiburg (Germany)

    2001-07-01

    Acute hepatic encephalopathy is a poorly defined syndrome of heterogeneous aetiology. We report a 49-year-old woman with alcoholic cirrhosis and hereditary haemorrhagic telangiectasia who developed acute hepatic coma induced by severe gastrointestinal bleeding. Laboratory analysis revealed excessively elevated blood ammonia. MRI showed lesions compatible with chronic hepatic encephalopathy and widespread cortical signal change sparing the perirolandic and occipital cortex. The cortical lesions resembled those of hypoxic brain damage and were interpreted as acute toxic cortical laminar necrosis. (orig.)

  16. Duloxetine-related posterior reversible encephalopathy syndrome

    OpenAIRE

    Zappella, Nathalie; Perier, Fran?ois; Pico, Fernando; Palette, Catherine; Muret, Alexandre; Merceron, Sybille; Girbovan, Andrei; Marquion, Fabien; Legriel,Stephane

    2016-01-01

    Abstract Background: Posterior reversible encephalopathy syndrome (PRES) has well-established links with several drugs. Whether a link also exists with serotonin?norepinephrine reuptake inhibitor such as duloxetine is unclear. Methods: We report on a patient who developed PRES with a coma and myoclonus related to hypertensive encephalopathy a few days after starting duloxetine treatment. Magnetic resonance imaging was performed and catecholamine metabolites assayed. Results: The patient achie...

  17. Disrupted glutamate-glutamine cycle in acute encephalopathy with biphasic seizures and late reduced diffusion

    Energy Technology Data Exchange (ETDEWEB)

    Takanashi, Jun-ichi; Terai, Masaru [Tokyo Women' s Medical University Yachiyo Medical Center, Department of Pediatrics, Yachiyo-shi (Japan); Mizuguchi, Masashi [The University of Tokyo, Department of Developmental Medical Sciences, Graduate School of Medicine, Tokyo (Japan); Barkovich, A.J. [University of California San Francisco, Department of Radiology and Biomedical Imaging, San Francisco, CA (United States)

    2015-11-15

    Acute encephalopathy with biphasic seizures and late reduced diffusion (AESD) is the most common subtype of infectious pediatric encephalopathy in Japan. It is sometimes difficult to make an early diagnosis of AESD; excitotoxicity is postulated to be the pathogenesis based on elevated glutamine (Gln) and glutamate (Glu) complex (Glx = Glu + Gln) observed on MR spectroscopy. It is uncertain whether Gln or Glu contributes to the elevated Glx, or whether MR spectroscopy is useful for an early diagnosis. Five Japanese patients with AESD (three boys and two girls, 1 year of age) were enrolled in this study. MR spectroscopy was acquired from the frontal white matter (repetition time (TR) of 5000 ms, echo time (TE) of 30 ms) with a 1.5- or 3.0-T scanner. MR spectroscopy was performed four times for two patients, three times for one patient, and two times for two patients. Quantification of Glu and Gln was performed using LCModel. Glu was elevated in three of four studies on days 1-4 and became normal or low afterward. Gln was normal in three studies on days 1-2, elevated in all seven studies on days 4-12, and became normal or low afterward. These findings suggest that MR spectroscopy may be useful for an early diagnosis. Acute Glu elevation changes to subacute Gln elevation, suggesting that a disrupted Glu-Gln cycle may play an important role. (orig.)

  18. A case of Hashimoto`s encephalopathy presenting with seizures and psychosis

    Directory of Open Access Journals (Sweden)

    Min-Joo Lee

    2012-03-01

    Full Text Available Hashimoto’s encephalopathy (HE is a rare, poorly understood, autoimmune disease characterized by symptoms of acute or subacute encephalopathy associated with increased anti-thyroid antibody levels. Here, we report a case of a 14-year-old girl with HE and briefly review the literature. The patient presented with acute mental changes and seizures, but no evidence of infectious encephalitis. In the acute stage, the seizures did not respond to conventional antiepileptic drugs, including valproic acid, phenytoin, and topiramate. The clinical course was complicated by the development of acute psychosis, including bipolar mood, insomnia, agitation, and hallucinations. The diagnosis of HE was supported by positive results for antithyroperoxidase and antithyroglobulin antibodies. Treatment with methylprednisolone was effective; her psychosis improved and the number of seizures decreased. HE is a serious but curable, condition, which might be underdiagnosed if not suspected. Anti-thyroid antibodies must be measured for the diagnosis. HE should be considered in patients with diverse neuropsychiatric manifestations.

  19. Staged multidisciplinary step-up management for necrotizing pancreatitis

    NARCIS (Netherlands)

    da Costa, D. W.; Boerma, D.; van Santvoort, H. C.; Horvath, K. D.; Werner, J.; Carter, C. R.; Bollen, T. L.; Gooszen, H. G.; Besselink, M. G.; Bakker, O. J.

    2014-01-01

    Some 15 per cent of all patients with acute pancreatitis develop necrotizing pancreatitis, with potentially significant consequences for both patients and healthcare services. This review summarizes the latest insights into the surgical and medical management of necrotizing pancreatitis. General

  20. Staged multidisciplinary step-up management for necrotizing pancreatitis

    NARCIS (Netherlands)

    Costa, D.W. da; Boerma, D.; Santvoort, H.C. van; Horvath, K.D.; Werner, J.; Carter, C.R.; Bollen, T.L.; Gooszen, H.G.; Besselink, M.G.; Bakker, O.J.

    2014-01-01

    BACKGROUND: Some 15 per cent of all patients with acute pancreatitis develop necrotizing pancreatitis, with potentially significant consequences for both patients and healthcare services. METHODS: This review summarizes the latest insights into the surgical and medical management of necrotizing

  1. Rat Brain Biogenic Amine Levels during Acute and Subacute ...

    African Journals Online (AJOL)

    Rat Brain Biogenic Amine Levels during Acute and Subacute Phosphamidon Treatment with Reference to Behavioral Tolerance. ... African Research Review ... The present study examines if the levels of amine neurotransmitter substances in rat brain regions are altered during acute and sub-acute treatment with an ...

  2. Differentiation of ruminant transmissible spongiform encephalopathy isolate types, including bovine spongiform encephalopathy and CH1641 scrapie

    NARCIS (Netherlands)

    Jacobs, J.G.; Sauer, M.; Keulen, van L.J.M.; Tang, Y.; Bossers, A.; Langeveld, J.P.M.

    2011-01-01

    With increased awareness of the diversity of transmissible spongiform encephalopathy (TSE) strains in the ruminant population, comes an appreciation of the need for improved methods of differential diagnosis. Exposure to bovine spongiform encephalopathy (BSE) has been associated with the human TSE,

  3. Necrotizing Soft-Tissue Infections: Clinical Guidelines

    Science.gov (United States)

    2009-10-01

    streptococcal toxic shock syndrome associated with necrotizing fasciitis: case re- port and review. Pharmacotherapy 1999;19:1094–8. 36. Simmonds M. Necrotising...fasciitis and group A streptococ- cus toxic shock-like syndrome in pregnancy : treatment with plasmapheresis and immunoglobulin. Int J Obstet Anesth

  4. Intestinal proteome changes during infant necrotizing enterocolitis

    DEFF Research Database (Denmark)

    Jiang, Pingping; Smith, Birgitte; Qvist, Niels

    2013-01-01

    between necrotic and vital small-intestine sections and 23 proteins were identified for colon sections. Five proteins were similarly affected in the small intestine and colon: histamine receptors (HRs), actins, globins, immunoglobulin, and antitrypsin. Two heat shock proteins (HSPs) were affected...

  5. Necrotizing fasciitis : plain radiographic and CT findings

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Chang Dae; Park, Jeong Hee; Jeon, Hae Jeong; Lim, Jong Nam; Heo, Tae Haeng; Park, Dong Rib [Konkuk Univ. College of Medicine, Seoul (Korea, Republic of)

    1996-11-01

    To evaluate the plain radiographic and CT findings of the necrotizing fasciitis. We retrospectively reviewed the radiologic findings of 4 cases with necrotizing fasciitis. Three cases were proven pathologically. We evaluated pattern and extent of the gas shadows in plain films. CT findings were analysed, with emphasis on : (a) gas pattern, (b) extent, (c) location and involved site, (d) associated focal abscess, and (e) swelling of the adjacent muscles. On plain radiographs, four cases showed streaky or mottled gas densities in the pelvis, three cases in the perineum, one case in the abdomen, and two cases in the thigh. On CT images, gas pattern was mottled and streaky appearance with swelling of the adjacent muscles. Gas shadows located in the extraperitoneal space in four cases, fascial layer in four cases, and subcutaneous layer in four cases. There were gas shadows in pelvic wall, perineum, abdominal wall, buttock, thigh, and scrotum. Focal low density lesion suggestive of focal abscess was not visualized. Plain radiography is useful for early diagnosis of the necrotizing fasciitis and CT is very useful for detection of precise location and extent of the disease. CT is also useful for differentiation of necrotizing fasciitis from focal abscess and cellulitis.

  6. Subacute toxicity assessment of annatto in rat

    OpenAIRE

    Bautista, Ana Rita Pedreira Lapa; E.L.T. Moreira; Batista, Márcio Santos; Miranda, M.S.; Gomes, I. C. S.

    2004-01-01

    Texto completo:acesso restrito. p. 625-629 Increased human use of annatto (Bixa orellana L), a red yellow food colorant, demands generation of toxicity data. The toxic effects of annatto powder (bixin 27%) have been assessed following administration of a subacute regimen (4 weeks, 20 doses) in Wistar male and female rats. A full study with three dose levels was considered unnecessary since no sign of toxicity had been noted in a preliminary experiment with 1000 mg/kg body weight/day as ...

  7. Subacute sclerosing panencephalitis: a case report.

    Science.gov (United States)

    Praticò, A D; Saporito, M; Iacono, O; Castellano-Chiodo, D; Pavone, P

    2011-02-01

    Subacute sclerosing panencephalitis (SSPE) is a progressive neurological disorder of childhood and early adolescence caused by persistent defective measles virus. Clinical manifestations appear many years after the acute measles infection. The incidence of SSPE has substantially declined after the introduction of an effective vaccine. We report a case of a child with SSPE that began with atonia, dysarthria, and intellectual deterioration without the presence of any particular EEG anomalies. We have reported this girl who was affected by this severe affliction in the hope that, because of the rarity of SSPE, it would not go undiagnosed.

  8. Subacute Cutaneous Lupus Erythematosus Triggered by Radiotherapy

    Directory of Open Access Journals (Sweden)

    I. Kolm

    2013-08-01

    Full Text Available Background: The origin of collagen autoimmune diseases is not fully understood. Some studies postulate a mechanism of molecular mimicry or heterologous immunity following viral infections triggering autoimmunity. Apart from infections, other exogenous factors such as visible light or X-rays have been reported to incite autoimmunity. Case Report: We report a case of histologically and serologically confirmed subacute lupus erythematosus (SCLE following radiotherapy for breast cancer. Discussion: The close temporal and spatial correlation between radiotherapy and onset of SCLE in this patient suggests that an autoimmune reaction may have been triggered locally by functionally altering the immune system and breaking self-tolerance.

  9. Cereals Bond Trounces Subacute Rumen Acidosis

    OpenAIRE

    Akbar Nikkhah

    2015-01-01

    This perspective article provides a feasible ideology based on which modern ruminant enterprises will learn to vigilantly include mixtures of hard and soft cereal grains in optimizing rumen environment. Subacute Rumen Acidosis (SARA), variably defined as a common and economically important metabolic disease, occurs arguably when rumen pH declines below 5.8-6 for a long-lasting period of time of several hours. Prolonged SARA reduces high-producing dairy and bee...

  10. Chronic traumatic encephalopathy and athletes.

    Science.gov (United States)

    Meehan, William; Mannix, Rebekah; Zafonte, Ross; Pascual-Leone, Alvaro

    2015-10-27

    Recent case reports have described athletes previously exposed to repetitive head trauma while participating in contact sports who later in life developed mood disorders, headaches, cognitive difficulties, suicidal ideation, difficulties with speech, and aggressive behavior. Postmortem discoveries show that some of these athletes have pathologic findings that are collectively termed chronic traumatic encephalopathy (CTE). Current hypotheses suggest that concussions or perhaps blows to the head that do not cause the signs and symptoms necessary for making the diagnosis of concussion, so-called subconcussive blows, cause both the clinical and pathologic findings. There are, however, some athletes who participate in contact sports who do not develop the findings ascribed to CTE. Furthermore, there are people who have headaches, mood disorders, cognitive difficulties, suicidal ideation, and other clinical problems who have neither been exposed to repeated head trauma nor possessed the pathologic postmortem findings of those currently diagnosed with CTE. The current lack of prospective data and properly designed case-control studies limits the current understanding of CTE, leading to debate about the causes of the neuropathologic findings and the clinical observations. Given the potential for referral and recall bias in available studies, it remains unclear whether or not the pathologic findings made postmortem cause the presumed neurobehavioral sequela and whether the presumed risk factors, such as sports activity, cerebral concussions, and subconcussive blows, are solely causative of the clinical signs and symptoms. This article discusses the current evidence and the associated limitations. © 2015 American Academy of Neurology.

  11. Chronic traumatic encephalopathy: a review.

    Science.gov (United States)

    Saulle, Michael; Greenwald, Brian D

    2012-01-01

    Chronic traumatic encephalopathy (CTE) is a progressive neurodegenerative disease that is a long-term consequence of single or repetitive closed head injuries for which there is no treatment and no definitive pre-mortem diagnosis. It has been closely tied to athletes who participate in contact sports like boxing, American football, soccer, professional wrestling and hockey. Risk factors include head trauma, presence of ApoE3 or ApoE4 allele, military service, and old age. It is histologically identified by the presence of tau-immunoreactive NFTs and NTs with some cases having a TDP-43 proteinopathy or beta-amyloid plaques. It has an insidious clinical presentation that begins with cognitive and emotional disturbances and can progress to Parkinsonian symptoms. The exact mechanism for CTE has not been precisely defined however, research suggest it is due to an ongoing metabolic and immunologic cascade called immunoexcitiotoxicity. Prevention and education are currently the most compelling way to combat CTE and will be an emphasis of both physicians and athletes. Further research is needed to aid in pre-mortem diagnosis, therapies, and support for individuals and their families living with CTE.

  12. Chronic Traumatic Encephalopathy: A Review

    Directory of Open Access Journals (Sweden)

    Michael Saulle

    2012-01-01

    Full Text Available Chronic traumatic encephalopathy (CTE is a progressive neurodegenerative disease that is a long-term consequence of single or repetitive closed head injuries for which there is no treatment and no definitive pre-mortem diagnosis. It has been closely tied to athletes who participate in contact sports like boxing, American football, soccer, professional wrestling and hockey. Risk factors include head trauma, presence of ApoE3 or ApoE4 allele, military service, and old age. It is histologically identified by the presence of tau-immunoreactive NFTs and NTs with some cases having a TDP-43 proteinopathy or beta-amyloid plaques. It has an insidious clinical presentation that begins with cognitive and emotional disturbances and can progress to Parkinsonian symptoms. The exact mechanism for CTE has not been precisely defined however, research suggest it is due to an ongoing metabolic and immunologic cascade called immunoexcitiotoxicity. Prevention and education are currently the most compelling way to combat CTE and will be an emphasis of both physicians and athletes. Further research is needed to aid in pre-mortem diagnosis, therapies, and support for individuals and their families living with CTE.

  13. Joseph Haydn's encephalopathy: new aspects.

    Science.gov (United States)

    Blahak, Christian; Bäzner, Hansjörg; Hennerici, Michael G

    2015-01-01

    With increasing age, Joseph Haydn complained of progressive forgetfulness preventing him from composing for about the last 8 years of his life. He spent his days more and more inactive and immobilized, suffering from a disabling gait disturbance. Still, most biographers consider diffuse atherosclerosis and congestive heart failure to be reasons for Haydn's medical condition and physical decline during the last years of his life. A more sophisticated and detailed inspection of documents and sources, however, leads to the diagnosis of subcortical vascular encephalopathy (SVE), caused by progressive cerebral small vessel disease. Important features of the disease are mood changes, urinary symptoms, and in particular a characteristic gait disturbance, while dementia is only mild and occurs later in the course. Haydn was severely disabled by the symptoms of SVE for several years and often reported difficulties in the completion of his last oratorio "Die Jahreszeiten" (The Seasons). Subsequently, the disease prevented him from composing another large oratorio, "Das jüngste Gericht" (The last judgement), which had been already drafted. Finally, the progress of SVE stopped his long career as a composer and conductor at the age of 73 years. © 2015 Elsevier B.V. All rights reserved.

  14. Bovine Spongiform Encephalopathy (BSE), or Mad Cow Disease

    Science.gov (United States)

    ... Search Form Controls Cancel Submit Search the CDC Bovine Spongiform Encephalopathy (BSE), or Mad Cow Disease Note: ... gov . Recommend on Facebook Tweet Share Compartir BSE (bovine spongiform encephalopathy) is a progressive neurological disorder of ...

  15. Irreversible encephalopathy after treatment with high-dose intravenous metronidazole.

    NARCIS (Netherlands)

    Groothoff, M.V.R.; Hofmeijer, J.; Sikma, M.A.; Meulenbelt, J.

    2010-01-01

    BACKGROUND: Encephalopathy associated with metronidazole is rare and, in most cases, reversible following discontinuation. OBJECTIVE: We describe a case of fatal encephalopathy after treatment with high-dose intravenous metronidazole and the potential causes of the irreversibility. CASE SUMMARY: A

  16. Irreversible Encephalopathy After Treatment With High-Dose Intravenous Metronidazole

    NARCIS (Netherlands)

    Groothoff, Miriam V. R.; Hofmeijer, Jannette; Sikma, Maaike A.; Meulenbelt, Jan

    Background: Encephalopathy associated with metronidazole is rare and, in most cases, reversible following discontinuation. Objective: We describe a case of fatal encephalopathy after treatment with high-dose intravenous metronidazole and the potential causes of the irreversibility. Case summary: A

  17. Hypertensive encephalopathy in a patient with neonatal thyrotoxicosis

    NARCIS (Netherlands)

    Pijnenburg, MWH; Zweens, MJ; Bink, MTE; Odink, RJ

    1999-01-01

    Neonatal hyperthyroidism may give rise to serious cardiovascular complications. A girl with severe thyrotoxicosis in whom hypertensive encephalopathy developed is described. Conclusion Neonatal thyrotoxicosis can give rise to hypertension and may lead to hypertensive encephalopathy.

  18. Clinical profile of subacute sclerosing panencephalitis.

    Science.gov (United States)

    Akram, Muhammad; Naz, Farrah; Malik, Akbar; Hamid, Haroon

    2008-08-01

    To describe the clinical manifestations of subacute sclerosing panencephalitis in children. Case series. This study was conducted in the Department of Neurology at The Children's Hospital and the Institute of Child Health, Lahore, from April 2005 to April 2007. Fifty patients were diagnosed as subacute sclerosing panencephalitis during the study period. Their diagnosis was based on a detailed history, clinical examination, presence of antimeasles antibodies in Cerebrospinal Fluid (CSF) and typical electroencephalogram (EEG). The findings were described as average, mean and percentages. Fifty patients were included in this study. The average age of the patients was 8 years. Thirty-eight (76%) were males and 12 (24%) were females. The average duration of symptoms before presentation was 66.72 days. History of measles infection was present in 31 patients (62%) and measles vaccination in 43 patients (86%). Motor regression was present in all (100%) patients and cognition decline in 43 patients (86%). Seizures were focal (10%), generalized tonicclonic (16%) and myoclonic (74%). Burst-suppression pattern Electroencephalogram (EEG) and the antimeasles antibody in CSF were positive in 100% of patients. SSPE is an indicator of high incidence of measles infection among the paediatric population even among vaccinated children. Males are more common sufferers. SSPE can present with different types of seizures, cognition decline and motor regression being supported by suggestive EEG and presence of anti-measles antibodies in CSF.

  19. Outcome determinants of subacute low back pain.

    Science.gov (United States)

    Karjalainen, Kaija; Malmivaara, Antti; Mutanen, Pertti; Pohjolainen, Timo; Roine, Risto; Hurri, Heikki

    2003-12-01

    Descriptive prognostic study. To identify outcome determinants of subacute low back pain. The factors predicting recovery from prolonged back pain among working adults are largely unknown. One hundred sixty-four employed patients with subacute (duration of pain 4-12 weeks) daily low back pain were recruited from primary health care to a randomized study. Data on potential predictive factors were collected before randomization. In multiple regressions using repeated measures analysis, the treatment received was adjusted when determining the impact of the predictive factors. Dependent outcome variables used were pain, perceived functional disability, generic health-related quality of life, satisfaction with care, days on sick leave, use of health care, and costs of health care consumption measured, at 3-, 6-, and 12-month follow-ups. Age and intensity of pain at baseline predicted most of the outcomes. The perceived risk of not recovering was a stronger determinant of outcome than gender, education, or self-rated health status (which did not have any predictive value) or body mass index, expectations of treatment effect, satisfaction with work, or the presence of radicular symptoms below the knee (only slight predictive value). The only factors predicting the duration of sick leave were the duration of sick leave at baseline and the type of occupation. Age and intensity of pain are the strongest predictors of outcome. Accumulation of days on sick leave is predicted by the duration of sick leave at entry and the type of work, but not by pain, perceived disability, or satisfaction with work.

  20. Electroencephalography and Brain MRI Patterns in Encephalopathy.

    Science.gov (United States)

    Wabulya, Angela; Lesser, Ronald P; Llinas, Rafael; Kaplan, Peter W

    2016-04-01

    Using electroencephalography (EEG) and histology in patients with diffuse encephalopathy, Gloor et al reported that paroxysmal synchronous discharges (PSDs) on EEG required combined cortical gray (CG) and "subcortical" gray (SCG) matter pathology, while polymorphic delta activity (PDA) occurred in patients with white matter pathology. In patients with encephalopathy, we compared EEG findings and magnetic resonance imaging (MRI) to determine if MRI reflected similar pathological EEG correlations. Retrospective case control study of 52 cases with EEG evidence of encephalopathy and 50 controls without evidence of encephalopathy. Review of clinical, EEG and MRI data acquired within 4 days of each other. The most common EEG finding in encephalopathy was background slowing, in 96.1%. We found PSDs in 0% of cases with the combination of CG and SCG abnormalities. Although 13.5% (n=7) had PSDs on EEG; 3 of these had CG and 4 had SCG abnormalities. A total of 73.1% (38/52) had white matter abnormalities-of these 28.9% (11/38) had PDA. PSDs were found with either CG or "SCG" MRI abnormalities and did not require a combination of the two. In agreement with Gloor et al, PDA occurred with white matter MRI abnormalities in the absence of gray matter abnormalities. © EEG and Clinical Neuroscience Society (ECNS) 2015.

  1. Branched-chain amino acids for hepatic encephalopathy

    DEFF Research Database (Denmark)

    Als-Nielsen, B; Koretz, R L; Kjaergard, L L

    2003-01-01

    Hepatic encephalopathy may be caused by a decreased plasma ratio of branched-chain amino acids (BCAA) to aromatic amino acids. Treatment with BCAA may therefore have a beneficial effect on patients with hepatic encephalopathy.......Hepatic encephalopathy may be caused by a decreased plasma ratio of branched-chain amino acids (BCAA) to aromatic amino acids. Treatment with BCAA may therefore have a beneficial effect on patients with hepatic encephalopathy....

  2. Wernicke encephalopathy in a patient with liver failure

    OpenAIRE

    Zhao, Pan; Zhao, Yanling; Wei, Zhenman; Chen, Jing; Yan, Lilong

    2016-01-01

    Abstract Early recognition and diagnosis of Wernicke encephalopathy is pivotal for the prognosis of this medical emergency, especially in patients with liver failure which predisposes individuals to develop hepatic encephalopathy. For these patients, distinguishing between hepatic encephalopathy and Wernicke encephalopathy is a challenge in real-world clinical practice. A male patient with 21-year medical history of liver cirrhosis presented diarrhea and ascites. One month before this visit, ...

  3. Necrotizing fasciitis of the neck: case presentation

    Directory of Open Access Journals (Sweden)

    Emil Marginean

    2017-10-01

    Full Text Available Necrotizing fasciitis represents a severe and rare infectious disease, which is accompanied by extended necrosis of subcutaneous tissues and fascia layers, having as a result gangrene of the teguments. To reduce mortality associated with this disease, a rapid diagnosis is necessary as well as aggressive surgical treatment, accompanied by adequate, aggressive antibiotic therapy. As a rule, etiology is teeth related, with the condition more common in immunology-depressed patients. This article presents the experience of the authors regarding a case presentation of necrotizing fasciitis with typical clinical expression and management of the disease in an immunological-depressed patient. Further data on diagnosis, microbiology, clinical manifestations, therapy principles, and prognosis are yet needed for atypical cases.

  4. Life-threatening cervical necrotizing fasciitis.

    Science.gov (United States)

    Helmy, A S; Salah, M A; Nawara, H A; Khatab, H; Khalaf, H A; Abd el-Maguid, N

    1997-12-01

    Eight cases of cervical necrotizing fasciitis are presented. Three were odontogenic, two were pharyngeal in origin and three were primary or idiopathic. Soft tissue gas was recognized in four patients. The bacteriology showed streptococci on the top of the list (50%), while for the idiopathic cases, it was monomicrobial and caused by staphylococci. Third generation cephalosporin and metronidazole represent good initial empirical antibacterial coverage. Histopathologically, all cases showed extensive necrosis of the debrided fascia and vascular thrombosis of the dermal vessels. The mortality rate was 3/8 (37.5%). Early diagnosis of cervical necrotizing fasciitis and initiation of definitive therapy in an intensive care environment is essential to minimize mortality. It is also important to recognize that this devastating infection may occur spontaneously, and it should be suspected in patients with unexplained soft tissue pain and tenderness.

  5. Concussion in Chronic Traumatic Encephalopathy.

    Science.gov (United States)

    Stein, Thor D; Alvarez, Victor E; McKee, Ann C

    2015-10-01

    Chronic traumatic encephalopathy (CTE) is a progressive neurodegenerative disease that occurs in association with repetitive mild traumatic brain injury. It is associated with a variety of clinical symptoms in multiple domains, and there is a distinct pattern of pathological changes. The abnormal tau pathology in CTE occurs uniquely in those regions of the brain that are likely most susceptible to stress concentration during trauma. CTE has been associated with a variety of types of repetitive head trauma, most frequently contact sports. In cases published to date, the mean length of exposure to repetitive head trauma was 15.4 years. The clinical symptoms of the disease began after a mean latency of 14.5 years with a mean age of death of 59.3 years. Most subjects had a reported history of concussions with a mean of 20.3. However, 16 % of published CTE subjects did not have a history of concussion suggesting that subconcussive hits are sufficient to lead to the development of CTE. Overall, the number of years of exposure, not the number of concussions, was significantly associated with worse tau pathology in CTE. This suggests that it is the chronic and repetitive nature of head trauma, irrespective of concussive symptoms, that is the most important driver of disease. CTE and exposure to repetitive head trauma is also associated with a variety of other neurodegenerations, including Alzheimer disease. In fact, amyloid β peptide deposition is altered and accelerated in CTE and is associated with worse disease. Here, we review the current exposure, clinical, and pathological associations of CTE.

  6. [Wernicke encephalopathy: Guiding thiamine prescription].

    Science.gov (United States)

    Boulanger, A S; Paquette, I; Létourneau, G; Richard-Devantoy, S

    2017-05-01

    Wernicke's encephalopathy (WE) is a medical emergency. The objective of this paper is to systematically review the literature published over the past 15 years pertaining to prophylactic and curative treatment of WE with thiamine. A systematic literature search was performed using Medline to include all studies published between January 1, 2000 and December 31, 2015. Of the 316 abstracts identified, 20 met the final inclusion criteria. The evidence on the use of prophylactic thiamine was quite heterogeneous. The use of thiamine in this context largely depended on the evaluation of an individual's risk of developing WE. Use of prophylactic thiamine in low-risk patients is not universally indicated. When prescribed in this sub-population, the oral route is suggested but may be insufficient owing to its limited intestinal absorption and the high risk of non-compliance. High-risk patients need parenteral treatment with a recommended posology of 250 mg daily for 3 to 5 days. Intramuscular route is preferred in the outpatient setting, whereas intravenous route is suggested for inpatients. In cases where the diagnosis of WE is suspected or confirmed, a curative treatment with high-dose IV thiamine is justified. The evidence widely accepted in the literature is much clearer in this condition, with treatment regimens consisting of 500 mg IV 3 times daily for 3 to 5 days, followed by 250 mg IV daily for a minimum of 3 to 5 additional days. The literature does indicate that thiamine should be prescribed at high dosages, with the parenteral routes indicated in hospital settings and in high-risk patients. Based on the current literature review, we suggest treatment algorithms guiding thiamine prescription for WE. Copyright © 2016 L'Encéphale, Paris. Published by Elsevier Masson SAS. All rights reserved.

  7. Acute Necrotizing Pancreatitis Associated with Vildagliptin

    OpenAIRE

    Purnima Kunjathaya; Pradeep Kakkadasam Ramaswami; Anupama Nagar Krishnamurthy; Naresh Bhat

    2013-01-01

    Context To report a case of acute necrotizing pancreatitis in a patient receiving vildagliptin. Case report A 49-year-old manpresented to us with severe abdominal pain and was diagnosed to have pancreatitis three weeks after the commencement of vildagliptin for the treatment of uncontrolled type 2 diabetes mellitus. His serum amylase was 2,215 U/L at admission, with contrast enhanced computed tomography (CECT) of the abdomen and pelvis showing features of acute pancreatitis. The patient had a...

  8. Sialometaplasia necrotizing in dog - Case report.

    Directory of Open Access Journals (Sweden)

    Suellen Ramos Barboza

    2016-09-01

    Full Text Available ABSTRACT. Barboza S.R., Braga L.S.F., Maestri L.F. de P., Monteiro B.S., Rassele A.C., Santos R.V., Vicente G. de C. & Gava M.G. [Sialometaplasia necrotizing in dog - Case report.] Sialometaplasia necrotizante em cão - Relato de caso. Revista Brasileira de Medicina Veterinária, 38(3:214-216, 2016. Setor de Clínica Médica de Animais de Pequeno Porte e Patologia Animal, Universidade Vila Velha, Avenida Comissário José Dantas de Melo, 21, Boa Vista, Vila Velha, ES 29102-920, Brasil. E-mail: suellenramosvet@hotmail.com The sialometaplasia is a rare disease of unknown cause in dogs and cats, which usually affects the submandibular salivary gland, caused by trauma, physical-chemical or biological attack in blood vessels that can produce ischemic changes, causing necrosis, scaly inflammation and metaplasia of glandular epithelium and ducts. Animals affected by sialometaplasia necrotizing show pain, an increase in salivary glands and regional lymphadenopathy. Ultrasonography associated with biopsy of the affected tissue are effective in diagnosing the disease. The treatment is surgical and symptomatic. In this study, we tried to report the case of a half-breed dog, 6 years old, who presented the symptoms and histopathological diagnosis of necrotizing sialometaplasia.

  9. Necrotizing Fasciitis in Paroxysmal Nocturnal Hemoglobinuria

    Directory of Open Access Journals (Sweden)

    Pusem Patir

    2015-01-01

    Full Text Available Paroxysmal nocturnal hemoglobinuria (PNH is a rare, progressive, and life-threatening hematopoietic stem cell disorder characterized by complement-mediated intravascular hemolysis and a prothrombotic state. Patients with PNH might have slightly increased risk of infections due to complement-associated defects subsequent to CD59 deficiency. Here, we report a rare case of a 65-year-old male patient with necrotic ulcers on both legs, where the recognition of pancytopenia and microthrombi led to the diagnosis of PNH based on FLAER (FLuorescent AERolysin flow cytometric analysis. He was subsequently started on eculizumab therapy, with starting and maintenance doses set as per drug labelling. Progression of the patient’s leg ulcers during follow-up, with fulminant tissue destruction, purulent discharge, and necrotic patches, led to a later diagnosis of necrotizing fasciitis due to Pseudomonas aeruginosa and Klebsiella pneumonia infection. Courses of broad-spectrum antibiotics, surgical debridement, and superficial skin grafting were applied with successful effect during ongoing eculizumab therapy. This case highlights the point that it is important to maintain treatment of underlying disorders such as PNH in the presence of life-threatening infections like NF.

  10. Meningoencefalite necrotizante de cão Maltês Necrotizing meningoencephalitis of Maltese dog

    Directory of Open Access Journals (Sweden)

    Kalan Bastos Violin

    2008-06-01

    Full Text Available A Meningoencefalite Necrotizante (MEN é uma encefalopatia causada por uma disfunção inflamatória de característica necrotizante. O objetivo deste relato é descrever os aspectos clínicos e anatomopatológicos da Meningoencefalite Necrotizante (MEN em um cão Maltês. A doença tem um caráter necrótico único e está relacionada intimamente à Encefalite do Cão Pug (ECP devido a suas semelhanças, bem como à Leucoencefalite Necrotizante (LEN. Embora o primeiro relato de caso de ECP tenha mais de 15 anos e o primeiro relato de caso de MEN em Maltês tenha 11 anos, há muito a ser revelado sobre a etiologia e os mecanismos imunopatológicos da doença. Neste trabalho, relata-se o caso de um cão Maltês com sinais que foram compatíveis com a MEN. Foram detectadas nas imagens macroscópicas, cavitação cerebral, e na microscopia, perda de células do parênquima em certas regiões do córtex cerebral. A partir dessas descobertas descreve-se o primeiro caso de MEN em cão Maltês no Brasil.The Necrotizing Meningoencephalitis (NME is an encephalopathy caused by an inflammatory dysfunction with necrotic characterization. The aim of this report is to describe the anatomopathological features of the NME in a Maltese dog. The disease has a unique necrotic pattern and is closely related to Pug Dog Encephalitis (PDE because of their similarity as well as to Necrotizing Leukoencephalitis (NLE. Although the first PDE report has more than 15 years and the first Maltese NME report has 11 years there is a lot to be unveiled about the etiologic and the immunopathologic mechanisms of the disease. Here we report one case of a Maltese dog with signs that were compatible with NME. The gross morphology pictures with the cerebral cavitation and the histological loss of parenchymal cells in some regions of the cerebral cortex were detected. Based on these findings, we describe the first case of NME in Maltese dog in Brazil.

  11. Subacute sclerosing panencephalitis presenting as neuromyelitis optica.

    Science.gov (United States)

    Raut, Tushar Premraj; Singh, Maneesh Kumar; Garg, Ravindra Kumar; Naphade, Pravin Umakant

    2012-12-14

    Subacute sclerosing panencephalitis (SSPE) is a slowly progressing inflammatory and degenerative disorder of the brain caused by a mutant measles virus. The diagnosis of SSPE is based on characteristic clinical and EEG findings (periodic complexes) and demonstration of elevated antibody titres against measles in cerebrospinal fluid. SSPE can have atypical clinical features at the onset. The authors here report a case of a 3-year-old child who presented with vision loss followed 15 months later by quadriparesis with bladder involvement. These clinical features resembled that of neuromyelitis optica. However, as the disease progressed, appearance of myoclonic jerks, periodic discharges on EEG and positive cerebrospinal fluid serology for measles led to the final diagnosis of SSPE.

  12. Basal ganglia lesions in subacute sclerosing panencephalitis

    Directory of Open Access Journals (Sweden)

    Kelson James Almeida

    Full Text Available ABSTRACT The parieto-occipital region of the brain is the most frequently and severely affected in subacute sclerosing panencephalitis (SSPE. The basal ganglia, cerebellum and corpus callosum are less commonly involved. We describe a patient with SSPE confirmed by neuropathology based on brain magnetic resonance imaging showing extensive basal ganglia involvement and no significant involvement of other cortical structures. Though rarely described in SSPE, clinicians should be aware of this involvement. SSPE should be kept in mind when changes in basal ganglia signal are seen on brain magnetic resonance imaging with or without involvement of other regions of the human brain to avoid erroneous etiological diagnosis of other pathologies causing rapidly progressive dementia.

  13. Subacute Sclerosing Panencephalitis Presenting with Hemidystonia

    Directory of Open Access Journals (Sweden)

    Hepsen Mine Serin

    2014-06-01

    Full Text Available In this paper, we present a case of subacute sclerosing panencephalitis (SSPE in an 11-year-old boy who presented with hemidystonia. Electroencephalogram (EEG revealed periodic epileptiform discharges which did not disappear with diazepam induction. His cranial magnetic resonance imaging was normal. SSPE diagnosis was considered and it was confirmed with the identification of measles antibodies in cerebrospinal fluid. SSPE is a progressive disease. Hemidystonia is not an expected presentation of SSPE. We aimed to emphasize that SSPE may present with different clinical findings such as hemidystonia. (The Me­di­cal Bul­le­tin of Ha­se­ki 2014; 52: 137-9

  14. Subacute sclerosing panencephalitis and chronic viral encephalitis.

    Science.gov (United States)

    Anlar, Banu

    2013-01-01

    Subacute sclerosing panencephalitis (SSPE) is a chronic infection of the central nervous system associated with the presence of mutant measles virus in the brain. It presents as a progressive, usually fatal disease. The diagnosis is based on clinical criteria and an elevated titer of measles antibodies in the cerebrospinal fluid (CSF). Electroencephalography and imaging studies provide supportive laboratory data. A brain biopsy is indicated only when CSF serology is negative or equivocal in a suspected case to assess the presence of inclusion bodies, measles virus antigens, or viral RNA. Among many drugs and methods tried in the treatment, the highest rate of stabilization or improvement was obtained with intraventricular human lymphoblastoid interferon-α and oral inosiplex. Further research for more available and efficient therapeutic regimens is warranted. Measles and SSPE are preventable by maintenance of high rates of immunization in the population. Copyright © 2013 Elsevier B.V. All rights reserved.

  15. Subacute cutaneous lupus erythematosus presenting as poikiloderma.

    LENUS (Irish Health Repository)

    Hughes, R

    2012-02-01

    Subacute cutaneous lupus erythematosus (SCLE) is a recognised variant of lupus erythematosus (LE), which accounts for 10-15% of all cases of cutaneous LE, occurring most commonly in young to middle-aged white women. Diagnosis is based on the detection of anti-Ro\\/SS-A antibodies in the skin and serum, characteristic clinical and histological cutaneous involvement, and relatively mild systemic involvement. Several unusual variants of SCLE have been reported including erythrodermic SCLE, SCLE with vitiligo-like lesions, acral SCLE and bullous SCLE. Poikoilodermatous SCLE is a recognised but rare variant of SCLE. There are currently only two case reports, comprising five individual cases, in the literature. We present a case of SCLE in which the main clinical findings were an extensive photodistributed poikilodermatous rash and alopecia.

  16. Subacute transverse myelitis with Lyme profile dissociation

    Directory of Open Access Journals (Sweden)

    Ajjan, Mohammed

    2008-06-01

    Full Text Available Introduction: Transverse myelitis is a very rare neurologic syndrome with an incidence per year of 1-5 per million population. We are presenting an interesting case of subacute transverse myelitis with its MRI (magnetic resonance imaging and CSF (cerebrospinal fluid findings. Case: A 46-year-old African-American woman presented with decreased sensation in the lower extremities which started three weeks ago when she had a 36-hour episode of sore throat. She reported numbness up to the level just below the breasts. Lyme disease antibodies total IgG (immunoglobulin G and IgM (immunoglobulin M in the blood was positive. Antinuclear antibody profile was within normal limits. MRI of the cervical spine showed swelling in the lower cervical cord with contrast enhancement. Cerebrospinal fluid was clear with negative Borrelia Burgdorferi IgG and IgM. Herpes simplex, mycoplasma, coxiella, anaplasma, cryptococcus and hepatitis B were all negative. No oligoclonal bands were detected. Quick improvement ensued after she was given IV Ceftriaxone for 7 days. The patient was discharged on the 8th day in stable condition. She continued on doxycycline for 21 days. Conclusions: Transverse myelitis should be included in the differential diagnosis of any patient presenting with acute or subacute myelopathy in association with localized contrast enhancement in the spinal cord especially if flu-like prodromal symptoms were reported. Lyme disease serology is indicated in patients with neurological symptoms keeping in mind that dissociation in Lyme antibody titers between the blood and the CSF is possible.

  17. Subacute Thyroiditis Developed in Identical Twins Two Years Apart

    National Research Council Canada - National Science Library

    HAMAGUCHI, Erika; NISHIMURA, Yasuyuki; KANEKO, Shuichi; TAKAMURA, Toshinari

    2005-01-01

    .... Subacute thyroiditis was diagnosed and prednisone was administered. Two years later, her identical twin sister, who lives separately, was referred to our hospital because of neck pain, low-grade fever, and palpitation...

  18. Subacute Sclerosing Panencephalitis of the Brainstem as a Clinical Entity

    National Research Council Canada - National Science Library

    Pavan S. Upadhyayula; Jason Yang; John K. Yue; Joseph D. Ciacci

    2017-01-01

    Subacute sclerosing panencephalitis (SSPE) is a rare progressive neurological disorder of early adolescence caused by persistent infection of the measles virus, which remains prevalent worldwide despite an effective vaccine...

  19. Angiosarcoma of the Heart Presenting as Subacute Pericarditis.

    Science.gov (United States)

    Joseph, Alexandra L; Wilklow, Frank E; Olivier, John J; Joseph, Georges J; Glancy, David Luke

    2017-03-15

    A young woman, who presented with what appeared to be subacute pericarditis, was found to have primary angiosarcoma of the heart, a condition that is nearly always fatal regardless of the therapy. Copyright © 2016 Elsevier Inc. All rights reserved.

  20. Necrotizing sialometaplasia of the palate. Ulcerative or necrotizing stage of leukokeratosis nicotina palati?

    Science.gov (United States)

    Philipsen, H P; Petersen, J K; Simonsen, B H

    1976-12-01

    A typical case of the recently described tumor-suspect lesion, necrotizing sialometaplasia (NS) of the palate, in a 54-year old Caucasian male is presented. Results of complete blood- and urinanalysis including serum electrophoresis and labial salivary gland biopsy strongly pointed at a local etiologic factor. Previous statements that the disease represents a new entity are questioned. The present authors favor the idea that NS is the necrotizing (ulcerative) or terminal stage of leukokeratosis nicotina palati (nicotinic stomatitis). It is of particular importance that this lesion is not diagnosed as a malignancy, as it heals spontaneously and uneventfully.

  1. Subacute cannabinoid treatment: anticonvulsant activity and withdrawal excitability in mice.

    OpenAIRE

    Karler, R.; Turkanis, S. A.

    1980-01-01

    1 The effects of subacute treatment with cannabidiol, delta 9-tetrahydrocannabinol (delta 9-THC), phenytoin and phenobarbitone on anticonvulsant activity and on withdrawal excitability in mice were compared in three electrically induced seizure-threshold tests. 2 In the maximal electroshock-threshold test, subacute treatment did not alter the anticonvulsant activity of cannabidiol, phenytoin or phenobarbitone, but tolerance developed to delta 9-THC. 3 In the 60 Hz electroshock-threshold test,...

  2. Subacute sclerosing panencephalitis: brain stem involvement in a peculiar pattern

    Energy Technology Data Exchange (ETDEWEB)

    Senol, U. [Akdeniz University, Antalya (Turkey). Faculty of Medicine; Haspolat, S. [Akdeniz University, Antalya (Turkey). Dept. of Child Neurology; Cevikol, C. [Akdeniz University, Antalya (Turkey). Dept. of Radiodiagnostics; Saatci, I. [Hacettepe University (Turkey). Medical Faculty

    2000-12-01

    The most common pattern in subacute sclerosing panencephalitis, is in the cerebral hemisphere white matter on T2-weighted images with or without atrophy. Brain-stem lesions are rare. We report brain-stem involvement in two children with subacute sclerosing panencephalitis. A peculiar pattern, with involvement of the pons with extension to both middle cerebellar peduncles and substantia nigra but sparing the pontine tegmentum, is suggested. (orig.)

