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Hermann's Syndrome


Presentation

  • […] doi: 10.1097/ICB.0000000000000347 Case Report Abstract In Brief Author Information Authors Article Metrics Metrics To present a case report of a rare ocular manifestation of Proteus syndrome — rhegmatogenous retinal detachment.[journals.lww.com]
  • Abstract A 30-year-old, right-handed female presented 2 weeks postpartum with acute-onset severe headache, vertigo, and vomiting.[karger.com]
  • It may be noted in the treatment records that these signs are not present continuously, or the signs may be present at one examination and not appear at another.[nymetrodisability.com]
  • The presently reported ultrastructural findings are in line with such concept. The present study represents the first attempt using skin ultrastruture for distinguishing EDSH and JHS from FM.[omicsonline.org]
  • Diagnosis of Boerhaave syndrome can be difficult, because often no classic symptoms are present and delays in presentation for medical care are common. [1] Although Boerhaave syndrome classically presents as the Mackler triad of chest pain, vomiting,[emedicine.medscape.com]
Epilepsy
  • What percentage of the population has chronic temporal lobe epilepsy?" A. "Temporal lobe epilepsy (TLE) is a very common type of focal (localization-related) epilepsy that can be difficult to control with medications.[epilepsy.com]
  • Von Allmen is chief of pediatric epilepsy for the Texas Comprehensive Epilepsy Program at the Mischer Neuroscience Institute and medical director of the Children’s Memorial Hermann Hospital Pediatric Epilepsy Monitoring Unit.[utphysicians.com]
  • Hermann has served as a contributing editor of the AES journal Epilepsy Currents and as a member of the editorial boards of Epilepsy Research and The Journal of Epilepsy.[neurology.wisc.edu]
  • “If you eliminate the onchocerciasis, the epilepsy really disappears.”[sciencemag.org]
Vomiting
  • Abstract A 30-year-old, right-handed female presented 2 weeks postpartum with acute-onset severe headache, vertigo, and vomiting.[karger.com]
  • Dodgson described his headaches as "bilious", referring to severe vomiting of bile. We found that some children with mononucleosis suffered hallucinations of images that were shrinking or enlarging.[neurology.org]
  • Boerhaave syndrome, a transmural perforation of the esophagus, should be distinguished from Mallory-Weiss syndrome, a nontransmural esophageal tear that is also associated with vomiting.[emedicine.medscape.com]
  • On query, he denied hematuria, urostomy site discharge, flank pain, vomiting, diarrhea, or bloody stools.[cureus.com]
Suggestibility
  • We found it difficult to suggest an alternative. That is one reason why I wel come the expression "apallic syndrome".[books.google.com]
  • Researchers had suggested as early as the 1960s that high rates of epilepsy in Tanzania, with similar nodding symptoms, might be related to onchocerciasis.[sciencemag.org]
  • Although insulin resistance and the metabolic syndrome are associated with obesity (assessed by BMI), it has been suggested that a substantial number of obese individuals have normal metabolic function.[docsopinion.com]
  • This suggested that these cells had engulfed Aβ and could present it as an antigen.[alzforum.org]
  • However, similarity does not mean common genetic and molecular origin for both conditions although it possibly suggests the presence of related defects.[omicsonline.org]
Seizure
  • McDonald Critchley noted that patients with migraine or seizures arising in their parietal lobes perceived objects as smaller (micropsia) or larger (macropsia)than they were.[neurology.org]
  • There is, of course, tremendous interest in new and more effective ways to treat and prevent seizures, which is extremely important.[epilepsy.com]
  • Over time the seizures often worsened, leaving the children severely disabled. Many died of malnutrition, accidents, or secondary infections.[sciencemag.org]
  • Neurological Non-specific EEG anomalies occur in 72% of the patients and one quarter of them develop seizures (transient childhood epilepsy, grand mal seizures).[ojrd.biomedcentral.com]
  • AES is the nation’s 3,000-member professional society for epilepsy physicians (epileptologists), scientists, and allied professionals dedicated to the treatment, prevention and cure of seizures and epilepsy. Dr.[neurology.wisc.edu]
Ataxia
  • […] of childhood Lateral medullary syndrome Lateral pontine syndrome Medial medullary syndrome Medial pontine syndrome References [ edit ] External links [ edit ] v t e Lesions of spinal cord and brain Spinal cord / vascular myelopathy sensory: Sensory ataxia[en.wikipedia.org]
  • Classically, the syndrome involves the oculomotor nerve fascicles and superior cerebellar peduncle, leading to ipsilateral CN III palsy and limb ataxia respectively 1-4.[radiopaedia.org]
  • Infarction of the vestibular nuclei may lead to vomiting, vertigo, and nystagmus, while injury to the inferior cerebellar peduncle may lead to ipsilateral ataxia.[karger.com]
Cerebellar Ataxia
  • At the time he referred to this finding as "delirium cordis". [1] The eponymous " Nothnagel's syndrome " is named after him, a disorder characterized by ipsilateral oculomotor palsy and contralateral cerebellar ataxia. [2] He died in Vienna.[en.wikipedia.org]
Neglect
  • Although this latter article is listed in PubMed, both of these publications appear to be completely neglected in today’s scientific literature.[clinchem.aaccjnls.org]
  • Dysdiadochokinesia Intention tremor ) medial ( Cerebellar ataxia ) Basal ganglia Chorea Dystonia Parkinson's disease Cortex ACA syndrome MCA syndrome PCA syndrome frontal lobe : Expressive aphasia Aboulia parietal lobe : Receptive aphasia Hemispatial neglect[en.wikipedia.org]
Tremor
  • CN 3, 4) Weber's syndrome ventral peduncle, PCA Benedikt syndrome ventral tegmentum, PCA Parinaud's syndrome dorsal, tumor Nothnagel's syndrome Claude's syndrome Other Alternating hemiplegia Cerebellum lateral ( Dysmetria Dysdiadochokinesia Intention tremor[en.wikipedia.org]
  • Tremor Other Hyperkinet Mov (N Y). 2016;6:402. Zétola VF, Verschoor B, Lima FM, Ottmann FE, Doubrawa E, Paiva E, et al. [Hemiballism-hemichorea with non-ketotic hyperglycemia: movement disorder related to diabetes mellitus].[revistas.ces.edu.co]