  3. Qualifying and quantifying minimal hepatic encephalopathy

    DEFF Research Database (Denmark)

    Morgan, Marsha Y; Amodio, Piero; Cook, Nicola A

    2016-01-01

    Minimal hepatic encephalopathy is the term applied to the neuropsychiatric status of patients with cirrhosis who are unimpaired on clinical examination but show alterations in neuropsychological tests exploring psychomotor speed/executive function and/or in neurophysiological variables. There is ......Minimal hepatic encephalopathy is the term applied to the neuropsychiatric status of patients with cirrhosis who are unimpaired on clinical examination but show alterations in neuropsychological tests exploring psychomotor speed/executive function and/or in neurophysiological variables...... analytical techniques may provide better diagnostic information while the advent of portable wireless headsets may facilitate more widespread use. A large number of other diagnostic tools have been validated for the diagnosis of minimal hepatic encephalopathy including Critical Flicker Frequency......, the Inhibitory Control Test, the Stroop test, the Scan package and the Continuous Reaction Time; each has its pros and cons; strengths and weaknesses; protagonists and detractors. Recent AASLD/EASL Practice Guidelines suggest that the diagnosis of minimal hepatic encephalopathy should be based on the PHES test...

  4. PRIONS AND THE TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHIES

    Science.gov (United States)

    This book chapter is an invited, scholarly review of the mechanism(s) of TSEs for the 2nd edition of Metabolic Encephalopathies. Each chapter in the book assumes a professional knowledge of neuroscience and biochemistry, and the focus of the book is on the metabolic basis of dise...

  5. Pathogenesis of bovine spongiform encephalopathy in sheep

    NARCIS (Netherlands)

    Keulen, van L.J.M.; Vromans, M.E.W.; Dolstra, C.H.; Bossers, A.; Zijderveld, van F.G.

    2008-01-01

    The pathogenesis of bovine spongiform encephalopathy (BSE) in sheep was studied by immunohistochemical detection of scrapie-associated prion protein (PrPSc) in the gastrointestinal, lymphoid and neural tissues following oral inoculation with BSE brain homogenate. First accumulation of PrPSc was

  6. Wernicke's Encephalopathy in a Nigerian with Schizophrenia ...

    African Journals Online (AJOL)

    While Wernicke's encephalopathy (WE) is a well-characterized syndrome in alcoholism and malnutrition, little is written of its prevalence or presentation in patients with psychiatric illness. We present a case of a 37-year-old Nigerian male with schizophrenia and malnutrition who presented with delirium and ophthalmoplegia ...

  7. Autopsy prevalence of Wernicke's encephalopathy in alcohol ...

    African Journals Online (AJOL)

    Autopsy prevalence of Wernicke's encephalopathy in alcohol-related disease. ... The histological lesions were classified as either acute (5l, acute on chronic (9) or chronic (3) according to defined pathological criteria Macroscopic abnormalities were not obvious in any of the patients in the study group. Chart analysis ...

  8. Necrotizing fasciitis developing from a brown recluse spider bite.

    Science.gov (United States)

    Majeski, J

    2001-02-01

    A 20-year retrospective case series was analyzed to identify the brown recluse spider bite as a cause of necrotizing fasciitis. Data from 31 consecutive patients with necrotizing fasciitis were analyzed. Of the 31 patients with necrotizing fasciitis a brown recluse spider bite was found to be the initial cause in two patients. Both patients with spider bites delayed in obtaining medical treatment, and secondary infection of the necrotic tissue occurred. One patient was diagnosed by frozen section tissue biopsy, and the second patient was diagnosed by clinical examination. All patients in this series had immediate aggressive operative debridement. Both patients survived with functional limbs. There were no deaths in this large series. Necrotizing fasciitis can be caused by a secondarily infected brown recluse spider bite. Successful treatment of necrotizing fasciitis from any cause is associated with early diagnosis, immediate surgical debridement, and supplemental enteral or parenteral nutrition.

  9. Serial diffusion-weighted imaging in subacute sclerosing panencephalitis.

    Science.gov (United States)

    Kanemura, Hideaki; Aihara, Masao

    2008-06-01

    Subacute sclerosing panencephalitis may be associated with clinical features of frontal lobe dysfunction. We previously reported that frontal lobe volume falls significantly as clinical stage progresses, using three-dimensional magnetic resonance imaging-based brain volumetry. The hypothesis that frontal volume increases correlate with clinical improvement, however, was not tested in our previous study. Therefore, we reevaluated our patient with subacute sclerosing panencephalitis, to determine whether apparent diffusion coefficient maps can characterize the clinical course of subacute sclerosing panencephalitis. We studied an 8-year-old boy with subacute sclerosing panencephalitis, using serial diffusion-weighted imaging magnetic resonance imaging, and measured the regional apparent diffusion coefficient. The regional apparent diffusion coefficient of the frontal lobe decreased significantly with clinical progression, whereas it increased to within normal range during clinical improvements. The apparent diffusion coefficient of the other regions did not change. These results suggest that the clinical signs of patients with subacute sclerosing panencephalitis are attributable to frontal lobe dysfunction, and that apparent diffusion coefficient measurements may be useful in predicting the clinical course of subacute sclerosing panencephalitis.

  10. Necrotizing Fasciitis of vulva: A report of two cases

    Directory of Open Access Journals (Sweden)

    Jamal A

    2000-08-01

    Full Text Available Vulvar necrotizing fascitis is an uncommon infectious disorder. Since the first reported cases almost 100 years, ago, necrotizing fasciitis continues to present a diagnostic and therapeutic challenge. What usually begins as a subtle infection can become life-threatening. We report two cases of vulvar necrotizing fasciitis, one after posterior colporrhaphy in a woman with four risk factors and the other in a young woman without any risk factor.

  11. Wernicke’s encephalopathy following hyperemesis gravidarum

    Directory of Open Access Journals (Sweden)

    Leila Pourali

    2016-06-01

    Full Text Available Background: ″Wernicke’s Korsakoff″ syndrome is the most important complication of severe thiamine deficiency. The term refers to two different syndromes, each representing a different stage of the disease. Wernicke’s encephalopathy (WE is an acute syndrome requiring emergent treatment to prevent death and neurologic morbidity. Korsakoff syndrome (KS refers to a chronic neurologic condition that usually occurs as a consequence of WE. It is a rare complication of hyperemesis gravidarum that confusion, ocular signs, and gait ataxia are the most prevalent symptoms, respectively. Typical brain lesions of wernicke’s encephalopathy (WE are observed at autopsy in 0.4 to 2.8 percent of the general population in the western world and the majority of affected patients are alcoholic. The prevalence of wernicke’s encephalopathy lesions seen on autopsy was 12.5% of alcohol abusers in one report. Among those who with alcohol-related death, it has been reported to be even higher, 29 to 59%. The aim of this study was to report a case of wernicke’s encephalopathy following hyperemesis gravidarum. Case Presentation: A 28-year-old-pregnant woman in 19th weeks of gestation referred to the hospital with hyperemesis, gait ataxia, and dysarthria before that she had hyperemesis gravidarum with weight loss and unresponsive to outpatient and inpatient medical therapy. MRI showed hyperdense lesion around thalamus which was characteristic of wernicke’s encephalopathy. Rapid improvement in patient’s condition occurred after high dose thiamine infusion. Conclusion: In hyperemesis gravidarum, presence of either symptoms of ocular or mental disorder or ataxia must be considered to rule out and appropriate treatment of Wernicke’s syndrome which can cause maternal and fetal death.

  12. Fatal necrotizing fasciitis due to necrotic toxin-producing Escherichia coli strain

    Directory of Open Access Journals (Sweden)

    C. Gallois

    2015-11-01

    Full Text Available We report a fatal case of necrotizing soft tissues infection caused by an Escherichia coli strain belonging to phylogenetic group C and harbouring numerous virulence factors reported to be part of a pathogenicity island (PAI such as PAI IIJ96 and conserved virulence plasmidic region.

  13. Growth of Necrotic Cores in Vulnerable Plaque

    Science.gov (United States)

    Fok, Pak-Wing

    2011-03-01

    Plaques are fatty deposits that grow mainly in arteries and develop as a result of a chronic inflammatory response. Plaques are called vulnerable when they are prone to mechanical rupture. Vulnerable Plaques (VPs) are characterized by lipid-rich, necrotic cores that are heavily infiltrated with macrophages. The rupture of VPs releases thrombogenic agents into the bloodstream, usually resulting in myocardial infarctions. We propose a quantitative model to predict the development of a plaque's necrotic core. By solving coupled reaction-diffusion equations for macrophages and dead cells, we explore the joint effects of hypoxic cell death and chemo-attraction to Ox-LDL, a molecule that is strongly linked to atherosclerosis. Our model predicts cores that have approximately the right size and shape. Normal mode analysis and subsequent calculation of the smallest eigenvalues allow us to compute the times required for the system to reach its steady state. This study allows us to make quantitative predictions for how quickly vulnerable plaques develop and how their growth depends on system parameters such as chemotactic coefficients and cell death rates.

  14. Candesartan mediates microcirculation in acute necrotizing pancreatitis.

    Science.gov (United States)

    Bostanci, H; Sahin, T T; Dikmen, K; Dikmen, A U; Yuksel, O; Gulbahar, O; Poyraz, A; Tekin, E

    2015-01-01

    In the present study we aimed to determine the effect of an AT-II antagonist candesartan on pancreatic microcirculation in an experimental model of acute necrotizing pancreatitis. There were five study groups with 10 animals in each. Pancreatitis was induced by intravenous infusion of cerulein and coadministration of glycodeoxycholate into biliopancreatic canal. Candesartan is given at 6th and 18th hour to the 24th and 48th hour groups, respectively. At 24th and 48th hours; following anaesthesia laparotomy was performed and laser Doppler flowmetry was performed in the pancreatic tissue of the animals. Following scarification blood samples were obtained for amylase, myeloperoxidase, IL-6 and tumour necrosis factor alpha. Tissue samples from the pancreas were obtained for histopathological analysis, endothelial cell apoptosis (TUNEL assay) and matrix metalloproteinase-9 immunohistochemistry. Pancreatic microcirculation was higher in the candesartan treated groups (p candesartan treated groups (p candesartan treated groups (p 0.05). Tissue matrix metalloproteinase -9 levels were found to be reduced with candesartan treatment (p candesartan in the early phases of acute necrotizing pancreatitis effective on microcirculation of pancreatic tissue (Tab. 3, Fig. 6, Ref. 28).

  15. Subacute toxicity assessment of annatto in rat.

    Science.gov (United States)

    Bautista, A R P L; Moreira, E L T; Batista, M S; Miranda, M S; Gomes, I C S

    2004-04-01

    Increased human use of annatto (Bixa orellana L), a red yellow food colorant, demands generation of toxicity data. The toxic effects of annatto powder (bixin 27%) have been assessed following administration of a subacute regimen (4 weeks, 20 doses) in Wistar male and female rats. A full study with three dose levels was considered unnecessary since no sign of toxicity had been noted in a preliminary experiment with 1000 mg/kg body weight/day as was recommended by the OECD guideline. In this study, annatto administered by gavage at a dose level of 2000 mg/kg/day decreased male body weight gain, but had no effect on either food intake or food conversion efficiency. Haematological and plasma biochemical examination as well necropsy performed at the end of administration (29th day) and observation (43rd day) periods revealed no alterations related with annatto administration. Kidney apoptosis occurred in 20% treated female rats in restricted areas without proliferation or tubular segments modification. The precise nature of apoptosis was not investigated in the present study. These findings suggest that annatto was no toxic to the rat.

  16. Subacute sclerosing panencephalitis: clinical and demographic characteristics.

    Science.gov (United States)

    Rafique, Arshad; Amjad, Nida; Chand, Prem; Zaidi, Syed Sohail Zahoor; Rana, Muhammad Suleman; Ahmed, Khalid; Ibrahim, Shahnaz

    2014-08-01

    To determine the clinical and demographic characteristics of children diagnosed with Subacute sclerosing panencephalitis (SSPE). Case series. The Aga Khan University Hospital, Karachi, from January 2000 to June 2012. A retrospective analysis was done, regarding medical charts of 43 children under the age of 16 years with a discharge diagnosis of SSPE. Demographic and clinical characteristics were recorded. RESULTS were expressed as percentages. Most of the 43 patients were male (72%). The average age at presentation was 8.7 years with average duration of symptoms being 100.6 days. History of measles was present in 17 patients (39.5%). All children had seizures at presentation and 65% had cognitive impairment. Most patients required poly therapy for control of seizures. Sodium valproate was the most commonly used anti-epileptic agent; Isoprinosine was tried in 22 (51%) patients. CSF for antimeasles antibodies was positive in approximately 86% of the 40 (93%) children. EEG showed burst suppression pattern in 36 (83.7%) cases. Forty-two patients (97.6%) were discharged home in a vegetative state. SSPE is progressive neurodegenerative disorder. It can be prevented by timely immunization against measles. Measles antibody in the CSF is diagnostic for SSPE and is helpful in early diagnosis. Most patients experience a gradual but progressive decline in motor and cognitive functions.

  17. Subacute posttraumatic ascending myelopathy: a literature review.

    Science.gov (United States)

    Zhang, J; Wang, G

    2017-07-01

    A literature review. Our aim was to summarise the history, epidemiology, aetiological mechanism, pathological study, clinical and radiological evaluation, treatment and prognosis of subacute posttraumatic ascending myelopathy (SPAM). Medical literature on SPAM were searched in the PubMed, Medline, Ovid and Embase databases. The cases of SPAM reported in literature were analysed, and the history, epidemiology, aetiological mechanism, pathological study, clinical and radiological evaluation, treatment and prognosis of SPAM were summarised. SPAM remains a relatively rare disorder occurring within the first few weeks after spinal cord injury (SCI). The incidence rate ranges from 0.42% to 1% among all SCI. SPAM is likely to occur in young and middle-aged male patients. Risk factors of SPAM include complete injury, low blood pressure, early postoperative mobilisation and nonsurgical treatment. SPAM is well recognised according to typical clinical manifestation and magnetic resonance imaging characteristics. There is no effective therapy for this neurological deterioration. The prognosis of SPAM is poor. There is usually a slight improvement of one or more level(s) below the maximal level of deterioration. The mortality rate is approximately 10%.

  18. Necrotizing fasciitis in association with Ludwig's angina - A case report.

    Science.gov (United States)

    Kavarodi, A M

    2011-07-01

    A 28 year old male diabetic patient developed Ludwig's angina which subsequently evolved into cervicofacial necrotizing fasciitis. The differential characteristic of Ludwig's angina and cervicofacial necrotizing fasciitis, as it relates to this rare presentation is discussed. The clinical and radiological features, pathophysiology, diagnosis and the management that resulted in a successful outcome are presented.

  19. Cervical necrotizing fasciitis: A potentially fatal disease with varied ...

    African Journals Online (AJOL)

    Necrotizing fasciitis was recognized centuries ago by physicians. It is a rapidly progressive and potentially fatal soft‑tissue infection that is typified by soft‑tissue necrosis, especially affecting the subcutaneous tissues and fascia. Cervico‑facial necrotizing fasciitis is said to be uncommon, but when it occurs, it is often of ...

  20. la sialometaplasie necrosante necrotizing sialometaplasia of the plate

    African Journals Online (AJOL)

    It is often confused clinically and histopathologically with malignancies, such as squamous cell carcinoma or mucoepidermoid carcinoma. We report a case of NS in ... Keywords: Necrotizing sialometaplasia; oral; palate; histology. CAS CLINIQUE .... Necrotizing sialometaplasia of the nasal cavity. Am J Oto- laryngol. 1982 ...

  1. Sodium valproate-related hyperammonaemic encephalopathy.

    Science.gov (United States)

    Pegg, Emily Jane; Zaman, Fawad

    2014-04-10

    A 59-year-old man with a background of poststroke epilepsy, lung cancer, chronic obstructive pulmonary disease and hypertension, presented to the medical assessment unit with acute confusion and altered consciousness. Medications included sodium valproate, aspirin and antihypertensives. On examination he was confused, with his Glasgow Coma Scale fluctuating between 10 and 14. Routine blood tests, thyroid function tests, serum sodium valproate level, urine dip, CT of the brain and cerebrospinal fluid analysis were all normal. EEG revealed changes consistent with an encephalopathic process. Serum ammonia was elevated (75 µg/dL), consistent with a diagnosis of valproate-related hyperammonaemic encephalopathy. Sodium valproate was changed to a different antiepileptic drug and his confusion gradually resolved. Valproate-related hyperammonaemic encephalopathy is a treatable condition which should be considered as a diagnosis in anyone taking sodium valproate with new onset confusion, even in the presence of therapeutic sodium valproate levels and normal liver function tests.

  2. Nonconvulsive Status Epilepticus in Hepatic Encephalopathy

    Directory of Open Access Journals (Sweden)

    Hyung Kim

    2011-05-01

    Full Text Available We discuss a case of a 64-year-old male with a history of liver failure presenting with altered mental status, initially diagnosed with hepatic encephalopathy but ultimately diagnosed with nonconvulsive status epilepticus (NCSE by electroencephalogram (EEG. NCSE is a difficult diagnosis to make, given no clear consensus on diagnostic criteria. Especially in the intensive care unit setting of persistent altered mental status with no clear etiology, NCSE must be considered in the differential diagnosis, as the consequences of delayed diagnosis and treatment can be substantial. EEG can be useful in the evaluation of patients with hepatic encephalopathy who have persistently altered levels of consciousness despite optimal medical management. [West J Emerg Med. 2011;12(4:372–374.

  3. Molecular & Genetic Investigation of Tau in Chronic Traumatic Encephalopathy

    Science.gov (United States)

    2015-10-01

    AWARD NUMBER: W81XWH-14-1-0399 TITLE: Molecular & Genetic Investigation of Tau in Chronic Traumatic Encephalopathy PRINCIPAL INVESTIGATOR: John F...Include area code) October 2015 Annual Report 30 Sep 2014 - 29 Sep 2015 Molecular & Genetic Investigation of Tau in Chronic Traumatic Encephalopathy John...available, work will commence. Tau, genetics, susceptibility, MAPT, chronic traumatic encephalopathy , Alzheimer disease U U U U 1 USAMRMC Table of

  4. MINIM AL HEPATIC ENCEPHALOPATHY IN AL COHOLIC CIRRHOSIS

    OpenAIRE

    Kavya; Jegan Niwas; Sarah; Rajasekaran

    2015-01-01

    BACKGROUND : Minimal hepatic Encephalopathy (MHE) has severe and important health implications which affects the quality of life as well as the survival of patients with liver disease. Psychometric hepatic encephalopathy score (PHES) has been validated for diagnosis of MHE. AIM OF THE STUDY : To detect the prevalence of minimal hepatic encephalopathy (MHE) in alcoholic cirrhosis patients and to compare the patterns of alcohol consumption in patients with MHE t...

  5. 'Khatatonia' - cathinone-induced hypertensive encephalopathy.

    Science.gov (United States)

    Bede, P; El-Kininy, N; O'Hara, F; Menon, P; Finegan, E; Healy, D

    2017-12-01

    Khat consumption is an under-recognised cause of hypertensive encephalopathy and intraparenchymal brain haemorrhage. We report the radiological findings of extensive periventricular, subcortical and brain stem white matter pathology of a patient who had consumed excessive amounts of Khat. The Khat plant contains cathinone, an amphetamine-like alkaloid which has been associated with chronic hypertensive end-organ damage, but is seldom considered a cause of cerebrovascular events in northern Europe.

  6. Wernicke's encephalopathy induced by hyperemesis gravidarum

    Science.gov (United States)

    Palacios-Marqués, Ana; Delgado-García, Silvia; Martín-Bayón, Tina; Martínez-Escoriza, Juan Carlos

    2012-01-01

    Wernicke's encephalopathy (WE) is a reversible neurological emergency caused by thiamine deficiency. Prolonged vomiting in pregnancy results in thiamine depletion. The early recognition of its clinical signs and symptoms is essential to establish the suspected diagnosis and can be confirmed by MRI. Prompt administration of thiamine is important for preventing the occurrence of sequelae in the mother and for improving the fetal prognostic. We report a case of WE induced by hyperemesis gravidarum with a good maternal and fetal outcome. PMID:22684836

  7. Wernicke's Encephalopathy Complicating Hyperemesis during Pregnancy

    OpenAIRE

    Mohamed Adnane Berdai; Smael Labib; Mustapha Harandou

    2016-01-01

    Wernicke’s encephalopathy is caused by severe thiamine deficiency; it is mostly observed in alcoholic patients. We report the case of a 28-year-old woman, at 17 weeks of gestational age, with severe hyperemesis gravidarum. She presented with disturbance of consciousness, nystagmus, ophthalmoplegia, and ataxia. The resonance magnetic imagery showed bilaterally symmetrical hyperintensities of thalamus and periaqueductal area. The case was managed with very large doses of thiamine. The diagnosis...

  8. Vitamin-Responsive Epileptic Encephalopathies in Children

    Directory of Open Access Journals (Sweden)

    Satish Agadi

    2013-01-01

    Full Text Available Untreated epileptic encephalopathies in children may potentially have disastrous outcomes. Treatment with antiepileptic drugs (AEDs often may not control the seizures, and even if they do, this measure is only symptomatic and not specific. It is especially valuable to identify potential underlying conditions that have specific treatments. Only a few conditions have definitive treatments that can potentially modify the natural course of disease. In this paper, we discuss the few such conditions that are responsive to vitamin or vitamin derivatives.

  9. Vitamin-Responsive Epileptic Encephalopathies in Children

    OpenAIRE

    Satish Agadi; Quach, Michael M.; Zulfi Haneef

    2013-01-01

    Untreated epileptic encephalopathies in children may potentially have disastrous outcomes. Treatment with antiepileptic drugs (AEDs) often may not control the seizures, and even if they do, this measure is only symptomatic and not specific. It is especially valuable to identify potential underlying conditions that have specific treatments. Only a few conditions have definitive treatments that can potentially modify the natural course of disease. In this paper, we discuss the few such conditio...

  10. COMPLEX THERAPY FOR HYPERTENSIVE AND MIXED ENCEPHALOPATHY

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    Sof'ya Alekseevna Rumyantseva

    2009-01-01

    Full Text Available Arterial hypertension (AH is one of the main causes of the occurrence and progression of different types of vascular pathology. AH-associated functional and morphological impairments of the brain are the severe symptom complexes of hypertensive encephalopathy (HE, which require continuous correction. The measures for the prevention and treatment of all cardiovascular diseases, including HE, involve adequate correction of AH, correction of energy neuronal homeostatic disorders, as well as a harmonious combination of psychotherapeutic and pharmacological exposures

  11. Norovírus Associated Encephalopathy

    OpenAIRE

    Salva, I; Brito, MJ; Farela Neves, J

    2011-01-01

    clinical presentation is self limited. It is classified into five groups (genogroups I through V). There are numerous reports of neurologic complications, namely afebrile seizures, but only two reports of associated encephalopathy. Case Report: A 12 month old girl with previous history of a pneumonia treated with amoxicillin-clavulanic acid and clarythromycin, presented in our emergency department with strabismus, ataxia for 3 days, later associated with vomiting and diarrhea. On admission...

  12. Hemorrhagic Encephalopathy From Acute Baking Soda Ingestion

    Directory of Open Access Journals (Sweden)

    Adrienne Hughes

    2016-09-01

    Full Text Available Baking soda is a readily available household product composed of sodium bicarbonate. It can be used as a home remedy to treat dyspepsia. If used in excessive amounts, baking soda has the potential to cause a variety of serious metabolic abnormalities. We believe this is the first reported case of hemorrhagic encephalopathy induced by baking soda ingestion. Healthcare providers should be aware of the dangers of baking soda misuse and the associated adverse effects.

  13. Hemorrhagic Encephalopathy From Acute Baking Soda Ingestion

    OpenAIRE

    Adrienne Hughes; Alisha Brown; Matthew Valento

    2016-01-01

    Baking soda is a readily available household product composed of sodium bicarbonate. It can be used as a home remedy to treat dyspepsia. If used in excessive amounts, baking soda has the potential to cause a variety of serious metabolic abnormalities. We believe this is the first reported case of hemorrhagic encephalopathy induced by baking soda ingestion. Healthcare providers should be aware of the dangers of baking soda misuse and the associated adverse effects. [West J Emerg Med. 20XX;XX(X...

  14. Hemorrhagic Encephalopathy From Acute Baking Soda Ingestion.

    Science.gov (United States)

    Hughes, Adrienne; Brown, Alisha; Valento, Matthew

    2016-09-01

    Baking soda is a readily available household product composed of sodium bicarbonate. It can be used as a home remedy to treat dyspepsia. If used in excessive amounts, baking soda has the potential to cause a variety of serious metabolic abnormalities. We believe this is the first reported case of hemorrhagic encephalopathy induced by baking soda ingestion. Healthcare providers should be aware of the dangers of baking soda misuse and the associated adverse effects.

  15. Brain-aluminium concentration in dialysis encephalopathy.

    Science.gov (United States)

    McDermott, J R; Smith, A I; Ward, M K; Parkinson, I S; Kerr, D N

    1978-04-29

    Brain-aluminium concentrations were found to be significantly higher in 7 patients dying with dialysis encephalopathy (mean 15.9 microgram aluminium/g dry weight) than in 11 dialysed controls (4.4 microgram/g) and in 2 uraemic patients who were not dialysed (2.7 microgram/g). The grey matter from the patients with dialysis encephalopathy contained about three times as much aluminium as white matter. The results suggest that dialysis with untreated and/or softened tap-water (aluminium concentration 0.1-1.2 mg/1) makes the major contribution to brain-aluminium levels; dialysis with deionised water (aluminium concentration normally less than 0.02 mg/1) and intake of phosphate-binding AL(OH)3 gel are less important. Brain aluminium levels remain elevated for up to four years after restoration of good renal function by transplantation. The association of dialysis encephalopathy with high levels of aluminium in the brain and in the dialysis water emphasises the potential neurotoxicity of aluminium in man.

  16. Epileptic encephalopathies (including severe epilepsy syndromes).

    Science.gov (United States)

    Covanis, Athanasios

    2012-09-01

    Epileptic encephalopathies represent a group of devastating epileptic disorders that appear early in life and are characterized by pharmacoresistant generalized or focal seizures, persistent severe electroencephalography (EEG) abnormalities, and cognitive dysfunction or decline. The ictal and interictal epileptic discharges are age-specific and are the main etiologic factors causing cognitive deterioration. This is most obvious in the idiopathic group. In the symptomatic group, the most common causes are structural, congenital, or acquired and rarely some metabolic disorders. In certain cases, clinical and EEG abnormalities persist and may evolve from one type to another as the child grows older. Various factors trigger and sustain the underlying pathophysiologic process and the ongoing epileptic and epileptiform activity during the most critical periods of brain maturation, perpetuating their deleterious effect on the brain. Immune-mediated mechanisms may have a role, suggested by certain encephalopathies responding to immune-modulating treatments and by the finding of various autoimmune antibodies. The chance of a better cognitive outcome improves with early diagnosis and treatment that is appropriate and effective. Current antiepileptic drugs are, in general, not effective: we urgently need new trials in this very special epileptic category. This article briefly reviews the most common epileptic encephalopathies and analyzes the most important clinical issues. Wiley Periodicals, Inc. © 2012 International League Against Epilepsy.

  17. BLOOD BIOMARKERS FOR EVALUATION OF PERINATAL ENCEPHALOPATHY

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    Ernest Marshall Graham

    2016-07-01

    Full Text Available Recent research in identification of brain injury after trauma shows many possible blood biomarkers that may help identify the fetus and neonate with encephalopathy. Traumatic brain injury shares many common features with perinatal hypoxic-ischemic encephalopathy. Trauma has a hypoxic component, and one of the 1st physiologic consequences of moderate-severe traumatic brain injury is apnea. Trauma and hypoxia-ischemia initiate an excitotoxic cascade and free radical injury followed by the inflammatory cascade, producing injury in neurons, glial cells and white matter. Increased excitatory amino acids, lipid peroxidation products and alteration in microRNAs and inflammatory markers are common to both traumatic brain injury and perinatal encephalopathy. The blood-brain barrier is disrupted in both leading to egress of substances normally only found in the central nervous system. Brain exosomes may represent ideal biomarker containers, as RNA and protein transported within the vesicles are protected from enzymatic degradation. Evaluation of fetal or neonatal brain derived exosomes that cross the blood-brain barrier and circulate peripherally has been referred to as the liquid brain biopsy. A multiplex of serum biomarkers could improve upon the current imprecise methods of identifying fetal and neonatal brain injury such as fetal heart rate abnormalities, meconium, cord gases at delivery, and Apgar scores. Quantitative biomarker measurements of perinatal brain injury and recovery could lead to operative delivery only in the presence of significant fetal risk, triage to appropriate therapy after birth and measure the effectiveness of treatment.

  18. Covert Hepatic Encephalopathy: Can My Patient Drive?

    Science.gov (United States)

    Shaw, Jawaid; Bajaj, Jasmohan S

    2017-02-01

    Liver cirrhosis is a public health problem and hepatic encephalopathy is one of its main complications, which can be either overt meaning thereby evident and readily diagnosed, or covert/minimal (covert hepatic encephalopathy-CHE) needing psychometric testing for diagnosis. Patients with CHE hepatic encephalopathy have deficits in multiple domains including visuospatial assessment, attention, response inhibition, working memory, along with psychomotor speed to name a few areas. These patients have poor navigational skills, get fatigued easily, and demonstrate poor insight into their driving deficits. The combination of all these leads them to have poor driving skills leading to traffic violations and crashes as demonstrated not only on the simulation testing but also in real-life driving events. There are multiple psychometric tests for CHE testing but these are not easily available and there is no uniform consensus on the gold standard testing as of yet. It does not automatically connote that all patients who test positive on driving simulation testing are unfit to drive. The physicians are encouraged to take driving history from the patient and the caregivers on every encounter and focus their counseling efforts more on patients with recent history of traffic crashes, with abnormal simulation studies and history of alcohol cessation within last year. As physicians are not trained to determine fitness to drive, their approach toward CHE patients in regards to driving restrictions should be driven by ethical principles while as respecting the local laws.

  19. Subacute Sclerosing Panencephalitis in a Child with Human Immunodeficiency Virus Co-Infection

    OpenAIRE

    Maurya, Pradeep Kumar; Thakkar, Mayur Deepak; Kulshreshtha, Dinkar; Singh, Ajai Kumar; Thacker, Anup Kumar

    2016-01-01

    Subacute sclerosing panencephalitis is a fatal infectious disease of childhood caused by persistence of the measles virus in the brain. The effect of human immunodeficiency virus (HIV) co-infection on subacute sclerosing panencephalitis remains elusive and rare. We report a child who developed subacute sclerosing panencephalitis following a short latency period and a rapidly progressive course with HIV co-infection.

  20. Control methods for cattle feedstuffs aimed at prevention of Bovine spongiform encephalopathy (BSE

    Directory of Open Access Journals (Sweden)

    Nešić Ksenija

    2006-01-01

    Full Text Available In the course of the last decades of the twentieth century, more than 30 new diseases were determined for the first time in history. Bovine spongiform encephalopathy (BSE, or "mad cow disease" is one of them. The disease implies the subacute neurodegenerative transmission of spongiform encephalopathy and it was diagnosed and described for the first time in Great Britain in 1986. A theory has been established that BSE is spread through feedstuffs, more precisely, meat-bone flour which contains infective proteins of ruminants, and legislature has been passed throughout the world with the objective of preventing the entry of meat-bone flour into the food chain. The complete ban of the use of meat-bone flour for all farm animals (with the exception of fish flour for non-ruminants and an adequate thermal treatment in the production of meat-bone flour (133ºC, 3 bar, 20 min are the elements on which the European Union (EU legislature is based. The regulations in our country include a ban on the use of meat-bone flour in cattle feedstuffs and a ban on imports of beef proteins. The implementation of this legislature throughout the world requires the corresponding analytical means. At the present time, there are several available possibilities: optic microscopy, PCR, immunoprobes, spectroscopic methods, and several others which are still being examined for use for this purpose. All the analytical methods are being applied with the objective of controlling the implementation of the current regulations, but also in order to discover possible cross contamination that could take place in factories of animal feedstuffs, during transportation, storage, or on farms, in particular when there are no separate lines for feedstuffs that contains meat-bone flour and others in which even its traces are banned. In order to secure the successful control and prevention of bovine spongiform encephalopathy in our country, as well as to secure the unhindered continuation of

  1. Surgical management of necrotizing sialometaplasia of palate

    Directory of Open Access Journals (Sweden)

    S M Balaji

    2015-01-01

    Full Text Available Necrotizing sialometaplasia (NSM is a rare benign, inflammatory disease of both major and minor salivary glands, although more commonly reported in the minor glands of the palate. The characteristic clinical presentation can perplex the clinician and may be mistaken for a malignant neoplasm, such as mucoepidermoid carcinoma, as well as invasive squamous cell carcinoma. The clinical and histological similarity between this entity and a malignant lesion may result in unnecessary or mis-treatment. Though clinically mimics malignancy, NSM is considered to be a self-limiting disease, and takes about 3-12 weeks to resolve. Majority of the case resolves itself or by supportive and symptomatic treatment. Surgical intervention is rarely required in NSM except the diagnostic biopsy. Herein we report the clinical, histopathological feature and surgical management of a case of NSM of hard palate in a young adult male.

  2. Impact of probiotics on necrotizing enterocolitis.

    Science.gov (United States)

    Underwood, Mark A

    2017-02-01

    A large number of randomized placebo-controlled clinical trials and cohort studies have demonstrated a decrease in the incidence of necrotizing enterocolitis with administration of probiotic microbes. These studies have prompted many neonatologists to adopt routine prophylactic administration of probiotics while others await more definitive studies and/or probiotic products with demonstrated purity and stable numbers of live organisms. Cross-contamination and inadequate sample size limit the value of further traditional placebo-controlled randomized controlled trials. Key areas for future research include mechanisms of protection, optimum probiotic species or strains (or combinations thereof) and duration of treatment, interactions between diet and the administered probiotic, and the influence of genetic polymorphisms in the mother and infant on probiotic response. Next generation probiotics selected based on bacterial genetics rather than ease of production and large cluster-randomized clinical trials hold great promise for NEC prevention. Copyright © 2016 Elsevier Inc. All rights reserved.

  3. Necrotizing sialometaplasia of palate: a case report

    Energy Technology Data Exchange (ETDEWEB)

    Krishna, Sowmya [V.S Dental College and Hospital, Karnataka (India); Ramnarayan BK [Dayanada Sagar College of Dental Sciences and Hospital, Karnataka (India)

    2011-03-15

    Necrotizing sialometaplasia (NS) which mimics malignancy both clinically and histopathologically is an uncommon benign, self-limiting inflammatory disease of the mucus-secreting minor salivary glands. The lesion is believed to be the result of vascular ischemia that may be initiated by trauma. Till date, the diagnosis of NS remains a challenge. This report demonstrates a case of NS in a 73-year-old male patient who presented with an ulcerative lesion in his palate. He had a history of local trauma and was long-term user of salbutamol inhaler. An incisional biopsy was carried out and the diagnosis was established through history, clinical examination, histopathology using Hematoxylin and Eosin stain. The patient was given symptomatic treatment and the lesion healed in about 7 weeks.

  4. Statin-induced autoimmune necrotizing myositis

    Directory of Open Access Journals (Sweden)

    Katarzyna Ząber

    2016-02-01

    Full Text Available Myositides comprise a large group of disorders involving limb muscle weakness. In differential diagnosis we have to consider idiopathic myositides, myositides associated with other diseases, and those induced by external factors, e.g. drug-induced. Statins are commonly used drugs, but many patients experience a broad spectrum of adverse effects including symptoms from skeletal muscle. Physicians should pay special attention to patients reporting muscle weakness lasting longer than 12 weeks, despite statin withdrawal, as well as other symptoms: dysphagia, disturbed grip function, elevated creatinine kinase (CK levels and abnormal electromyography. The reported case deals with the problem of differential diagnosis of drug-induced muscle injury, polymyositis with a recently reported myopathy – statin-induced autoimmune necrotizing myositis, related to anti-HMGCR antibodies.

  5. Pure White Cell Aplasia and Necrotizing Myositis

    Directory of Open Access Journals (Sweden)

    Peter Geon Kim

    2016-01-01

    Full Text Available Pure white cell aplasia (PWCA is a rare hematologic disorder characterized by the absence of neutrophil lineages in the bone marrow with intact megakaryopoiesis and erythropoiesis. PWCA has been associated with autoimmune, drug-induced, and viral exposures. Here, we report a case of a 74-year-old female who presented with severe proximal weakness without pain and was found to have PWCA with nonspecific inflammatory necrotizing myositis and acute liver injury on biopsies. These findings were associated with a recent course of azithromycin and her daily use of a statin. Myositis improved on prednisone but PWCA persisted. With intravenous immunoglobulin and granulocyte-colony stimulating factor therapies, her symptoms and neutrophil counts improved and were sustained for months.

  6. Epilepsy in children with subacute sclerosing panencephalitis.

    Science.gov (United States)

    Jović, Nebojša J

    2013-01-01

    Subacute sclerosing panencephalitis (SSPE) is a rare, progressive, fatal neurodegenerative disease of childhood and early adolescence caused by defective measles virus. The initial symptoms of SSPE usually involve regression in cognitive functioning and behavior or recurrent myoclonic jerks. Seizures revealing SSPE and epilepsy during the clinical course can occur. The aim of the study was to analyze clinical and EEG characteristics of both initially occurred seizures and epilepsy which developed in the course of the disease. Retrospective study was carried out on 19 children (14 boys, 5 girls) with SSPE diagnosed and treated at our Clinic from 1995 to 2010. Seizures revealed SSPE in our patients aged from 6.5 to 11.5 years (mean 8.6 years). SSPE onset ranged from 4.5 to 16.5 years (mean 10.05). Complete vaccination was performed in nine patients. Cognitive and behavioral decline was preceeded by 6-18 months in two children with intractable focal motor seizures with secondary generalization, one child with complex partial seizures and one with atypical absences. During the clinical course of the disease epilepsy developed in 10 (52.6%) cases, including four patients with seizures as the initial SSPE sign. It occurred mainly in the first year, while in three cases seizures appeared between 1 and 5 years of the disease evolution. Myoclonus was present independently from seizures. No significant inter-group differences were found relating to the type of SSPE progression and history of epilepsy. The only child with fulminant SSPE presented with initial seizures. Favorable seizure control was achieved in 60.0% patients. Intractable epilepsy developed in four patients. Atypical SSPE presentation can include mainly focal intractable seizures. Epilepsy developed during clinical course in 52.6% cases. No significant influence was found of the history of epilepsy on the type of SSPE progression.

  7. Subacute sclerosing panencephalitis: A clinical appraisal.

    Science.gov (United States)

    Jagtap, Sujit Abajirao; Nair, M D; Kambale, Harsha J

    2013-10-01

    Subacute sclerosing panencephalitis (SSPE) is a rare chronic, progressive encephalitis affecting primarily children and young adults, caused by a persistent infection of immune resistant measles virus. The aim of the present study is to describe the clinical profile and natural history of patients with SSPE. We collected data of patients with SSPE during 2004-2010 who fulfilled Dyken's criteria. We analyzed demographical, clinical, electrophysiological, and imaging features. Study included 34 patients, 26 (76.5%) males with age of onset from 3 to 31 years. Twenty one patients were below 15 years of age formed childhood SSPE and 13 above 15 years of age constituted adult onset group. 85.3% had low-socioeconomic status. Eleven received measles vaccination and seven were unvaccinated. 59.9% patients had measles history. Most common presenting symptom was scholastic backwardness (52.5%) followed by seizures (23.5%). Three patients each had cortical blindness, macular degeneration, decreased visual acuity, and optic atrophy. Electroencephalographic (EEG) showed long interval periodic complexes and cerebrospinal fluid anti-measles antibody was positive in all. Magnetic resonance imaging was done in 70.5% with was abnormal in 52.5%. Mean incubation period of SSPE after measles was 9.6 years. The follow-up duration was 1-10 years, (average of 2 years). Only one patient died from available data of follow-up, 9 were stable and 10 deteriorated in the form of progression of staging. SSPE is common in low-socioeconomic status. The profile of adult onset did not differ from childhood onset SSPE, except for a longer interval between measles infection and presence of the ophthalmic symptom as presenting feature in adult onset group.

  8. Epilepsy in children with subacute sclerosing panencephalitis

    Directory of Open Access Journals (Sweden)

    Jović Nebojša J.

    2013-01-01

    Full Text Available Introduction. Subacute sclerosing panencephalitis (SSPE is a rare, progressive, fatal neurodegenerative disease of childhood and early adolescence caused by defective measles virus. The initial symptoms of SSPE usually involve regression in cognitive functioning and behavior or recurrent myoclonic jerks. Seizures revealing SSPE and epilepsy during the clinical course can occur. Objective. The aim of the study was to analyze clinical and EEG characteristics of both initially occurred seizures and epilepsy which developed in the course of the disease. Methods. Retrospective study was carried out on 19 children (14 boys, 5 girls with SSPE diagnosed and treated at our Clinic from 1995 to 2010. Seizures revealed SSPE in our patients aged from 6.5 to 11.5 years (mean 8.6 years. Results. SSPE onset ranged from 4.5 to 16.5 years (mean 10.05. Complete vaccination was performed in nine patients. Cognitive and behavioral decline was preceeded by 6-18 months in two children with intractable focal motor seizures with secondary generalization, one child with complex partial seizures and one with atypical absences. During the clinical course of the disease epilepsy developed in 10 (52.6% cases, including four patients with seizures as the initial SSPE sign. It occurred mainly in the first year, while in three cases seizures appeared between 1 and 5 years of the disease evolution. Myoclonus was present independently from seizures. No significant inter-group differences were found relating to the type of SSPE progression and history of epilepsy. The only child with fulminant SSPE presented with initial seizures. Favorable seizure control was achieved in 60.0% patients. Intractable epilepsy developed in four patients. Conclusion. Atypical SSPE presentation can include mainly focal intractable seizures. Epilepsy developed during clinical course in 52.6% cases. No significant influence was found of the history of epilepsy on the type of SSPE progression.

  9. Subacute sclerosing panencephalitis: A clinical appraisal

    Directory of Open Access Journals (Sweden)

    Sujit Abajirao Abajirao

    2013-01-01

    Full Text Available Introduction: Subacute sclerosing panencephalitis (SSPE is a rare chronic, progressive encephalitis affecting primarily children and young adults, caused by a persistent infection of immune resistant measles virus. The aim of the present study is to describe the clinical profile and natural history of patients with SSPE. Methods: We collected data of patients with SSPE during 2004-2010 who fulfilled Dyken′s criteria. We analyzed demographical, clinical, electrophysiological, and imaging features. Results: Study included 34 patients, 26 (76.5% males with age of onset from 3 to 31 years. Twenty one patients were below 15 years of age formed childhood SSPE and 13 above 15 years of age constituted adult onset group. 85.3% had low-socioeconomic status. Eleven received measles vaccination and seven were unvaccinated. 59.9% patients had measles history. Most common presenting symptom was scholastic backwardness (52.5% followed by seizures (23.5%. Three patients each had cortical blindness, macular degeneration, decreased visual acuity, and optic atrophy. Electroencephalographic (EEG showed long interval periodic complexes and cerebrospinal fluid anti-measles antibody was positive in all. Magnetic resonance imaging was done in 70.5% with was abnormal in 52.5%. Mean incubation period of SSPE after measles was 9.6 years. The follow-up duration was 1-10 years, (average of 2 years. Only one patient died from available data of follow-up, 9 were stable and 10 deteriorated in the form of progression of staging. Conclusion: SSPE is common in low-socioeconomic status. The profile of adult onset did not differ from childhood onset SSPE, except for a longer interval between measles infection and presence of the ophthalmic symptom as presenting feature in adult onset group.

  10. Current therapies and future perspectives in subacute sclerosing panencephalitis.

    Science.gov (United States)

    Tatli, Burak; Ekici, Bariş; Ozmen, Meral

    2012-04-01

    Subacute sclerosing panencephalitis is a progressive neurological disorder of children and young adults caused by a measles virus that became defective by persisting in the host. According to the results of clinical trials, antiviral and/or immunomodulatory therapy can slow the progression of the disease and improve life expectancy in patients. However, its long-term effects and eventual outcome remain debatable due to conflicting results and its lack of effect on the rapidly progressive form of the disease. Possible future therapies for subacute sclerosing panencephalitis are RNAi and antiapoptotic agents, which are currently in the hypothetical and experimental stages of research.

  11. Probiotics for people with hepatic encephalopathy.

    Science.gov (United States)

    Dalal, Rohan; McGee, Richard G; Riordan, Stephen M; Webster, Angela C

    2017-02-23

    Hepatic encephalopathy is a disorder of brain function as a result of liver failure or portosystemic shunt or both. Both hepatic encephalopathy (clinically overt) and minimal hepatic encephalopathy (not clinically overt) significantly impair patient's quality of life and daily functioning, and represent a significant burden on healthcare resources. Probiotics are live micro-organisms, which when administered in adequate amounts, may confer a health benefit on the host. To determine the beneficial and harmful effects of probiotics in any dosage, compared with placebo or no intervention, or with any other treatment for people with any grade of acute or chronic hepatic encephalopathy. This review did not consider the primary prophylaxis of hepatic encephalopathy. We searched The Cochrane Hepato-Biliary Group Controlled Trials Register, CENTRAL, MEDLINE, Embase, Science Citation Index Expanded, conference proceedings, reference lists of included trials, and the World Health Organization International Clinical Trials Registry Platform until June 2016. We included randomised clinical trials that compared probiotics in any dosage with placebo or no intervention, or with any other treatment in people with hepatic encephalopathy. We used standard methodological procedures expected by The Cochrane Collaboration. We conducted random-effects model meta-analysis due to obvious heterogeneity of participants and interventions. We defined a P value of 0.05 or less as significant. We expressed dichotomous outcomes as risk ratio (RR) and continuous outcomes as mean difference (MD) with 95% confidence intervals (CI). We included 21 trials with 1420 participants, of these, 14 were new trials. Fourteen trials compared a probiotic with placebo or no treatment, and seven trials compared a probiotic with lactulose. The trials used a variety of probiotics; the most commonly used group of probiotic was VSL#3, a proprietary name for a group of eight probiotics. Duration of administration

  12. Acute and subacute toxicity of copper sulfate pentahydrate (CuSO(4)5.H(2)O) in the guppy (Poecilia reticulata).

    Science.gov (United States)

    Park, Keehae; Heo, Gang-Joon

    2009-03-01

    Chemicals are used for treatment of aquatic diseases, but there is little data available about copper sulfate in small ornamental fish. The aim of the present study was to determine the TLm(24h) and evaluate the toxicity of copper sulfate in the guppy (Poecilia reticulata). The fish were subjected to an acute toxicity test for 24 hr, and the results showed a TLm(24h) value of 1.17 ppm. Severe hyperplasia and exfoliation of the epithelial cells of gill lamellae and obstruction of the internal cavities of renal tubules with necrotized renal epithelial cells sloughed from the basement membrane were observed. However, no significant changes, except for mild curling of gill lamellae, were found in a subacute toxicity test in which fish were exposed to 1/10 of the TLm(24h) value for 1 week. Therefore, use of less than 0.12 ppm of copper sulfate may be recommended as a therapeutic level.

  13. Necrotizing sialometaplasia of the palate: A case report

    Directory of Open Access Journals (Sweden)

    Ashwarya Trivedi

    2014-01-01

    Full Text Available Necrotizing sialometaplasia is a benign, self-limiting, reactive inflammatory disorder of the salivary tissue, which mimics malignancy both clinically and histopathologically. The etiology is unknown, although it most likely represents a local ischemic event, infectious process or perhaps an immune response to an unknown allergen. A case of necrotizing sialometaplasia of the palate in a 40-year-old male patient is presented. Histopathological examination is necessary for the diagnosis of necrotizing sialometaplasia because the clinical features of this condition can mimic other diseases, particularly malignant neoplasms.

  14. Necrotizing Fasciitis of the Nose Complicated with Cavernous Sinus Thrombosis

    Directory of Open Access Journals (Sweden)

    D. Swaminath

    2014-01-01

    Full Text Available Necrotizing fasciitis is a rapidly progressive life threatening bacterial infection of the skin, the subcutaneous tissue, and the fascia. We present a case of necrotizing fasciitis involving the nose complicated by cavernous sinus thrombosis. Few cases of septic cavernous sinus thrombosis have been reported to be caused by cellulitis of the face but necrotizing fasciitis of the nose is rare. It is very important to recognize the early signs of cavernous thrombosis. Treatment for septic cavernous sinus thrombosis is controversial but early use of empirical antibiotics is imperative.

  15. The Spectrum of Disease in Chronic Traumatic Encephalopathy

    Science.gov (United States)

    McKee, Ann C.; Stein, Thor D.; Nowinski, Christopher J.; Stern, Robert A.; Daneshvar, Daniel H.; Alvarez, Victor E.; Lee, Hyo-Soon; Hall, Garth; Wojtowicz, Sydney M.; Baugh, Christine M.; Riley, David O.; Kubilus, Caroline A.; Cormier, Kerry A.; Jacobs, Matthew A.; Martin, Brett R.; Abraham, Carmela R.; Ikezu, Tsuneya; Reichard, Robert Ross; Wolozin, Benjamin L.; Budson, Andrew E.; Goldstein, Lee E.; Kowall, Neil W.; Cantu, Robert C.

    2013-01-01

    Chronic traumatic encephalopathy is a progressive tauopathy that occurs as a consequence of repetitive mild traumatic brain injury. We analysed post-mortem brains obtained from a cohort of 85 subjects with histories of repetitive mild traumatic brain injury and found evidence of chronic traumatic encephalopathy in 68 subjects: all males, ranging…

  16. Wernicke's encephalopathy after vertical banded gastroplasty for morbid obesity.

    OpenAIRE

    Seehra, H.; MacDermott, N.; Lascelles, R. G.; Taylor, T V

    1996-01-01

    Thiamine deficiency is known to lead to certain neurological sequelae including Wernicke- Korsakoff encephalopathy. Signs attributable to this condition include ataxia, ophthalmoplegia, nystagmus, and mental confusion. Recognised predisposing conditions include alcoholism gastric carcinoma, pyloric obstruction, hyperemesis gravidarum, and prolonged intravenous feeding. We have recently encountered two cases of Wernicke's encephalopathy after vertical banded gastroplasty for morbid obesity . O...

  17. Necrotizing pulmonary aspergillosis: regarding two clinical cases

    Directory of Open Access Journals (Sweden)

    S. Carreira

    2011-03-01

    Full Text Available The authors present two case reports of necrotizing pulmonary aspergillosis. This disease is part of a spectrum of clinical conditions caused by the inhalation of Aspergillus spores. The necrotizing pulmonary aspergillosis (NPA corresponds to an indolent, destructive process of the lung due to invasion by Aspergillus species, usually A. fumigatus. The diagnosis is confirmed by a histological demonstration of tissue invasion by Aspergillus species and its growth on culture. Due to the difficulty in confirming the diagnosis, the following diagnosis criteria were established and when combined are highly indicative of NPA: characteristic clinical and radiological findings, elevation of inflammatory markers and either serological results positive for Aspergillus or the isolation of Aspergillus from respiratory samples. Active tuberculosis, non tuberculosis mycobacteriosis, cavitary histoplasmosis and coccidioidomycosis should be excluded. It is necessary to raise the level of suspicion and perform the adequate diagnostic tests in order to start therapy and avoiding disease progression. Resumo: Os autores apresentam dois casos clínicos de aspergilose pulmonar necrotizante. Esta patologia faz parte de um espectro de condições clínicas provocadas pela inalação de esporos do fungo Aspergillus. A aspergilose pulmonar necrotizante (APN corresponde a um processo indolente de destruição do pulmão pelo Aspergillus, geralmente A. fumigatus. O diagnóstico definitivo faz-se através da demonstração histológica de invasão tecidual pelo Aspergillus e do seu crescimento em cultura2. Pela dificuldade em obter um diagnóstico definitivo foram estabelecidos os seguintes critérios de diagnóstico que, quando reunidos, são fortemente indicativos de APN: aspectos clínicos e radiológicos consistentes com o diagnóstico, elevação dos parâmetros inflamatórios (PCR, VS e marcadores serol

  18. Histopathological and imaging modifications in chronic ethanolic encephalopathy.

    Science.gov (United States)

    Folescu, Roxana; Zamfir, Carmen Lăcrămioara; Sişu, Alina Maria; Motoc, Andrei Gheorghe Marius; Ilie, Adrian Cosmin; Moise, Marius

    2014-01-01

    Chronic abuse of alcohol triggers different types of brain damage. The Wernicke-Korsakoff syndrome gets together Wernicke's encephalopathy and Korsakoff's syndrome. Another type of encephalopathy associated with chronic ethanol consumption is represented by the Marchiafava-Bignami malady or syndrome, an extremely rare neurological disorder, which is characterized by a demielinization of corpus callosum, extending as far as a necrosis. Because the frequency of ethanolic encephalopathy is increased and plays a major role in the sudden death of ethanolic patients, we have studied the chronic ethanolic encephalopathy both in deceased and in living patients, presenting different pathologies related to the chronic ethanol consumption. The present study investigated the effects of chronic ethanolic encephalopathy on the central nervous system based both on the histopathological exam of the tissular samples and the imaging investigation, such as MRI and CT.

  19. Reversible cortical blindness in a case of hepatic encephalopathy

    Directory of Open Access Journals (Sweden)

    Amlan Kanti Biswas

    2016-01-01

    Full Text Available Hepatic encephalopathy is a frequent and often fatal manifestation of chronic liver disease. The pathogenesis of hepatic encephalopathy is believed to be multifactorial including impaired blood-brain barrier function, imbalance between the excitatory and inhibitory neurotransmitters in cortex, accumulation of various toxic and false neurotransmitters, and lack of nutrients like oxygen and glucose. Signs and symptoms of hepatic encephalopathy varies and commonly ranges from personality changes, disturbed consciousness, sleep pattern alternation, intellectual deterioration, speech disturbances, asterixis to frank coma and even death. Reversible or transient cortical blindness is rare manifestation of hepatic encephalopathy. It may even precede the phase of altered consciousness in such patients. Very few similar cases have been reported worldwide. Hence, we would like to report a case of transient cortical blindness in a patient of hepatic encephalopathy.

  20. Hashimoto encephalopathy with pegylated interferon alfa-2b and ribavirin.

    Science.gov (United States)

    Deutsch, Melanie; Koskinas, John; Tzannos, Konstatinos; Vassilopoulos, Dimitrios; Mailis, Antonis; Tolis, George; Hadziyannis, Stephanos

    2005-10-01

    To report an instance of Hashimoto encephalopathy probably resulting from pegylated interferon alfa-2b and ribavirin. A 36-year-old woman with a 10-year history of autoimmune thyroiditis presented with symptoms and signs consistent with Hashimoto encephalopathy during therapy with pegylated interferon alfa-2b and ribavirin for chronic hepatitis C. Hashimoto encephalopathy is a rare autoimmune condition that occurs in patients with Hashimoto thyroiditis and high titers of antithyroid antibodies. It is characterized by a variety of nonspecific neuropsychiatric symptoms, increased cerebrospinal fluid protein level, and abnormal brain imaging and electroencephalogram. Prompt response to corticosteroids is observed in most cases. As of August 29, 2005, this is the first report of such an association. An objective causality assessment revealed that the Hashimoto encephalopathy was probably caused by the patient's medications. Hashimoto encephalopathy may rarely be triggered by interferon alfa therapy in susceptible patients.

  1. Hospital epidemiology of emergent cervical necrotizing fasciitis

    Directory of Open Access Journals (Sweden)

    Shaikh Nissar

    2010-01-01

    Full Text Available Background : Necrotizing fasciitis (NF is a surgical emergency. It is a rapidly progressing infection of the fascia and subcutaneous tissue and could be fatal if not diagnosed early and treated properly. NF is common in the groin, abdomen, and extremities but rare in the neck and the head. Cervical necrotizing fasciitis (CNF is an aggressive infection of the neck and the head, with devastating complications such as airway obstruction, pneumonia, pulmonary abscess, jugular venous thrombophlebitis, mediastinitis, and septic shock associated with high mortality. Aim : To assess the presentation, comorbidities, type of infection, severity of disease, and intensive care outcome of CNF. Methods : Medical records of the patients treated for NF in the surgical intensive care unit (SICU from January 1995 to February 2005 were reviewed retrospectively. Results : Out of 94 patients with NF, 5 (5.3% had CNF. Four patients were male. The mean age of our patients was 41.2 ± 14.8 years. Sixty percent of patients had an operative procedure as the predisposing factor and 80% of patients received nonsteroidal anti-inflammatory drugs (NSAIDs. The only comorbidity associated was diabetes mellitus (DM in 3 patients (60%. Sixty percent of the cases had type1 NF. Mean sequential organ failure assessment (SOFA score on admission to the ICU was 8.8 ± 3.6. All patients had undergone debridement at least two times. During the initial 24 h our patients received 5.8 ± 3.0 l of fluid, 2.0 ± 1.4 units of packed red blood cells (PRBC, 4.8 ± 3.6 units of fresh frozen plasma (FFP, and 3.0 ± 4.5 units of platelet concentrate. The mean number of days patients were intubated was 5.2 ± 5.1 days and the mean ICU stay was 6.4 ± 5.2 days. Sixty percent of cases had multiorgan dysfunction (MODS and one patient died, resulting in a mortality rate of 20%. Conclusion : According to our study, CNF represents around 5% of NF patients. CNF was higher among male patients and in

  2. Cardiovascular dysfunction in infants with neonatal encephalopathy.

    LENUS (Irish Health Repository)

    Armstrong, Katey

    2012-04-01

    Severe perinatal asphyxia with hypoxic ischaemic encephalopathy occurs in approximately 1-2\\/1000 live births and is an important cause of cerebral palsy and associated neurological disabilities in children. Multiorgan dysfunction commonly occurs as part of the asphyxial episode, with cardiovascular dysfunction occurring in up to a third of infants. This narrative paper attempts to review the literature on the importance of early recognition of cardiac dysfunction using echocardiography and biomarkers such as troponin and brain type natriuretic peptide. These tools may allow accurate assessment of cardiac dysfunction and guide therapy to improve outcome.

  3. Chronic traumatic encephalopathy and the availability cascade.

    Science.gov (United States)

    Solomon, Gary S; Sills, Allen

    2014-09-01

    Chronic traumatic encephalopathy (CTE) in sports has been known for > 85 years, and has experienced a resurgence of interest over the past decade, both in the media and in the scientific community. However, there appears to be a disconnection between the public's perception of CTE and the currently available scientific data. The cognitive bias known as the "availability cascade" has been suggested as a reason to explain this rift in knowledge. This review summarizes and updates the history of CTE in sports, discusses recent epidemiological and autopsy studies, summarizes the evidence base related to CTE in sports, and offers recommendations for future directions.

  4. Chronic Traumatic Encephalopathy: Known Causes, Unknown Effects.

    Science.gov (United States)

    Iacono, Diego; Shively, Sharon B; Edlow, Brian L; Perl, Daniel P

    2017-05-01

    Chronic traumatic encephalopathy (CTE) is a neuropathologic diagnosis typically made in human brains with a history of repetitive traumatic brain injury (rTBI). It remains unknown whether CTE occurs exclusively after rTBI, or whether a single TBI (sTBI) can cause CTE. Similarly, it is unclear whether impact (eg, motor vehicle accidents) and non-impact (eg, blasts) types of energy transfer trigger divergent or common pathologies. While it is established that a history of rTBI increases the risk of multiple neurodegenerative diseases (eg, dementia, parkinsonism, and CTE), the possible pathophysiologic and molecular mechanisms underlying these risks have yet to be elucidated. Published by Elsevier Inc.

  5. Post-partum posterior reversible encephalopathy syndrome

    DEFF Research Database (Denmark)

    Aaen, Anne Albers; Jeppesen, Jørgen; Obaid, Hayder

    2015-01-01

    Posterior reversible encephalopathy syndrome (PRES) is a complex clinical condition with vasogenic subcortical oedema caused by hypertension. Oedema is often seen on magnetic resonance imaging. The wide clinical spectrum ranges from headaches to vision loss and even death. Early diagnosis...... and treatment is important for the reversibility of the condition. In this case report we emphasize the importance of blood pressure control in a post-partum woman, who had a rather complicated pregnancy. The symptoms of PRES were not recognized immediately because of failure to use and acknowledge a blood...

  6. STXBP1 encephalopathy

    DEFF Research Database (Denmark)

    Stamberger, Hannah; Nikanorova, Marina; Willemsen, Marjolein H.

    2016-01-01

    %) with epileptic spasms or tonic seizures as main seizure type. We found no correlation between severity of seizures and severity of ID or between mutation type and seizure characteristics or cognitive outcome. Neurologic comorbidities including autistic features and movement disorders are frequent. We also report......, and the degree of ID. Accordingly, we hypothesize that seizure severity and ID present 2 independent dimensions of the STXBP1-E phenotype. STXBP1-E may be conceptualized as a complex neurodevelopmental disorder rather than a primary epileptic encephalopathy....

  7. Chronic Traumatic Encephalopathy and Movement Disorders: Update.

    Science.gov (United States)

    Tarazi, Apameh; Tator, Charles H; Tartaglia, Maria Carmela

    2016-05-01

    Association of repetitive brain trauma with progressive neurological deterioration has been described since the 1920s. Punch drunk syndrome and dementia pugilistica (DP) were introduced first to explain symptoms in boxers, and more recently, chronic traumatic encephalopathy (CTE) has been used to describe a neurodegenerative disease in athletes and military personal with a history of multiple concussions. Although there are many similarities between DP and CTE, a number of key differences are apparent especially when comparing movement impairments. The aim of this review is to compare clinical and pathological aspects of DP and CTE with a focus on disorders of movement.

  8. Repetitive Head Impacts and Chronic Traumatic Encephalopathy.

    Science.gov (United States)

    McKee, Ann C; Alosco, Michael L; Huber, Bertrand R

    2016-10-01

    Chronic traumatic encephalopathy (CTE) is a distinctive neurodegenerative disease that occurs as a result of repetitive head impacts. CTE can only be diagnosed by postmortem neuropathologic examination of brain tissue. CTE is a unique disorder with a pathognomonic lesion that can be reliably distinguished from other neurodegenerative diseases. CTE is associated with violent behaviors, explosivity, loss of control, depression, suicide, memory loss and cognitive changes. There is increasing evidence that CTE affects amateur athletes as well as professional athletes and military veterans. CTE has become a major public health concern. Published by Elsevier Inc.

  9. Bovine Spongiform Encephalopathy (BSE, Mad Cow Disease

    Directory of Open Access Journals (Sweden)

    G. K. Bruckner

    1997-07-01

    Full Text Available Mad Cow Disease or BSE (Bovine Spongiform Encephalopathy became a household name internationally and also in South Africa. International hysteria resulted following reports of a possible link between a disease diagnosed in cattle in Britain and a variant of the disease diagnosed in humans after the presumed ingestion or contact with meat from infected cattle. The European Union instituted a ban on the importation of beef from the United Kingdom during March 1996 that had a severe effect on the beef industry in the UK and also resulted in a world wide consumer resistance against beef consumption.

  10. Bilirubin encephalopathy due to Rh incompatibility

    Directory of Open Access Journals (Sweden)

    Taísa Roberta Ramos Nantes de Castilho

    2011-06-01

    Full Text Available The authors present the case of a newborn of an Rh-factorsensitizedmother, who received early hospital discharge while icteric only to be readmitted at an Emergency Service at five days of age with signs of kernicterus. Despite treatment given, the neonate progressed with a clinical picture of bilirubin encephalopathy. The lack of interaction between the obstetric and neonatal teams, premature hospital discharge, and lack of concern of neonatologists with jaundice in a full-term infant are highlighted as causes of a condition that should have disappeared if there had been adequateprevention.

  11. Fetal encephalopathy after maternal anaphylaxis. Case report.

    Science.gov (United States)

    Luciano, R; Zuppa, A A; Maragliano, G; Gallini, F; Tortorolo, G

    1997-01-01

    Fetal hypoxic-ischemic encephalopathy can be diagnosed at birth by means of cerebral ultrasound scanning. The morphological appearance of the lesions depends on the time elapsed between the insult and examination of the brain. We report a case of a neonate affected by multicystic encephalomalacia and corpus callosum atrophy attributable to an episode of maternal anaphylactic shock which occurred at 27 weeks of gestation following intravenous iron injection. The diagnosis was made by means of a cerebral ultrasound scan performed at birth and confirmed by magnetic resonance. This case demonstrates that maternal severe acute hypotension during pregnancy can cause fetal cerebral damage similar to the hypoxicischemic injuries occurring in the perinatal period.

  12. Evaluation of acute and subacute toxicities of aqueous ethanolic ...

    African Journals Online (AJOL)

    We investigated the acute and subacute toxicities of hydro-ethanolic extract of leaves of Senna alata (L.) Roxb. in Swiss mice and Wistar albino rats. The mice were divided into 6 groups of 10 animals and each group received once by intra gastric gavages 0, 4, 8, 12, 16, 20 times 1000 mg/kg dose of extract. Distilled water ...

  13. Resolving MRI abnormalities with progression of subacute sclerosing panencephalitis

    Energy Technology Data Exchange (ETDEWEB)

    Winer, J.B. (Saint Mary' s Hospital, Birmingham (UK)); Pires, M.; Kermode, A.; Ginsberg, L. (National Hospital for Nervous Diseases, Birmingham (UK)); Rossor, M. (Saint Mary' s Hospital, Birmingham (UK) National Hospital for Nervous Diseases, Birmingham (UK))

    1991-04-01

    Serial MRI was performed on a 15 year old girl with Subacute Sclerosing Pan-encephalitis (SSPE). After a period of remission she entered a phase of progressive deterioration. A repeat MRI showed significant resolution of the previous abnormalities. Her pathology and MRI scans are discussed.

  14. Epilepsia partialis continua in children with fulminant subacute sclerosing panencephalitis.

    Science.gov (United States)

    Kravljanac, Ruzica; Jovic, Nebojsa; Djuric, Milena; Nikolic, Ljubica

    2011-12-01

    Various inflammatory diseases of central nervous system, including subacute sclerosing panencephalitis, could cause epilepsia partialis continua. Two boys with epilepsia partialis continua with onset in terminal phase of atypical subacute sclerosing panencephalitis have been reported. Children were not vaccinated against measles, and the second case had history of measles at an early age. In both cases, the onset of subacute sclerosing panencephalitis was characterized by altered behavior and cognitive decline with very fast mental and neurological deterioration. One boy was suffering from complex partial seizures and myoclonic jerks synchronous with periodic electroencephalographic pattern. Diagnosis was proved by increased titers of antimeasles antibodies in both serum and cerebrospinal fluid. In terminal phase of the disease, epilepsia partialis continua of localized group of the muscles was diagnosed, with good response to intravenous infusion of midazolam. Surface electroencephalographic recordings during epilepsia partialis continua did not show the epileptic discharges. During the terminal phase of the disease, no other type of seizures and movement disorders were recognized, except epilepsia partialis continua. In spite of the treatment, period from the onset of disease to death lasted less than 3 months, suggesting very fulminant course of subacute sclerosing panencephalitis.

  15. High-Dose Intravenous Ribavirin Therapy for Subacute Sclerosing Panencephalitis

    Science.gov (United States)

    Hosoya, Mitsuaki; Shigeta, Shiro; Mori, Shuichi; Tomoda, Akemi; Shiraishi, Seiji; Miike, Teruhisa; Suzuki, Hitoshi

    2001-01-01

    Two patients with subacute sclerosing panencephalitis (SSPE) were treated safely and effectively with high doses of intravenous ribavirin combined with intraventricular alpha interferon. The ribavirin concentrations maintained in the serum and cerebrospinal fluid were higher than those which inhibit SSPE virus replication in vitro and in vivo. PMID:11181386

  16. A close look at EEG in subacute sclerosing panencephalitis.

    Science.gov (United States)

    Demir, Nurhak; Cokar, Ozlem; Bolukbasi, Feray; Demirbilek, Veysi; Yapici, Zuhal; Yalcinkaya, Cengiz; Direskeneli, Guher Saruhan; Yentur, Sibel; Onal, Emel; Yilmaz, Gulden; Dervent, Aysin

    2013-08-01

    To define atypical clinical and EEG features of patients with subacute sclerosing panencephalitis that may require an overview of differential diagnosis. A total of 66 EEGs belonging to 53 (17 females and 36 males) consecutive patients with serologically confirmed subacute sclerosing panencephalitis were included in this study. Patient files and EEG data were evaluated retrospectively. EEGs included in the study were sleep-waking EEGs and/or sleep-waking video-EEG records with at least 2 hours duration. Cranial MRIs of the patients taken 2 months before or after the EEG records were included. Age range at the onset of the disease was 15 to 192 months (mean age: 80.02 months). Epilepsy was diagnosed in 21 (43%) patients. Among epileptic seizures excluding myoclonic jerks, generalized tonic-clonic type constituted the majority (58%). Tonic seizures were documented during the video-EEG recordings in four patients. Epileptogenic activities were found in 56 (83%) EEG recordings. They were localized mainly in frontal (58%), posterior temporal, parietal, occipital (26%), and centrotemporal (8%) regions. Multiple foci were detected in 26 recordings (39%). Epileptiform activities in the 39 (59%) EEGs appeared as unilateral or bilateral diffuse paroxysmal discharges. Recognition of uncommon clinical and EEG findings of subacute sclerosing panencephalitis, especially in countries where subacute sclerosing panencephalitis has not been eliminated yet, could be helpful in prevention of misdiagnosis and delay in the management of improvable conditions.

  17. Treatment of acute and subacute dorsal perilunate fracture dislocations

    Directory of Open Access Journals (Sweden)

    Levent Kucuk

    2014-04-01

    Outcomes: Results of the perilunate fracture dislocations treated in acute or subacute phase by open reduction and internal fixation via dorsal approach are satisfactory. There is a strong demand for prospective, randomized studies to compare the results of different treatment modalities. [Hand Microsurg 2014; 3(1.000: 1-7

  18. Evaluation of acute and subacute toxicity of Alstonia congensis ...

    African Journals Online (AJOL)

    The extract demonstrated good hypoglycaemic effects by lowering the plasma sugar level and also had some beneficial and reduction effects on cardiovascular risk factors. There was no evidence of drug-induced symptoms or death at all the doses of the extract administered in acute study but subacute results revealed a ...

  19. Acute and Subacute Toxic Study of Aqueous Leaf Extract of ...

    African Journals Online (AJOL)

    HP

    Results: For the acute toxicit test, no death and signs of poisoning were observed in the treated groups. In the subacute .... Subcute toxicity test. The animals were divided into six groups of six animals each. The treatments were given by intraperitoneal injection. Group 1 served ... produce significant changes in behavior,.

  20. Speech and Language Therapy for Aphasia following Subacute Stroke

    NARCIS (Netherlands)

    Koyuncu, E.; Çam, P.; Altinok, N.; Çalli, D.E.; Yarbay Duman, T.; Özgirgin, N.

    2016-01-01

    The aim of this study was to investigate the time window, duration and intensity of optimal speech and language therapy applied to aphasic patients with subacute stroke in our hospital. The study consisted of 33 patients being hospitalized for stroke rehabilitation in our hospital with first stroke

  1. Acute and subacute toxicities of defatted ethanolic extract of Moringa ...

    African Journals Online (AJOL)

    Moringa oleifera seeds are widely accepted as a nutritional supplement. The seeds are consumed and are sold on the shelf of nature, herbal shops, pharmacy and supermarkets. They are consumed as herbal remedy for various diseases. This study was designed to evaluate the acute and sub-acute toxicity of defatted ...

  2. Virtual Reality Training for Upper Extremity in Subacute Stroke (VIRTUES)

    DEFF Research Database (Denmark)

    Brunner, Iris; Skouen, Jan Sture; Hofstad, Håkon

    2017-01-01

    Objective: To compare the effectiveness of upper extremity virtual reality rehabilitation training (VR) to time-matched conventional training (CT) in the subacute phase after stroke. Methods: In this randomized, controlled, single-blind phase III multicenter trial, 120 participants with upper...

  3. Clinical Characteristics of Transplant-associated Encephalopathy in Children.

    Science.gov (United States)

    Lee, Yun Jeong; Yum, Mi Sun; Kim, Eun Hee; Kim, Min Jee; Kim, Kyung Mo; Im, Ho Joon; Kim, Young Hwue; Park, Young Seo; Ko, Tae Sung

    2017-03-01

    We aimed to analyze characteristics of encephalopathy after both hematopoietic stem cell and solid organ pediatric transplantation. We retrospectively reviewed medical records of 662 pediatric transplant recipients (201 with liver transplantation [LT], 55 with heart transplantation [HT], and 67 with kidney transplantation [KT], 339 with allogeneic hematopoietic stem cell transplantation [HSCT]) who received their graft organs at Asan Medical Center between January 2000 and July 2014. Of the 662 patients, 50 (7.6%) experienced encephalopathy after transplantation. The incidence of encephalopathy was significantly different according to the type of organ transplant: LT, 16/201 (8.0%), HT, 13/55 (23.6%), KT, 5/67 (7.5%), and HSCT, 16/339 (4.7%) (P encephalopathy (n = 14) was the most common encephalopathy for all transplant types, but particularly after HSCT. Hypertensive encephalopathy was the most common after KT and HT, whereas metabolic encephalopathy was the most common after LT. The median time to encephalopathy onset also differed according to the transplant type: 5 days after KT (range 0-491 days), 10 days after HT (1-296 days), 49.5 days after HSCT (9-1,405 days), and 39 days after LT (1-1,092 days) (P = 0.018). The mortality rate among patients with encephalopathy was 42.0% (n = 21/50). Only 5 patients died of neurologic complications. Transplant-associated encephalopathy presented different characteristics according to the type of transplant. Specialized diagnostic approach for neurologic complications specific to the type of transplant may improve survival and quality of life in children after transplantation.

  4. Pulp revascularization in an immature necrotic tooth: a case report.

    Science.gov (United States)

    Gelman, Richard; Park, Helen

    2012-01-01

    Immature permanent teeth damaged by caries or trauma can present a challenge to dentistry. Currently, triple antibiotic paste (TAP) containing ciprofloxacin, metronidazole, and minocycline is used to attempt revascularization in necrotic immature teeth. Therefore, the purpose of this report was to present a case of pulp revascularization in an immature necrotic tooth. An 8-year-old male presented with trauma to the permanent maxillary left and right central incisors. Upon clinical and radiographic examination, the left central incisor was deemed necrotic. Revascularization therapy was performed over multiple visits. At 11 months follow-up, healing of the periapical area and apexogenesis were found to be complete. With an increasing breadth of clinical evidence and practitioner acceptance, regenerative techniques may become a standard technique in treating immature necrotic permanent teeth.

  5. The phylum Synergistetes in gingivitis and necrotizing ulcerative gingivitis

    National Research Council Canada - National Science Library

    Baumgartner, Angelica; Thurnheer, Thomas; Lüthi-Schaller, Helga; Gmür, Rudolf; Belibasakis, Georgios N

    2012-01-01

    The clinical manifestation of necrotizing ulcerative gingivitis (NUG) is distinct from that of common gingivitis in that it is characterized by local necrosis of the gingival tissues, rapid onset, pain and extensive bleeding...

  6. Diabetic Muscle Infarction Masquerading as Necrotizing Fasciitis

    Directory of Open Access Journals (Sweden)

    Kalyana C. Janga

    2017-01-01

    Full Text Available A 43-year-old male patient with past medical history of diabetes mellitus (DM, end stage renal disease (ESRD on hemodialysis (HD, congestive heart failure (CHF, obstructive sleep apnea (OSA, and chronic anemia presented with complaints of left thigh pain. A computerized tomogram (CT of the thigh revealed evidence of edema with no evidence of a focal collection or gas formation noted. The patient’s clinical symptoms persisted and he underwent magnetic resonance imaging (MRI of his thigh which was reported to show small areas of muscle necrosis with fluid collection. These findings in the acute setting concerned necrotizing fasciitis. After careful discussion following a multidisciplinary approach, a decision was made to perform a fasciotomy with tissue debridement. The patient was treated with IV antibiotics and discharged with a vacuum assisted wound drain. The surgical pathology revealed evidence of muscle edema with necrosis. Seven weeks later the patient presented with similar complaints on the other thigh (right thigh. MRI of the thighs revealed worsening edema with features suggestive of myositis and possible muscle infarction. A CT guided biopsy of the right quadriceps muscle revealed fibrotic interstitial connective tissue and no evidence of necrosis. This favored a diagnosis of diabetic muscle infarction. The disease was managed with pain control, strict diabetes management, and aggressive dialysis.

  7. Problems of ileostomy in necrotizing enterocolitis.

    Science.gov (United States)

    Haberlik, A; Höllwarth, M E; Windhager, U; Schober, P H

    1994-01-01

    Exteriorization of the intestine and resection of the gangrenous bowel are major therapeutic regimens for necrotizing enterocolitis (NEC). Ileostomy associated complications are well known, therefore the time of ileostomy closure is a matter for discussion. Between 1975 and 1992, 84 patients with NEC were treated surgically. Ileostomies were performed in 37 children (44%). Of these 37 neonates, 9 (7M, 2F) died. In the remaining 28 patients (16M, 12F) with a mean gestational age of 35.8 weeks and a mean birth weight of 2412 g, ileostomies were performed between the 2nd and 11th days after birth. On average, the stomies were in function for 91 days, and within this period the average weekly weight gain was 153 g. Nineteen patients of this group did not show any problems attributable to the ileostomy. In 9 patients (32%) complications occurred, requiring a preplanned closure of the stoma. Postinflammatory strictures of bowel were diagnosed in 9 patients and resection of the stenotic intestine was performed at the same time as stoma closure. In conclusion, an appropriate weight gain can be achieved in patients with an ileostomy with an adequate feeding regimen. In otherwise uncomplicated cases, ileostomy closure can be delayed by up to 10 weeks when simultaneous surgical correction of additional intestinal strictures is possible. In one-third of patients with an ileostomy, however, complications may occur and urge a preplanned closure of the stoma.

  8. Medical image of the week: necrotizing pancreatitis

    Directory of Open Access Journals (Sweden)

    Desai H

    2015-08-01

    Full Text Available No abstract available. Article truncated after 150 words. A 60-year-old man with a past medical history significant for coronary artery disease status post percutaneous coronary intervention was admitted to Banner University Medical Center for acute pancreatitis complicated by a pericardial effusion requiring pericardiocentesis. The following day, the patient developed severe shortness of breath requiring increasing amounts of supplemental oxygen. The patient was emergently transferred to ICU for noninvasive bilevel positive airway pressure ventilation, but he subsequently required intubation. Throughout his worsening condition, he denied any abdominal pain, only relaying ongoing substernal chest pain. His troponins, however, remained negative and echocardiography failed to show any reaccumulation of the pericardial effusion. CT scan of the chest failed to show any pulmonary embolism. But, CT abdomen displayed acute pancreatitis complicated by peripancreatic gas consistent with necrotizing pancreatitis (Figure 1. Emergent laparotomy was completed. There were no signs of stomach or duodenal perforation. Purulent fluid was removed from the lesser sac and ...

  9. Necrotizing gastritis due to Bacillus cereus in an immunocompromised patient.

    Science.gov (United States)

    Le Scanff, J; Mohammedi, I; Thiebaut, A; Martin, O; Argaud, L; Robert, D

    2006-04-01

    Bacillus cereus is increasingly being acknowledged as a serious bacterial pathogen in immunocompromised patients. We present a case of acute necrotizing gastritis caused by B. cereus in a 37-year-old woman with acute myeloblastic leukemia, who recovered following total parenteral nutrition and treatment with imipenem and vancomycin. B. cereus was isolated from gastric mucosa and blood cultures. Up to now, no case of acute necrotizing gastritis due to this organism has been reported.

  10. Triple diagnostics for early detection of ambivalent necrotizing fasciitis.

    Science.gov (United States)

    Hietbrink, Falco; Bode, Lonneke G; Riddez, Louis; Leenen, Luke P H; van Dijk, Marijke R

    2016-01-01

    Necrotizing fasciitis is an uncommon, rapidly progressive and potential lethal condition. Over the last decade time to surgery decreased and outcome improved, most likely due to increased awareness and more timely referral. Early recognition is key to improve mortality and morbidity. However, early referral frequently makes it a challenge to recognize this heterogeneous disease in its initial stages. Signs and symptoms might be misleading or absent, while the most prominent skin marks might be in discrepancy with the position of the fascial necrosis. Gram staining and especially fresh frozen section histology might be a useful adjunct. Retrospective analysis of 3 year period. Non-transferred patients who presented with suspected necrotizing fasciitis are included. ASA classification was determined. Mortality was documented. In total, 21 patients are included. Most patients suffered from severe comorbidities. In 11 patients, diagnoses was confirmed based on intra-operative macroscopic findings. Histology and/or microbiotic findings resulted in 6/10 remaining patients in a change in treatment strategy. In total, 17 patients proved to suffer necrotizing fasciitis. In the cohort series 2 patients died due to necrotizing fasciitis. In the early phases of necrotizing fasciitis, clinical presentation can be ambivalent. In the present cohort, triple diagnostics consisting of an incisional biopsy with macroscopic, histologic and microbiotic findings was helpful in timely identification of necrotizing fasciitis.

  11. Clinical presentation of chronic traumatic encephalopathy

    Science.gov (United States)

    Daneshvar, Daniel H.; Baugh, Christine M.; Seichepine, Daniel R.; Montenigro, Philip H.; Riley, David O.; Fritts, Nathan G.; Stamm, Julie M.; Robbins, Clifford A.; McHale, Lisa; Simkin, Irene; Stein, Thor D.; Alvarez, Victor E.; Goldstein, Lee E.; Budson, Andrew E.; Kowall, Neil W.; Nowinski, Christopher J.; Cantu, Robert C.; McKee, Ann C.

    2013-01-01

    Objective: The goal of this study was to examine the clinical presentation of chronic traumatic encephalopathy (CTE) in neuropathologically confirmed cases. Methods: Thirty-six adult male subjects were selected from all cases of neuropathologically confirmed CTE at the Boston University Center for the Study of Traumatic Encephalopathy brain bank. Subjects were all athletes, had no comorbid neurodegenerative or motor neuron disease, and had next-of-kin informants to provide retrospective reports of the subjects' histories and clinical presentations. These interviews were conducted blind to the subjects' neuropathologic findings. Results: A triad of cognitive, behavioral, and mood impairments was common overall, with cognitive deficits reported for almost all subjects. Three subjects were asymptomatic at the time of death. Consistent with earlier case reports of boxers, 2 relatively distinct clinical presentations emerged, with one group whose initial features developed at a younger age and involved behavioral and/or mood disturbance (n = 22), and another group whose initial presentation developed at an older age and involved cognitive impairment (n = 11). Conclusions: This suggests there are 2 major clinical presentations of CTE, one a behavior/mood variant and the other a cognitive variant. PMID:23966253

  12. The why and wherefore of hepatic encephalopathy

    Directory of Open Access Journals (Sweden)

    Grover VPB

    2015-12-01

    Full Text Available Vijay PB Grover, Joshua M Tognarelli, Nicolas Massie, Mary ME Crossey, Nicola A Cook, Simon D Taylor-Robinson Liver Unit, Division of Diabetes, Endocrinology and Metabolism, Department of Medicine, Imperial College London, London, UK Abstract: Hepatic encephalopathy is a common neuropsychiatric abnormality, which complicates the course of patients with liver disease. It was probably first described by Hippocrates over 2000 years ago, who said that "those whose madness arises from phlegm are quiet and neither shout nor make a disturbance, while those whose madness arises from bile shout, play tricks and will not keep still, but are always up to some mischief". He was presumably describing the differences between patients with pneumonia and acute liver failure. Despite the fact that the syndrome was probably first recognized thousands of years ago, the exact pathogenesis still remains unclear. Furthermore, a precise definition of the syndrome is lacking, as are definitive methods of diagnosing this condition. It is important as both patients with cirrhosis and the general population with whom they interact may be affected as a consequence. At a minimum, the individual may be affected by impaired quality of life, impaired ability to work, and slowed reaction times, which are relevant to the population at large if affected individuals operate heavy machinery or drive a car. Pathogenic mechanisms, diagnostic tools, and treatment options are discussed. Keywords: hepatic encephalopathy, cirrhosis, ammonia, pathology, treatment, rifaximin, lactulose

  13. Prions and animal transmissible spongiform encephalopathies

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    Juntes Polona

    2017-01-01

    Full Text Available Background. Transmissible spongiform encephalopathies (TSEs or prion diseases are a unique group of neurodegenerative diseases of animals and humans, which always have a fatal outcome and are transmissible among animals of the same or different species. Scope and Approach. The aim of this work is to review some recent data about animal TSEs, with the emphasis on their causative agents and zoonotic potential, and to discuss why the surveillance and control measures over animal TSEs should remain in force. Key Findings and Conclusions. We still have incomplete knowledge of prions and prion diseases. Scrapie has been present for a very long time and controlled with varied success. Bovine spongiform encephalopathy (BSE emerged unnoticed, and spread within a few years to epidemic proportions, entailing enormous economic consequences and public concerns. Currently, the classical BSE epidemic is under control, but atypical cases do, and probably will, persist in bovine populations. The Chronic Wasting Disease (CWD of the cervids has been spreading in North America and has recently been detected in Europe. Preventive measures for the control of classical BSE remain in force, including the feed ban and removal of specified risk materials. However, active BSE surveillance has considerably decreased. In the absence of such preventive and control measures, atypical BSE cases in healthy slaughtered bovines might persist in the human food chain, and BSE prions might resurface. Moreover, other prion strains might emerge and spread undetected if the appropriate preventive and surveillance measures were to cease, leaving behind inestimable consequences.

  14. [Clinical Features and Treatment of Hashimoto Encephalopathy].

    Science.gov (United States)

    Maki, Yoshimitsu; Takashima, Hiroshi

    2016-09-01

    Hashimoto encephalopathy (HE) is characterized by heterogeneous neurological symptoms. HE is diagnosed based on three criteria-the presence of antithyroid antibodies, neurological symptoms from the cerebrum and/or cerebellum, and a positive response to immunotherapy. We clinically analyzed 18 patients (3 men, 15 women; age range, 38-81years) diagnosed with HE in our hospital from May 2013 to January 2016. Eleven patients showed sensory abnormalities such as strong pain, deep muscle pain, dysesthesia, paresthesia, or neuralgia. Surprisingly, the majority of the pain was distributed in a manner that was not explainable anatomically. Seventeen patients showed motor disturbances, such as weakness, paresis of extremities, or dexterity movement disorder, and eight patients showed give-way weakness, which is disruption of continuous muscle contraction. Other symptoms indicative of brain-related anomalies such as tremor, dystonia, involuntary movements, cerebellar ataxia, parkinsonism, memory loss, and chronic fatigue were also seen. In most patients, such motor, sensory, or higher brain functions were markedly improved with immunosuppressive therapies such as prednisolone, azathioprine, or immunoadsorption therapy. Although give-way weakness and anatomically unexplainable pain are typically considered as being psychogenic in origin, the presence of these symptoms is indicative of HE. HE exhibits diffuse involvement of the entire brain and thus, these symptoms are explainable. We propose that physicians should not diagnose somatoform disorders without first excluding autoimmune encephalopathy.

  15. Probiotics in management of hepatic encephalopathy.

    Science.gov (United States)

    Sharma, Barjesh Chander; Singh, Jatinderpal

    2016-12-01

    Gut microflora leads to production of ammonia and endotoxins which play important role in the pathogenesis of hepatic encephalopathy (HE). There is relationship between HE and absorption of nitrogenous substances from the intestines. Probiotics play a role in treatment of HE by causing alterations in gut flora by decreasing the counts of pathogen bacteria, intestinal mucosal acidification, decrease in production and absorption of ammonia, alterations in permeability of gut, decreased endotoxin levels and changes in production of short chain fatty acids. Role of gut microbiota using prebiotics, probiotics and synbiotics have been evaluated in the management of minimal hepatic encephalopathy (MHE), overt HE and prevention of HE. Many studies have shown efficacy of probiotics in reduction of blood ammonia levels, treatment of MHE and prevention of HE. However these trials have problems like inclusion of small number of patients, short treatment durations, variability in HE/MHE related outcomes utilized and high bias risk, errors of systematic and random types. Systematic reviews also have shown different results with one systematic review showing clinical benefits whereas another concluded that probiotics do not have any role in treatment of MHE or HE. Also practical questions on optimal dose, ideal combination of organisms, and duration of treatment and persistence of benefits on long term follow-up are still to be clarified. At present, there are no recommendations for use of probiotics in patients with HE.

  16. Biotin deficiency in the rat as a model for reduced pyruvate carboxylase activity

    NARCIS (Netherlands)

    Schrijver, Jacobus

    1978-01-01

    The investigations described in this thesis are a contribution to the study of Leigh's disease (Subacute Necrotizing Encephalomyelopathy, SNE). SNE resembles in neuropathology Wernicke's encephalopathy, which is caused by thiamine deficiency. The scope and the purpose of the present study is given

  17. Wernicke encephalopathy in a patient with liver failure

    Science.gov (United States)

    Zhao, Pan; Zhao, Yanling; Wei, Zhenman; Chen, Jing; Yan, Lilong

    2016-01-01

    Abstract Early recognition and diagnosis of Wernicke encephalopathy is pivotal for the prognosis of this medical emergency, especially in patients with liver failure which predisposes individuals to develop hepatic encephalopathy. For these patients, distinguishing between hepatic encephalopathy and Wernicke encephalopathy is a challenge in real-world clinical practice. A male patient with 21-year medical history of liver cirrhosis presented diarrhea and ascites. One month before this visit, he was noted to have poor appetite and progressive fatigue. After admission, although several major symptoms, including diarrhea, ascites, hyponatremia, and hypoproteinemia, were greatly improved through appropriate treatments, his laboratory indicators were not changed much. His appetite was not reversed at discharge. On the 5th day after discharge, the patient suddenly became reluctant to speak and did not remember the recent happenings. Simultaneously, unsteady gait and strabismus occurred. On the basis of clinical manifestations and brain magnetic resonance imaging scan results, the patient was diagnosed as Wernicke encephalopathy and these relative symptoms were resolved after intravenous vitamin B1. To our knowledge, this is the second case report of Wernicke encephalopathy developing in a critically ill cirrhotic patient without hepatocellular carcinoma or operative intervention. Wernicke encephalopathy may be underdiagnosed in these patients and this case raises physicians’ awareness of its possible onset. PMID:27399058

  18. Role of diffusion weighted MR in the discrimination diagnosis of the cystic and/or necrotic head and neck lesions

    Energy Technology Data Exchange (ETDEWEB)

    Koc, Osman [Selcuk University, Meram Faculty of Medicine, Department of Radiology, Konya 42080 (Turkey); Paksoy, Yahya [Selcuk University, Meram Faculty of Medicine, Department of Radiology, Konya 42080 (Turkey)]. E-mail: yahyapaksoy@yahoo.com; Erayman, Ibrahim [Selcuk University, Meram Faculty of Medicine, Department of Infectious Disease, Konya (Turkey); Kivrak, Ali Sami [Selcuk University, Meram Faculty of Medicine, Department of Radiology, Konya 42080 (Turkey); Arbag, Hamdi [Selcuk University, Meram Faculty of Medicine, Department of Otolaryngology-Head and Neck Surgery, Konya (Turkey)

    2007-05-15

    Purpose: The purpose was to determine whether the diffusion weighted imaging (DWI) was able to differentiate necrotic tumor or metastatic lesions from infected necrotic lesions such as abscesses and necrotic lymphadenitis in the neck. Materials and methods: DWI was performed on 37 consecutive patients with 85 head and neck necrotic and cystic lesions. The lesions were classified into four categories: metastatic lymph node involvement including lymphoma, necrotic tumor, abscesses and necrotic lymphadenitis. Each lesion was histopathologically studied and proved. Results: In 12 patients, there were 35 necrotic lymphadenitis (necrotic tuberculosis lymphadenitis, n = 18; necrotic nonspecific suppurative lymphadenitis, n = 17). Of the 15 necrotic metastatic nodes, 11 lesions were lymphomatous involvement and 4 lesions were other tumor involvement. Other 11 patients have abscesses. Thirteen primary tumoral necrotic lesions arose in the neck of nine patients. All of the abscesses and necrotic lympadenitis showed hyperintensity on DWI, in contrast to necrotic tumor and necrotic nodal metastasis that showed hypointensity on DWI. DWI successfully differentiated metastatic nodes and necrotic tumors from necrotic lymphadenitis and abscesses. Conclusion: DWI may be supportive for differentiating necrotic tumor lesions such as necrotic tumor and metastatic necrotic nodes from the infective necrotic lesions such as necrotic lymphadenitis and abscesses in the head and neck.

  19. Necrotizing Enterocolitis: Old Problem with New Hope

    Directory of Open Access Journals (Sweden)

    Shu-Fen Wu

    2012-06-01

    Full Text Available The incidence of necrotizing enterocolitis (NEC and mortality rate associated with this disease are not decreasing despite more than three decades of intensive research investigation and advances in neonatal intensive care. Although the etiology of NEC is not clearly elucidated, the most accepted hypothesis at present is that enteral feeding in the presence of intestinal hypoxia-ischemia-reperfusion, and colonization with pathogens provokes an inappropriately accentuated inflammatory response by the immature intestinal epithelial cells of the preterm neonate. However, delayed colonization of commensal flora with dysbiotic flora with a predominance of pathologic microorganisms plays a fundamental role in the pathogenesis of NEC. Recent studies have further identified that NEC infants have less diverse flora compared to age-matched controls without NEC. Increased gastric residual volume may be an early sign of NEC. An absolute neutrophil count of <1.5 × 109/L and platelets below 100 × 109/L are associated with an increased risk for mortality and gastrointestinal morbidity. Nonspecific supportive medical management should be initiated promptly. Sudden changes in vital signs such as tachycardia or impending shock may indicate perforation. A recent meta-analysis investigating using probiotics for prevention of NEC with a total of 2176 preterm very low birth weight infants found a success rate of just 1/25. Careful monitoring of the residual volume, and of serious changes in hemograms and vital signs may help in early diagnosis and prediction of when to perform medical or early surgical intervention. In term of prevention, administration of oral probiotics containing Bifidobacterium and Lactobacillus is a simple and safe method that attempts to early establish of commensal flora balance to inhibit pathogenic flora and an inflammatory response.

  20. Epidemiology of necrotizing meningoencephalitis in Pug dogs.

    Science.gov (United States)

    Levine, J M; Fosgate, G T; Porter, B; Schatzberg, S J; Greer, K

    2008-01-01

    Although the histopathologic features of necrotizing meningoencephalitis (NME) have been described previously, little information is available concerning the signalment, geographic distribution, seasonal onset, treatment, and survival of affected dogs. Sixty Pugs with NME and 14 contemporaneous control Pugs with other intracranial diseases (non-NME group). Pugs that were euthanized or died because of intracranial disease were prospectively obtained. All dogs had necropsy, histopathology, and testing for various infectious diseases and were subsequently divided into NME and non-NME groups. Signalment, geographic distribution, seasonal onset, treatment, and survival were compared between groups. In Pugs with NME, median age at onset of clinical signs was 18 months (range, 4-113 months). A greater proportion of female dogs were present in the NME group (40/60) compared with the control group (6/14). Pugs with NME had a significantly lower mean weight (7.81 kg) than control Pugs (9.79 kg) (P= .012). Mean survival in Pugs with NME was 93 days (range, 1-680 days), with dogs receiving any form of treatment living significantly longer than those that were not treated (P= .003). Anticonvulsive drugs were the only treatment significantly associated with longer survival (P= .003). NME appears to be a common cause of intracranial signs in Pugs, based on the high proportion of NME dogs reported in this population. Pugs with NME are most commonly young adult female dogs. Although further investigation is needed to determine the optimal treatment of NME, anticonvulsive drugs appear to beneficially affect duration of survival.

  1. Necrotizing soft tissue infection in pregnancy

    Directory of Open Access Journals (Sweden)

    Nestorović Milica

    2017-01-01

    Full Text Available Introduction. Necrotizing soft tissue infection (NSTI is a life-threatening condition, characterized by widely spread necrosis of skin, subcutaneous fat, fascia and muscles. Treatment involves surgical debridement and broad-spectrum antimicrobial therapy. Mortality is still high due to diagnostic delays. NSTI is rare in general population, there are even less literature data of this condition in pregnancy. Timely diagnosis and therapy is crucial for outcome of these patients. Clinicians should have in mind NSTI in patients with perianal infections, especially in cases where immunosuppressive role of pregnancy is present. Case outline. We present a case of a 21-year-old pregnant woman with NSTI spreading from perianal region. The patient was admitted to hospital in the 31st week of otherwise healthy twin pregnancy one day after incision of perianal abscess. At admission she was examined by a gynecologist; vital signs were stable, laboratory results showed the presence of infection. She was referred for another surgical procedure and broad-spectrum antibiotics were prescribed. The next morning the patient complained of intense abdominal pain. Clinical exam revealed only discrete redness of the skin tender on palpation, crepitating. She was immediately referred to surgery. Intraoperative findings revealed massive soft tissue infection spreading up to the chest wall. Wide skin incisions and debridement were performed. The patient developed septic shock and after initial resuscitation gynecologist confirmed intrauterine death of twins and indicated labor induction. Over the next few days the patient’s general condition improved. On several occasions the wounds were aggressively debrided under general anesthesia, which left the patient with large abdominal wall defect. Twenty-three days after the initial operation, the defect was reconstructed with partial-thickness skin grafts, providing satisfactory results. Conclusion. Diagnosis and outcome of

  2. Dengue viral infections as a cause of encephalopathy

    Directory of Open Access Journals (Sweden)

    Malavige G

    2007-01-01

    Full Text Available The aim of this study was to determine the clinical characteristics and poor prognostic factors associated with high mortality in dengue encephalopathy. Fifteen patients with confirmed dengue infections, who developed encephalopathy, were recruited from two tertiary care hospitals in Colombo, Sri Lanka. Among the factors that contributed to encephalopathy were: Acute liver failure (73%, electrolyte imbalances (80% and shock (40%. Five (33.3% patients developed seizures. Disseminated intravascular coagulation was seen in five (33.3%. Secondary bacterial infections were observed in 8 (53.3% of our patients. The overall mortality rate was 47%.

  3. Current concepts in the assessment and treatment of hepatic encephalopathy.

    LENUS (Irish Health Repository)

    Cash, W J

    2012-02-01

    Hepatic encephalopathy (HE) is defined as a metabolically induced, potentially reversible, functional disturbance of the brain that may occur in acute or chronic liver disease. Standardized nomenclature has been proposed but a standardized approach to the treatment, particularly of persistent, episodic and recurrent encephalopathy associated with liver cirrhosis has not been proposed. This review focuses on the pathogenesis and treatment of HE in patients with cirrhosis. The pathogenesis and treatment of hepatic encephalopathy in fulminant hepatic failure is quite different and is reviewed elsewhere.

  4. Severe early onset ethylmalonic encephalopathy with West syndrome.

    Science.gov (United States)

    Papetti, Laura; Garone, Giacomo; Schettini, Livia; Giordano, Carla; Nicita, Francesco; Papoff, Paola; Zeviani, Massimo; Leuzzi, Vincenzo; Spalice, Alberto

    2015-12-01

    Ethylmalonic encephalopathy (EE) is a rare autosomal recessive disorder characterized by early onset encephalopathy, chronic diarrhoea, petechiae, orthostatic acrocyanosis and defective cytochrome c oxidase (COX) in muscle and brain. High levels of lactic, ethylmalonic and methylsuccinic acids are detected in body fluids. EE is caused by mutations in ETHE1 gene, a mitochondrial sulfur dioxygenase. Neurologic signs and symptoms include progressively delayed development, hypotonia, seizures, and abnormal movements. We report on the clinical, electroencephalographic and MRI findings of a baby with a severe early onset encephalopathy associated with novel ETHE1 gene mutation. This is the first case described in literature with an early pure epileptic onset, presenting with West syndrome.

  5. Isolated Brainstem Involvement in a Patient with Hypertensive Encephalopathy

    Directory of Open Access Journals (Sweden)

    Y. Osman

    2013-01-01

    Full Text Available Hypertensive encephalopathy typically presents with headache, confusion, and bilateral parietooccipital vasogenic edema. Brainstem edema in hypertensive encephalopathy usually occurs in association with typical supratentorial parieto-occipital changes and is usually asymptomatic. We report here a patient with hypertensive encephalopathy, with isolated brain stem involvement on magnetic resonance imaging (MRI. Rapid treatment of hypertension resulted in clinical and radiological improvement. Prompt recognition of the condition and aggressive treatment of hypertension in such patients is crucial to relieve edema and prevent life-threatening progression.

  6. Hyperfixation of Tc-99m ECD in subacute cortical infarction

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Jae Seung; Kweon, Sun Uck; Ryu, Jin Sook; Moon, Dae Hyuk; Lee, Hee Kyung [College of Medicine, Ulsan Univ., Seoul (Korea, Republic of)

    2001-07-01

    It has been known that hyperfixation of Tc-99m ECD (HF) is not shown in subacute cerebral infarction because the brain distribution of Tc-99m ECD reflects not only perfusion but also the metabolic status of brain tissue. However, we observed several cases with HF in the subacute pure cortical infarction. To find out the cause of HF in subacute cortical infarction. We assessed the difference in associated cerebral hemodynamics and clinical findings between the subacute cortical infarctions with and without HF. We reviewed 16 patients (63.8{+-}8.6 yr, M/F: 15/1) with pure cortical infarction not involving adjacent subcortical white matter on MRI. All patients underwent acetazolamide stress brain perfusion SPECT using Tc-99m ECD and MRI at subacute period (7.3{+-}4.4 days from ictus). Uptake of Tc-99m ECD in infarcted cortex was assessed visually comparing the contralateral side. To assess the difference in associate clinical findings between the infarctions with and without HF, rCVR of the cerebral territory including infarcted cortex, extent of Gd-enhancement on MRI. Intervals between SPECT and ictus, and the presence of associated ICA stenosis were evaluated. Infarctions were focal (n=8) or multifocal (n=8) and located in frontoparietal cortices on MRI. Twelve patients were accompanied with ipsilateral ICA stenosis. Resting SPECT showed increased cortical uptake (=HF) in 7 patients and decreased in 9. rCVR of the MCA territory was preserved in all of the 7 patients with HF, compared with 4 of the 9 patients without HF (p=0.03). Gd-enhancement was minimal in all of the 7 patients with HF, compared with of the 0 patients without HF (p=0.03). Presence of ipsilateral ICA stenosis and intervals from ictus were not different (p>0.1) Subacute cerebral cortical infarction with HF was more frequently associated with preserved rCVR and minimal destruction of the blood-brain barrier than that without HF. Our findings suggest that HF may result from luxury perfusion of

  7. Chronic traumatic encephalopathy: contributions from the Boston University Center for the Study of Traumatic Encephalopathy.

    Science.gov (United States)

    Riley, David O; Robbins, Clifford A; Cantu, Robert C; Stern, Robert A

    2015-01-01

    Chronic Traumatic Encephalopathy (CTE) is a neurodegenerative disease associated with repetitive brain trauma (RBT). Initially described in boxers, CTE has now been found in other contact sport athletes with a history of RBT. In recent years, there has been tremendous media attention regarding CTE, primarily because of the deaths of high profile American football players who were found to have CTE upon neuropathological examination. However, the study of CTE remains in its infancy. This review focuses on research from the Centre for the Study of Traumatic Encephalopathy (CSTE) at Boston University. This study reviews the formation of the CSTE, major CSTE publications and current ongoing research projects at the CSTE. The neuropathology of CTE has been well-described. Current research focuses on: methods of diagnosing the disease during life (including the development of biomarkers), examination of CTE risk factors (including genetic susceptibility and head impact exposure variables); description of the clinical presentation of CTE; development of research diagnostic criteria for Traumatic Encephalopathy Syndrome; and assessment of mechanism and pathogenesis. Current research at the BU CSTE is aimed at increasing understanding of the long-term consequences of repetitive head impacts and attempting to begin to answer several of the unanswered questions regarding CTE.

  8. A Case of Valproate Induced Hyperammonemic Encephalopathy

    Directory of Open Access Journals (Sweden)

    Surjit Tarafdar

    2011-01-01

    Full Text Available A 36-years-old man on phenytoin, levetiracetam, and sodium valproate presented with acute confusion. Routine investigations including serum valproate and phenytoin concentration were normal. His serum ammonia concentration was raised. His valproate was held and 2 days later he recovered with concordant normalisation of serum ammonia concentration. Urea acid cycle disorder was ruled out, and a diagnosis of valproate induced hyperammonemic encephalopathy (VHE was made. Asymptomatic hyperammonemia occurs in 15–50% of valproate-treated patients, and while the true incidence of VHE is not known, it is a recognized complication of sodium valproate treatment. VHE typically presents acutely with impaired consciousness, lethargy, and vomiting. Valproate concentrations may be in the therapeutic range, and liver function tests are typically “normal.” Treatment for VHE consists of ceasing valproate and providing supportive care. Some have advocated carnitine replacement.

  9. Chronic Traumatic Encephalopathy: The Impact on Athletes.

    Science.gov (United States)

    Galgano, Michael A; Cantu, Robert; Chin, Lawrence S

    2016-03-14

    Chronic traumatic encephalopathy (CTE) is a devastating neuropsychological condition afflicting a small percentage of athletes partaking in high-impact sports. The onset of symptoms lags years behind the inciting events. Repetitive minor head injuries are felt to be the main etiology behind CTE. Routine radiographic imaging generally is unremarkable in cases of CTE. Functional magnetic resonance imaging (fMRI), magnetic resonance spectroscopy (MRS), and diffusion tensor imaging (DTI) are advanced MRI-based sequences that have shown promise in detecting early radiographic findings that may be reflective of CTE. Progressive neuronal loss is the histopathological hallmark of this neurodegenerative disease. Strategizing earlier detection techniques is paramount in delivering optimal care to athletes afflicted with CTE.

  10. Transcranial electrostimulation in patients with alcoholic encephalopathy

    Directory of Open Access Journals (Sweden)

    Barylnik Yu.B.

    2010-09-01

    Full Text Available The method of transcranial electrostimulation (TES was used for treating patients with alcoholic encephalopathy against the background of the basic treatment, which includes nootropics, normotimics, soporifics, over-all strengthening therapy and other devices. The course of treatment consisted of 10 daily procedures lasting for 30 minutes. The TES influence was evaluated according to the clinical state, the neurologic status, including EEG (electroencephalogram, the psychometric scales were also used for evaluating the manifestation of depression, anxiety and working memory in comparison with appropriate indices in the control group of patients, who were being treated by the traditional method. TES led to normalization of health state, neurologic status and vegetative innervation, the reduction in pathologic inclination, which corresponded to general improvement of the state of patients, EEG indices and psychometric scales

  11. Brain MRI findings in Wernicke encephalopathy.

    Science.gov (United States)

    Wicklund, Meredith R; Knopman, David S

    2013-08-01

    A 71-year-old woman with myelofibrosis on chemotherapy experienced an acute illness with nausea, vomiting, and diarrhea. Two weeks later, she developed an acute confusional state characterized by disorientation and fluctuating alertness with normal speech and language. Her neurologic examination demonstrated an upper motor neuron pattern of right hemiparesis. She reported double vision though ophthalmoparesis was not appreciated. Her gait was normal. While hospitalized, she developed generalized tonic-clonic seizures. Brain MRI revealed a small area of restricted diffusion of the left precentral gyrus (figure). She was diagnosed with a stroke with secondary seizures; however, as the confusional state resolved, she developed profound retrograde and anterograde amnesia. Review of the brain MRI showed high T2 signal in the medial thalamus and contrast enhancement of the mamillary bodies; a diagnosis of Wernicke-Korsakoff syndrome was entertained and she was started on thiamine replacement. The encephalopathy and hemiparesis resolved though she remains severely amnestic.

  12. Does this patient have hypertensive encephalopathy?

    Science.gov (United States)

    Christopoulou, Foteini; Rizos, Evangelos C; Kosta, Paraskevi; Argyropoulou, Maria I; Elisaf, Moses

    2016-05-01

    A 63-year-old man was admitted to our hospital for further investigation and management of brain metastases. The patient was initially presented with a 4-day history of confusion. On the day of admission, the patient was confused, agitated, disorientated in place and time, and had visual disturbances. His blood pressure was repeatedly recorded high, with levels of systolic blood pressure between 170-210 mm Hg. A brain magnetic resonance imaging showed areas of high signal on T2 and fluid-attenuated inversion recovery images, located bilaterally in the white matter of the occipital regions and unilateral in the left frontal lobe, suggestive of posterior reversible encephalopathy syndrome. Aggressive treatment of hypertension resulted in complete resolution of the clinical and radiologic features of the syndrome. Copyright © 2016 American Society of Hypertension. Published by Elsevier Inc. All rights reserved.

  13. Wernicke encephalopathy and Creutzfeldt-Jakob disease.

    Science.gov (United States)

    Bertrand, A; Brandel, J P; Grignon, Y; Sazdovitch, V; Seilhean, D; Faucheux, B; Privat, N; Brault, J L; Vital, A; Uro-Coste, E; Pluot, M; Chapon, F; Maurage, C A; Letournel, F; Vespignani, H; Place, G; Degos, C F; Peoc'h, K; Haïk, S; Hauw, J J

    2009-06-01

    We assessed the prevalence of Wernicke encephalopathy (WE) in all 657 cases suspected of Creutzfeldt-Jakob (CJD) referred from 2001 to 2006 to the French Neuropathology Network of CJD. Clinical, biological and imaging data were reviewed when the diagnosis of WE was made at autopsy. No CJD was found in five cases suspected of sporadic CJD. In these five cases, myoclonus had been observed in four, CSF 14-3-3 protein in two. In 14 other cases, WE was combined with CJD, 13 of which were sporadic. These belonged mainly to the molecular variants of sporadic CJD associated with a long duration of disease. This stresses the necessity of remaining alert to the diagnosis of WE when CJD is suspected.

  14. Is chronic traumatic encephalopathy a real disease?

    Science.gov (United States)

    Randolph, Christopher

    2014-01-01

    Chronic traumatic encephalopathy (CTE) has received widespread media attention and is treated in the lay press as an established disease, characterized by suicidality and progressive dementia. The extant literature on CTE is reviewed here. There currently are no controlled epidemiological data to suggest that retired athletes are at increased risk for dementia or that they exhibit any type of unique neuropathology. There remain no established clinical or pathological criteria for diagnosing CTE. Despite claims that CTE occurs frequently in retired National Football League (NFL) players, recent studies of NFL retirees report that they have an all-cause mortality rate that is approximately half of the expected rate, and even lower suicide rates. In addition, recent clinical studies of samples of cognitively impaired NFL retirees have failed to identify any unique clinical syndrome. Until further controlled studies are completed, it appears to be premature to consider CTE a verifiable disease.

  15. Posterior reversible encephalopathy syndrome: A case report

    Directory of Open Access Journals (Sweden)

    Kostić Dejan

    2015-01-01

    Full Text Available Posterior reversible encephalopathy syndrome (PRES is characterized by the following symptoms: seizures, impaired consciousness and/or vision, vomiting, nausea, and focal neurological signs. Diagnostic imaging includes examination by magnetic resonance (MR and computed tomography (CT, where brain edema is visualized bi-laterally and symmetrically, predominantly posteriorly, parietally, and occipitally. Case report. We presented a 73-year-old patient with the years-long medical history of hipertension and renal insufficiency, who developed PRES with the symptomatology of the rear cranium. CT and MR verified changes in the white matter involving all lobes on both sides of the brain. After a two-week treatment (antihypertensive, hypolipemic and rehydration therapy clinical improvement with no complications occurred, with complete resolution of changes in the white matter observed on CT and MR. Conclusion. PRES is a reversible syndrome in which the symptoms withdraw after several days to several weeks if early diagnosis is made and appropriate treatment started without delay.

  16. Subacute thyroiditis (de Quervain) presenting as a painless cold nodule

    Energy Technology Data Exchange (ETDEWEB)

    Bartels, P.C.; Boer, R.O.

    1987-09-01

    A 49-yr-old woman presented with a solid, painless, nontender nodule in the left thyroid lobe. Thyroid scintigraphy revealed a solitary cold area in the left lobe and a slightly decreased 24-hr radioactive iodine thyroid uptake (9%). Although there were no specific clinical or biochemical signs suggesting thyroiditis needle aspiration cytology showed the presence of a subacute thyroiditis. Approximately 1 mo later the entire thyroid gland was affected leading to a completely suppressed thyroid radioiodine uptake and elevated serum thyroid hormone concentrations. This case illustrates that in the early phase of the disease, subacute thyroiditis may present as a solitary, painless, cold nodule and should be considered in the differential diagnosis of such lesions.

  17. Measles-vaccinated Israeli boy with subacute sclerosing panencephalitis.

    Science.gov (United States)

    Har-Even, Ronly; Aichenbaum, Sergio; Rabey, Jose M; Livne, Amir; Bistritzer, Tzvy

    2011-06-01

    Subacute sclerosing panencephalitis is a rare neurologic disorder of childhood and adolescence. We describe a 16-year-old boy who manifested the disease despite proper vaccinations. He was hospitalized because of bedwetting, involuntary limb movements, abnormal speech, and balance disturbances. Immunoglobulin G antibodies against measles were strongly positive, with a high relative cerebrospinal fluid/serum ratio. Polymerase chain reaction for measles produced negative results. Electroencephalography registered slow activity with high voltage discharges every few seconds, and with triphasic complex morphology. Magnetic resonance imaging revealed diffuse white matter changes, mostly around the posterior regions and lateral ventricles. Treatment with valproic acid, levetiracetam, carbamazepine, and intravenous immunoglobulin G was ineffective. Inosiplex and interferon-β-1a were also administrated. The patient became comatose, with generalized myoclonic jerks, and died 1 year later. An autopsy was not performed. This patient illustrates that subacute sclerosing panencephalitis should be suspected among young vaccinated subjects. Copyright © 2011 Elsevier Inc. All rights reserved.

  18. Quantitative Risk Assessment of Bovine Spongiform Encephalopathy

    Science.gov (United States)

    Tsutsui, Toshiyuki; Kasuga, Fumiko

    Bovine spongiform encephalopathy (BSE) is a progressive neurological disease of cattle affecting the central nervous system and was first diagnosed in the United Kingdom (UK) in 1986 (Wells et al., 1987). This disease is one of the transmissible spongiform encephalopathy (TSE) which includes Creutzfeldt-Jakob disease (CJD) in humans and scrapie in sheep. The causative agent of TSE is considered to be an abnormal form of prion protein. However, the details of its pathogenic mechanism have not been fully identified. Scrapie, which causes neurological symptoms in sheep and goats, has existed in the UK for 200 years (Hoinville, 1996) and spread across the rest of the world in the 1900s (Detwiler & Baylis, 2003). There has been no report so far that scrapie can be transmitted to humans. Initially, BSE was also considered as a disease affecting only animals. However, a variant type of Creutzfeldt-Jakob disease (vCJD) was first reported in the UK, and exposure to a BSE agent was suspected (Collinge, Sidle, Meads, Ironside, & Hill, 1996). vCJD is clinically and pathologically different from the sporadic type of CJD, and age at clinical onset of vCJD is younger than sporadic type (Will et al., 1996). Since the UK government announced the possible association between BSE and vCJD in 1996, BSE has become a huge public health concern all over the world. Of particular concern about vCJD, the fatal disease in younger age, distorted consumer confidence in beef safety, and as a result reduced beef consumption has been seen in many BSE-affected countries.

  19. Subacute Sclerosing Panencephalitis in a Child with Human Immunodeficiency Virus Co-Infection.

    Science.gov (United States)

    Maurya, Pradeep Kumar; Thakkar, Mayur Deepak; Kulshreshtha, Dinkar; Singh, Ajai Kumar; Thacker, Anup Kumar

    2016-12-01

    Subacute sclerosing panencephalitis is a fatal infectious disease of childhood caused by persistence of the measles virus in the brain. The effect of human immunodeficiency virus (HIV) co-infection on subacute sclerosing panencephalitis remains elusive and rare. We report a child who developed subacute sclerosing panencephalitis following a short latency period and a rapidly progressive course with HIV co-infection. © 2016 Marshfield Clinic.

  20. Two successive necrotic lesions secondary to presumed loxosceles envenomation.

    Science.gov (United States)

    Tarullo, David B; Jacobsen, Ryan C; Algren, D Adam

    2013-06-01

    Brown recluse spider (Loxosceles reclusa) envenomations with subsequent necrotic skin lesions occur infrequently, and systemic loxoscelism is rarer still. We report a case of 2 successive developing necrotic lesions, each on adjacent medial aspects of the legs, secondary to presumed Loxosceles envenomation. A 31-year-old man with no significant past medical history presented to the emergency department with 2, large, necrotic lesions, 1 on each medial thigh. They had progressed over the course of 1 month from small blisters to large necrotic lesions with eschar. He underwent surgical debridement without skin grafting with no further complications. Bites from recluse spiders that progress to necrosis usually present as single lesions. The differential diagnoses for a necrotic skin lesion is large. The presence of more than 1 lesion argues against Loxosceles envenomation; however, in the absence of underlying infection, systemic diseases, immunodeficiency, or malignancy, the diagnosis must be considered if the case presents in an endemic area. Brown recluse spiders rarely bite multiple times, thus confounding the diagnosis of an already nonspecific clinical finding. Copyright © 2013 Wilderness Medical Society. Published by Elsevier Inc. All rights reserved.

  1. Encephalopathy in Wilson disease: copper toxicity or liver failure?

    National Research Council Canada - National Science Library

    Ferenci, Peter; Litwin, Tomasz; Seniow, Joanna; Czlonkowska, Anna

    2015-01-01

    Hepatic encephalopathy (HE) is a complex syndrome of neurological and psychiatric signs and symptoms that is caused by portosystemic venous shunting with or without liver disease irrespective of its etiology...

  2. Uremic encephalopathy and other brain disorders associated with renal failure.

    Science.gov (United States)

    Seifter, Julian Lawrence; Samuels, Martin A

    2011-04-01

    Kidney failure is one of the leading causes of disability and death and one of the most disabling features of kidney failure and dialysis is encephalopathy. This is probably caused by the accumulation of uremic toxins. Other important causes are related to the underlying disorders that cause kidney failure, particularly hypertension. The clinical manifestations of uremic encephalopathy include mild confusional states to deep coma, often with associated movement disorders, such as asterixis. Most nephrologists consider cognitive impairment to be a major indication for the initiation of renal replacement therapy with dialysis with or without subsequent transplantation. Sleep disorders, including Ekbom's syndrome (restless legs syndrome) are also common in patients with kidney failure. Renal replacement therapies are also associated with particular neurologic complications including acute dialysis encephalopathy and chronic dialysis encephalopathy, formerly known as dialysis dementia. The treatments and prevention of each are discussed. © Thieme Medical Publishers.

  3. Hepatic encephalopathy in acute-on-chronic liver failure.

    Science.gov (United States)

    Lee, Guan-Huei

    2015-10-01

    The presence of hepatic encephalopathy (HE) within 4 weeks is part of the criteria for defining acute-on-chronic liver failure (ACLF). The pathophysiology of HE is complex, and hyperammonemia and cerebral hemodynamic dysfunction appear to be central in the pathogenesis of encephalopathy. Recent data also suggest that inflammatory mediators may have a significant role in modulating the cerebral effect of ammonia. Multiple prospective and retrospective studies have shown that hepatic encephalopathy in ACLF patients is associated with higher mortality, especially in those with grade III-IV encephalopathy, similar to that of acute liver failure (ALF). Although significant cerebral edema detected by CT in ACLF patients appeared to be less common, specialized MRI imaging was able to detect cerebral edema even in low grade HE. Ammonia-focused therapy constitutes the basis of current therapy, as in the treatment of ALF. Emerging treatment strategies focusing on modulating the gut-liver-circulation-brain axis are discussed.

  4. Posterior reversible encephalopathy syndrome: An atypical postpartum complication

    National Research Council Canada - National Science Library

    Paul, Debashish; Kulkarni, SachinNarayan; Choudhury, MiliDas; Maity, GD

    2016-01-01

    Posterior reversible encephalopathy syndrome (PRES) is presented by headache, altered mental status, blurring of vision, vomiting and seizure in conjunction with radiological finding of posterior cerebral white matter edema...

  5. Safety, efficacy, and patient acceptability of rifaximin for hepatic encephalopathy

    DEFF Research Database (Denmark)

    Kimer, Nina; Krag, Aleksander; Gluud, Lise L

    2014-01-01

    Hepatic encephalopathy is a complex disease entity ranging from mild cognitive dysfunction to deep coma. Traditionally, treatment has focused on a reduction of ammonia through a reduced production, absorption, or clearance. Rifaximin is a nonabsorbable antibiotic, which reduces the production of ...... and safety of long-term treatment with rifaximin and evaluate effects of combination therapy with lactulose and branched-chain amino acids for patients with liver cirrhosis and hepatic encephalopathy....... of ammonia by gut bacteria and, to some extent, other toxic derivatives from the gut. Clinical trials show that these effects improve episodes of hepatic encephalopathy. A large randomized trial found that rifaximin prevents recurrent episodes of hepatic encephalopathy. Most patients were treated...

  6. Gene Panel Testing in Epileptic Encephalopathies and Familial Epilepsies

    DEFF Research Database (Denmark)

    Møller, Rikke S.; Larsen, Line H.G.; Johannesen, Katrine M.

    2016-01-01

    to epileptic encephalopathies (EEs). Potentially causative variants were evaluated by literature and database searches, submitted to bioinformatic prediction algorithms, and validated by Sanger sequencing. If possible, parents were included for segregation analysis. We identified a presumed disease...

  7. [Follow-up of newborns with hypoxic-ischaemic encephalopathy].

    Science.gov (United States)

    Martínez-Biarge, M; Blanco, D; García-Alix, A; Salas, S

    2014-07-01

    Hypothermia treatment for newborn infants with hypoxic-ischemic encephalopathy reduces the number of neonates who die or have permanent neurological deficits. Although this therapy is now standard of care, neonatal hypoxic-ischaemic encephalopathy still has a significant impact on the child's neurodevelopment and quality of life. Infants with hypoxic-ischaemic encephalopathy should be enrolled in multidisciplinary follow-up programs in order to detect impairments, to initiate early intervention, and to provide counselling and support for families. This article describes the main neurodevelopmental outcomes after term neonatal hypoxic-ischaemic encephalopathy. We offer recommendations for follow-up based on the infant's clinical condition and other prognostic indicators, mainly neonatal neuroimaging. Other aspects, such as palliative care and medico-legal issues, are also briefly discussed. Copyright © 2013 Asociación Española de Pediatría. Published by Elsevier Espana. All rights reserved.

  8. Paracoccidioidomycosis: acute-subacute clinical form, juvenile type*

    Science.gov (United States)

    Marques, Silvio Alencar; Lastória, Joel Carlos; de Camargo, Rosangela Maria Pires; Marques, Mariangela Esther Alencar

    2016-01-01

    The authors report aspects of paracoccidioidomycosis, acute-subacute clinical form, juvenile type, in a 19-year-old female patient. Paracoccidioidomycosis, juvenile type, classically occurs in young patients, both sexes, with lymphoma-like aspects as initial presentation. However, following the natural history of the disease the lymph nodes assume patterns of infectious disease, as an abscess and fistulae. Systemic dissemination of the disease can occur and lethality and morbidity are significant in this clinical presentation. PMID:27438214

  9. Herbal hepatitis: subacute hepatic necrosis secondary to chaparral leaf.

    Science.gov (United States)

    Katz, M; Saibil, F

    1990-04-01

    A 33-year-old woman developed subacute hepatic necrosis after several months of ingestion of Chaparral Leaf, an herbal product. Symptoms first appeared 3 months after she began taking the tablets. The patient unwittingly carried out a clinical challenge by reducing, then increasing, the dose of pills. The public and the medical profession must be wary of all "harmless" nonprescription medications, whether purchased in pharmacies or elsewhere.

  10. Visual deterioration as presentation of subacute sclerosing panencephalitis.

    Science.gov (United States)

    Johnston, H M; Wise, G A; Henry, J G

    1980-01-01

    A 7-year-old boy presented with deteriorating vision and macular degenerative changes. A month later he had developed unusual behaviour and increasing forgetfulness. An electroencephalogram showing periodic complexes, and high measles complement-fixation titres in the cerebrospinal fluid and blood, confirmed the diagnosis of subacute sclerosing panencephalitis. Four months after the onset of visual symptoms he started having myoclonic jerks. Images Fig. 1 Fig. 2 PMID:7436466

  11. Subacute sclerosing panencephalitis. Changes on CT scan during acute relapse

    Energy Technology Data Exchange (ETDEWEB)

    Modi, G.; Bill, P. (Wentworth Hospital, Durban (South Africa). Dept. of Medicine); Campbell, H. (Wentworth Hospital, Durban (South Africa). Dept. of Radiology)

    1989-11-01

    A 19-year-old female patient presented in an acute state of akinetic mutism. Serological analysis of serum and cerebrospinal fluid demonstrated the presence of antibodies to measles virus. CT scan carried out during this acute phase of relapse demonstrated white matter enhancement affecting the cortical white matter of the frontal lobes and corpus callosum. These features indicate that active demyelination occurs during acute relapse in subacute sclerosing panencephalitis (SSPE) and suggest that immunotherapy should be considered during this acute phase. (orig.).

  12. Antibodies to borna disease virus in subacute sclerosing panencephalitis.

    Science.gov (United States)

    Güngör, Serdal; Anlar, Banu; Turan, Nuri; Yilmaz, Hüseyin; Helps, Chris R; Harbour, Dave A

    2005-09-01

    Mechanisms causing persistence and reactivation of measles virus in subacute sclerosing panencephalitis (SSPE) are unknown. Borna disease virus (BDV) frequently causes latent or persistent infection in the nervous system. We investigated a possible association of these viruses in SSPE. Although BDV seropositivity was similar in SSPE and control groups, SSPE patients with high antibodies to BDV had earlier and more rapid disease. The findings suggest that BDV might be involved in the course, but not in the etiopathogenesis, of SSPE.

  13. Subacute osteoporotic compression fracture: Misleading magnetic resonance appearance

    Energy Technology Data Exchange (ETDEWEB)

    Frager, D.; Elkin, C.; Swerdlow, M.; Bloch, S.

    1988-03-01

    Three patients with benign subacute osteoporotic vertebral compression fractures are presented. T/sub 1/ weighted magnetic resonance (MR) images (SE 500/30) showed decreased vertebral signal. Because the results of the MR examination were thought to indicate malignant disease, extensive medical workups, including one biopsy, were pursued in all three patients. Routine (SE 500/30) spin-echo pulse sequences cannot definitively distinguish between benign and malignant vertebral compression fractures.

  14. Subacute bacterial endocarditis (SBE due to Streptococcus gordonii

    Directory of Open Access Journals (Sweden)

    Raffaella Battista

    2009-12-01

    Full Text Available Endocarditis is an inflammatory state of the endothelium that promotes thrombus formation and tissue damage on the surface of heart valves. Recent studies have reported endocarditis mortality rates ranging from 12% to 46% (2008. The Streptococcus gordonii is a normal inhabitant of the human oral cavity. It is a component of the microbial communities responsible of plaque formation, associated with dental caries and also regarded as the main causative agent in the development of subacute bacterial endocarditis (SBE.

  15. Protease production by Streptococcus sanguis associated with subacute bacterial endocarditis.

    OpenAIRE

    Straus, D. C.

    1982-01-01

    A viridans streptococcus (Streptococcus sanguis biotype II) isolated from the blood of a patient with subacute bacterial endocarditis was examined for protease production. In broth culture, extracellular proteolytic enzymes were not produced by this organism until after the early exponential phase of growth, with maximal protease production occurring during the stationary phase. Four distinct proteases were isolated and purified from the supernatant fluids of stationary-phase cultures, employ...

  16. Mealtime interruptions, assistance and nutritional intake in subacute care.

    Science.gov (United States)

    Porter, Judi; Wilton, Anita; Collins, Jorja

    2016-09-01

    Protected mealtimes is an initiative to support increased nutritional intake for all hospitalised patients, particularly those who are malnourished. The increased focus on maximising independence of patients in the subacute setting may provide a supportive environment for implementing these strategies. The aim of the present study was to gain insight into subacute ward practices at mealtimes under usual conditions (i.e. at baseline) where no protected mealtimes policy was implemented. Participants were patients aged ≥65 years recruited from subacute care facilities at a large healthcare network in Victoria, Australia. Participants were observed at mealtimes and mid meals (i.e. morning tea, afternoon tea and supper) to determine daily energy and protein intake, provision of mealtime assistance and mealtime interruptions. Almost all participants received assistance when it was needed, with positive and negative interruptions experienced by 56.2% and 76.2% of participants, respectively. There was an energy deficit of approximately 2 MJ per day between average intake and estimated requirements. In conclusion, mealtime practices were suboptimal, with particularly high rates of negative interruptions. Protected mealtimes is one strategy that may improve the mealtime environment to support patients' dietary intake. Prospective studies are needed to evaluate its implementation and effects.

  17. EFFECT OF POSITIONAL RELEASE TECHNIQUE IN SUBJECTS WITH SUBACUTE TRAPEZITIS

    Directory of Open Access Journals (Sweden)

    Sweety Charles Carvalho

    2014-06-01

    Full Text Available Background: Trapezius stretching combined with positional release technique (PRT have found effective in trapezitis, studies are limited to find which technique has shown effective over the other due to lack of control group. The purpose of the study is to find the effect of PRT on pain intensity, functional disability and range of motion in subjects with subacute trapezitis. Method: An experimental study design, selected subjects with subacute trapezitis was randomized into Study and Control group. Total 40 subjects, 20 subjects in each group; data was collected who completed the study. Control group received passive trapezius muscle stretching while Study group received positional release technique with passive trapezius muscle stretching for 8 sessions in 2 weeks. Outcome measurements such as Visual Analogue scale, Neck disability index and cervical Range of motion were measured. Results: There is statistically significant difference (p<0.05 showing improvement in means of VAS, NDI and Range of motion before and after intervention within the groups and there is statistically significant difference when the post-intervention means after 2 weeks of treatment were compared between Study and Control group. Conclusion: It is concluded that the Positional Release Technique with trapezius stretching found to be significantly more added effect than trapezius stretching alone in improving pain, functional disability and cervical movements for subjects with subacute trapezitis.

  18. Cerebrospinal fluid nitric oxide levels in subacute sclerosing panencephalitis.

    Science.gov (United States)

    Yilmaz, Deniz; Yüksel, Deniz; Senbil, Nesrin; Eminzade, Sude; Kilinç, Kamer; Anlar, Banu; Gürer, Yavuz

    2009-09-01

    Oxidative damage plays a role in neurodegenerative diseases. Levels of cerebrospinal fluid nitrite and nitrate levels (oxidation products that provide an indirect estimation of nitric oxide) were investigated in relation to clinical and laboratory features in subacute sclerosing panencephalitis (n = 47) and age-matched control (n = 43) groups. Significantly decreased levels of nitrite (median, 4.91 micromol/L) and nitrate (median, 6.14 micromol/L) were found in the patients. Nitrite and nitrate levels did not correlate with clinical or laboratory findings, except for presence of myoclonus. Cerebrospinal fluid nitrite levels of subacute sclerosing panencephalitis patients without myoclonic jerks were significantly higher than in those with myoclonus (median, 15.63 vs 4.34 micromol/L, respectively). The higher levels of nitrite in these patients can be explained by short disease duration and early stages of disease. Nitrate levels in subacute sclerosing panencephalitis patients with myoclonus (median, 9.26 micromol/L) were higher than in those without myoclonus (median, 4.25 micromol/L). Microbleeding resulting in conversion of nitrite to nitrate and increased production of superoxide can be suggested as possible mechanisms underlying these findings.

  19. Masticatory function in subacute TMD patients before and after treatment.

    Science.gov (United States)

    Pereira, L J; Steenks, M H; de Wijer, A; Speksnijder, C M; van der Bilt, A

    2009-06-01

    Masticatory function can be impaired in temporomandibular disorders (TMDs) patients. We investigated whether treatment of subacute non-specific TMD patients may influence oral function and clinical outcome measures. Fifteen patients with subacute TMD participated in the study. We quantified masticatory performance, maximum voluntary bite force, muscle activity and chewing cycle duration before and after treatment. Masticatory performance and bite force of patients were compared with the results obtained for an age- and gender-matched group of subjects without TMD complaints. Furthermore, we determined possible changes in anamnestic and clinical scores from questionnaires (mandibular function impairment questionnaire; MFIQ), pain scores and clinical outcome measures. Maximum bite force significantly increased, although the values after treatment were still significantly lower than those of the subjects without TMD complaints. The corresponding electromyography values did not show significant change after treatment. The masticatory performance of the patients remained unaltered; patients chewed significantly less efficient than controls. The average duration of chewing cycles significantly decreased after treatment. We observed a significant improvement in MFIQ scores. During the clenching and chewing tasks, the visual analogue scale scores were significantly higher than before these tasks. We may conclude that subacute temporomandibular joint disorders negatively influence chewing behaviour. Bite force, chewing cycle duration and also perceived mandibular function significantly improved after treatment, although the masticatory performance remained unaltered.

  20. Hypothesis of interference to superinfection between bovine spastic paresis and bovine spongiform encephalopathy; suggestions for experimentation, theoretical and practical interest.

    Science.gov (United States)

    Ledoux, Jean-Marie

    2004-01-01

    Sub-acute transmissible spongiform encephalopathies (TSEs) or prion diseases are diseases of little known etiology. The origin of these diseases would appear to be an abnormal protease-resistant prion protein (PrP(res)) which would be infectious by directly inducing its defective conformation to the normal native protein (PrP(C)). This hypothesis does not account for certain aspects of TSEs, such as interference to superinfection: in laboratory animals, inoculation by means of an attenuated strain with a long incubation period protects against later infection by a very virulent strain with a short incubation period. The hypothesis is put forward that there exists a possibility of interference to superinfection between neurodegenerative diseases of unknown origin, thought to be similar to TSEs, and a later infection by a TSE. The study of this interference between bovine spastic paresis (BSP) and bovine spongiform encephalopathy (BSE) could be used as a model for this hypothesis. BSP is a very rare disease among cattle, of unknown etiology; it is curable, in the very early stages, by using tryptophan and especially lithium, potentiated by copper and manganese. An etiology close to that of TSEs has been suggested on several occasions. If interference could be demonstrated between BSP and BSE, interesting data would be provided concerning the etiology, the pathogenesis and possibly the treatment and prevention of these diseases. Notably, such data could lead to the development of a treatment and a prevention with lithium and amino acids precursors of neuromediators (tryptophan, tyrosine, glutamic acid, etc.), as well as the developing of a vaccine to combat TSEs, especially BSE and scrapie.

  1. Branched-chain amino acids for people with hepatic encephalopathy

    DEFF Research Database (Denmark)

    Gluud, Lise Lotte; Dam, Gitte; Les, Iñigo

    2017-01-01

    -chain amino acids (BCAA) versus control interventions has evaluated if BCAA may benefit people with hepatic encephalopathy. Objectives: To evaluate the beneficial and harmful effects of BCAA versus any control intervention for people with hepatic encephalopathy. Search methods: We identified trials through...... included randomised clinical trials, irrespective of the bias control, language, or publication status. Data collection and analysis: The authors independently extracted data based on published reports and collected data from the primary investigators. We changed our primary outcomes in this update...

  2. Early Recognition of Chronic Traumatic Encephalopathy Through FDDNP PET Imaging

    Science.gov (United States)

    2017-10-01

    characteristic distribution is felt to be the cardinal pathologic feature of Chronic Traumatic Encephalopathy. This project will examine whether FDDNP PET...chronic traumatic encephalopathy (CTE). Pathological series have indicated that a characteristic feature of CTE is accumulation of tau protein in the...with age . Table 1 - Regional uptake in ROIs with Age , Years of Pro Fighting, and Number of Pro Fights (Pearson’s correlations; ns – non significant

  3. Diagnosis and Management of Epileptic Encephalopathies in Children

    Directory of Open Access Journals (Sweden)

    Puneet Jain

    2013-01-01

    Full Text Available Epileptic encephalopathies refer to a group of disorders in which the unremitting epileptic activity contributes to severe cognitive and behavioral impairments above and beyond what might be expected from the underlying pathology alone, and these can worsen over time leading to progressive cerebral dysfunction. Several syndromes have been described based on their electroclinical features (age of onset, seizure type, and EEG pattern. This review briefly describes the clinical evaluation and management of commonly encountered epileptic encephalopathies in children.

  4. Early progressive encephalopathy in boys and MECP2 mutations.

    Science.gov (United States)

    Kankirawatana, P; Leonard, H; Ellaway, C; Scurlock, J; Mansour, A; Makris, C M; Dure, L S; Friez, M; Lane, J; Kiraly-Borri, C; Fabian, V; Davis, M; Jackson, J; Christodoulou, J; Kaufmann, W E; Ravine, D; Percy, A K

    2006-07-11

    MECP2 mutations mainly occur in females with Rett syndrome. Mutations have been described in 11 boys with progressive encephalopathy: seven of nine with affected sisters and two de novo. The authors report four de novo occurrences: three pathogenic and one potentially pathogenic. Common features include failure to thrive, respiratory insufficiency, microcephaly, and abnormal motor control. MECP2 mutations should be assessed in boys with progressive encephalopathy and one or more of respiratory insufficiency, abnormal movements or tone, and intractable seizures.

  5. Pathology of the Superior Colliculus in Chronic Traumatic Encephalopathy.

    Science.gov (United States)

    Armstrong, Richard A; McKee, Ann C; Cairns, Nigel J

    2017-01-01

    To investigate neuropathological changes in the superior colliculus in chronic traumatic encephalopathy. The densities of the tau-immunoreactive neurofibrillary tangles, neuropil threads, dot-like grains, astrocytic tangles, and neuritic plaques, together with abnormally enlarged neurons, typical neurons, vacuolation, and frequency of contacts with blood vessels, were studied across the superior colliculus from pia mater to the periaqueductal gray in eight chronic traumatic encephalopathy and six control cases. Tau-immunoreactive pathology was absent in the superior colliculus of controls but present in varying degrees in all chronic traumatic encephalopathy cases, significant densities of tau-immunoreactive neurofibrillary tangles, NT, or dot-like grains being present in three cases. No significant differences in overall density of the tau-immunoreactive neurofibrillary tangles, neuropil threads, dot-like grains, enlarged neurons, vacuoles, or contacts with blood vessels were observed in control and chronic traumatic encephalopathy cases, but chronic traumatic encephalopathy cases had significantly lower mean densities of neurons. The distribution of surviving neurons across the superior colliculus suggested greater neuronal loss in intermediate and lower laminae in chronic traumatic encephalopathy. Changes in density of the tau-immunoreactive pathology across the laminae were variable, but in six chronic traumatic encephalopathy cases, densities of tau-immunoreactive neurofibrillary tangles, neuropil threads, or dot-like grains were significantly greater in intermediate and lower laminae. Pathological changes were not correlated with the distribution of blood vessels. The data suggest significant pathology affecting the superior colliculus in a proportion of chronic traumatic encephalopathy cases with a laminar distribution which could compromise motor function rather than sensory analysis.

  6. Clinical predictors and differential diagnosis of posterior reversible encephalopathy syndrome

    OpenAIRE

    della Faille, Laetitia; Fieuws, Steffen; Van Paesschen, W.

    2017-01-01

    The aim of our study is to determine the clinical predictors and the differential diagnosis of posterior reversible encephalopathy syndrome (PRES) in patients presenting with acute neurological symptoms and risk factors for PRES. Using the diagnostic algorithm for PRES from Fugate and Rabinstein (Lancet Neurol 14(9):914-925, 1), we carried out a retrospective study on 220 patients, presenting with acute neurological symptoms such as seizures, encephalopathy, headache, visual disturbances or o...

  7. Clinical predictors and differential diagnosis of posterior reversible encephalopathy syndrome

    OpenAIRE

    Faille, Laetitia della; Fieuws, S.; Van Paesschen, W.

    2017-01-01

    The aim of our study is to determine the clinical predictors and the differential diagnosis of posterior reversible encephalopathy syndrome (PRES) in patients presenting with acute neurological symptoms and risk factors for PRES. Using the diagnostic algorithm for PRES from Fugate and Rabinstein (Lancet Neurol 14(9):914?925, 1), we carried out a retrospective study on 220 patients, presenting with acute neurological symptoms such as seizures, encephalopathy, headache, visual disturbances or o...

  8. Wernicke's encephalopathy after vertical banded gastroplasty for morbid obesity.

    Science.gov (United States)

    Seehra, H.; MacDermott, N.; Lascelles, R. G.; Taylor, T. V.

    1996-01-01

    Thiamine deficiency is known to lead to certain neurological sequelae including Wernicke- Korsakoff encephalopathy. Signs attributable to this condition include ataxia, ophthalmoplegia, nystagmus, and mental confusion. Recognised predisposing conditions include alcoholism gastric carcinoma, pyloric obstruction, hyperemesis gravidarum, and prolonged intravenous feeding. We have recently encountered two cases of Wernicke's encephalopathy after vertical banded gastroplasty for morbid obesity . Other neurological sequelae are recognised after vertical banded gastroplasty, including Guillain-Barre syndrome, psychosis, and pseudoathetosis, but the causes are multifactorial. PMID:8601118

  9. Wernicke encephalopathy after obesity surgery: a systematic review.

    Science.gov (United States)

    Singh, Sonal; Kumar, Abhay

    2007-03-13

    To characterize the clinical features, risk factors, radiographic findings, and prognosis of Wernicke encephalopathy after bariatric surgery. We performed a systematic review of MEDLINE, Embase, Ovid, ISI (Science Citation Index), and Google Scholar for case reports, case series, or cohort studies of Wernicke encephalopathy after bariatric surgery. We found 32 cases (27 of whom were women) reported, from 2 weeks to 18 months after the procedure. Most patients had vomiting as a risk factor (n = 25) and presented with the triad of Wernicke encephalopathy (confusion, ataxia, and nystagmus; n = 21). Optic neuropathy, papilledema, deafness, seizures, asterixis, weakness, and sensory and motor neuropathy were also reported. Characteristic radiographic findings were hyperintense signals in the periaqueductal gray area and dorsal medial nucleus of the thalamus; radiographs were normal in 15 patients. One series from Brazil reported 4 patients (among 50 patients) with Wernicke encephalopathy; all presented with vomiting and concomitant peripheral neuropathy at a median of 2.5 months (1.5 to 3 months) after bariatric surgery. Another series identified 2 of 23 patients (both women) with Wernicke encephalopathy after bariatric surgery. Wernicke encephalopathy after bariatric surgery usually occurs between 4 and 12 weeks postoperatively, especially in young women with vomiting. Atypical neurologic features are common. The diagnosis is mainly clinical, because radiographic findings are normal in some patients. Prospective studies to determine the prevalence of this problem and protocols for preventive thiamine supplementation need evaluation.

  10. Endoplasmic reticulum stress implicated in chronic traumatic encephalopathy.

    Science.gov (United States)

    Lucke-Wold, Brandon P; Turner, Ryan C; Logsdon, Aric F; Nguyen, Linda; Bailes, Julian E; Lee, John M; Robson, Matthew J; Omalu, Bennet I; Huber, Jason D; Rosen, Charles L

    2016-03-01

    Chronic traumatic encephalopathy is a progressive neurodegenerative disease characterized by neurofibrillary tau tangles following repetitive neurotrauma. The underlying mechanism linking traumatic brain injury to chronic traumatic encephalopathy has not been elucidated. The authors investigate the role of endoplasmic reticulum stress as a link between acute neurotrauma and chronic neurodegeneration. The authors used pharmacological, biochemical, and behavioral tools to assess the role of endoplasmic reticulum stress in linking acute repetitive traumatic brain injury to the development of chronic neurodegeneration. Data from the authors' clinically relevant and validated rodent blast model were compared with those obtained from postmortem human chronic traumatic encephalopathy specimens from a National Football League player and World Wrestling Entertainment wrestler. The results demonstrated strong correlation of endoplasmic reticulum stress activation with subsequent tau hyperphosphorylation. Various endoplasmic reticulum stress markers were increased in human chronic traumatic encephalopathy specimens, and the endoplasmic reticulum stress response was associated with an increase in the tau kinase, glycogen synthase kinase-3β. Docosahexaenoic acid, an endoplasmic reticulum stress inhibitor, improved cognitive performance in the rat model 3 weeks after repetitive blast exposure. The data showed that docosahexaenoic acid administration substantially reduced tau hyperphosphorylation (t = 4.111, p chronic traumatic encephalopathy. Docosahexaenoic acid therefore warrants further investigation as a potential therapeutic agent for the prevention of chronic traumatic encephalopathy.

  11. Clinical predictors and differential diagnosis of posterior reversible encephalopathy syndrome.

    Science.gov (United States)

    Faille, Laetitia Della; Fieuws, S; Van Paesschen, W

    2017-06-01

    The aim of our study is to determine the clinical predictors and the differential diagnosis of posterior reversible encephalopathy syndrome (PRES) in patients presenting with acute neurological symptoms and risk factors for PRES. Using the diagnostic algorithm for PRES from Fugate and Rabinstein (Lancet Neurol 14(9):914-925, 1), we carried out a retrospective study on 220 patients, presenting with acute neurological symptoms such as seizures, encephalopathy, headache, visual disturbances or other focal neurological signs that appear in the clinical setting of risk factors such as hypertension/blood pressure fluctuations, chemotherapy, renal failure, autoimmune disorders, or eclampsia, in whom imaging of the brain was performed to exclude PRES. Seventeen percent of patients had a radiologically confirmed diagnosis of PRES. Univariable logistic regression showed a significant association between PRES and epileptic seizures, encephalopathy, hypertension, chemotherapy and renal failure. Multivariable logistic regression of acute neurological symptoms and risk factors showed a significant association of epileptic seizures, encephalopathy, visual disturbances, hypertension and chemotherapy with PRES. Using these variables to predict PRES yielded a discriminative ability (AUC) equal to 0.793. Diagnoses when PRES was not confirmed included primary or secondary headaches (26%), toxic-metabolic encephalopathy (21%), vascular pathology (12%) and other less frequent disorders. Epileptic seizures, encephalopathy, visual disturbances, hypertension, renal failure and chemotherapy were the best clinical predictors of PRES, while headache, immune suppression and autoimmune disease were not useful for the clinical diagnosis of PRES in our study.

  12. Quantification of gut lesions in a subclinical necrotic enteritis model

    DEFF Research Database (Denmark)

    Gholamiandehkordi, Ahmad R.; Timbermont, Leen; Lanckriet, Anouk

    2007-01-01

    Currently Clostridium perfringens-induced necrotic enteritis is a major problem in broiler flocks. In the present study, broilers were inoculated with a combination of Eimeria maxima or overdose coccidial vaccine (one inoculation) with C. perfringens (repeated inoculations). Single C. perfringens......, E. maxima or an overdose of live coccidial vaccine inoculations did not result in grossly visible necrotic gut lesions, while combined inoculation resulted in typical necrotic lesions at approximately 4 days after inoculations with C. perfringens in approximately one-half of the inoculated animals...... defects. The villus length and the villus length/crypt depth ratio were also analysed. This approach proved to be discriminative between single E. maxima infection, overdose of coccidial vaccine or C. perfringens inoculations and the non-inoculated control group, and between the double...

  13. Staged multidisciplinary step-up management for necrotizing pancreatitis.

    Science.gov (United States)

    da Costa, D W; Boerma, D; van Santvoort, H C; Horvath, K D; Werner, J; Carter, C R; Bollen, T L; Gooszen, H G; Besselink, M G; Bakker, O J

    2014-01-01

    Some 15 per cent of all patients with acute pancreatitis develop necrotizing pancreatitis, with potentially significant consequences for both patients and healthcare services. This review summarizes the latest insights into the surgical and medical management of necrotizing pancreatitis. General management strategies for the treatment of complications are discussed in relation to the stage of the disease. Frequent clinical evaluation of the patient's condition remains paramount in the first 24-72 h of the disease. Liberal goal-directed fluid resuscitation and early enteral nutrition should be provided. Urgent endoscopic retrograde cholangiopancreatography is indicated when cholangitis is suspected, but it is unclear whether this is appropriate in patients with predicted severe biliary pancreatitis without cholangitis. Antibiotic prophylaxis does not prevent infection of necrosis and antibiotics are not indicated as part of initial management. Bacteriologically confirmed infections should receive targeted antibiotics. With the more conservative approach to necrotizing pancreatitis currently advocated, fine-needle aspiration culture of pancreatic or extrapancreatic necrosis will less often lead to a change in management and is therefore indicated less frequently. Optimal treatment of infected necrotizing pancreatitis consists of a staged multidisciplinary 'step-up' approach. The initial step is drainage, either percutaneous or transluminal, followed by surgical or endoscopic transluminal debridement only if needed. Debridement is delayed until the acute necrotic collection has become 'walled-off'. Outcome following necrotizing pancreatitis has improved substantially in recent years as a result of a shift from early surgical debridement to a staged, minimally invasive, multidisciplinary, step-up approach. © 2013 BJS Society Ltd. Published by John Wiley & Sons Ltd.

  14. Cutaneous necrotic ulceration due to BCG re-vaccination

    DEFF Research Database (Denmark)

    Gyldenløve, Mette; Andersen, Ase Bengård; Halkjær, Liselotte Brydensholt

    2012-01-01

    The case report describes a severe local reaction with large cutaneous necrotic ulcer following bacillus Calmette-Guérin (BCG) re-vaccination. This is a very rare adverse event, and only a few reports have been described in the literature.......The case report describes a severe local reaction with large cutaneous necrotic ulcer following bacillus Calmette-Guérin (BCG) re-vaccination. This is a very rare adverse event, and only a few reports have been described in the literature....

  15. Necrotizing Soft Tissue Infection Occurring after Exposure to Mycobacterium marinum

    Directory of Open Access Journals (Sweden)

    Shivani S. Patel

    2014-01-01

    Full Text Available Cutaneous infections caused by Mycobacterium marinum have been attributed to aquarium or fish exposure after a break in the skin barrier. In most instances, the upper limbs and fingers account for a majority of the infection sites. While previous cases of necrotizing soft tissue infections related to M. marinum have been documented, the importance of our presenting case is to illustrate the aggressive nature of M. marinum resulting in a persistent necrotizing soft tissue infection of a finger that required multiple aggressive wound debridements, followed by an amputation of the affected extremity, in order to hasten recovery.

  16. Epileptic seizures in nonalcoholic Wernicke's encephalopathy: a case report and literature review.

    Science.gov (United States)

    Shang, Wenjin; Chen, Xiuhui; Li, Xunhua; Chen, Hongbing; Tang, Shujin; Hong, Hua

    2017-09-06

    Wernicke encephalopathy (WE) is characterized by eye signs, cerebellar dysfunction, and confusion. Epileptic seizures are rare in nonalcoholic WE. We reviewed the clinical, laboratory, radiological, and prognostic characteristics of nonalcoholic WE accompanied by epileptic seizures. We reported 1 case and searched similar cases using PubMed, WoK, Ovid, and Embase. WE was diagnosed according to dietary deficiencies, clinical symptoms and brain magnetic resonance imaging (MRI). We reviewed 13 patients (median age, 27 years; 5 men) with clear histories of thiamine deficiency and symptoms of typical WE. The type of epileptic seizures reported in the 13 cases reviewed was generically reported as seizures or convulsions in 4 patients; 7 patients had generalized tonic-clonic seizures, 1 partial seizure, and 1 generalized convulsive status epileptics. Two patients had epileptic seizures as the first symptom of WE. Laboratory tests mainly indicated metabolic acidosis and electrolyte disturbances. Electroencephalography may present as normal patterns, increased slow waves or epileptic discharge. Six patients had cortical lesions on brain MRI. These lesions were usually diffuse and band-like, and sometimes involved all lobes either symmetrically or asymmetrically, with the frontal lobe as the most susceptible area. All cortical lesions were accompanied by non-cortical lesions typical of WE. Brain MRI abnormalities, after thiamine treatment, mostly disappeared on follow-up MRIs. The patients had good prognoses. Only 1 patient had repeated seizures, and there were no comas or deaths. Patients with nonalcoholic WE accompanied by seizures are young and generally have good prognoses. Most patients experienced generalized convulsive seizures, which may have been related to abnormal cerebral cortical metabolism due to subacute thiamine deficiency.

  17. The clinical features of 17 patients with steroid-responsive encephalopathy associated with autoimmune thyroiditis

    Directory of Open Access Journals (Sweden)

    CHEN Hai

    2013-06-01

    Full Text Available Objective To investigate the onset pattern, clinical manifestations, laboratory findings and imaging features of 17 Chinese patients with steroid-responsive encephalopathy associated with autoimmune thyroiditis (SREAT. Methods The clinical data of 17 SREAT patients were collected. Retrospective analysis of their clinical features, as well as their serum levels of anti-thyroid, cerebrospinal fluid (CSF biochemical indicators, MRI and therapy was performed. Results The initial symptoms of those patients were seizures (4 cases, psychiatric symptoms (4 cases, hypomnesis (4 cases, walking unsteadiness (2 cases, headache (2 cases and dysarthria (1 case. Three cases were acute onset, 5 cases subacute onset and 9 cases chronic onset. The anti-thyroid peroxidase antibody (anti-TPO of 17 cases were significantly increased, average (928.63 ± 406.28 × 10 3 IU/L. The anti?thyroglobulin antibody (anti-TG of 15 cases was increased, average (601.27 ± 1014.12 × 10 3 IU/L. The protein in CSF was mildly increased, average (513.75 ± 283.15 mg/L. The EEG of 5 patients presented slow wave and the EEG of 2 patients showed epileptiform discharge. The brain MRI of 11 patients showed multifocal lesions in frontal lobe, temporal lobe, parietal lobe, basal ganglia, centrum ovale, corpus callosum, thalamus, cerebellum, and brain stem. The findings of clinical immunological index and tumor markers were normal. Besides, the prognosis of 11 patients treated with methylprednisolone and 3 patients treated with dexamethasone were good. Recurrence occurred in 2 patients. Conclusion Basically, the clinical features of Chinese SREAT patients present seizures, hypomnesis and psychiatric symptoms associated with increased anti-thyroid and multifocal lesions in gray and white matter of brain.

  18. Necrotic ulcer: a manifestation of leukemia cutis.

    Science.gov (United States)

    Aksu, Ayse Esra Koku; Saracoglu, Zeynep Nurhan; Sabuncu, Ilham; Ciftci, Evrim; Gulbas, Zafer; Isiksoy, Serap

    2012-01-01

    A 71-year-old man presented to our dermatological clinic with a 3-month history of a wound on his leg. He complained of weakness for the past few months. On his dermatological examination he had a 3x3-cm necrotic ulcer on his left tibia (Figure 1). On physical examination, there was 1 x 1-cm axillary lymphadenopathy. There was no other lymph node enlargement, hepatosplenomegaly, or gingival hypertrophy. Peripheral blood results showed 2.4x103/mm3 leukocytes (normal range 4-11 x 103/mm3) with 66% neutrophils. The hemoglobin value was 10.1 g/dL (13-18 g/dL), and the platelet count was 63x103/mm3 (150-440 x 103/mm3). No blasts were detected in a peripheral blood smear. His lactate dehydrogenase level was 567 U/L (240-480 U/L). All other results of blood chemistry were within normal limits. Punch biopsy of the skin lesion showed ulceration and dense dermal acute and chronic inflammation. There was a superficial and deep perivascular and periadnexal infiltrate of neoplastic cells composed of relatively abundant eosinophilic cytoplasm and large nuclei with blastic chromatin and occasional small nucleoli (Figure 2). Mitotic figures were prominent. Immunohistochemical stains were performed, and the neoplastic cells were CD3, CD20, CD138, and S100 protein negative. Myeloperoxidase and CD68 were positive. The histopathological findings were consistent with leukemic infiltration. Examination of bone marrow biopsy revealed that the blastic cells constituted more than 20% of the bone marrow cellularity. Cytogenetic analysis of bone marrow aspiration with fluorescence in situ hybridization was negative for inversion 16, t(8;21) and t(15;7). Histochemical stains for myeloperoxidase, sudan black, periodic acid-Schiff, and alpha naphthyl acetate were also negative. Blastic cells were DR, CD13, CD117, and CD34 positive and CD5, CD7, CD10, CD14, CD19, CD20, CD33, CD41, CD56, CD64, and CD79 negative according to flow cytometry immunophenotyping. Blastic cells were 35% in the bone

  19. Progressive limbic encephalopathy: Problems and prospects

    Directory of Open Access Journals (Sweden)

    Sadanandavalli Retnaswami Chandra

    2014-01-01

    Full Text Available Background: It was observed that a good number of patients presenting with psychiatric manifestations when investigated later because of unresponsiveness to treatment or late development of organic features turned out to be treatable limbic syndromes. Introduction: The aim of this study is to assess the patients presenting with new onset neuropsychiatric symptoms satisfying the criteria for probable limbic encephalitis. Patients and Methods: Patients referred to neurology department following a period of treatment for neuropsychiatric symptoms, which did not respond to conventional treatment were analyzed using Electroencephalography (EEG, magnetic resonance imaging, cerebrospinal fluid, screening for malignancy Vasculitic work-up, histopathology and autoantibody done when feasible. Results: There were 22 patients satisfying criteria for probable limbic encephalitis. Their mean age was 34.5 years. Symptoms varied from unexplained anxiety, panic and depression, lack of inhibition, wandering, incontinence, myoclonus, seizures and stroke like episodes. Three had systemic malignancy, 10 had chronic infection, one each with vasculitis, acute disseminated encephalomyelitis, Hashimoto encephalitis and two each with non-convulsive status, cryptogenic and Idiopathic inflammation. Conclusion: All patients who present with new onset neuropsychiatric symptoms need to be evaluated for sub-acute infections, inflammation, autoimmune limbic encephalitis and paraneoplastic syndrome. A repeated 20 minute EEG is a very effective screening tool to detect organicity.

  20. Minimal hepatic encephalopathy characterized by parallel use of the continuous reaction time and portosystemic encephalopathy tests

    DEFF Research Database (Denmark)

    Lauridsen, M M; Schaffalitzky de Muckadell, O B; Vilstrup, H

    2015-01-01

    based vs. paper and pencil). To compare results of the Continuous Reaction time (CRT) and the Portosystemic Encephalopathy (PSE) tests in a large unselected cohort of cirrhosis patients without clinically detectable brain impairment and to clinically characterize the patients according to their test...... results. The CRT method is a 10-minute computerized test of a patient's motor reaction time stability (CRTindex) to 150 auditory stimuli. The PSE test is a 20-minute paper-pencil test evaluating psychomotor speed. Both tests were performed at the same occasion in 129 patients. Both tests were normal...

  1. Stain- and Culture-Negative Necrotizing Granulomas in an Urban Population

    National Research Council Canada - National Science Library

    Burke, A; Paulk, A

    2016-01-01

    Context: A recent study found that 10% of necrotizing granulomas are idiopathic. We aimed to study the rate of stain-negative and culturenegative necrotizing granulomas within an urban population. Design...

  2. Higher Grades and Repeated Recurrence of Hepatic Encephalopathy May Be Related to High Serum Manganese Levels.

    Science.gov (United States)

    Kobtan, Abdelrahman A; El-Kalla, Ferial S; Soliman, Hanan H; Zakaria, Soha S; Goda, Mohamed A

    2016-02-01

    Hepatic encephalopathy is a serious complication of liver failure. Until now, the precise pathophysiologic mechanisms are not fully determined. It has been demonstrated that manganese plays an important role in the pathogenesis of hepatic encephalopathy. Therefore, we studied manganese levels in serum of cirrhotic patients with hepatic encephalopathy in relation to grading and recurrence of hepatic encephalopathy. One hundred persons were enrolled in the study, 80 cirrhotic patients with or without encephalopathy and 20 healthy controls. Hepatic encephalopathy was diagnosed clinically and by laboratory findings. Serum manganese levels were measured in all participants. The grading of hepatic encephalopathy was significantly correlated to the severity of liver dysfunction. The mean serum manganese level was significantly higher in cirrhotic patients than in controls and in cirrhotic patients with encephalopathy than in those without encephalopathy. It was also significantly higher in patients with advanced grading of hepatic encephalopathy. Serum manganese level was positively correlated to number of recurrences of encephalopathy during a 6-month follow-up period. Serum manganese levels were able to predict recurrence of hepatic encephalopathy within 6 months following the episode. Serum manganese levels are positively correlated to the modified Child-Pugh score of cirrhosis as well as grading and number of recurrences of hepatic encephalopathy. Higher manganese levels seem to be related to worsening of the condition, and its measurement may be used as a predictor of repeated recurrences.

  3. [Method and procedures in bacteriological study of necrotic teeth].

    Science.gov (United States)

    Rodríguez-Ponce, A; López Campos, A; López Paz, J; Pazos Sierra, R

    1991-01-01

    Research was conducted of 160 radicular canals with necrotic pulp. Results of different bacteriological analyses are presented. Culture analyses in aerobic and anaerobic media, resulted in the isolation of Staphylococcus Epidermidis, Streptococcus Viridans and Corynebacterium sp in the group studied, as the most frequent bacteria. There was no evidence of a specific germ linked with the pulp necrosis.

  4. Necrotizing Fasciitis In A Preterm, HIV Infected Baby | Olutekunbi ...

    African Journals Online (AJOL)

    Necrotizing fasciitis (NF) is a rapidly progressive life threatening bacterial infection of the soft tissues. It is commoner in the adult population where it is associated with systemic and local disease conditions such as diabetes mellitus, intravenous drug abuse, dental lesions, trauma and immunosuppression. It is rare in ...

  5. Carbohydrate maldigestion induces necrotizing enterocolitis in preterm pigs

    Science.gov (United States)

    Necrotizing enterocolitis (NEC) is a major gastrointestinal disorder in preterm infants. Key risk factors for NEC are enteral feeding and microbial colonization. Maldigestion of carbohydrate secondary to immature digestive function has been suspected to cause bacterial overgrowth and NEC. We investi...

  6. Clostridium perfringens, necrotic enteritis and its vaccination in broiler chickens

    Science.gov (United States)

    Clostridium perfringens type A strains are the main etiological factors for necrotic enteritis (NE), one of the economically important gastrointestinal diseases in poultry responsible for the annual loss of 2 billion dollars in US poultry industry. NE has gained worldwide importance during the last...

  7. Novel Phaeoacremonium species associated with necrotic wood of Prunus trees

    NARCIS (Netherlands)

    Damm, U.; Mostert, L.; Crous, P.W.; Fourie, P.H.

    2008-01-01

    The genus Phaeoacremonium is associated with opportunistic human infections, as well as stunted growth and die-back of various woody hosts, especially grapevines. In this study, Phaeoacremonium species were isolated from necrotic woody tissue of Prunus spp. (plum, peach, nectarine and apricot) from

  8. Challenges in the management of cervicofacial necrotizing fasciitis ...

    African Journals Online (AJOL)

    Background: Necrotizing fascitis is uncommon but a life threatening cervico-facial infection, which is characterized by aggressive spread of inflammation and necrosis of the tissues. In our environment clinical presentation is often late and patient's management at this stage is very challenging. We present the challenges in ...

  9. Necrotizing meningoencephalitis in a large mixed-breed dog.

    Science.gov (United States)

    Estey, Chelsie M; Scott, Steven J; Cerda-Gonzalez, Sofia

    2014-12-01

    A 4-year-old 26-kg (57.2-lb) spayed female Staffordshire Bull Terrier mix was evaluated because of a 24-hour history of cluster seizures. Neurologic examination revealed altered mentation and multifocal intracranial signs; MRI was performed. The MRI findings included multifocal, asymmetric forebrain lesions affecting both the gray and white matter, an area suggestive of focal necrosis, and loss of corticomedullary distinction. A midline shift and caudal transtentorial herniation were noted, suggestive of greater than normal intracranial pressure. Because the dog's clinical signs worsened despite medical treatment and additional evidence of increased intracranial pressure, bilateral craniectomy and durectomy were performed. Histologic evaluation of a brain biopsy specimen revealed bilateral and asymmetric areas of necrosis in the subcortical white matter and adjacent gray matter. At the periphery of the necrotic areas, there was increased expression of glial fibrillary acidic protein and Virchow-Robin spaces were expanded by CD3+ lymphocytes. Results of immunohistochemical analysis of brain tissue were negative for canine distemper virus, Neospora canis, and Toxoplasma gondii. These clinical, imaging, and histopathologic findings were compatible with necrotizing meningoencephalitis. The dog's neurologic status continued to worsen following surgery. Repeated MRI revealed ongoing signs of increased intracranial pressure, despite the bilateral craniectomy. The owners elected euthanasia. To the author's knowledge, this is the first report of necrotizing meningoencephalitis in a large mixed-breed dog. Necrotizing meningoencephalitis should be considered as a differential diagnosis in dogs other than small or toy breeds that have signs suggestive of inflammatory disease.

  10. Umbilical arterial necrotic vasculopathy associated with fetal ascites.

    Science.gov (United States)

    Rossi, Giuliana; Cosmi, Erich; Trevisanuto, Daniele; Cappellari, Ambra; Andrisani, Alessandra; Visentin, Silvia; Chiarelli, Silvia; Zanardo, Vincenzo

    2010-01-01

    Immune and nonimmune neonatal ascites may be part of hydrops fetalis or may be an isolated finding. However, a significant percentage of nonimmune ascites do not have an identifiable pathogenesis and are considered idiopathic. We report a case of fetal ascites and umbilical arterial necrotic vasculopathy, an association not previously described. 2010 S. Karger AG, Basel.

  11. Severe necrotizing cutaneous reaction to topical 5-fluorouracil

    Directory of Open Access Journals (Sweden)

    Bhalla M

    2003-01-01

    Full Text Available 5-flourouracil (5-FU has been used for the treatment of various malignant and pre-malignant conditions. It has also been used to treat viral warts as a topical agent. It usually does not produce any significant adverse effects when used topically. Severe necrotizing cutaneous reaction following topical 5-FU used for the treatment of warts is being reported.

  12. Case Report: Extremity Necrotizing Fasciitis: Four-year Experience ...

    African Journals Online (AJOL)

    Patients and Method: This is a retrospective observational study. The medical records of patients diagnosed with Necrotizing fasciitis of the extremities and managed solely or jointly with the Plastic/Reconstructive surgeons, by the orthopaedic surgical service of Ahmadu Bello University Teaching Hospital, Zaria between ...

  13. Necrotizing enterocolitis. New thoughts about pathogenesis and potential treatments.

    Science.gov (United States)

    MacKendrick, W; Caplan, M

    1993-10-01

    Necrotizing enterocolitis (NEC) remains a major cause of morbidity and mortality in premature infants. An incomplete understanding of its pathogenesis has hampered efforts to devise an effective preventative strategy. New insights into the pathogenesis of NEC, particularly at the cellular and biochemical level, however, offer a rational basis for the development of new approaches to this disease.

  14. Neonatal scrotal wall necrotizing fasciitis (Fournier gangrene): a case report

    LENUS (Irish Health Repository)

    Zgraj, Oskar

    2011-12-12

    Abstract Introduction Necrotizing fasciitis in neonates is rare and is associated with almost 50% mortality. Although more than 80 cases of neonates (under one month of age) with necrotizing fasciitis have been reported in the literature, only six of them are identified as originating in the scrotum. Case presentation We report the case of a four-week-old, full-term, otherwise-healthy Caucasian baby boy who presented with an ulcerating lesion of his scrotal wall. His scrotum was explored because of a provisional diagnosis of missed torsion of the testis. He was found to have necrotizing fasciitis of the scrotum. We were able to preserve the testis and excise the necrotic tissue, and with intravenous antibiotics there was a successful outcome. Conclusions Fournier gangrene is rarely considered as part of the differential diagnosis in the clinical management of the acute scrotum. However, all doctors who care for small babies must be aware of this serious condition and, if it is suspected, should not hesitate in referring the babies to a specialist pediatric surgical center immediately.

  15. Identification of cultivable microorganisms from primary teeth with necrotic pulps.

    Science.gov (United States)

    Ledezma-Rasillo, Gildardo; Flores-Reyes, Hector; Gonzalez-Amaro, Ana M; Garrocho-Rangel, Arturo; Ruiz-Rodriguez, M del Socorro; Pozos-Guillen, Amaury J

    2010-01-01

    The objective of this study was to identify cultivable microorganisms from primary teeth with necrotic pulps. This experimental study included 21 patients of both sexes between 4 and 7 years of age with necrotic pulps in primary teeth. Twenty-one maxillary and mandibular molars containing at least 1 necrotic canal, an abscess or sinus tract, one or more radiolucent areas in the furcation or periapical region, teeth having at least two thirds of root length, and carious lesions directly exposed to the oral environment were included. After antisepsis of the oral cavity, anesthesia of the affected tooth, and isolation and disinfection of the operative field, 3 sterile absorbent paper points were sequentially placed for 30 seconds for the collection of samples. The samples were immediately processed in an anaerobic chamber, and all isolated microorganisms were identified. Anaerobic species (anaerobic facultative and moderate anaerobes) were isolated in all root canals; 68.4% of root canal samples studied showed a polymicrobial nature. Most of the isolate consisted of Bifidobacterium Spp2 and Streptococcus intermedius. Other less frequently encountered species were Actinomyces israelii, Bifidobacterium spp 1, Clostridium spp, and Candida albicans. Results indicate the existence of combinations of bacterial species in root canal infections of the primary dentition with necrotic pulps, anaerobic bacteria predominating.

  16. Necrotizing hepatitis associated with enteric salmonellosis in an alpaca

    Science.gov (United States)

    2004-01-01

    Abstract Salmonella typhimurium was isolated from the feces of an alpaca suffering anorexia and weight loss. Multifocal necrotizing and suppurative hepatitis consistent with bacterial infection was found in the liver biopsies. Enteric salmonellosis may be associated with milder physical and clinicopathological changes in camelids than in other large animal species. PMID:15144106

  17. Severe necrotizing infection of the perineum: beyond necrosectomy ...

    African Journals Online (AJOL)

    The evolution of systemic inflammatory response syndrome (SIRS) to septic shock is a continuum that can be stemmed using dedicated and early goal directed interventions. In the setting of necrotizing soft tissue infection, mortality approaches 100% when debridement is delayed or altogether omitted. Volume depletion ...

  18. Unusual presentation of necrotizing fasciitis in an HIV exposed infant

    African Journals Online (AJOL)

    Necrotizing fasciitis(NF) is a potentially life threatening soft tissue infection characterized by rapidly spreading inflammation with necrosis of fascia, subcutaneous tissues and overlying skin and is associated with signs of systemic toxicity. We present a case report of an uncommon presentation of NF in an HIV exposed infant.

  19. Management of necrotizing enterocolitis: experience at a tertiary ...

    African Journals Online (AJOL)

    Introduction Necrotizing enterocolitis (NEC) is the most common surgical emergency in the neonatal intensive care unit. The aim of this study was to determine the incidence of NEC and identify the factors predicting the surgical management and also to determine the mortality due to. NEC at our tertiary care neonatal unit in ...

  20. Report a possible correlation between necrotizing ulcerative gingivitis and mononucleosis

    Directory of Open Access Journals (Sweden)

    Rosa Francinne Miranda

    2011-12-01

    Full Text Available Necrotizing ulcerative gingivitis is a relatively uncommon periodontal disease, characterized by ulceration, necrosis, pain and gingival bleeding. Factors often related to its occurrence include stress and systemic viral infections, such as those caused by cytomegalovirus and Epstein-Barr virus type 1, the latter being also considered the causative agent of infectious mononucleosis. This article aims to describe a clinical case of a female patient who presented with necrotizing ulcerative gingivitis associated with a clinical picture of infectious mononucleosis, as well as to review the literature concerning a possible correlation between these pathologies. This patient presented to our health care facility with necrotizing ulcerative gingivitis accompanied by lymphadenopathy, fever and prostration, after laboratory tests, Epstein-Barr virus type 1 infection was confirmed, as well as the co-occurrence of pathologies: necrotizing ulcerative gingivitis and infectious mononucleosis. Symptom remission in both disorders also occurred concomitantly, after instruction in plaque control measures and palliative medication for control of systemic symptoms. Therefore, although there is no scientific validation of an association between these two pathologies, it is imperative that all diagnostic alternatives be considered and investigated, in order to establish the therapeutic approach most appropriate to the patient.

  1. Retrospective evaluation of necrotizing fasciitis in university college ...

    African Journals Online (AJOL)

    2011-11-12

    Nov 12, 2011 ... under-reported in the dental literature. Aims: To report our experience with cases seen and treated .... Retrospective evaluation of necrotizing fasciitis extremities. Reports of CNF in the literature were mostly .... Surgical Memoirs of the war of rebellion. New York: Reverside. Press; 1871. p. 146-70. 3. Meleny ...

  2. Predicting success of catheter drainage in infected necrotizing pancreatitis

    NARCIS (Netherlands)

    Hollemans, Robbert A.; Bollen, Thomas L.; Van Brunschot, Sandra; Bakker, Olaf J.|info:eu-repo/dai/nl/314099050; Ali, Usama Ahmed; Van Goor, Harry; Boermeester, Marja A.; Gooszen, Hein G.; Besselink, Marc G.; Van Santvoort, Hjalmar C.|info:eu-repo/dai/nl/304821721

    2016-01-01

    Introduction: At least 30% of patients with infected necrotizing pancreatitis are successfully treated with catheter drainage alone. It is currently not possible to predict which patients also need necrosectomy. We evaluated predictive factors for successful catheter drainage. Methods: This was a

  3. Predicting Success of Catheter Drainage in Infected Necrotizing Pancreatitis

    NARCIS (Netherlands)

    Hollemans, Robbert A.; Bollen, Thomas L.; van Brunschot, Sandra; Bakker, Olaf J.; Ahmed Ali, Usama; van Goor, Harry; Boermeester, Marja A.; Gooszen, Hein G.; Besselink, Marc G.; van Santvoort, Hjalmar C.

    2016-01-01

    At least 30% of patients with infected necrotizing pancreatitis are successfully treated with catheter drainage alone. It is currently not possible to predict which patients also need necrosectomy. We evaluated predictive factors for successful catheter drainage. This was a post hoc analysis of 130

  4. Predicting Success of Catheter Drainage in Infected Necrotizing Pancreatitis

    NARCIS (Netherlands)

    Hollemans, R.A.; Bollen, T.L.; Brunschot, S. van; Bakker, O.J.; Ali, U. Ahmed; Goor, H. van; Boermeester, M.A.; Gooszen, H.G.; Besselink, M.G.; Santvoort, H.C. van

    2016-01-01

    INTRODUCTION: At least 30% of patients with infected necrotizing pancreatitis are successfully treated with catheter drainage alone. It is currently not possible to predict which patients also need necrosectomy. We evaluated predictive factors for successful catheter drainage. METHODS: This was a

  5. Cerebral and splanchnic oxygenation and necrotizing enterocolitis in preterm infants

    NARCIS (Netherlands)

    Schat, Trijntje Eelkje

    2015-01-01

    Necrotizing enterocolitis (NEC) is the most common and deadliest gastrointestinal disease in preterm infants. Mortality rates of NEC can be as high as 40%. Furthermore, NEC is associated with adverse neurological outcomes at school age and gastrointestinal complications in the long term, such as

  6. Subacute combined degeneration of the spinal cord in a Nigerian child

    African Journals Online (AJOL)

    2015-12-19

    Dec 19, 2015 ... Abstract: Background: Vitamin. B12 deficiency has been reported to be associated with a spectrum of neurological disorders among which is subacute combined de- generation of the spinal cord. Method: We report a case of subacute combined degeneration of the spinal cord secondary to vitamin B12 ...

  7. Subacute combined degeneration of the spinal cord in a Nigerian child

    African Journals Online (AJOL)

    Background: Vitamin B12 deficiency has been reported to be associated with a spectrum of neurological disorders among which is subacute combined degeneration of the spinal cord. Method: We report a case of subacute combined degeneration of the spinal cord secondary to vitamin B12 deficiency and discussed the ...

  8. Psychiatric phenotypes in chronic traumatic encephalopathy.

    Science.gov (United States)

    Mahar, Ian; Alosco, Michael L; McKee, Ann C

    2017-09-06

    Chronic traumatic encephalopathy (CTE) is a neurodegenerative disorder involving cognitive, motor, and psychiatrically-relevant symptoms resulting from repetitive head impacts. Psychiatric phenotypes of CTE, including depression and suicidality, present particular challenges for CTE research, given that the diagnosis requires postmortem neuropathological examination. The pathognomonic lesion of CTE is the perivascular accumulation of hyperphosphorylated tau (ptau) protein at the depths of cortical sulci. These lesions are found in the earliest disease stages, and with advancing pathological severity, ptau deposition occurs in widespread brain regions in a four-stage scheme of severity. We review the psychiatric phenotypes of individuals neuropathologically diagnosed with CTE, and suggest that earlier CTE stages hold particular interest for psychiatric CTE research. In the early CTE stages, there is ptau pathology in frontal cortex and axonal loss in the frontal white matter, followed by progressive ptau neurofibrillary degeneration in the amygdala and hippocampus. Neuropathological changes in the frontal and medial temporal lobes may underlie psychiatric phenotypes. Additional insight into the association between CTE pathology and psychiatric sequelae may come from advancements in in vivo methods of CTE detection. Further epidemiological, clinical, and postmortem studies are needed to validate the nature of psychiatric sequelae in CTE. Copyright © 2017. Published by Elsevier Ltd.

  9. A critical review of chronic traumatic encephalopathy.

    Science.gov (United States)

    Iverson, Grant L; Gardner, Andrew J; McCrory, Paul; Zafonte, Ross; Castellani, Rudy J

    2015-09-01

    Chronic traumatic encephalopathy (CTE) has been described in the literature as a neurodegenerative disease with: (i) localized neuronal and glial accumulations of phosphorylated tau (p-tau) involving perivascular areas of the cerebral cortex, sulcal depths, and with a preference for neurons within superficial cortical laminae; (ii) multifocal axonal varicosities and axonal loss involving deep cortex and subcortical white matter; (iii) relative absence of beta-amyloid deposits; (iv) TDP-43 immunoreactive inclusions and neurites; and (v) broad and diverse clinical features. Some of the pathological findings reported in the literature may be encountered with age and other neurodegenerative diseases. However, the focality of the p-tau cortical findings in particular, and the regional distribution, are believed to be unique to CTE. The described clinical features in recent cases are very similar to how depression manifests in middle-aged men and with frontotemporal dementia as the disease progresses. It has not been established that the described tau pathology, especially in small amounts, can cause complex changes in behavior such as depression, substance abuse, suicidality, personality changes, or cognitive impairment. Future studies will help determine the extent to which the neuropathology is causally related to the diverse clinical features. Copyright © 2015 Elsevier Ltd. All rights reserved.

  10. Corpus callosum atrophy in Wernicke's encephalopathy.

    Science.gov (United States)

    Lee, Soon-Tae; Jung, Young-Min; Na, Duk L; Park, Seong Ho; Kim, Manho

    2005-10-01

    Neuropathologic changes in Wernicke's encephalopathy (WE) involve variable brain structures. Corpus callosum involvement in WE, however, is largely unknown. The authors investigated the degree and the pattern of corpus callosum changes in WE according to the etiologies. Nineteen patients with WE (between 34 and 81 years) and 19 age- and sex-matched control participants were included. The total cross-sectional callosal area and 5 callosal subregions (C1-C5) were measured by tracing outer margins in the midsagittal sections. Subregions were determined by placing radial dividers with 10 rays. The pixel numbers for corpus callosums were calculated, and the values obtained were adjusted for head size variations. The causes of WE were alcoholism (10), intestinal surgery (5), anorexia (3), and hyperemesis gravidarum (1). The mean size of the total corpus callosum was significantly reduced in alcoholic WE (P< .001; 527.8 +/- 70.8 mm2 for alcoholic WE; 664.6 +/- 58.1 mm2 for the corresponding controls), but not in nonalcoholic WE. In subregion analysis, prefrontal callosum (C2) atrophy was the most prominent in alcoholic WE. In contrast, only splenium (C5) was atrophied in nonalcoholic WE. The degree of atrophy did not change throughout the follow-up period (mean 5.3 weeks). This study suggests that the extent and location of corpus callosum atrophy differs between alcoholic WE and nonalcoholic WE, implying separate contribution of alcohol neurotoxicity and nutritional deficiency.

  11. Neuroimaging of Wernicke's encephalopathy and Korsakoff's syndrome.

    Science.gov (United States)

    Jung, Young-Chul; Chanraud, Sandra; Sullivan, Edith V

    2012-06-01

    There is considerable evidence that neuroimaging findings can improve the early diagnosis of Wernicke's encephalopathy (WE) in clinical settings. The most distinctive neuroimaging finding of acute WE are cytotoxic edema and vasogenic edema, which are represented by bilateral symmetric hyperintensity alterations on T2-weighted MR images in the periphery of the third ventricle, periaqueductal area, mammillary bodies and midbrain tectal plate. An initial bout of WE can result in Korsakoff's syndrome (KS), but repeated bouts in conjunction with its typical comorbidity, chronic alcoholism, can result in signs of tissue degeneration in vulnerable brain regions. Chronic abnormalities identified with neuroimaging enable examination of brain damage in living patients with KS and have expanded the understanding of the neuropsychological deficits resulting from thiamine deficiency, alcohol neurotoxicity, and their comorbidity. Brain structure and functional studies indicate that the interactions involving the thalamus, mammillary bodies, hippocampus, frontal lobes, and cerebellum are crucial for memory formation and executive functions, and the interruption of these circuits by WE and chronic alcoholism can contribute substantially to the neuropsychological deficits in KS.

  12. The mechanisms and treatment of asphyxial encephalopathy

    Directory of Open Access Journals (Sweden)

    Guido eWassink

    2014-02-01

    Full Text Available Acute post-asphyxial encephalopathy occurring around the time of birth remains a major cause of death and disability. The recent seminal insight that allows active neuroprotective treatment is that even after profound asphyxia (the primary phase, many brain cells show initial recovery from the insult during a short latent phase, typically lasting approximately 6 h, only to die hours to days later after a secondary deterioration characterized by seizures, cytotoxic edema, and progressive failure of cerebral oxidative metabolism. Although many of these secondary processes are potentially injurious, they appear to be primarily epiphenomena of the ‘execution’ phase of cell death. Animal and human studies designed around this conceptual framework have shown that moderate cerebral hypothermia initiated as early as possible but before the onset of secondary deterioration, and continued for a sufficient duration to allow the secondary deterioration to resolve, has been associated with potent, long-lasting neuroprotection. Recent clinical trials show that while therapeutic hypothermia significantly reduces morbidity and mortality, many babies still die or survive with disabilities. The challenge for the future is to find ways of improving the effectiveness of treatment. In this review, we will dissect the known mechanisms of hypoxic-ischemic brain injury in relation to the known effects of hypothermic neuroprotection.

  13. Pathogenetic aspects of alcoholic encephalopathy treatment

    Directory of Open Access Journals (Sweden)

    Shchetinin S.G.

    2010-12-01

    Full Text Available Alcohol is considered to be the most common exogenous toxins, causing encephalopathy. The defeat of almost all parts of the nervous system should be assigned to the special features of ethanol. Neurophysiological mechanisms of development of substance dependence are based in the stem and limbic structures of the brain that are involved in ensuring the regulation of emotional state, mood, motivation sphere, psychophysical tone of human behavior in general and its adaptation to the environment. Stress or disruption of the normal functioning of these structures can lead to the formation of abstinence syndrome, affective disorders in remission and craving for alcohol. Dopaminergic and opioid (endorphin system play an important role in the genesis of various mental and motor disorders. In some way alcohol dependence can be regarded as an endorfinodefitsitnoe disease with a pathogenetic point of view. Activating of opioidereal system by trans-cranial electrical stimulation promotes the restoration of disturbed emotional, cognitive and autonomic functions, reduces craving for alcohol and in that way increases the effectiveness of rehabilitation treatment

  14. The neuropathology of chronic traumatic encephalopathy.

    Science.gov (United States)

    McKee, Ann C; Stein, Thor D; Kiernan, Patrick T; Alvarez, Victor E

    2015-05-01

    Repetitive brain trauma is associated with a progressive neurological deterioration, now termed as chronic traumatic encephalopathy (CTE). Most instances of CTE occur in association with the play of sports, but CTE has also been reported in association with blast injuries and other neurotrauma. Symptoms of CTE include behavioral and mood changes, memory loss, cognitive impairment and dementia. Like many other neurodegenerative diseases, CTE is diagnosed with certainty only by neuropathological examination of brain tissue. CTE is a tauopathy characterized by the deposition of hyperphosphorylated tau (p-tau) protein as neurofibrillary tangles, astrocytic tangles and neurites in striking clusters around small blood vessels of the cortex, typically at the sulcal depths. Severely affected cases show p-tau pathology throughout the brain. Abnormalities in phosphorylated 43 kDa TAR DNA-binding protein are found in most cases of CTE; beta-amyloid is identified in 43%, associated with age. Given the importance of sports participation and physical exercise to physical and psychological health as well as disease resilience, it is critical to identify the genetic risk factors for CTE as well as to understand how other variables, such as stress, age at exposure, gender, substance abuse and other exposures, contribute to the development of CTE. © 2015 International Society of Neuropathology.

  15. Chronic traumatic encephalopathy and other neurodegenerative proteinopathies

    Directory of Open Access Journals (Sweden)

    Maria Carmela Tartaglia

    2014-01-01

    Full Text Available Chronic traumatic encephalopathy (CTE is described as a slowly progressive neurodegenerative disease believed to result from multiple concussions. Traditionally, concussions were considered benign events and although most people recover fully, about 10% develop a post-concussive syndrome with persisting neurological, cognitive and neuropsychiatric symptoms. CTE was once thought to be unique to boxers, but it has now been observed in many different athletes having suffered multiple concussions as well as in military personal after repeated blast injuries. Much remains unknown about the development of CTE but its pathological substrate is usually tau, similar to that seen in Alzheimer’s disease and frontotemporal lobar degeneration. The aim of this perspective is to compare and contrast clinical and pathological CTE with the other neurodegenerative proteinopathies and highlight that there is an urgent need for understanding the relationship between concussion and the development of CTE as it may provide a window into the development of a proteinopathy and thus new avenues for treatment.

  16. [Definition and diagnostic principles of encephalopathy].

    Science.gov (United States)

    Göllnitz, G

    1968-01-01

    Definition of the collecting terms "early infantile brain damages" and "encephalopathy" as well as temporal delimination of the prenatal, perinatal and postnatal injury period. The diagnostical value of anamnesis, of neurological findings (tone, reflex-mechanisms in the different infantile developmental stages), motometric examinations, radiographs of cranial and hand skeleton, electroencephalogram, pneumoencephalogram and liquor is discussed thoroughly. The termed slighter early infantile brain damages are somatic the easier to demonstrate the earlier after birth this is performed. If the children are prejudged not till 4-6 years, the diagnostical pains transpose first of all to psychosomatic methods, except some basic somatic examinations. By reason of a review of the infantile patients in the children's department for neuropsychiatry at Rostock during the the last five years, the main point of diagnosis is formed by turns: a very profound and detailed developmental anamnesis, extensive examinations of the motoric function and coordination, radiographs of cranial and hand skeleton, electroencephalograms at several course-controls and at provocation-controls, examinations of the corporal proportions, vegetative and endocrine function tests as well as a pneumencephalogram may be called in additional. This methods are fewer suitable for routine examinations. They demand to express a strict opinion on indication. Non of the mentioned examinations has a reliability of 100%. The more extensive the diagnostical base, the more frequent controls are performed, the more reliable are the results.

  17. Biotin and Thiamine Responsive Basal Ganglia Disease--A vital differential diagnosis in infants with severe encephalopathy.

    Science.gov (United States)

    Ygberg, Sofia; Naess, Karin; Eriksson, Mats; Stranneheim, Henrik; Lesko, Nicole; Barbaro, Michela; Wibom, Rolf; Wang, Chen; Wedell, Anna; Wickström, Ronny

    2016-05-01

    We report two siblings of Swedish origin with infantile Biotin and Thiamine Responsive Basal Ganglia Disease (BTRBG). Initial symptoms were in both cases lethargia, with reduced contact and poor feeding from the age of 5 weeks. Magnetic resonance imaging showed altered signal in the basal ganglia, along with grey and white matter abnormalities. The diagnosis BTRBG was not recognized in the first sibling who died at the age of 8 weeks. The second sibling was started on biotin and thiamine immediately upon development of symptoms, leading to clinical improvement and partial reversion of the magnetic resonance imaging findings. Genetic analysis of the SLC19A3 gene identified two mutations, c.74dupT and c.1403delA, carried in compound heterozygous form in both boys, each inherited from one parent. The first mutation has previously been described in children with BTRBG, and the second mutation is novel. Although the clinical picture in BTRGB is very severe it is also rather unspecific and the diagnosis may be missed. This report highlights the importance of considering biotin and thiamine treatment also in a European infant born to non-consanguineous parents, who presents with symptoms of acute/subacute encephalopathy. Copyright © 2016 European Paediatric Neurology Society. Published by Elsevier Ltd. All rights reserved.

  18. Neuropsychological and educational problems at school age associated with neonatal encephalopathy

    National Research Council Canada - National Science Library

    Marlow, N; Rose, A S; Rands, C E; Draper, E S

    2005-01-01

    .... To investigate neurocognitive and behavioural outcomes after neonatal encephalopathy. Sixty five children with neonatal encephalopathy, identified using the Trent Neonatal Survey database for 1992-1994, were followed up at the age of 7 years...

  19. Prognostic factors for disability and sick leave in patients with subacute non-malignant pain

    DEFF Research Database (Denmark)

    Valentin, Gitte H; Pilegaard, Marc S; Vaegter, Henrik B

    2016-01-01

    OBJECTIVE: This systematic review aims to identify generic prognostic factors for disability and sick leave in subacute pain patients. SETTING: General practice and other primary care facilities. PARTICIPANTS: Adults (>18 years) with a subacute (≤3-month) non-malignant pain condition. Eligibility...... reporting of all factors examined. TRIAL REGISTRATION NUMBER: CRD42014008914.......OBJECTIVE: This systematic review aims to identify generic prognostic factors for disability and sick leave in subacute pain patients. SETTING: General practice and other primary care facilities. PARTICIPANTS: Adults (>18 years) with a subacute (≤3-month) non-malignant pain condition. Eligibility...... criteria were cohort studies investigating the prediction of disability or long-term sick leave in adults with a subacute pain condition in a primary care setting. 19 studies were included, referring to a total of 6266 patients suffering from pain in the head, neck, back and shoulders. PRIMARY...

  20. Midazolam or diazepam administration during electroencephalography helps to diagnose subacute sclerosing panencephalitis (SSPE).

    Science.gov (United States)

    Yilmaz, Kutluhan; Sahin, Derya Aydin

    2010-08-01

    Although diagnostic contribution of intravenous diazepam administration during electroencephalography (EEG) recording in subacute sclerosing panencephalitis has been known, no another drug with less potential side effects has been studied in this procedure. In this study, diazepam is compared with midazolam in 25 subacute sclerosing panencephalitis-diagnosed children and 10 children with subacute sclerosing panencephalitis-suggesting symptoms, normal EEG findings and no certain diagnosis. Neither midazolam nor diazepam affected typical periodic slow-wave complexes. However, in the patients with atypical EEG abnormalities, midazolam, like diazepam, attenuated sharp or sharp-and-slow waves, and therefore made the identification of periodic slow-wave paroxysms easier. In the patients with normal EEGs, both midazolam and diazepam revealed typical periodic complexes on EEG recording in the same 3 patients. Cerebrospinal fluid examination verified the diagnosis of subacute sclerosing panencephalitis. The findings suggest that midazolam or diazepam administration increases the contribution of EEG recording in atypical cases with subacute sclerosing panencephalitis.

  1. Quantitative EEG in hospital encephalopathy: review and microstate analysis.

    Science.gov (United States)

    Sarkis, Rani A; Lee, Jong Woo

    2013-10-01

    Hospital-acquired encephalopathy is a widely prevalent disorder. The quantitative changes in EEG associated with this condition have long been noted, including slowing of the background frequency and changes in the frequency band power. EEG has had limited clinical use, despite its ability to continuously track clinical severity. We review the development of the use of EEG and particularly quantitative EEG in the assessment of hospital-acquired encephalopathy. Recent advances in EEG technology have included network and microstate analyses, and continuous EEG monitoring, leading to renewed interest in the use of quantitative EEG. We describe the development of microstate analysis that has allowed novel quantitative analysis of the resting state background. We examined the microstates of 16 inpatients with encephalopathy and 20 control patients. The global variance explained by the four standard resting microstates was smaller in patients with encephalopathy. This suggests a decrease in microstate stability, indicating a breakdown in the resting state network dynamics. Modern analysis and acquisition techniques hold the promise of renewed interest in quantitative EEG techniques in the assessment of hospital-acquired encephalopathy.

  2. Posterior reversible encephalopathy syndrome in a patient with lupus nephritis

    Directory of Open Access Journals (Sweden)

    Huseyin Kadikoy

    2012-01-01

    Full Text Available Posterior reversible encephalopathy syndrome (PRES is characterized by acute onset of headache, nausea, focal neurological deficits or seizures along with radiological findings of white matter defects in the parietal and occipital lobes. Causes of PRES include uremia, hypertensive encephalopathy, eclampsia and immunosuppressive medications. Usually, the treat-ment of choice involves correcting the underlying abnormality. We describe an unusual case of recurrent PRES caused by uremia during a lupus flare in a patient with biopsy-proven Class IV Lupus Nephritis (LN with vasculitis. PRES in systemic lupus erythematosis (SLE is a rare clin-ical phenomenon and, when reported, it is associated with hypertensive encephalopathy. Our patient did not have hypertensive crisis, but had uremic encephalopathy. The patient′s PRES-related symptoms resolved after initiation of hemodialysis. The temporal correlation of the correc-tion of the uremia and the resolution of the symptoms of PRES show the etiology to be uremic encephalopathy, making this the first reported case of uremia-induced PRES in Class IV LN with vasculitis.

  3. Recent advances in hepatic encephalopathy [version 1; referees: 4 approved

    Directory of Open Access Journals (Sweden)

    Victoria Liere

    2017-09-01

    Full Text Available Hepatic encephalopathy describes the array of neurological alterations that occur during acute liver failure or chronic liver injury. While key players in the pathogenesis of hepatic encephalopathy, such as increases in brain ammonia, alterations in neurosteroid levels, and neuroinflammation, have been identified, there is still a paucity in our knowledge of the precise pathogenic mechanism. This review gives a brief overview of our understanding of the pathogenesis of hepatic encephalopathy and then summarizes the significant recent advances made in clinical and basic research contributing to our understanding, diagnosis, and possible treatment of hepatic encephalopathy. A literature search using the PubMed database was conducted in May 2017 using “hepatic encephalopathy” as a keyword, and selected manuscripts were limited to those research articles published since May 2014. While the authors acknowledge that many significant advances have been made in the understanding of hepatic encephalopathy prior to May 2014, we have limited the scope of this review to the previous three years only.

  4. Chronotypology and melatonin alterations in minimal hepatic encephalopathy

    Directory of Open Access Journals (Sweden)

    Polychronopoulos Panagiotis

    2009-05-01

    Full Text Available Abstract Background "Minimal (subclinical hepatic encephalopathy" is a term that describes impairment of every day life activities in cirrhosis patients without clinical neurologic abnormalities. Melatonin diurnal pattern disruption and metabolic changes due to liver insufficiency can affect the human biologic clock. Our study was conducted to measure plasma melatonin levels in an attempt to correlate plasma melatonin abnormalities with liver insufficiency severity, and describe chronotypology in cirrhosis patients with minimal encephalopathy. Methods Twenty-six cirrhotic patients enrolled in the study and thirteen patients without liver or central nervous system disease served as controls. All patients had full clinical and biochemical evaluation, chronotypology analysis, neurological evaluation, melatonin profile and quality of life assessment. Results Cirrhotic patients with minimal encephalopathy exhibit melatonin secretion abnormalities. Cirrhosis patients with more severe hepatic insufficiency (Child-Pugh score > 5 had significantly (p Chronotypology analysis revealed Morning Type pattern in 88% of cirrhosis patients. Discussion The presence of abnormal plasma melatonin levels before the onset of clinical hepatic encephalopathy, and the finding that patients with more severe cirrhosis have lower evening melatonin levels are the most important findings of this study. Despite these melatonin abnormalities, chronotypology revealed Morning Type pattern in 23 of 26 cirrhosis patients. We believe these findings are important and deserve further study. Conclusion Melatonin abnormalities occur in cirrhosis patients without clinical encephalopathy, are related to liver insufficiency severity, may influence chronotypology patterns, and certainly deserve further investigation.

  5. Subacute cutaneous lupus erythematosus inducing unilateral sensorimotor neuropathy.

    Science.gov (United States)

    Psarelis, Savvas; Georgiades, Fanourios; Ioannou, Antreas; Xenophontos, Eleni; Georgiou, Georgios; Papanicolaou, Eleni

    2017-05-01

    We describe the case of a 32-year-old male with previous history of subacute cutaneous lupus erythematosus (SCLE) who presented with arthritis followed by a unilateral lower-limb sensorimotor impairment, without biochemical or immunological marker abnormalities. Our patient currently satisfies only three of the systemic lupus international collaborating clinics criteria. Management of such patients is challenging due to lack of examples in the literature, with this case being the first described where a seronegative patient with SCLE demonstrated neurological involvement. A brief review of the available literature is included.

  6. Atypical clinical course subacute sclerosing panencephalitis presenting as acute Encephalitis

    Science.gov (United States)

    Komur, Mustafa; Arslankoylu, Ali E; Okuyaz, Cetin; Kuyucu, Necdet

    2012-01-01

    We report a 14-year-old boy who presented with loss of consciousness and gait instability. The electroencephalogram (EEG) showed generalized slowing with irregular activity and cerebral magnetic imaging revealed asymmetrical nonspecific signals on basal ganglia. His second electroencephalogram revealed periodical generalized high-voltage slow wave complexes which did not disappear with diazepam induction. Subacute sclerosing panencephalitis (SSPE) was considered and the diagnosis was confirmed with the identification of measles antibodies in cerebrospinal fluid. Our findings show that SSPE should be in mind in the differential diagnosis of meningoencephalitis and acute disseminated encephalomyelitis and highlight the significance of EEG in the diagnosis of unidentified cases. PMID:23248691

  7. Catatonia as presenting clinical feature of subacute sclerosing panencephalitis.

    Science.gov (United States)

    Dayal, Prabhoo; Balhara, Yatan Pal Singh

    2014-01-01

    Catatonia is not a usual clinical presentation of subacute sclerosing panencephalitis (SSPE), especially in the initial stages of illness. However, there is only one reported case of SSPE presenting as catatonia among children. In this report, however, there were SSPE-specific changes on EEG and the catatonia failed to respond to lorazepam. We describe a case of SSPE in a child presenting as catatonia that presented with clinical features of catatonia and did not have typical EEG findings when assessed at first contact. He responded to lorazepam and EEG changes emerged during the course of follow-up.

  8. Computed tomographic findings of early subacute sclerosing panencephalitis

    Energy Technology Data Exchange (ETDEWEB)

    Pedersen, H.; Wulff, C.H.

    1982-03-01

    Computed tomography of the brain (CT) was carried out at the early stages of subacute sclerosing panencephalitis (SSPE) in three children. The lateral ventricles were very small and the hemispheric sulci and interhemispheric fissures were not visible in all three patients in contrast to severe atrophy found at a later stage in one patient. The early CT abnormalities were revealed at the same time as the titres of measles antibodies in blood and cerebrospinal fluid were elevated, and the characteristic periodic complexes in the electroencephalogram established the diagnosis of SSPE. The CT changes indicating brain swelling reflect the reactive changes of this slow virus infection.

  9. Oxidant and antioxidant status in children with subacute sclerosing panencephalitis.

    Science.gov (United States)

    Caksen, Hüseyin; Ozkan, Mustafa; Cemek, Mustafa; Cemek, Fatma

    2014-11-01

    We analyzed serum alpha-tocopherol, beta-carotene, retinol, and ascorbic acid levels and malondialdehyde and reduced glutathione concentrations on erythrocyte and cerebrospinal fluid in 30 patients with subacute sclerosing panencephalitis to evaluate oxidant and antioxidant status. Serum alpha-tocopherol, beta-carotene, retinol, ascorbic acid levels, and erythrocyte and cerebrospinal fluid reduced glutathione concentrations were decreased; however, erythrocyte and cerebrospinal fluid malondialdehyde levels were increased in the patients. Cerebrospinal fluid malondialdehyde levels were different between clinical stages of the disease (P panencephalitis and that antioxidants were increased as defense mechanisms of the organism against oxidative damage. © The Author(s) 2013.

  10. The relationship between plasma free fatty acids and experimentally induced hepatic encephalopathy in the rat

    NARCIS (Netherlands)

    Smit, J. J.; Bosman, D. K.; Jörning, G. G.; de Haan, J. G.; Maas, M. A.; Chamuleau, R. A.

    1991-01-01

    Two experimental models of hepatic encephalopathy in the rat have been investigated in order to study the postulated relationship between plasma free fatty acids concentration (C6 - C22:0) and the degree of hepatic encephalopathy. As a model of chronic hepatic encephalopathy, porta caval shunted

  11. Unusual Reversible MR Signal Abnormalities In Hypertensive Encephalopathy : A Case Report

    Directory of Open Access Journals (Sweden)

    Nalini A

    2000-01-01

    Full Text Available Hypertensive encephalopathy is an acute neurological emergency comprising of headache, seizures, visual disturbances and altered sensorium associated with elevated systemic blood pressure. We report a patient who suffered from two episodes of hypertensive encephalopathy secondary to primary renal disease, with the unusual MRI features involving the cerebellar lobes secondary to hypertensive encephalopathy and subsequent resolution.

  12. Wernicke’s Encephalopathy Complicating Hyperemesis during Pregnancy

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    Mohamed Adnane Berdai

    2016-01-01

    Full Text Available Wernicke’s encephalopathy is caused by severe thiamine deficiency; it is mostly observed in alcoholic patients. We report the case of a 28-year-old woman, at 17 weeks of gestational age, with severe hyperemesis gravidarum. She presented with disturbance of consciousness, nystagmus, ophthalmoplegia, and ataxia. The resonance magnetic imagery showed bilaterally symmetrical hyperintensities of thalamus and periaqueductal area. The case was managed with very large doses of thiamine. The diagnosis of Wernicke’s encephalopathy was confirmed later by a low thiamine serum level. The patient was discharged home on day 46 with mild ataxia and persistent nystagmus. Wernicke’s encephalopathy is a rare complication of hyperemesis gravidarum. It should be diagnosed as early as possible to prevent long-term neurological sequela or death. Thiamine supplementation in pregnant women with prolonged vomiting should be initiated, especially before parenteral dextrose infusion. Early thiamine replacement will reduce maternal morbidity and fetal loss rate.

  13. A Critical Case of Wernicke's Encephalopathy Induced by Hyperemesis Gravidarum

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    Byung Ju Kang

    2015-05-01

    Full Text Available Wernicke’s encephalopathy is a reversible but potentially critical disease caused by thiamine deficiency. Most patients complain of symptoms such as ophthalmoplegia, ataxia and confusion. Heavy alcohol drinking is commonly associated with the disease, but other clinical conditions also can provoke it. In pregnant women, hyperemesis gravidarum can lead to the depletion of body thiamine due to poor oral intake and a high metabolic demand. We report a case of Wernicke’s encephalopathy following hyperemesis gravidarum in a 36-year-old female at 20 weeks of pregnancy, who visited our hospital because of shock with vaginal bleeding. This case suggests that although the initial presentation may include atypical symptoms (e.g., shock or bleeding, Wernicke’s encephalopathy should be considered, and thiamine replacement should be performed in pregnant women with neurologic symptoms and poor oral intake.

  14. Neuroimaging findings in acute Wernicke's encephalopathy: review of the literature.

    Science.gov (United States)

    Zuccoli, Giulio; Pipitone, Nicolò

    2009-02-01

    Wernicke's encephalopathy is an acute neurological syndrome resulting from thiamine (vitamin B1) deficiency. Early recognition is important because timely thiamine supplementation can reverse the clinical features of the disease. The aim of this article is to provide an update on the typical and atypical neuroimaging findings of the acute phase of the disease. Wernicke's encephalopathy is characterized by a quite distinct pattern of MR alterations, which include symmetrical alterations in the thalami, mamillary bodies, tectal plate, and periaqueductal area, but atypical alterations may also been seen. A thorough knowledge of the neuroimaging findings of Wernicke's encephalopathy will assist in arriving at an early diagnosis, thus reducing the morbidity and mortality associated with this disease.

  15. Clinical and radiological features of hypertensive brainstem encephalopathy

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    Xiao-qiu LI

    2015-07-01

    Full Text Available Objective To discuss the diagnosis and treatment of hypertensive brainstem encephalopathy. Methods  The clinical and imaging data of 3 cases of hypertensive brainstem encephalopathy were summarized and analyzed for the purpose of improving the acumen in diagnosis and treatment. Results All the 3 patients showed relatively mild clinical symptoms, and they were misdiagnosed in different degrees during the treatment, but their clinical symptoms were improved by rapid and effective antihypertensive therapy. Cerebral CT and MRI scans revealed extensive abnormal signals in brain stem, with or without supratentorial lesions and brain stem hemorrhage. The lesions as revealed by imaging were improved significantly after treatment. Conclusions Clinical-radiographic dissociation is the classic feature of hypertensive brainstem encephalopathy. The clinical symptoms and lesions as shown by imaging could be improved after active treatment. DOI: 10.11855/j.issn.0577-7402.2015.06.03

  16. EPILEPTIC ENCEPHALOPATHY WITH CONTINUOUS SPIKES-WAVES ACTIVITY DURING SLEEP

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    E. D. Belousova

    2012-01-01

    Full Text Available The author represents the review and discussion of current scientific literature devoted to epileptic encephalopathy with continuous spikes-waves activity during sleep — the special form of partly reversible age-dependent epileptic encephalopathy, characterized by triad of symptoms: continuous prolonged epileptiform (spike-wave activity on EEG in sleep, epileptic seizures and cognitive disorders. The author describes the aspects of classification, pathogenesis and etiology, prevalence, clinical picture and diagnostics of this disorder, including the peculiar anomalies on EEG. The especial attention is given to approaches to the treatment of epileptic encephalopathy with continuous spikeswaves activity during sleep. Efficacy of valproates, corticosteroid hormones and antiepileptic drugs of other groups is considered. The author represents own experience of treatment this disorder with corticosteroids, scheme of therapy and assessment of efficacy.

  17. Hippocampal sclerosis and chronic epilepsy following posterior reversible encephalopathy syndrome.

    Science.gov (United States)

    Kapina, Viktoria; Vargas, Maria-Isabel; Wohlrab, Gabriele; Vulliemoz, Serge; Fluss, Joel; Seeck, Margitta

    2013-12-01

    Chronic epilepsy has rarely been reported after posterior reversible encephalopathy syndrome (PRES) and the association with hippocampal sclerosis has been suggested only once before. We report the case of a girl admitted at the age of 8 years with idiopathic nephrotic syndrome. On the second day of admission, she presented with focal complex seizures and cerebral MRI showed posterior encephalopathy and no hippocampal sclerosis. MRI after one month confirmed the diagnosis of PRES. The seizures recurred and the girl developed pharmacoresistant epilepsy and was admitted to our hospital for further investigation. Cerebral MRI three years after the diagnosis of PRES showed hippocampal sclerosis which was not present on the initial MRI. We conclude that there is a triggering role of PRES in the development of hippocampal sclerosis. Hippocampal sclerosis may have resulted from seizure-associated damage, alternatively, hypertensive encephalopathy may have led to hippocampal damage via a vascular mechanism.

  18. Molecular chaperones and hypoxic-ischemic encephalopathy

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    Cong Hua

    2017-01-01

    Full Text Available Hypoxic-ischemic encephalopathy (HIE is a disease that occurs when the brain is subjected to hypoxia, resulting in neuronal death and neurological deficits, with a poor prognosis. The mechanisms underlying hypoxic-ischemic brain injury include excitatory amino acid release, cellular proteolysis, reactive oxygen species generation, nitric oxide synthesis, and inflammation. The molecular and cellular changes in HIE include protein misfolding, aggregation, and destruction of organelles. The apoptotic pathways activated by ischemia and hypoxia include the mitochondrial pathway, the extrinsic Fas receptor pathway, and the endoplasmic reticulum stress-induced pathway. Numerous treatments for hypoxic-ischemic brain injury caused by HIE have been developed over the last half century. Hypothermia, xenon gas treatment, the use of melatonin and erythropoietin, and hypoxic-ischemic preconditioning have proven effective in HIE patients. Molecular chaperones are proteins ubiquitously present in both prokaryotes and eukaryotes. A large number of molecular chaperones are induced after brain ischemia and hypoxia, among which the heat shock proteins are the most important. Heat shock proteins not only maintain protein homeostasis; they also exert anti-apoptotic effects. Heat shock proteins maintain protein homeostasis by helping to transport proteins to their target destinations, assisting in the proper folding of newly synthesized polypeptides, regulating the degradation of misfolded proteins, inhibiting the aggregation of proteins, and by controlling the refolding of misfolded proteins. In addition, heat shock proteins exert anti-apoptotic effects by interacting with various signaling pathways to block the activation of downstream effectors in numerous apoptotic pathways, including the intrinsic pathway, the endoplasmic reticulum-stress mediated pathway and the extrinsic Fas receptor pathway. Molecular chaperones play a key role in neuroprotection in HIE. In

  19. Is vaccination against transmissible spongiform encephalopathy feasible?

    Science.gov (United States)

    Wisniewski, T; Chabalgoity, J A; Goni, F

    2007-04-01

    Prion diseases are a unique category of illness, affecting both animals and humans, where the underlying pathogenesis is related to a conformation change of the cellular form of a normal, self-protein called a prion protein (PrP(c) [C for cellular]) to a pathological and infectious conformation known as scrapie form (PrPsc [Sc for scrapie]). Currently, all prion diseases are without effective treatment and are universally fatal. The emergence of bovine spongiform encephalopathy and variant Creutzfeldt-Jakob disease has highlighted the need to develop possible therapies. In Alzheimer's disease (AD), which has similarities to prion diseases, both passive and active immunisation have been shown to be highly effective at preventing disease and cognitive deficits in model animals. In a human trial of active vaccination in AD, despite indications of cognitive benefits in patients with an adequate humoral response, 6% of patients developed significant complications related to excessive cell-mediated immunity. This experience highlights that immunotherapies designed to be directed against a self-antigen have to finely balance an effective humoral immune response with potential autoimmune toxicity. Many prion diseases have the gut as a portal of infectious agent entry. This makes mucosal immunisation a potentially very attractive method to partially or completely prevent prion entry across the gut barrier and to also produce a modulated immune response that is unlikely to be associated with any toxicity. The authors' recent results using an attenuated Salmonella vaccine strain expressing the prion protein show that mucosal vaccination can partially protect against prion infection from a peripheral source, suggesting the feasibility of this approach.

  20. Potentially modifiable factors contributing to sepsis-associated encephalopathy.

    Science.gov (United States)

    Sonneville, Romain; de Montmollin, Etienne; Poujade, Julien; Garrouste-Orgeas, Maïté; Souweine, Bertrand; Darmon, Michael; Mariotte, Eric; Argaud, Laurent; Barbier, François; Goldgran-Toledano, Dany; Marcotte, Guillaume; Dumenil, Anne-Sylvie; Jamali, Samir; Lacave, Guillaume; Ruckly, Stéphane; Mourvillier, Bruno; Timsit, Jean-François

    2017-08-01

    Identifying modifiable factors for sepsis-associated encephalopathy may help improve patient care and outcomes. We conducted a retrospective analysis of a prospective multicenter database. Sepsis-associated encephalopathy (SAE) was defined by a score on the Glasgow coma scale (GCS) encephalopathy. After adjusting for baseline characteristics, site of infection, and type of admission, the following factors remained independently associated with sepsis-associated encephalopathy: acute renal failure [adjusted odds ratio (aOR) = 1.41, 95% confidence interval (CI) 1.19-1.67], hypoglycemia 10 mmol/l (aOR = 1.37, 95% CI 1.09-1.72), hypercapnia >45 mmHg (aOR = 1.91, 95% CI 1.53-2.38), hypernatremia >145 mmol/l (aOR = 2.30, 95% CI 1.48-3.57), and S. aureus (aOR = 1.54, 95% CI 1.05-2.25). Sepsis-associated encephalopathy was associated with higher mortality, higher use of ICU resources, and longer hospital stay. After adjusting for age, comorbidities, year of admission, and non-neurological SOFA score, even mild alteration of mental status (i.e., a score on the GCS of 13-14) remained independently associated with mortality (adjusted hazard ratio = 1.38, 95% CI 1.09-1.76). Acute renal failure and common metabolic disturbances represent potentially modifiable factors contributing to sepsis-associated encephalopathy. However, a true causal relationship has yet to be demonstrated. Our study confirms the prognostic significance of mild alteration of mental status in patients with sepsis.

  1. Subacute cannabinoid treatment: anticonvulsant activity and withdrawal excitability in mice.

    Science.gov (United States)

    Karler, R; Turkanis, S A

    1980-03-01

    1 The effects of subacute treatment with cannabidiol, delta 9-tetrahydrocannabinol (delta 9-THC), phenytoin and phenobarbitone on anticonvulsant activity and on withdrawal excitability in mice were compared in three electrically induced seizure-threshold tests. 2 In the maximal electroshock-threshold test, subacute treatment did not alter the anticonvulsant activity of cannabidiol, phenytoin or phenobarbitone, but tolerance developed to delta 9-THC. 3 In the 60 Hz electroshock-threshold test, the activity of delta 9-THC and cannabidiol did not change, but tolerance developed to phenobarbitone, and there was an increase in sensitivity to phenytoin. 4 In the 6 Hz electroshock-threshold test, there was an increase in sensitivity to both delta 9-THC and cannabidiol, there was tolerance to phenobarbitone, while the activity of phenytoin did not change. 5 Although tolerance developed in some of the seizure-threshold tests to delta 9-THC and phenobarbitone, tolerance to cannabidiol and phenytoin did not develop in any of the tests. 6 Hyperexcitability followed withdrawal from only delta 9-THC (6 Hz and 60 Hz electroshock-threshold tests) and phenobarbitone (maximal electroshock-threshold and 60 Hz electroshock-threshold tests). 7 The delta 9-THC withdrawal hyperexcitability suggests that the use of marihuana may jeopardize the control of seizures in epileptics.

  2. Changes of resting cerebral activities in subacute ischemic stroke patients

    Directory of Open Access Journals (Sweden)

    Ping Wu

    2015-01-01

    Full Text Available This study aimed to detect the difference in resting cerebral activities between ischemic stroke patients and healthy participants, define the abnormal site, and provide new evidence for pathological mechanisms, clinical diagnosis, prognosis prediction and efficacy evaluation of ischemic stroke. At present, the majority of functional magnetic resonance imaging studies focus on the motor dysfunction and the acute stage of ischemic stroke. This study recruited 15 right-handed ischemic stroke patients at subacute stage (15 days to 11.5 weeks and 15 age-matched healthy participants. A resting-state functional magnetic resonance imaging scan was performed on each subject to detect cerebral activity. Regional homogeneity analysis was used to investigate the difference in cerebral activities between ischemic stroke patients and healthy participants. The results showed that the ischemic stroke patients had lower regional homogeneity in anterior cingulate and left cerebrum and higher regional homogeneity in cerebellum, left precuneus and left frontal lobe, compared with healthy participants. The experimental findings demonstrate that the areas in which regional homogeneity was different between ischemic stroke patients and healthy participants are in the cerebellum, left precuneus, left triangle inferior frontal gyrus, left inferior temporal gyrus and anterior cingulate. These locations, related to the motor, sensory and emotion areas, are likely potential targets for the neural regeneration of subacute ischemic stroke patients.

  3. Neuronal loss is an early component of subacute sclerosing panencephalitis.

    Science.gov (United States)

    Yüksel, Deniz; Diren, Barış; Ulubay, Hakan; Altunbaşak, Sakir; Anlar, Banu

    2014-09-02

    We performed diffusion tensor imaging (DTI) and magnetic resonance spectroscopy (MRS) studies in a group of patients with subacute sclerosing panencephalitis (SSPE) in order to estimate the pathologic process underlying the phenotypic variability. Patients with SSPE who had MRI including DTI and MRS examinations were evaluated according to their clinical status as determined by the SSPE Scoring System and their mental age as determined by tests appropriate for age and developmental level. Comparisons of fractional anisotropy (FA) and apparent diffusion coefficient (ADC) values and metabolite ratios of frontal periventricular white matter, parieto-occipital periventricular white matter, and globus pallidus in both hemispheres were made between control and SSPE groups, and between SSPE subgroups. Control (n = 18) and SSPE (n = 39) groups differed in all DTI and MRS parameters except FA, choline (Cho), and Cho/creatine (Cr). SSPE cases had higher ADC and lower N-acetylaspartate (NAA), NAA/Cho, and NAA/Cr in all regions of interest, suggesting cell loss. Disease progression rate and neurologic deficit appeared to be associated with the degree of ADC elevation and NAA reduction: the group with severe global deterioration had the lowest NAA (230.75 ± 197.97 in forceps minor), and rapid progression was associated with acute reduction in NAA. The combination of MRS and diffusion MRI findings suggests neuronal loss can be a primary target in rapidly or subacutely progressing SSPE, and preservation or regeneration of axonal structure may be beneficial in chronic cases. © 2014 American Academy of Neurology.

  4. Subacute Sclerosing Panencephalitis of the Brainstem as a Clinical Entity.

    Science.gov (United States)

    Upadhyayula, Pavan S; Yang, Jason; Yue, John K; Ciacci, Joseph D

    2017-11-07

    Subacute sclerosing panencephalitis (SSPE) is a rare progressive neurological disorder of early adolescence caused by persistent infection of the measles virus, which remains prevalent worldwide despite an effective vaccine. SSPE is a devastating disease with a characteristic clinical course in subcortical white matter; however, atypical presentations of brainstem involvement may be seen in rare cases. This review summarizes reports to date on brainstem involvement in SSPE, including the clinical course of disease, neuroimaging presentations, and guidelines for treatment. A comprehensive literature search was performed for English-language publications with keywords "subacute sclerosing panencephalitis" and "brainstem" using the National Library of Medicine PubMed database (March 1981-September 2017). Eleven articles focusing on SSPE of the brainstem were included. Predominant brainstem involvement remains uncharacteristic of SSPE, which may lead to misdiagnosis and poor outcome. A number of case reports have demonstrated brainstem involvement associated with other intracranial lesions commonly presenting in later SSPE stages (III and IV). However, brainstem lesions can appear in all stages, independent of higher cortical structures. The varied clinical presentations complicate diagnosis from a neuroimaging perspective. SSPE of the brainstem is a rare but important clinical entity. It may present like canonical SSPE or with unique clinical features such as absence seizures and pronounced ataxia. While SSPE generally progresses to the brainstem, it can also begin with a primary focus of infection in the brainstem. Awareness of varied SSPE presentations can aid in early diagnosis as well as guide management and treatment.

  5. Subacute Sclerosing Panencephalitis of the Brainstem as a Clinical Entity

    Directory of Open Access Journals (Sweden)

    Pavan S. Upadhyayula

    2017-11-01

    Full Text Available Subacute sclerosing panencephalitis (SSPE is a rare progressive neurological disorder of early adolescence caused by persistent infection of the measles virus, which remains prevalent worldwide despite an effective vaccine. SSPE is a devastating disease with a characteristic clinical course in subcortical white matter; however, atypical presentations of brainstem involvement may be seen in rare cases. This review summarizes reports to date on brainstem involvement in SSPE, including the clinical course of disease, neuroimaging presentations, and guidelines for treatment. A comprehensive literature search was performed for English-language publications with keywords “subacute sclerosing panencephalitis” and “brainstem” using the National Library of Medicine PubMed database (March 1981–September 2017. Eleven articles focusing on SSPE of the brainstem were included. Predominant brainstem involvement remains uncharacteristic of SSPE, which may lead to misdiagnosis and poor outcome. A number of case reports have demonstrated brainstem involvement associated with other intracranial lesions commonly presenting in later SSPE stages (III and IV. However, brainstem lesions can appear in all stages, independent of higher cortical structures. The varied clinical presentations complicate diagnosis from a neuroimaging perspective. SSPE of the brainstem is a rare but important clinical entity. It may present like canonical SSPE or with unique clinical features such as absence seizures and pronounced ataxia. While SSPE generally progresses to the brainstem, it can also begin with a primary focus of infection in the brainstem. Awareness of varied SSPE presentations can aid in early diagnosis as well as guide management and treatment.

  6. Fever of unknown origin: subacute thyroiditis versus typhoid fever.

    Science.gov (United States)

    Cunha, Burke A; Thermidor, Marjorie; Mohan, Sowjanya; Valsamis, Ageliki S; Johnson, Diane H

    2005-01-01

    Fever of unknown origin (FUO) is not infrequently a diagnostic dilemma for clinicians. Common infectious causes include endocarditis and abscesses in adults, and noninfectious causes include neoplasms and certain collagen vascular diseases, for example, polymyalgia rheumatica, various vasculitides, and juvenile rheumatoid arthritis (adult Still's disease). Subacute thyroiditis is a rare cause of FUO. Among the infectious causes of FUO, typhoid fever is relatively uncommon. We present a case of FUO in a traveler returning from India whose initial complaints were that of left-sided neck pain and angle of the jaw pain, which initially suggested the diagnosis of subacute thyroiditis. After an extensive FUO workup, when typhoid fever is a likely diagnostic possibility, an empiric trial of anti- Salmonella therapy has diagnostic and therapeutic significance. The presence of relative bradycardia, and response to quinolone therapy, was the basis of the clinical diagnosis of typhoid fever as the explanation for this patients FUO. This case illustrates the diagnostic difficulties in assessing patients with FUO with few diagnostic findings.

  7. Speech and language therapy for aphasia following subacute stroke

    Directory of Open Access Journals (Sweden)

    Engin Koyuncu

    2016-01-01

    Full Text Available The aim of this study was to investigate the time window, duration and intensity of optimal speech and language therapy applied to aphasic patients with subacute stroke in our hospital. The study consisted of 33 patients being hospitalized for stroke rehabilitation in our hospital with first stroke but without previous history of speech and language therapy. Sixteen sessions of impairment-based speech and language therapy were applied to the patients, 30-60 minutes per day, 2 days a week, for 8 successive weeks. Aphasia assessment in stroke patients was performed with Gülhane Aphasia Test-2 before and after treatment. Compared with before treatment, fluency of speech, listening comprehension, reading comprehension, oral motor evaluation, automatic speech, repetition and naming were improved after treatment. This suggests that 16 seesions of speech and language therapy, 30-60 minutes per day, 2 days a week, for 8 successive weeks, are effective in the treatment of aphasic patients with subacute stroke.

  8. Possible progression of subacute lupus erythematosus--case report.

    Science.gov (United States)

    Brănişteanu, Daciana Elena; Lăbonţu, Andreea; Ciobanu, Delia; Stoleriu, Gabriela; Brănişteanu, D; Oanţă, A

    2014-01-01

    Subacute lupus erythematosus (SLE) is a specific form of lupus erythematosus characterized by prevalently cutaneous manifestations usually with a good prognosis. It is more common in patients aged 15 to 70 years, and there is a female predilection. This form accounts for 10% of all lupus erythematosus cases. We present the case of a 57-year-old male patient diagnosed at age 35 with chronic psoriasiform subacute lupus erythematosus, pathologically confirmed at the Iaşi Dermatology Clinic. At the age of 54 years he had multiple ischemic strokes, followed by deterioration of general status, and at 56 years deep vein thrombosis in the right leg. The patient presented the erythematous-squamous lesions specific to psoriasiform SLE localized both on the upper third of the body and knees and associated with submucosal lesions of the lower lip, oral mucosa and appendages. The patient also presented hypo- and hyperpigmentated atrophic scar-like lesions. Laboratory tests performed during the last two admissions showed the presence of anti-ds DNA and antiphospholipid antibodies, inflammatory syndrome, and nitrogen retention syndrome. Treatment consisted of systemic and local dermatocorticoids and associated medication, emollient lotions and creams with SPF 50+, with slowly favorable progression. The peculiarity of the case lies in the chronic progression without significant systemic involvement for 19 years, and then in 2 years the antiphospholipid antibody syndrome and a shift to systemic lupus erythematosus to occur.

  9. Protease production by Streptococcus sanguis associated with subacute bacterial endocarditis.

    Science.gov (United States)

    Straus, D C

    1982-01-01

    A viridans streptococcus (Streptococcus sanguis biotype II) isolated from the blood of a patient with subacute bacterial endocarditis was examined for protease production. In broth culture, extracellular proteolytic enzymes were not produced by this organism until after the early exponential phase of growth, with maximal protease production occurring during the stationary phase. Four distinct proteases were isolated and purified from the supernatant fluids of stationary-phase cultures, employing a combination of ion-exchange column chromatography, gel filtration column chromatography, and polyacrylamide gel electrophoresis. All four proteases could be eluted from a diethylaminoethyl cellulose column at a sodium chloride gradient concentration of 0.25 M but were separable by gel filtration chromatography on a Sephadex G-100 column. They varied in molecular weights as determined by gel filtration and sodium dodecyl sulfate-polyacrylamide gel electrophoresis from approximately 13,000 to 230,000. All four proteases had pH optima of between 8.0 and 9.0, and two of the proteases were active against casein, human serum albumin, and gelatin but were not active against elastin and collagen. The remaining two proteases were able to degrade only casein and gelatin. These results show that S. sanguis is able to excrete maximal levels of potentially destructive enzymes when the organisms are not actively multiplying. This finding may explain some of the damage caused in heart tissue by these organisms during subacute bacterial endocarditis. Images PMID:6759404

  10. Pancreatic encephalopathy- a rare complication of severe acute biliary pancreatitis

    Directory of Open Access Journals (Sweden)

    Vlad Denis Constantin

    2014-10-01

    Full Text Available Background. Pancreatic encephalopathy is a rare complication of severe acute pancreatitis, with high mortality, being difficult to diagnose and treat, thus requiring continuous research regarding its management. Materials and Methods. Of 20 patients diagnosed with severe acute pancreatitis on admission at Department of Emergency and Admission (DEA, from January 1st 2010 to March 31st 2014, 5 cases complicated by pancreatic encephalopathy were analyzed using a descriptive observational, retrospective, single-center study. Results. The study shows different types of diagnostic algorithm and therapeutical approaches, in correlation with morbidity and mortality rates. Conclusions. Our study highlighted the fact that speed is critical, early management being the key to outcome.

  11. Branched-chain amino acids for people with hepatic encephalopathy

    DEFF Research Database (Denmark)

    Gluud, Lise Lotte; Dam, Gitte; Les, Iñigo

    2015-01-01

    -chain amino acids (BCAA) versus control interventions has evaluated if BCAA may benefit people with hepatic encephalopathy. OBJECTIVES: To evaluate the beneficial and harmful effects of BCAA versus any control intervention for people with hepatic encephalopathy. SEARCH METHODS: We identified trials through...... control, language, or publication status. DATA COLLECTION AND ANALYSIS: The authors independently extracted data based on published reports and collected data from the primary investigators. We changed our primary outcomes in this update of the review to include mortality (all cause), hepatic...

  12. Diffusion weighted MR imaging of acute Wernicke's encephalopathy.

    Science.gov (United States)

    Chung, Tae-Ick; Kim, Joong-Seok; Park, Soung-Kyeong; Kim, Beum-Saeng; Ahn, Kook-Jin; Yang, Dong-Won

    2003-03-01

    We report a case of Wernicke's encephalopathy in which diffusion-weighted MR images demonstrated symmetrical hyperintense lesions in the paraventricular area of the third ventricles and medial thalami. Apparent diffusion coefficient mapping showed isointensity in the aforementioned areas. Diffusion-weighted MR images may provide evidence of vasogenic edema associated with thiamine deficiency, proven in the histopathology of experimental animals. In addition, diffusion-weighted MRI has many advantages over T2 or FLARE-weighted brain MRI in detecting structural and functional abnormalities in Wernicke's encephalopathy. Copyright 2002 Elsevier Science Ireland, Ltd.

  13. Risk factors and outcome of Shigella encephalopathy in Bangladeshi children.

    Science.gov (United States)

    Afroze, Farzana; Ahmed, Tahmeed; Sarmin, Monira; Smsb Shahid, Abu; Shahunja, K M; Shahrin, Lubaba; Chisti, Mohammod Jobayer

    2017-04-01

    Although, Shigella encephalopathy, a serious extra-intestinal complication of shigellosis, significantly increases the risks of death, data are very limited on predicting factors particularly related to electrolyte profiles in children below five years of age with Shigella encephalopathy. Our objective was to determine the clinical as well as laboratory predicting factors and outcome of children with Shigella encephalopathy. In this unmatched case-control design, children aged 2-59 months having a positive stool culture for Shigella and who had their serum electrolytes been done from July 2012 to June 2015 were studied. Children with Shigella encephalopathy, defined as having abnormal mentation, constituted the cases, and those without encephalopathy constituted the controls. During the study period, we identified a total of 541 children less than five years of age, who had Shigella in their stool culture. Only 139 children fulfilled the study criteria and among them 69 were cases and 70 were controls. The cases more often had fatal outcome compared to the controls (7% vs. 0%, P = 0.02). In logistic regression analysis, the cases were independently associated with shorter duration (1.2 ± 0.4 days) of diarrhea prior to admission, dehydrating diarrhea, sepsis and hyponatremia (pShigella isolates, S. flexneri (88/139, 63%) and S. sonnei(34/139, 24%) were the dominant species. S. dysenteriae was not isolated throughout the study period. S.sonnei was more frequently isolated from the cases (24/69, 35%) than the controls (10/70, 14%), whereas the isolation of S. flexneri was comparable between the groups (40/69, 58% vs 48/70, 69%). A total of 94 (67.6%) isolates were resistant to trimethoprim-sulphamethoxazole, 84 (60.4%) to ciprofloxacin, 66/138 (48%) to ampicillin, 5 (3.5%) to ceftriaxone, 17 (12.2%) to mecillinum and 35 (25%) to azithromycin. The case-fatality-rate was significantly higher among the children with Shigella encephalopathy compared to those without

  14. Risk factors and outcome of Shigella encephalopathy in Bangladeshi children.

    Directory of Open Access Journals (Sweden)

    Farzana Afroze

    2017-04-01

    Full Text Available Although, Shigella encephalopathy, a serious extra-intestinal complication of shigellosis, significantly increases the risks of death, data are very limited on predicting factors particularly related to electrolyte profiles in children below five years of age with Shigella encephalopathy. Our objective was to determine the clinical as well as laboratory predicting factors and outcome of children with Shigella encephalopathy.In this unmatched case-control design, children aged 2-59 months having a positive stool culture for Shigella and who had their serum electrolytes been done from July 2012 to June 2015 were studied. Children with Shigella encephalopathy, defined as having abnormal mentation, constituted the cases, and those without encephalopathy constituted the controls. During the study period, we identified a total of 541 children less than five years of age, who had Shigella in their stool culture. Only 139 children fulfilled the study criteria and among them 69 were cases and 70 were controls. The cases more often had fatal outcome compared to the controls (7% vs. 0%, P = 0.02. In logistic regression analysis, the cases were independently associated with shorter duration (1.2 ± 0.4 days of diarrhea prior to admission, dehydrating diarrhea, sepsis and hyponatremia (p<0.05 for all. Among 139 Shigella isolates, S. flexneri (88/139, 63% and S. sonnei(34/139, 24% were the dominant species. S. dysenteriae was not isolated throughout the study period. S.sonnei was more frequently isolated from the cases (24/69, 35% than the controls (10/70, 14%, whereas the isolation of S. flexneri was comparable between the groups (40/69, 58% vs 48/70, 69%. A total of 94 (67.6% isolates were resistant to trimethoprim-sulphamethoxazole, 84 (60.4% to ciprofloxacin, 66/138 (48% to ampicillin, 5 (3.5% to ceftriaxone, 17 (12.2% to mecillinum and 35 (25% to azithromycin.The case-fatality-rate was significantly higher among the children with Shigella encephalopathy

  15. Laboratory Examinations of Transmissible Spongiform Encephalopathies in Denmark during 2013

    DEFF Research Database (Denmark)

    Jensen, Tim Kåre

    , Chapter 2.4.6 and Chapter 2.7.13) regarding diagnostic examinations. The DTU-VET is the national reference laboratory of bovine spongiform encephalopathy (BSE) and TSE/Scrapie, and therefore the results of all neuropathological examinations on BSE and Scrapie in Denmark are given in the present report......The aim of this report is to give detailed information on the diagnostic examination on trans-missible spongiform encephalopathies (TSE) performed in Denmark during 2013. The present annual report is the 18th on this topic published by the National Veterinary Institute, Technical University...

  16. Laboratory examinations of transmissible spongiform encephalopathies in Denmark during 2016

    DEFF Research Database (Denmark)

    Jensen, Tim Kåre

    , Chapter 2.4.6 and Chapter 2.7.13) regarding diagnostic examinations.The DTU-VET is the national reference laboratory of bovine spongiform encephalopathy (BSE) and TSE/Scrapie, and therefore the results of all neuropathological examinations on BSE and Scrapie in Denmark are given in the present report......The aim of this report is to give detailed information on the diagnostic examination on trans-missible spongiform encephalopathies (TSE) performed in Denmark during 2016. The present annual report is the 21st on this topic published by the National Veterinary Institute, Technical University...

  17. Laboratory Examinations of Transmissible Spongiform Encephalopathies in Denmark during 2012

    DEFF Research Database (Denmark)

    Jensen, Tim Kåre

    , Chapter 2.4.6 and Chapter 2.7.13) regarding diagnostic examinations. The DTU-VET is the national reference laboratory of bovine spongiform encephalopathy (BSE) and TSE/Scrapie, and therefore the results of all neuropathological examinations on BSE and Scrapie in Denmark are given in the present report......The aim of this report is to give detailed information on the diagnostic examination on trans-missible spongiform encephalopathies (TSE) performed in Denmark during 2012. The present annual report is the 17th on this topic published by the National Veterinary Institute, Technical University...

  18. Duloxetine-related posterior reversible encephalopathy syndrome: A case report.

    Science.gov (United States)

    Zappella, Nathalie; Perier, François; Pico, Fernando; Palette, Catherine; Muret, Alexandre; Merceron, Sybille; Girbovan, Andrei; Marquion, Fabien; Legriel, Stephane

    2016-08-01

    Posterior reversible encephalopathy syndrome (PRES) has well-established links with several drugs. Whether a link also exists with serotonin-norepinephrine reuptake inhibitor such as duloxetine is unclear. We report on a patient who developed PRES with a coma and myoclonus related to hypertensive encephalopathy a few days after starting duloxetine treatment. Magnetic resonance imaging was performed and catecholamine metabolites assayed. The patient achieved a full recovery after aggressive antihypertensive therapy and intravenous anticonvulsant therapy. The clinical history, blood and urinary catecholamine and serotonin levels, and response to treatment strongly suggest that PRES was induced by duloxetine. Duloxetine should be added to the list of causes of PRES.

  19. Treatment of chronic hepatic encephalopathy with levodopa 1

    Science.gov (United States)

    Lunzer, Michael; James, I. M.; Weinman, J.; Sherlock, Sheila

    1974-01-01

    Three of six patients with chronic hepatic encephalopathy treated with levodopa showed a significant improvement. One patient was probably improved whilst the remaining two patients failed to show any benefit. Serial electroencephalography did not demonstrate significant changes. Treatment with levodopa was associated with an improvement in `speed-based' tasks as assessed by computerized psychometry. A significant rise in cerebral oxygen consumption was found during levodopa therapy. Gastrointestinal side effects were dose limiting. It is concluded that a therapeutic trial of levodopa in patients with chronic hepatic encephalopathy is indicated when the response to conventional therapy has been poor. PMID:4430473

  20. Septic Encephalopathy Characterized by Acute Encephalopathy with Biphasic Seizures and Late Reduced Diffusion and Early Nonconvulsive Status Epilepticus

    Directory of Open Access Journals (Sweden)

    Hiroshi Yamaguchi

    2016-01-01

    Full Text Available Infection, whether viral or bacterial, can result in various forms of brain dysfunction (encephalopathy. Septic encephalopathy (SE is caused by an excessive immune reaction to infection, with clinical features including disturbed consciousness and seizures. Acute encephalopathy with biphasic seizures and late reduced diffusion (AESD is usually accompanied by viral infection in children and is characterized by biphasic seizures and impaired consciousness. The initial neurologic symptom of AESD is typically a febrile seizure that frequently lasts longer than 30 minutes. However, the possible forms this seizure takes are unclear. For example, it is unknown if nonconvulsive status epilepticus (NCSE could be an early seizure symptomatic of AESD. In addition, thus far no cases of combined SE and AESD have been reported. Here, we describe the first reported case of SE with AESD that notably demonstrated NCSE as an early seizure.

  1. Anti-HMGCR Autoantibodies in European Patients With Autoimmune Necrotizing Myopathies

    Science.gov (United States)

    Allenbach, Yves; Drouot, Laurent; Rigolet, Aude; Charuel, Jean Luc; Jouen, Fabienne; Romero, Norma B.; Maisonobe, Thierry; Dubourg, Odile; Behin, Anthony; Laforet, Pascal; Stojkovic, Tania; Eymard, Bruno; Costedoat-Chalumeau, Nathalie; Campana-Salort, Emmanuelle; Tournadre, Anne; Musset, Lucile; Bader-Meunier, Brigitte; Kone-Paut, Isabelle; Sibilia, Jean; Servais, Laurent; Fain, Olivier; Larroche, Claire; Diot, Elisabeth; Terrier, Benjamin; De Paz, Raphael; Dossier, Antoine; Menard, Dominique; Morati, Chafika; Roux, Marielle; Ferrer, Xavier; Martinet, Jeremie; Besnard, Sophie; Bellance, Remi; Cacoub, Patrice; Arnaud, Laurent; Grosbois, Bernard; Herson, Serge; Boyer, Olivier; Benveniste, Olivier

    2014-01-01

    Abstract Necrotizing autoimmune myopathy (NAM) is a group of acquired myopathies characterized by prominent myofiber necrosis with little or no muscle inflammation. Recently, researchers identified autoantibodies (aAb) against 3-hydroxy-3-methylglutaryl-coenzyme A reductase (HMGCR) in patients with NAM, especially in statin-exposed patients. Here we report what is to our knowledge the first European cohort of patients with NAM. The serum of 206 patients with suspicion of NAM was tested for detection of anti-HMGCR aAb using an addressable laser bead immunoassay. Forty-five patients were found to be anti-HMGCR positive. Their mean age was 48.9 ± 21.9 years and the group was predominantly female (73.3%). Statin exposure was recorded in 44.4% of patients. Almost all patients had a muscular deficit (97.7%), frequently severe (Medical Research Council [MRC] 5 ≤3 in 75.5%). Subacute onset (<6 mo) was noted for most of them (64.4%). Nevertheless, 3 patients (6.6%) had a slowly progressive course over more than 10 years. Except for weight loss (20%), no extramuscular sign was observed. The mean CK level was high (6941 ± 8802 IU/L) and correlated with muscle strength evaluated by manual muscle testing (r = −0.37, p = 0.03). Similarly, anti-HMGCR aAb titers were correlated with muscular strength (r = −0.31; p = 0.03) and CK level (r = 0.45; p = 0.01). Mean duration of treatment was 34.1 ± 40.8 months, and by the end of the study no patient had been able to stop treatment. This study confirms the observation and description of anti-HMGCR aAb associated with NAM. The majority of patients were statin naive and needed prolonged treatments. Some patients had a dystrophic-like presentation. Anti-HMGR aAb titers correlated with CK levels and muscle strength, suggesting their pathogenic role. PMID:24797170

  2. Expression patterns of micro-RNAs 146a, 181a, and 155 in subacute sclerosing panencephalitis.

    Science.gov (United States)

    Yiş, Uluç; Tüfekçi, Uğur Kemal; Genç, Şermin; Çarman, Kürşat Bora; Bayram, Erhan; Topçu, Yasemin; Kurul, Semra Hız

    2015-01-01

    Subacute sclerosing panencephalitis is caused by persistent brain infection of mutated virus, showing inflammation, neurodegeneration, and demyelination. Although many factors are emphasized in the pathogenesis of subacute sclerosing panencephalitis, the exact mechanism of neurodegeneration remains unknown. Micro-RNAs are small, noncoding RNAs that regulate gene expression at the posttranscriptional levels. Micro-RNAs are essential for normal immune system development; besides they are also implicated in the pathogenesis of many chronic inflammatory disorders. The aim of this study is to investigate the expression patterns of micro-RNAs 146a, 181a, and 155 in peripheral blood mononuclear cells of patients with subacute sclerosing panencephalitis. We enrolled 39 patients with subacute sclerosing panencephalitis and 41 healthy controls. Quantitative analysis of micro-RNAs 146a, 181a, and 155 were performed using specific stem-loop primers followed by real-time polymerase chain reaction. All of 3 micro-RNAs were upregulated in subacute sclerosing panencephalitis patients. In addition, the level of micro-RNA 155 expression was higher in stage 3 patients. But, micro-RNA 146a and 181a expression levels showed no association or correlation with clinically relevant data. Alteration of peripheral blood mononuclear cell micro-RNAs in subacute sclerosing panencephalitis may shed new light on the pathogenesis of disease and may contribute to the aberrant systemic rise in mRNA levels in subacute sclerosing panencephalitis. © The Author(s) 2014.

  3. MRI findings in acute hyperammonemic encephalopathy resulting from decompensated chronic liver disease.

    Science.gov (United States)

    Sureka, Jyoti; Jakkani, Ravi Kanth; Panwar, Sanuj

    2012-06-01

    Hyperammonemic encephalopathy is a type of metabolic encephalopathy with diversified etiology. Hyperammonemia is the end result of several metabolic disorders such as congenital deficiencies of urea cycle enzymes, hepatic encephalopathy, Reye's syndrome and other toxic encephalopathies. Non-specific clinical presentation poses a great challenge in early diagnosis of this entity. Irrespective of the underlying etiology, hyperammonemia causes a distinctive pattern of brain parenchymal injury. The cingulate gyrus and insular cortex are more vulnerable to this type of toxic insult. Characteristic magnetic resonance imaging findings in combination with laboratory parameters can help to differentiate this entity from other metabolic encephalopathy and thus aiding in early diagnosis and treatment.

  4. Acute anter ior necrotizing scler itis: A case repor t

    Directory of Open Access Journals (Sweden)

    Yuen Keat Gan

    2016-09-01

    Full Text Available Necrotizing scleritis is an uncommon but potential disastrous infection to the eye. It is commonly caused by vaso-occlusive autoimmune diseases such as rheumatoid arthritis or surgically-induced, and rarely due to infections. In this article, we presented a rare case of necrotizing scleritis caused by herpes infection in an immunocompromised patient. A 49 years old, retroviral positive gentleman presented to our clinic with a painful, red right eye associated with watering, photophobia and blurring of vision. His right eye rapidly deteriorated leading to an impending perforation of the sclera despite intensive antimicrobial therapy. The patient was started on acyclovir ointment and subsequently improved remarkably salvaging the eye from the need of an evisceration. Although the visual prognosis was poor, structural integrity of the eye was achieved.

  5. Necrotizing fasciitis following drainage of Streptococcus milleri empyema.

    Science.gov (United States)

    Tcherveniakov, Peter; Svennevik, Eirik; Tzafetta, Kallirroi; Milton, Richard

    2010-03-01

    Streptococcus milleri (SM) is a heterogeneous group of Streptococci, which is a recognized cause of purulent infections of the mediastinal and pleural spaces. These infections are notoriously resistant and require aggressive surgical management. We present our experience with a 60-year-old patient, who developed necrotizing fasciitis of the chest wall after initial bedside drainage of a SM empyema. He required extensive debridement with significant soft tissue loss and subsequent latissimus dorsi flap reconstruction to cover the defect.

  6. Complementory Value of Sonography in Early Evaluation of Necrotizing Enterocolitis

    OpenAIRE

    Kamali, Karmella; Hosseini, Say?yed Reza; Ardakani, Say?yed Mostajab Razavi Nedjad; Farnoodi, Mohammad Reza

    2015-01-01

    BACKGROUND: Despite the widespread use of plain films to detect necrotizing enterocolitis (NEC), it is considered a time-consuming method, which exposes patients to radiation. We aimed to assess changes in ultrasonographic variables and to compare sonograhy and chest radiography in detecting early stages of NEC in suspected premature infants. MATERIAL AND METHODS: This case-control study was carried out in the years 2012–2013. We enrolled 67 premature neonates using a simple sampling method a...

  7. Acute hyperammonemic encephalopathy with features on diffusion-weighted images: Report of two cases

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Ja Young; Yu, In Kyu [Dept. of Radiology, Eulji University Hospital, Daejeon (Korea, Republic of)

    2015-02-15

    Acute hyperammonemic encephalopathy is a rare toxic encephalopathy caused by accumulated plasma ammonia. A few literatures are reported about MRI findings of acute hyperammonemic encephalopathy. It is different from the well-known chronic hepatic encephalopathy. The clinical symptom and MRI findings of acute hyperammonemic encephalopathy can be reversible with proper treatment. Acute hepatic encephalopathy involves the cingulate cortex, diffuse cerebral cortices, insula, bilateral thalami on diffusion-weighted imaging (DWI), and fluid-attenuated inversion-recovery. Acute hepatic encephalopathy might mimic hypoxic-ischemic encephalopathy because of their similar predominant involving sites. We experienced 2 cases of acute hyperammonemic encephalopathy consecutively. They showed restricted diffusion at the cingulate cortex, cerebral cortices, insula, and bilateral dorsomedial thalami on DWI. One patient underwent acute fulminant hepatitis A, the other patient with underlying chronic liver disease had acute liver failure due to hepatotoxicity of tuberculosis medication. In this report, we presented the characteristic features of DWI in acute hyperammonemic encephalopathy. In addition, we reviewed articles on MRI findings of acute hyperammonemic encephalopathy.

  8. [Differential Diagnosis of Immune-Mediated Encephalopathies: "Neurological Symptoms of Diffuse Brain Damage": A New Concept].

    Science.gov (United States)

    Maki, Yoshimitsu; Takashima, Hiroshi

    2017-10-01

    In recent years, incidence of autoimmune encephalopathies has increased. The diagnosis of the severe form of autoimmune encephalopathy is not difficult; however, milder forms can be misdiagnosed as general encephalopathies. We often treat Hashimoto's encephalopathy, which has diverse clinical symptoms and is often misdiagnosed as a psychosomatic disease. We have found that the neurological findings and symptoms of patients with Hashimoto's encephalopathy are similar to those of psychogenic diseases, such as giveway weakness and atypical sensory disorder. To understand the mechanism underlying these symptoms, we propose a new concept: neurological symptoms of diffuse brain damage. This theory is based on the premise that etiologically, symptoms observed were caused by diffuse, spotty, and shaded brain damage due to autoimmune encephalopathies. We also found similar neurological conditions in patients with anti-ganglionic acetylcholine receptor antibody-related encephalopathy, encephalopathies that developed after injection of the cervical cancer vaccine, and encephalopathies associated with Stiff person syndrome. In conclusion, the clinical features of autoimmune encephalopathy include the "neurological symptoms of diffuse brain damage" as well as the presence of antibodies. We could diagnose autoimmune encephalopathy more easily, using this new diagnostic concept.

  9. Percutaneous necrosectomy in patients with acute, necrotizing pancreatitis

    Energy Technology Data Exchange (ETDEWEB)

    Bruennler, T.; Langgartner, J.; Lang, S.; Salzberger, B.; Schoelmerich, J. [University Hospital of Regensburg, Department of Internal Medicine 1, Regensburg (Germany); Zorger, N.; Herold, T.; Feuerbach, S.; Hamer, O.W. [University Hospital of Regensburg, Department of Radiology, Regensburg (Germany)

    2008-08-15

    The objective of this retrospective study was to evaluate the outcome of patients with acute necrotizing pancreatitis treated by active percutaneous necrosectomy. By searching the radiological, surgical and internal medicine databases, all patients with acute necrotizing pancreatitis treated by active percutaneous necrosectomy between 1992 and 2004 were identified. Demographic, laboratory, and clinical data, and details about invasive procedures were collected by reviewing patient charts, radiological and surgical reports. The computed tomography severity index (CTSI) scores were determined by reviewing CT images. Eighteen patients were identified. Median Ranson score on admission was 2. The Acute Physiology and Chronic Health Evaluation (APACHE) II score was median 22. Median CTSI score was 7. Initially all patients were treated with CT-guided drainage placement. Because passive drainage proved not to be effective, subsequent minimally invasive, percutaneous necrosectomy was performed. Eight out of 18 patients recovered fully without the need for surgery. Ten of 18 patients required additional surgical necrosectomy. For one of ten patients, percutaneous necrosectomy allowed postponing surgery by 39 days. Four of ten surgically treated patients died: three from septic multiorgan failure, one from pulmonary embolism. Percutaneous minimally invasive necrosectomy can be regarded as a safe and effective complementary treatment modality in patients with necrotizing pancreatitis. It is suitable for a subset of patients to avoid or delay surgery. (orig.)

  10. Tomato necrotic ringspot virus, a new tospovirus isolated in Thailand.

    Science.gov (United States)

    Seepiban, Channarong; Gajanandana, Oraprapai; Attathom, Tipvadee; Attathom, Supat

    2011-02-01

    A new tospovirus isolated from naturally infected tomato plants grown in Nakhon Pathom province (Thailand) was characterized. Infected plants showed symptoms consisting of necrotic spots, necrotic ringspots and stem necrosis. This virus was detected using general antibodies that could recognize watermelon silver mottle virus (WSMoV), capsicum chlorosis virus (CaCV) and melon yellow spot virus (MYSV). However, it did not react with specific monoclonal antibodies (MAbs) to WSMoV and CaCV or a specific MAb to MYSV. The complete nucleotide sequences of S and M RNAs of the virus were determined. They were 3,023 and 4,716 nucleotides in length, respectively, and contained two ORFs in an ambisense arrangement. Sequence analysis indicated that amino acid sequence of the N protein shared 58.2%, 56.0% and 51.8% identity with those of CaCV, WSMoV and MYSV, respectively. The virus was experimentally transmitted by Thrips palmi and Ceratothripoides claratris. Based on our results, we conclude that this tospovirus isolate should be considered a member of a new species. The name tomato necrotic ringspot virus (TNRV) is proposed for this tospovirus.

  11. Necrotizing Fasciitis Complicating Pregnancy: A Case Report and Literature Review

    Directory of Open Access Journals (Sweden)

    Marinos Nikolaou

    2014-01-01

    Full Text Available Necrotizing fasciitis is a rare, life-threatening surgical infection in pregnancy with high rates of morbidity and mortality. A 15-year-old primigravid woman, at 28 weeks of gestation with no significant previous medical history, was admitted to our hospital complaining of severe left lower extremity pain and high fever the last 72 hours. During clinical examination, she had a swollen, erythematous and tender to palpation inflamed skin over the medial aspect of the upper thigh without any evidence of injury. Incision drainage was performed immediately and she received broad spectrum antibiotics. During initial laboratory examinations, diabetes mellitus was diagnosed. There was no clinical improvement over the following days. Magnetic resonance imaging (MRI revealed subcutaneous tissue inflammation and edema of infected tissues confirming the disease entity. Multidisciplinary therapy with immediate aggressive surgical debridement of necrotic tissues, multiple antibiotics, and intensive care monitoring was performed successfully. The patient’s postoperative course was uncomplicated and skin defect was closed with split thickness skin grafting. Our case emphasized the potential immunosuppressive role of pregnancy state in conjunction with diabetes mellitus in the development of severe necrotizing soft tissue infections.

  12. Necrotizing fasciitis of the perineum associated with a bartholin abscess.

    Science.gov (United States)

    Morris, Michael W; Aru, Marco; Gaugler, Andrew; Morris, Rachael F; Vanderlan, Wesley B

    2014-04-01

    Perineal gangrene was first described in healthy, young males more than two and one-half centuries ago. This disease, referred to commonly as Fournier gangrene, was marked by rapid progression, high mortality, and unknown etiology. In the last century the pathologic processes were described and accounts of perineal gangrene were reported in females. This disease still demonstrates a male predominance, but mortality does not demonstrate a gender predilection. We present a case of a Bartholin abscess progressing to necrotizing fasciitis of the perineum in a 53-year-old female following drainage and marsupialization. Perineal gangrene was treated successfully with serial debridements and a targeted antimicrobial regimen, with wound closure by secondary intention. Following retrospective case review, the exact pathophysiologic cause of progression to necrotizing fasciitis of the perineum is unclear though antibiotic resistance was a likely contributor. Clinical studies are necessary to investigate the differential incidence of this disease, which may result from diagnostic unawareness of necrotizing fasciitis of the perineum in females due to adherence to Fournier's original description or coding bias. Future clinical studies may define risk factors for disease better, and allow for standardized management and improved outcomes regardless of gender.

  13. Dental Pulp Revascularization of Necrotic Permanent Teeth with Immature Apices.

    Science.gov (United States)

    El Ashiry, Eman A; Farsi, Najat M; Abuzeid, Sawsan T; El Ashiry, Mohamed M; Bahammam, Hammam A

    The treatment of immature necrotic teeth with apical periodontitis presents challenges in endodontic and pediatric dentistry. Revascularization is a recent treatment for such cases as an alternative to conventional apexification. The purpose is to examine the effect of a pulpal revascularization procedure on immature necrotic teeth with apical periodontitis. Twenty patients were enrolled for pulp revascularization procedure by root canal disinfection using a triple antibiotic mixture for 1-2 weeks, followed by creating a blood clot, sealing the root canal orifice using white mineral trioxide aggregate and a coronal seal of composite resin. Patients were recalled periodically for up to 24 months. During follow-up, all patients were asymptomatic. Three cases of chronic apical periodontitis showed clinical disappearance of the sinus tract 2 weeks after treatment. Radiography revealed progressive periapical radiolucency resolution within the first 12 months. Within 12-24 months, the treated teeth showed progressive increases in dentinal wall thickness, root length and continued root development. Clinical and radiographic evidence showed successful revascularization treatments of immature necrotic permanent teeth with apical periodontitis. More studies are necessary to understand the underlying mechanisms and to perform histopathology of the pulp space contents after revascularization procedures.

  14. An evaluation of neuropeptide Y status in subacute sclerosing panencephalitis patients.

    Science.gov (United States)

    Abuhandan, M; Calik, M; Almaz, V; Taskın, A; Cakmak, A; Selek, S

    2013-01-01

    This study aimed to evaluate the neuropeptide Y values of patients with subacute sclerosing panencephalitis. The study comprised 38 patients diagnosed with subacute sclerosing panencephalitis at our clinic, who were being routinely followed-up, together with a control group of 36. Using the Risk and Haddad classification for clinical staging, 16 patients were determined as Stage II and 22 patients as Stage III. Neuropeptide Y values were measured by Enzyme Immunoassay methods. The neuropeptide Y values of the all patients with subacute sclerosing panencephalitis were found to be significantly high compared to the control group (ppanencephalitis.

  15. Subacute thyroiditis following influenza vaccine: a case report and literature review

    Directory of Open Access Journals (Sweden)

    Munther S. Momani

    2015-06-01

    Full Text Available Subacute thyroiditis following influenza vaccination is a rarely reported condition. Here, we review this condition and report the case of a 40-year-old male who developed subacute thyroiditis following the administration of an influenza vaccine containing the (H1N1 pandemic 2009 strain. Interestingly, the patient had history of pericarditis and his HLA typing showed HLA B35. Physicians should be aware of this complication for influenza vaccine and of the relationship of HLA B35 haplotype with the susceptibility to subacute thyroiditis and other autoimmune disorders.

  16. Neuropsychological functioning in Wernicke′s encephalopathy

    Directory of Open Access Journals (Sweden)

    Sushree Sangita Behura

    2015-01-01

    Full Text Available Context: Wernicke′s encephalopathy (WE is caused by thiamine (Vitamin B1 deficiency and most commonly found in chronic alcoholism and malnutrition. Clinically, the key features are mental status disturbances (global confusion, oculomotor abnormalities, and gait disturbances (ataxia. Apart from these clinical features, we can find deficits in neuropsychological functioning in patients with WE, which is more prominent after the improvement in the physical conditions. Neuropsychological functioning includes both basic cognitive processes (i.e., attention-concentration as well as higher order cognitive processes (i.e., memory, executive functioning, reasoning, which is much vital for the maintenance of quality of life of an individual. However, unfortunately, in most of the cases, neuropsychological functioning is ignored by the clinicians. Materials and Methods: In this study four case reports of WE have been presented. The patients were taken from the outdoor department of Mental Health Institute, S.C.B. Medical College, Cuttack, Odisha. Neuropsychological functioning was measured by administration of PGIBBD and Quality of Life was measured by WHO-QOL BREF Odia Version. Discussion: As described in the literature, among the three cardinal signs ( global confusion, ataxia, and ocular sings, the first two were present in all cases, but nystagmus was present in only two cases.Memory dysfunction was so disabling that the persons were unable to maintain a good Quality of Life and occupational impairment was prominent. There are disturbances in recent, remote memory, immediate recall, delayed recall, and attention and concentration, ultimately creating both physical and mental disability. PGI-BBD findings also suggest the overall impairment in neuropsychological functioning other than memory, that is, executive functioning, visual acuity, and depth perception. Findings of WHO-QOL BREF suggest the impairment of four domains of QOL in all the cases, but

  17. [Clinical features and outcomes of patients with Hashimoto's encephalopathy].

    Science.gov (United States)

    Tang, Yi; Xing, Yi; Zhang, Jin; Jia, Jianping

    2014-03-11

    As an ill-defined syndrome consisting of heterogeneous neurological symptoms and high serum antithyroid antibody titers, Hashimoto's encephalopathy typically responds to steroids. More serial clinical studies are required to characterize the clinical, laboratory and imaging features and outcomes. We analyzed retrospectively the clinical, laboratory, and imaging features and outcomes of 15 consecutive patients with Hashimoto's encephalopathy diagnosed at our hospital from 2005 to 2011. Cognitive impairment (11/15) and psychiatric symptoms (5/15) were the most frequent manifestations. Seizure (4/15) and myoclonus (1/15) were less common than previously described. Three (3/15) patients showed abnormal signals in hippocampus or temporal lobe related to memory disorders. Among 10 patients on steroid therapy, there were recovery (n = 5), improvement with residual deficits (n = 2) and relapse or no effect (n = 3). Among 5 patients on non-steroid, there were stable remission with antiepileptic drugs or general neurotrophic therapy (n = 3) and continuous deterioration (n = 2). Most patients respond well to steroids while someone improves without steroid therapy. In light of its reversible course, we recommend that Hashimoto's encephalopathy should always be considered in the differential diagnosis while evaluating disorders of central nervous system, even disorders those without manifestations of encephalopathy.

  18. Chronic liver disease and hepatic encephalopathy: Clinical profile ...

    African Journals Online (AJOL)

    2011-03-08

    Mar 8, 2011 ... Background: Hepatic encephalopathy (HE) is an important neuropsychiatry complication of liver disease causing significant morbidity and mortality worldwide. Efforts at ... Access this article online. Quick Response Code: Website: ... Brain imaging with computerized tomographic scan was done where ...

  19. About pathognomonic images: an infrequent case of acute encephalopathy

    Directory of Open Access Journals (Sweden)

    Alessandro Grasso

    2013-05-01

    Full Text Available BACKGROUND The occurrence of acute encephalopathy is a dramatic clinical dilemma when usual diagnostic techniques (blood tests, cerebral CT and cerebrospinal fluid analysis show no abnormalities. CLINICAL CASE We describe a case of a 73 years old man admitted in our Internal Medicine Unit for acute diarrhoea with vomiting and fever who developed a prolonged gastrointestinal dysmotility syndrome with poor nutritional intake. Although a parenteral support was provided, he developed acute encephalopathy followed by hypotension and lactic acidosis without evidence of renal and hepatic disease or glycemic alterations. Likewise, no cerebral CT and cerebrospinal fluid alterations were found. Conversely, cerebral MRI showed marked and diffuse DP-2 and FLAIR hyperintensity of the mesencephalic tectal plate, of the periaqueductal area, and of the periventricular region of the third ventricle including the median thalamic area. These MRI descriptions were considered pathognomonic of Wernicke encephalopathy. Thus, the immediate use of ev thiamine was followed by a prompt and complete recovery of neurological, hemodinamic and metabolic conditions. CONCLUSIONS Non-alcoholic Wernicke encephalopathy is a rare and dramatic clinical event with high mortality. In this context, brain MRI is the best diagnostic tool providing a typical picture.

  20. Benzodiazepine receptor antagonists for acute and chronic hepatic encephalopathy

    DEFF Research Database (Denmark)

    Als-Nielsen, B; Kjaergard, L L; Gluud, C

    2001-01-01

    The pathogenesis of hepatic encephalopathy is unknown. It has been suggested that liver failure leads to the accumulation of substances that bind to a receptor-complex in the brain resulting in neural inhibition which may progress to coma. Several trials have assessed benzodiazepine receptor...

  1. Posterior reversible encephalopathy syndrome in acute intermittent porphyria.

    Science.gov (United States)

    Zhao, Bi; Wei, QianQian; Wang, YunHan; Chen, YongPing; Shang, HuiFang

    2014-09-01

    Acute intermittent porphyria is an inherited disease that is rarely diagnosed in prepubertal children. It can affect the autonomic, peripheral, and central nervous system. Posterior reversible encephalopathy syndrome is a clinicoradiological entity characterized by headache, seizures, altered consciousness, and visual disorder associated with potentially reversible neuroradiological abnormalities predominantly in the parieto-occipital lobes. We report a child with acute intermittent porphyria who presented with radiological manifestations suggestive of posterior reversible encephalopathy syndrome. A 9-year-old girl underwent an appendectomy after developing abdominal pain. She subsequently developed bilateral visual disturbance, confusion, seizures, hypertension, tachycardia, nausea, vomiting, constipation, dark tea-colored urine, and recurrent abdominal pain. Initial brain magnetic resonance imaging revealed hyperintense gyriform lesions on T2-weighted images and hypointense to isointense lesions on T1-weighted images in both parieto-occipital lobes with mild enhancement. The diagnosis of acute intermittent porphyria was confirmed by increased urinary excretion of porphyrin precursors. Her clinical signs gradually improved after intravenous high-dose glucose treatment and symptomatic therapies. A repeat magnetic resonance imaging confirmed complete resolution of the parieto-occipital lesions, suggesting with posterior reversible encephalopathy syndrome. The association of abdominal pain, mental status changes, and autonomic dysfunction should arouse the suspicion of acute intermittent porphyria. Acute intermittent porphyria can be associated with posterior reversible encephalopathy syndrome. Copyright © 2014 Elsevier Inc. All rights reserved.

  2. Hypothermia therapy for newborns with hypoxic ischemic encephalopathy.

    Science.gov (United States)

    Silveira, Rita C; Procianoy, Renato S

    2015-01-01

    Therapeutic hypothermia reduces cerebral injury and improves the neurological outcome secondary to hypoxic ischemic encephalopathy in newborns. It has been indicated for asphyxiated full-term or near-term newborn infants with clinical signs of hypoxic-ischemic encephalopathy (HIE). A search was performed for articles on therapeutic hypothermia in newborns with perinatal asphyxia in PubMed; the authors chose those considered most significant. There are two therapeutic hypothermia methods: selective head cooling and total body cooling. The target body temperature is 34.5 °C for selective head cooling and 33.5 °C for total body cooling. Temperatures lower than 32 °C are less neuroprotective, and temperatures below 30 °C are very dangerous, with severe complications. Therapeutic hypothermia must start within the first 6h after birth, as studies have shown that this represents the therapeutic window for the hypoxic-ischemic event. Therapy must be maintained for 72 h, with very strict control of the newborn's body temperature. It has been shown that therapeutic hypothermia is effective in reducing neurologic impairment, especially in full-term or near-term newborns with moderate hypoxic-ischemic encephalopathy. Therapeutic hypothermia is a neuroprotective technique indicated for newborn infants with perinatal asphyxia and hypoxic-ischemic encephalopathy. Copyright © 2015 Sociedade Brasileira de Pediatria. Published by Elsevier Editora Ltda. All rights reserved.

  3. A novel encephalopathy in a thiamine-deficient dog resembling ...

    African Journals Online (AJOL)

    A novel encephalopathy in a thiamine-deficient dog resembling human Wernicke's disease with atypical MRI pattern. ... Thiamine is a water-soluble vitamin, which participates in several vital metabolic pathways involved in energy metabolism and neurotransmitter synthesis of mammals. In companion animals thiamine ...

  4. Another cause of vaccine encephalopathy: a case of Angelman syndrome.

    Science.gov (United States)

    Novy, Jan; Catarino, Claudia B; Chinthapalli, Krishna; Smith, Shelagh M; Clayton-Smith, Jill; Hennekam, Raoul C M; Hammond, Peter; Sisodiya, Sanjay M

    2012-05-01

    Dravet syndrome has been found recently as an important underlying condition in cases of alleged vaccine encephalopathy after pertussis vaccination, where vaccination seemed to have precipitated the occurrence of the disease without modifying the long-term course. We report on a patient diagnosed with Angelman syndrome in her fifth decade, in whom the intellectual disability and epilepsy had been assumed to be caused by a vaccine encephalopathy following smallpox vaccination. Clinical features of Angelman syndrome had faded away. The history of the present patient suggests that genetic conditions other than Dravet syndrome can be associated with an alleged vaccine encephalopathy. A history of vaccine encephalopathy is rare among patients with learning disability and refractory epilepsy (1.4% in our cohort), but it should lead to consideration of a comprehensive genetic work-up if Dravet syndrome is excluded. The early history of the patient, when available, should guide the investigations. Medico-legal aspects are also discussed. Copyright © 2012 Elsevier Masson SAS. All rights reserved.

  5. Another cause of vaccine encephalopathy: A case of Angelman syndrome

    NARCIS (Netherlands)

    Novy, Jan; Catarino, Claudia B.; Chinthapalli, Krishna; Smith, Shelagh M.; Clayton-Smith, Jill; Hennekam, Raoul C. M.; Hammond, Peter; Sisodiya, Sanjay M.

    2012-01-01

    Dravet syndrome has been found recently as an important underlying condition in cases of alleged vaccine encephalopathy after pertussis vaccination, where vaccination seemed to have precipitated the occurrence of the disease without modifying the long-term course. We report on a patient diagnosed

  6. A quantitative risk assessment for bovine spongiform encephalopathy in Japan

    NARCIS (Netherlands)

    Kadohira, M.; Stevenson, M.A.; Hogasen, H.R.; Koeijer, de A.A.

    2012-01-01

    A predictive case-cohort model was applied to Japanese data to analyze the interaction between challenge and stability factors for bovine spongiform encephalopathy (BSE) for the period 1985–2020. BSE risk in cattle was estimated as the expected number of detectable cases per year. The model was

  7. A case of chronic Wernicke's encephalopathy: A neuropsychological study

    NARCIS (Netherlands)

    Oudman, Erik; Van der Stigchel, Stefan; Postma, Albert; Wijnia, Jan W.; Nijboer, Tanja C W

    2014-01-01

    A 54-year-old woman was referred to our Korsakoff Center because of extensive cognitive problems following acute Wernicke's encephalopathy (WE). She had a relatively short history of alcohol abuse and was found lying on the floor in her home by her son. After 5 days without treatment, she was

  8. Hepatic Encephalopathy: Early Diagnosis in Pediatric Patients With Cirrhosis

    Science.gov (United States)

    DARA, Naghi; SAYYARI, Ali-Akbar; IMANZADEH, Farid

    2014-01-01

    Objective As acute liver failure (ALF) and chronic liver disease (cirrhosis) continue to increase in prevalence, we will see more cases of hepatic encephalopathy. Primary care physician are often the first to suspect it, since they are familiar with the patient’s usual physical and mental status. This serious complication typically occurs in patients with severe comorbidities and needs multidisciplinary evaluation and care. Hepatic encephalopathy should be considered in any patient with acute liver failure and cirrhosis who presents with neuropsychiatric manifestations, decrease level of consciousness (coma), change of personality, intellectual and behavioral deterioration, speech and motor dysfunction. Every cirrhotic patient may be at risk; potential precipitating factors should be addressed in regular clinic visits. The encephalopathy of liver disease may be prominent, or can be present in subtle forms, such as decline of school performance, emotional outbursts, or depression. “Subtle form” of hepatic encephalopathy may not be obvious on clinical examination, but can be detected by neurophysiologic and neuropsychiatric testing. PMID:24665321

  9. Antithyroperoxidase Antibodies in Encephalopathy : Diagnostic Marker or Incidental Finding?

    NARCIS (Netherlands)

    Dontje, B.; Van Santen, H. M.; Niermeijer, J. M.; Schonenberg-Meinema, D.; Van Trotsenburg, A. S P

    2016-01-01

    Patients with acute encephalopathy who are thoroughly examined for an underlying diagnosis and in whom infectious, metabolic, and malignant causes are excluded can form a true diagnostic dilemma. If antithyroperoxidase antibodies (anti-TPO abs) are present, the diagnosis steroid responsive

  10. MRI reveals reversible lesions resembling posterior reversible encephalopathy in porphyria

    Energy Technology Data Exchange (ETDEWEB)

    Celik, M. [Huesrev Gerede c, 128/4 Tesvikiye, 80690 Istanbul (Turkey); Department of Neurology, Sisli Etfal Education and Research Hospital, Sisli Etfal S., Sisli, Istanbul (Turkey); Forta, H.; Babacan, G. [Department of Neurology, Sisli Etfal Education and Research Hospital, Sisli Etfal S., Sisli, Istanbul (Turkey); Dalkilic, Tuerker [Department of Neurosurgery, Sisli Etfal Education and Research Hospital, Sisli Etfal S., Sisli, Istanbul (Turkey)

    2002-10-01

    We report a 20-year-old woman who had an attack of acute intermittent porphyria with seizures, hallucinations, autonomic and somatic neuropathy. T2-weighted MRI revealed multiple lesions which were no longer visible 3 months later. We suggest a similar mechanism to posterior reversible encephalopathy underlying cerebral symptoms in porphyria. (orig.)

  11. Laboratory Examinations of Transmissible Spongiform Encephalopathies in Denmark during 2014

    DEFF Research Database (Denmark)

    Jensen, Tim Kåre

    , Chapter 2.4.6 and Chapter 2.7.13) regarding diagnostic examinations. The DTU-VET is the national reference laboratory of bovine spongiform encephalopathy (BSE) and TSE/Scrapie, and therefore the results of all neuropathological examinations on BSE and Scrapie in Denmark are given in the present report...

  12. Models for discovery of targeted therapy in genetic epileptic encephalopathies.

    Science.gov (United States)

    Maljevic, Snezana; Reid, Christopher A; Petrou, Steven

    2017-10-01

    Epileptic encephalopathies are severe disorders emerging in the first days to years of life that commonly include refractory seizures, various types of movement disorders, and different levels of developmental delay. In recent years, many de novo occurring variants have been identified in individuals with these devastating disorders. To unravel disease mechanisms, the functional impact of detected variants associated with epileptic encephalopathies is investigated in a range of cellular and animal models. This review addresses efforts to advance and use such models to identify specific molecular and cellular targets for the development of novel therapies. We focus on ion channels as the best-studied group of epilepsy genes. Given the clinical and genetic heterogeneity of epileptic encephalopathy disorders, experimental models that can reflect this complexity are critical for the development of disease mechanisms-based targeted therapy. The convergence of technological advances in gene sequencing, stem cell biology, genome editing, and high throughput functional screening together with massive unmet clinical needs provides unprecedented opportunities and imperatives for precision medicine in epileptic encephalopathies. © 2017 International Society for Neurochemistry.

  13. Metformin inhibits glutaminase activity and protects against hepatic encephalopathy.

    Science.gov (United States)

    Ampuero, Javier; Ranchal, Isidora; Nuñez, David; Díaz-Herrero, María del Mar; Maraver, Marta; del Campo, José Antonio; Rojas, Ángela; Camacho, Inés; Figueruela, Blanca; Bautista, Juan D; Romero-Gómez, Manuel

    2012-01-01

    To investigate the influence of metformin use on liver dysfunction and hepatic encephalopathy in a retrospective cohort of diabetic cirrhotic patients. To analyze the impact of metformin on glutaminase activity and ammonia production in vitro. Eighty-two cirrhotic patients with type 2 diabetes were included. Forty-one patients were classified as insulin sensitizers experienced (metformin) and 41 as controls (cirrhotic patients with type 2 diabetes mellitus without metformin treatment). Baseline analysis included: insulin, glucose, glucagon, leptin, adiponectin, TNFr2, AST, ALT. HOMA-IR was calculated. Baseline HE risk was calculated according to minimal hepatic encephalopathy, oral glutamine challenge and mutations in glutaminase gene. We performed an experimental study in vitro including an enzymatic activity assay where glutaminase inhibition was measured according to different metformin concentrations. In Caco2 cells, glutaminase activity inhibition was evaluated by ammonia production at 24, 48 and 72 hours after metformina treatment. Hepatic encephalopathy was diagnosed during follow-up in 23.2% (19/82): 4.9% (2/41) in patients receiving metformin and 41.5% (17/41) in patients without metformin treatment (logRank 9.81; p=0.002). In multivariate analysis, metformin use [H.R.11.4 (95% CI: 1.2-108.8); p=0.034], age at diagnosis [H.R.1.12 (95% CI: 1.04-1.2); p=0.002], female sex [H.R.10.4 (95% CI: 1.5-71.6); p=0.017] and HE risk [H.R.21.3 (95% CI: 2.8-163.4); p=0.003] were found independently associated with hepatic encephalopathy. In the enzymatic assay, glutaminase activity inhibition reached 68% with metformin 100 mM. In Caco2 cells, metformin (20 mM) decreased glutaminase activity up to 24% at 72 hours post-treatment (p<0.05). Metformin was found independently related to overt hepatic encephalopathy in patients with type 2 diabetes mellitus and high risk of hepatic encephalopathy. Metformin inhibits glutaminase activity in vitro. Therefore, metformin use seems

  14. Metformin inhibits glutaminase activity and protects against hepatic encephalopathy.

    Directory of Open Access Journals (Sweden)

    Javier Ampuero

    Full Text Available AIM: To investigate the influence of metformin use on liver dysfunction and hepatic encephalopathy in a retrospective cohort of diabetic cirrhotic patients. To analyze the impact of metformin on glutaminase activity and ammonia production in vitro. METHODS: Eighty-two cirrhotic patients with type 2 diabetes were included. Forty-one patients were classified as insulin sensitizers experienced (metformin and 41 as controls (cirrhotic patients with type 2 diabetes mellitus without metformin treatment. Baseline analysis included: insulin, glucose, glucagon, leptin, adiponectin, TNFr2, AST, ALT. HOMA-IR was calculated. Baseline HE risk was calculated according to minimal hepatic encephalopathy, oral glutamine challenge and mutations in glutaminase gene. We performed an experimental study in vitro including an enzymatic activity assay where glutaminase inhibition was measured according to different metformin concentrations. In Caco2 cells, glutaminase activity inhibition was evaluated by ammonia production at 24, 48 and 72 hours after metformina treatment. RESULTS: Hepatic encephalopathy was diagnosed during follow-up in 23.2% (19/82: 4.9% (2/41 in patients receiving metformin and 41.5% (17/41 in patients without metformin treatment (logRank 9.81; p=0.002. In multivariate analysis, metformin use [H.R.11.4 (95% CI: 1.2-108.8; p=0.034], age at diagnosis [H.R.1.12 (95% CI: 1.04-1.2; p=0.002], female sex [H.R.10.4 (95% CI: 1.5-71.6; p=0.017] and HE risk [H.R.21.3 (95% CI: 2.8-163.4; p=0.003] were found independently associated with hepatic encephalopathy. In the enzymatic assay, glutaminase activity inhibition reached 68% with metformin 100 mM. In Caco2 cells, metformin (20 mM decreased glutaminase activity up to 24% at 72 hours post-treatment (p<0.05. CONCLUSIONS: Metformin was found independently related to overt hepatic encephalopathy in patients with type 2 diabetes mellitus and high risk of hepatic encephalopathy. Metformin inhibits glutaminase

  15. CADASIL: Migraine, Encephalopathy, Stroke and Their Inter-Relationships.

    Science.gov (United States)

    Tan, Rhea Yan Ying; Markus, Hugh Stephen

    2016-01-01

    Migraine is common in Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy (CADASIL) but its treatment responses are not well described, and its relationship to stroke risk unknown. Encephalopathy is a less common presentation; it has been suggested it is related to migraine. We characterised migraine patterns and treatment responses in CADASIL, and examined associations between migraine and both stroke risk and encephalopathy. 300 symptomatic CADASIL patients were prospectively recruited from a national referral clinic over a nineteen year period, from 1996 to 2015. Data was collected using a standardised questionnaire. Migraine was classified according to the International Classification of Headache Disorders, 3rd edition (beta version). A cross-sectional analysis was carried out on the data collected. Migraine was present in 226 (75.3%), and the presenting feature in 203 (67.7%). It was usually accompanied by aura (89.8%). Patients showed variable responses to a variety of drugs for migraine. Of 24 given triptans, 45.5% had consistent or partial responses. None had complications following triptans. Thirty-three (11.0%) patients experienced encephalopathy lasting on average 8.1 ± 3.4 days. Patients with migraine with aura had higher odds of encephalopathy (OR = 5.4; 95%CI 1.6-28.4; p = 0.002). Patients with confusional aura had higher odds of encephalopathy than those with other aura types (OR = 2.5, 95%CI = 1.0-5.8, p = 0.04). There was also no increase in risk of encephalopathy with sex or age at onset of migraine. Migraineurs had a lower stroke risk than non-migraineurs (HR = 0.46, 95%CI 0.3-0.6, p = 2.1x10-6). Migraine with aura is a prominent feature of CADASIL. Treatment responses are similar to those seen in the general migraine population and no complications were observed with triptans. Migraine with aura was associated with increased risk of encephalopathy suggesting they may share pathophysiological mechanisms

  16. The burden of hepatic encephalopathy in Latin America.

    Science.gov (United States)

    Dávalos Moscol, Milagros; Bustios Sanchez, Carla

    2011-06-01

    Hepatic encephalopathy (HE) is a neuropsychiatric syndrome characterized by changes in cognitive function, behavior, and personality, as well as by transient neurological symptoms and electroencephalographic changes, which occur in the context of acute or chronic liver failure. Cirrhosis is the main disease associated to HE, and it is known that its incidence is increasing worldwide. As a cause of mortality, cirrhosis is ranked 14 worldwide, but 10 in developed countries. It has been demonstrated that the incidence of liver disease is increasing, in part because of the ascending prevalence of NAFLD, HCV, HCC, as well of alcohol consumption. The real incidence of cirrhosis in Latin America is unknown, although in some Latin American countries that provided national data, cirrhosis death rates were between 5 and 17/100,000 for men and 3 and 5/100,000 for women. Disability, quality of life, and social aspects should be considered when assessing the impact of a disease. In this context, preliminary estimates of the global burden of disease attributable to chronic liver disease seem to be substantial. Hepatic encephalopathy, a main complication of liver failure, occurs in 30-45% of patients as overt encephalopathy, but when subclinical or minimal hepatic encephalopathy (MHE) is considered, estimates of the incidence of encephalopathy vary from 20 to 60%. In USA, the 2009 NIH Report on the Costs of Digestive Diseases stated that liver disease was the second most costly disease in direct and indirect costs (13.1 billion dollars). Although the economic cost of HE has not been assessed, it is obvious that the economic impact of HE on daily activities of living is extremely high, as the costs of diminished work performance and lost wages are substantial.

  17. Blood manganese levels in patients with hepatic encephalopathy.

    Science.gov (United States)

    Zerón, Hugo Mendieta; Rodríguez, Mónica Rodríguez; Montes, Sergio; Castañeda, Camilo Ríos

    2011-12-01

    Hepatic encephalopathy is an increasingly common disease. Identification of prognosis risk factors in patients with liver damage may lead to preventive actions, towards decreasing its mortality. Manganese (Mn) levels are increased in basal ganglia of patients with hepatic encephalopathy as well as in cases of cirrhotic and liver failure patients. The present is a clinical, prospective, prolective and observational study developed at the Internal Medicine Service from "Dr. Darío Fernández Fierro" General Hospital, ISSSTE, Mexico City. The objective of this work was to report whole blood Mn levels and mortality in encephalopathic patients. Consecutive patients over 18 years of age, diagnosed with hepatic encephalopathy were recruited at the emergency room service. An informed consent, signed by their families was collected. Patients' clinical characteristics, biochemical tests of renal function, hemoglobin, glucose, bilirubins and albumin levels were obtained along with a blood sample to analyze Mn. Patients evolution was followed up for 6 months. Blood Mn in patients [median, (range)] [20.5, (10.5-39.5) μg/L] were higher than blood levels from a group of healthy volunteers [7.5, (6.1-12.8) μg/L] (P<0.001). Among 9 patients studied four died, 2 women and 2 men, those patients showed higher (P=0.032) Mn levels [28, (17-39.5) μg/L] than those alive [13.5, (10.5-32) μg/L] after the follow up period. In this pilot study, Mn blood levels were higher in hepatic encephalopathy that died as consequence of the disease that those that survived in a 6 month follow up period. Blood Mn could be a potential prognosis factor for death in patients with hepatic encephalopathy. Copyright © 2011 Elsevier GmbH. All rights reserved.

  18. Case report: subacute tetraplegia in an immunocompromised patient.

    Science.gov (United States)

    Zeller, Daniel; Heidemeier, Anke; Grigoleit, Götz Ulrich; Müllges, Wolfgang

    2017-02-10

    Clinical reasoning in Neurology is based on general associations which help to deduce the site of the lesion. However, even "golden principles" may occasionally be deceptive. Here, we describe the case of subacute flaccid tetraparesis due to motor cortical lesions. To our knowledge, this is the first report to include an impressive illustration of nearly symmetric motor cortical involvement of encephalitis on brain MRI. A 51 year old immunocompromized man developed a high-grade pure motor flaccid tetraparesis over few days. Based on clinical presentation, critical illness polyneuromyopathy was suspected. However, brain MRI revealed symmetrical hyperintensities strictly limited to the subcortical precentral gyrus. An encephalitis, possibly due to CMV infection, turned out to be the most likely cause. While recognition of basic clinical patterns is indispensable in neurological reasoning, awareness of central conditions mimicking peripheral nervous disease may be crucial to detect unsuspected, potentially treatable conditions.

  19. Subacute brachial diplegia associated with West Nile virus myelitis.

    Science.gov (United States)

    Zafar, Sahar F; Ubogu, Eroboghene E

    2012-06-01

    Brachial diplegia is a clinical term used to describe weakness restricted to the upper extremities. We report a case of brachial diplegia associated with West Nile virus infection. A 48-year-old man developed severe painless bilateral upper extremity weakness within a few weeks of a flu-like illness. Clinical examination revealed marked periscapular, shoulder girdle, and humeral muscle atrophy and bilateral scapular winging, with near symmetrical bilateral hypotonic upper extremity weakness. This was associated with clinical signs of an encephalomyelopathy without cognitive or sensory deficits. Electrophysiological studies demonstrated a subacute disorder of motor neurons, their axons or both, involving the cervical and thoracic myotomes, with ongoing denervation. Serological studies confirmed recent West Nile virus (WNV) infection. Gradual improvement occurred following conservative supportive therapies. Progressive brachial diplegia is a rare neuromuscular presentation of WNV neuroinvasive disease. This case report adds to the clinical spectrum of WNV-induced neurologic sequelae. Copyright © 2012 Wiley Periodicals, Inc.

  20. Spectrophotometry of cerebrospinal fluid in subacute and chronic subdural haematomas

    Science.gov (United States)

    Kjellin, K. G.; Steiner, L.

    1974-01-01

    Spectrophotometric examinations were performed on cerebrospinal and subdural fluids in subacute (five patients) and chronic (20 patients) subdural haematomas, with special reference to the diagnostic aid of CSF spectrophotometry. Spectrophotometric xanthochromia of haemorrhagic origin was found in all CSFs examined, while definite visible xanthochromia was observed in only 28% and the CSF was judged as colourless in 52% of those cases. Characteristic bleeding patterns were found spectrophotometrically in all the 20 CSFs examined within 24 hours after lumbar puncture, haematoma patterns being detected in 90-95% of the cases. In many cases the electrophoretically separated protein fractions of CSF and subdural fluids were spectrophotometrically examined. In conclusion, CSF spectrophotometry is a simple, fast, and extremely sensitive method, which in our opinion should be used routinely in the diagnosis of suspected subdural haematomas, if lumbar puncture is not contraindicated. PMID:4140892

  1. Subacute Sclerosing Panencephalitis in a Toddler: Changing Epidemiological Trends

    Directory of Open Access Journals (Sweden)

    Roosy Aulakh

    2013-01-01

    Full Text Available Subacute sclerosing panencephalitis (SSPE is a devastating “slow virus” brain disease resulting from persistent measles virus infection of neurons. The age at presentation is usually 8 to 11 years with onset usually occurring 2–10 years after measles infection. We report a 2-and-half-year-old boy who presented with progressively increasing myoclonic jerks and subtle cognitive decline. He was diagnosed as a case of SSPE based on clinical features, typical electroencephalographic finding, and elevated cerebrospinal fluid/serum measles antibody titers. He had measles 4 months prior to onset of symptoms. This case along with review of recently published reports suggests progressively decreasing latency period between measles infection and onset of symptoms observed in cases with SSPE. Clinical implication would mean investigating for SSPE even in infants or toddlers with compatible clinical features and recent history of measles infection.

  2. Subacute sclerosing panencephalitis resembling Rasmussen's encephalitis on magnetic resonance imaging.

    Science.gov (United States)

    Jakkani, Ravi Kanth; Sureka, Jyoti; Panwar, Sanuj

    2015-09-01

    Subacute sclerosing panencephalitis (SSPE) is a rare, slowly progressing but invariably fatal disease that is related to a prior measles virus infection and most commonly affects paediatric patients. Magnetic resonance (MR) imaging is the modality of choice for determining such changes in white matter. SSPE typically demonstrates bilateral but asymmetric periventricular and subcortical white matter involvement. We herein report a rare case of unilateral white matter involvement in a 13-year-old boy with SSPE that closely simulated Rasmussen's encephalitis. To the best of our knowledge, this is the first report of an atypical presentation on MR imaging in which SSPE was a rare cause of unilateral brain parenchymal involvement in a patient with intractable seizures.

  3. Notes from the Field: Subacute Sclerosing Panencephalitis Death - Oregon, 2015.

    Science.gov (United States)

    Liko, Juventila; Guzman-Cottrill, Judith A; Cieslak, Paul R

    2016-01-15

    In 2015, the Oregon Health Authority was notified of the death of a boy with subacute sclerosing panencephalitis (SSPE), a rare and fatal complication of measles. The patient, aged 14 years, had reportedly been vaccinated against measles in the Philippines at age 8 months. However, the patient contracted measles at age 1 year while still in the Philippines. He had been well until 2012, when his neurodegenerative symptoms began. After the diagnosis of SSPE was made, the patient remained in home hospice care until his death. Investigators from the Oregon Health Authority and the Oregon Health and Science University reviewed the patient's medical records and interviewed the parents. Vaccination against measles can prevent not only acute measles and its complications, but also SSPE.

  4. Cerebral glucose metabolism in the course of subacute sclerosing panencephalitis

    Energy Technology Data Exchange (ETDEWEB)

    Huber, M.; Herholz, K.; Pawlik, G.; Szelies, B.; Juergens, R.H.; Heiss, W.D.

    1989-01-01

    Regional cerebral glucose metabolism was studied in a 15-year-old boy with subacute sclerosing panencephalitis before and after therapy with human interferon beta, using positron emission tomography of fluorine 18-2-fluoro-2-deoxyglucose. At first examination, metabolism was symmetrically decreased in the thalamus, cerebellum, and all cortical areas except prerolandic motor cortex, but increased in lentiform nucleus. A computed tomographic scan was normal. Six months later, bilateral focal necrosis centered in the previously hypermetabolic putamen was demonstrated by computed tomography and magnetic resonance imaging. The caudate nucleus and the superoposterior part of the putamen were spared, still showing increased metabolism. Corresponding with some clinical improvement, cortical glucose consumption rates had returned to a normal level.

  5. Subacute sclerosing panencephalitis (SSPE) the story of a vanishing disease.

    Science.gov (United States)

    Gadoth, Natan

    2012-10-01

    Subacute sclerosing panencephalitis (SSPE), is a devastating "slow virus" brain disease which affects young children who had measles some 6-7 years earlier. Although, the pandemic of SSPE during 1960-1980's was almost eradicated due to mass immunization, the disease is still taking the life of young children in countries where measles immunization is incomplete and in world regions where genetic polymorphism to this particular infection is present. The present review was written for the fortunate young generation of pediatricians and pediatric neurologists who probably have not seen a case of SSPE during their career, and for those who work in counties where the disease has not been eradicated. It is also a reminder that with full coverage of measles immunization this devastating disease can be fully eradicated. Copyright © 2012 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.

  6. Subacute Sclerosing Panencephalitis with Atypical Clinical and MRI Findings

    Directory of Open Access Journals (Sweden)

    Buse Rahime Hasırcı

    2016-04-01

    Full Text Available Subacute sclerosing panencephalitis (SSPE is a progressive inflammatory disorder of the central nervous system and a slow virus infection caused by aberrant measles virüs. Typical neurological manifestations include psychomotor impairment, progressive intellectual deterioration, myoclonic jerks and behavioral changes, with or without pyramidal symptoms. It usually affecting people aged 10 to 14 year. We report the case of an 17-year-old girl presenting with initial symptom of visual loss, seizures, a lack of SSPE specific EEG pattern, late onset and atypical fast progression of disease. The case highlights the importance of atypical clinical findings of SSPE at onset and also firstly disappearing, then appearing MRI findings at sequential images which can complicate the accurate diagnosis. High suspicion is needed because of its rareness.

  7. Atypical magnetic resonance imaging features in subacute sclerosing panencephalitis

    Directory of Open Access Journals (Sweden)

    Biplab Das

    2016-01-01

    Full Text Available Objectives: Subacute sclerosing panencephalitis (SSPE is rare chronic, progressive encephalitis that affects primarily children and young adults, caused by a persistent infection with measles virus. No cure for SSPE exists, but the condition can be managed by medication if treatment is started at an early stage. Methods and Results: Heterogeneity of imaging findings in SSPE is not very uncommon. But pial and gyral enhancements are very rarely noticed. Significant asymmetric onset as well as pial-gyral enhancements is not reported. Herein we present a case of 16 years adolescent of SSPE having remarkable asymmetric pial-gyral enhancements, which were misinterpreted as tubercular infection. Conclusion: Early diagnosis and treatment is encouraging in SSPE, although it is not curable with current therapy. Clinico-radiological and electrophysiological correlation is very important in diagnosis of SSPE, more gravely in patients having atypical image findings as in our index case.

  8. Mesenchymal stem cell application in children with subacute sclerosing panencephalitis.

    Science.gov (United States)

    Kuşkonmaz, Bariş; Uçkan, Duygu; Yalnizoğlu, Dilek; Günel, Mintaze; Karli Oğuz, Kader; Konuşkan, Bahadir; Anlar, Banu

    2015-09-01

    Subacute sclerosing panencephalitis (SSPE) is a serious, often fatal disease that responds poorly to current treatment modalities. Recently, the ability of mesenchymal stem cells (MSCs) to produce neurotrophic factors and inflammatory molecules has placed them among potential treatment agents for neurological conditions. We report the results of four patients treated with MSC for SSPE. The patients were followed up clinically, and by periodical laboratory evaluations, magnetic resonance imaging (MRI), and electroencephalography. One patient deteriorated to stage III of the disease, two patients remained in the same stage, and one died from disease progression and respiratory problems. Neurological findings and electroencephalography scores were consistent with the clinical course of the patient whereas MRI showed new inflammatory lesions in two patients. This is the first report of the application of MSC in SSPE. No benefit is demonstrated. © 2015 Mac Keith Press.

  9. Atypical magnetic resonance imaging features in subacute sclerosing panencephalitis.

    Science.gov (United States)

    Das, Biplab; Goyal, Manoj Kumar; Modi, Manish; Mehta, Sahil; Chakravarthi, Sudheer; Lal, Vivek; Vyas, Sameer

    2016-01-01

    Subacute sclerosing panencephalitis (SSPE) is rare chronic, progressive encephalitis that affects primarily children and young adults, caused by a persistent infection with measles virus. No cure for SSPE exists, but the condition can be managed by medication if treatment is started at an early stage. Heterogeneity of imaging findings in SSPE is not very uncommon. But pial and gyral enhancements are very rarely noticed. Significant asymmetric onset as well as pial-gyral enhancements is not reported. Herein we present a case of 16 years adolescent of SSPE having remarkable asymmetric pial-gyral enhancements, which were misinterpreted as tubercular infection. Early diagnosis and treatment is encouraging in SSPE, although it is not curable with current therapy. Clinico-radiological and electrophysiological correlation is very important in diagnosis of SSPE, more gravely in patients having atypical image findings as in our index case.

  10. Subacute Sclerosing Panencephalitis in a Toddler: Changing Epidemiological Trends

    Science.gov (United States)

    Aulakh, Roosy; Tiwari, Abhimanyu

    2013-01-01

    Subacute sclerosing panencephalitis (SSPE) is a devastating “slow virus” brain disease resulting from persistent measles virus infection of neurons. The age at presentation is usually 8 to 11 years with onset usually occurring 2–10 years after measles infection. We report a 2-and-half-year-old boy who presented with progressively increasing myoclonic jerks and subtle cognitive decline. He was diagnosed as a case of SSPE based on clinical features, typical electroencephalographic finding, and elevated cerebrospinal fluid/serum measles antibody titers. He had measles 4 months prior to onset of symptoms. This case along with review of recently published reports suggests progressively decreasing latency period between measles infection and onset of symptoms observed in cases with SSPE. Clinical implication would mean investigating for SSPE even in infants or toddlers with compatible clinical features and recent history of measles infection. PMID:24416610

  11. Progressive subacute Miller-Fisher syndrome successfully treated with plasmapheresis.

    Science.gov (United States)

    Ejma, Maria; Waliszewska-Prosół, Marta; Hofman, Anna; Budrewicz, Sławomir; Podemski, Ryszard; Bilińska, Małgorzata; Koszewicz, Magdalena

    2015-01-01

    Miller-Fisher Syndrome (MFS) is a rare acute polyneuropathy composed of the clinical triad of ataxia, areflexia and ophthalmoplegia, with a monophasic, self-limited course and spontaneous improvement. The authors present a 65-year-old man with Miller-Fisher syndrome consisting of bilateral ophthalmoplegia, trigeminal and facial nerve palsy, mild ataxia and peripheral neuropathy. The disease had a progressive, subacute course within 3 months. A high titer of anti-GQ1b antibodies was detected. As a result of plasmapheresis, complete recovery was achieved. The presented case was atypical in its clinical course and treatment. It could support the theory of the continuity between MFS, Bickerstaff brainstem encephalitis (BBE), and Guillain-Barré syndrome (GBS). Copyright © 2015 Polish Neurological Society. Published by Elsevier Urban & Partner Sp. z o.o. All rights reserved.

  12. Subacute bacterial endocarditis presenting as left upper quadrant abdominal pain

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    Yung-Ta Kao

    2013-09-01

    Full Text Available Infective endocarditis is a microbial infection of the endocardial surface of the heart. Its symptoms and signs are varied, and include fever, heart murmur, peripheral embolism, and heart failure. The diagnosis of subacute bacterial endocarditis (SBE is suggested by a history of an indolent process characterized by fever, fatigue, anorexia, and unexplained weight loss. These patients may have had an invasive procedure, such as dental work, or abused intravenous drugs prior to the diagnosis of SBE. Although uncommon, the patients may present with nonspecific symptoms caused by peripheral embolic events. Herein, we report a 25-year-old male diagnosed with SBE, who presented with the unusual symptom of sudden onset of left upper quadrant abdominal pain for 2 days. His clinical history is also discussed.

  13. Subacute liver failure by pseudocirrhotic metastatic breast cancer infiltration.

    Science.gov (United States)

    Jüngst, Christoph; Krämer, Jens; Schneider, Günther; Lammert, Frank; Zimmer, Vincent

    2013-01-01

    Hepatic metastases are common in the clinical course of breast cancer and typically appear as mass lesions. This report describes the case of a 70-year-old woman with a history of breast cancer and no previously known liver disease presenting with the first episode of variceal bleeding and subacute hepatic failure. Imaging studies indicated liver cirrhosis without signs of malignant focal lesions. Comprehensive diagnostic work-up was negative for specific causes of liver disease and provided no evidence for tumor recurrence. Finally transjugular liver biopsy revealed a marked diffuse desmoplastic infiltration by breast cancer cells. Malignant pseudocirrhosis is an unusual pattern of metastatic, tumor spread representing a rare but important differential diagnosis of progressive liver failure. Liver biopsy is the key procedure to establish the diagnosis as imaging studies may mimic cirrhosis.

  14. A rare pathogen for subacute osteomyelitis in adolescent: Serratia marcescens.

    Science.gov (United States)

    Turgut, Necmettin; Akgul, Turgut; Arzu, Ufuk; Batıbay, Sefa Giray; Ekinci, Mehmet; Şen, Cengiz; Korkmaz, Murat

    2015-01-01

    There are various pathogens reported for osteomyelitis. Osteomyelitis is bone infection which produces pain and fever, also threatens bone instability. It can lead to nonunion. The purpose of this report was to describe a case with union delay of the tibia due to serratia marcescens osteomyelitis. Serratia marcescens is an unexpected pathogen for subacute osteomyelitis in adolescence. Because of difficulty of diagnosis, treatment can be delayed or the situation can cause complications like nonunion or loss of function. Serratia marcescens is an unexpected pathogen for subacute osteomyelitis in adolescence. Because of difficulty of diagnosis, treatment can be delayed or cause complications like nonunion or loss of function. We present a meningomyelocele female adolescent operated with distal tibia varus osteotomy for correcting ankle valgus deformity. Insufficient healing was determined at osteotomy side on radiographs. The patient's erythrocyte sedimentation rate and CRP level was slightly higher with minimal clinical inflammation. MRI examination showed abscess formation at T2 imaging. Debridement, grafting and circular external fixation was performed. Sulperazon was started for drug therapy. Union was achieved after compression and distraction osteogenesis by circular external fixator. Orthopedic surgeons should be aware of opportunistic infections like serratia and keep in mind as a probable cause of disease. Osteomyelitis is one of our main problems in orthopedics. Serratia does not come to mind as a causative factor when we learn the patient has osteomyelitis. We give treatment for the most expected pathogens like staphylococcus species firstly. This shows us the importance of bone biopsies and wound culture tests. Presented case is diagnosed as serratia osteomyelitis after culture results and given treatment with antibiotics and debridement. Orthopedic surgeons should be aware of opportunistic infections like serratia and keep in mind when diagnosing the

  15. Assessment of acute and subacute toxic effects of the Saudi folk herb Retama raetam in rats

    Directory of Open Access Journals (Sweden)

    Mardi M. Algandaby

    2015-12-01

    Conclusion: Repeated administration of methanolic extract of RR (250 mg/kg has a low nephrotoxic subacute toxicity potential, while it might have hepatotoxic, nephrotoxic, and mutagenic effects at higher doses.

  16. Pending Laboratory Tests and the Hospital Discharge Summary in Patients Discharged To Sub-Acute Care

    National Research Council Canada - National Science Library

    Walz, Stacy E; Smith, Maureen; Cox, Elizabeth; Sattin, Justin; Kind, Amy J. H

    2011-01-01

    ...) tests at the time of hospital discharge for general medical patients. However, the prevalence and communication of pending labs within a high-risk population, specifically those patients discharged to sub-acute care (i.e...

  17. BILATERAL SUBRETINAL FLUID AND RETINAL VASCULOPATHY ASSOCIATED WITH SUBACUTE SCLEROSING PANENCEPHALITIS.

    Science.gov (United States)

    Agarwal, Aniruddha; Singh, Ramandeep; Kumar, Abiraj; Dogra, Mangat R; Gupta, Amod

    2017-01-01

    To report a case of bilateral retinopathy associated with subacute sclerosing panencephalitis. History and clinical examination, fluorescein angiography, and optical coherence tomography. We report a rare case of unilateral, followed by bilateral retinopathy, subretinal fluid, and vasculopathy in a young boy. History of missed measles vaccination, behavioral and neurologic symptoms, and electroencephalogram suggested a diagnosis of subacute sclerosing panencephalitis. Retinal imaging using optical coherence tomography was performed to document changes in the retinal microstructure through the natural course of the disease. Within 8 weeks, the changes progressed to retinal atrophy in both eyes. The progressive course of retinitis associated with subacute sclerosing panencephalitis can be monitored on optical coherence tomography. Retinitis is subacute sclerosing panencephalitis rapidly progressive from the acute stage to the stage of atrophy, involving full thickness of the retina.

  18. Subacute sclerosing panencephalitis presenting as schizophrenia with an alpha coma pattern in a child.

    Science.gov (United States)

    Kartal, Ayşe; Kurt, Ayşegül Neşe Çitak; Gürkaş, Esra; Aydin, Kurşad; Serdaroğlu, Ayşe

    2014-10-01

    Subacute sclerosing panencephalitis, a progressive neurodegenerative disorder of the central nervous system, can present atypically with uncharacteristic electroencephalographic (EEG) features at its onset albeit typically with progressive mental deterioration, behavioral changes, and myoclonic jerks. An atypical presentation of subacute sclerosing panencephalitis can lead to a delay in diagnosis, thus hindering early treatment. Herein, we describe a 14-year-old girl who presented with insomnia, amnesia, auditory and visual hallucinations. The patient's electroencephalography on admission showed an alpha coma pattern. In spite of antipsychiatric treatment (olanzapine 20 mg/d) for 3 months, a progressive deterioration in neurologic function was observed. Subacute sclerosing panencephalitis was suspected and diagnosis was confirmed by increased titers of measles antibodies in the cerebrospinal fluid. The attention of pediatricians should be drawn to psychiatric symptoms as possible initial presentations of subacute sclerosing panencephalitis in order to avoid needless diagnostic and treatment procedures. © The Author(s) 2013.

  19. Improving the welfare of dairy goats: Feeding behaviour identifies goats at risk of subacute rumen acidosis

    OpenAIRE

    Giger-Reverdin, Sylvie; Sauvant, Daniel; Duvaux-Ponter, Christine

    2013-01-01

    Main messages: Feeding behaviour is highly variable between animals. Feeding behaviour modifies rumen pH pattern and occurrence of subacute ruminal acidosis (SARA). Avoiding SARA increases animal welfare, milk production and therefore farm profit - ability.

  20. Systemic alendronate prevents resorption of necrotic bone during revascularization. A bone chamber study in rats

    Directory of Open Access Journals (Sweden)

    Aspenberg Per

    2002-08-01

    Full Text Available Abstract Background Avascular necrosis of bone (osteonecrosis can cause structural failure and subsequent deformation, leading to joint dysfunction and pain. Structural failure is the result of resorption of necrotic bone during revascularization, before new bone has formed or consolidated enough for loadbearing. Bone resorption can be reduced by bisphosphonates. If resorption of the necrotic bone could be reduced during the revascularization phase until sufficient new bone has formed, it would appear that structural failure could be avoided. Methods To test whether resorption of necrotic bone can be prevented, structural grafts were subjected to new bone ingrowth during systemic bisphosphonate treatment in a rat model. Results In rats treated with alendronate the necrotic bone was not resorbed, whereas it was almost entirely resorbed in the controls. Conclusion Systemic alendronate treatment prevents resorption of necrotic bone during revascularization. In patients with osteonecrosis, bisphosphonates may therefore prevent collapse of the necrotic bone.