Workup

  • Diagnostic testing at Children’s Memorial Hermann Hospital includes a detailed metabolic workup, spinal tap and high-resolution imaging to look for structural defects.[med.uth.edu]
  • Diagnostic testing at Children’s Memorial Hermann Hospital includes a detailed metabolic workup, spinal tap, and high-resolution imaging to look for structural defects.[utphysicians.com]

Treatment

  • In-utero treatment of ABS has recently become an option when the risk to the mother and baby are minimized. It is the first time surgical treatment has been performed in utero for a non-life threatening birth defect.[fetalhealthfoundation.org]
  • Expert Panel on Detection, Evaluation, and Treatment of High Blood Cholesterol in Adults.[clinchem.aaccjnls.org]
  • “We are hoping that having a place that specializes in the cause, diagnosis, and treatment of West Syndrome will improve outcomes.” Dr.[utphysicians.com]
  • “We are hoping that having a place that specializes in the cause, diagnosis and treatment of West Syndrome will improve outcomes.”[med.uth.edu]
  • It may be noted in the treatment records that these signs are not present continuously, or the signs may be present at one examination and not appear at another.[nymetrodisability.com]

Prognosis

  • Prognosis - Reynolds Neri Hermann syndrome Not supplied. Treatment - Reynolds Neri Hermann syndrome Not supplied. Resources - Reynolds Neri Hermann syndrome Not supplied.[checkorphan.org]
  • The prognosis of a stroke can be determined by its severity and results of serial neuropsychological assessment. Statement of Ethics The authors have no ethical conflicts to disclose. References Pearce JMS: Wallenberg's syndrome.[karger.com]
  • Prolonged bleeding has been reported following strabismus surgery in patients with the syndrome. [10] Prognosis [ edit ] The course of HPS has been mild in rare instances of the disorder, [11] however, the general prognosis is still considered to be poor[en.wikipedia.org]
  • Prognosis The prognosis of Boerhaave syndrome is directly contingent on its early recognition and appropriate intervention. [1] Early diagnosis allows prompt surgical repair.[emedicine.medscape.com]

Etiology

  • The points which have concerned my lawyer friends have not been matters of diagnosis, or of morbid anatomy, or of etiology. The fac tual problem which has been put before me was to make some approximate assessment as to the expectation of life.[books.google.com]
  • CONCLUSIONS: AIWS has multiple etiologies, generation of hallucinations reflect abnormal cerebral perfusion and its symptoms were not first described by Lippman, but probably by William Gowers.[neurology.org]
  • They are organised into groups, and further divided into clinical, etiological or histopathological sub-types.[orpha.net]
  • Further investigation is necessary to elucidate the whether the high level of cortisol is connected with some of the BMS etiological factors or is respond to stress connected with permanent pain sensations.[bib.irb.hr]
  • The etiology (cause) of fibromyalgia remains unknown to this day.[invisibleillnessforum.com]

Epidemiology

  • A clinical–histological and retrospective epidemiological study of 46 cases. Med Oral, 5 (2000), pp. 367-372 [15] M. Hisatomi, J. Asaumi, H. Konouchi, Y. Honda, T. Wakasa, K. Kishi.[elsevier.pt]
  • "Hermansky-Pudlak Syndrome: Background, Pathophysiology, Epidemiology". 13 June 2017. "Hermansky-Pudlak Syndrome". Hermansky–Pudlak Syndrome. University of Washington, Seattle. 1993. Retrieved 24 November 2008.[en.wikipedia.org]
  • Grear KE, Bushnell CD: Stroke and pregnancy: clinical presentation, evaluation, treatment, and epidemiology. Clin Obstet Gynecol 2013;56:350-359. Feske SK: Stroke in pregnancy. Semin Neurol 2007;27:442-452.[karger.com]
  • Abnormal hair implantation 58 (45) Minor Features Cutaneous syndactyly, toes II/III 46 (45) Webbed/short neck 42 (38) Cryptorchidism 28 (29) Hearing loss 23 (43) Palatal defects (including uvula) 18 (38) Strabismus 18 (45) Congenital heart defects 9 (45) Epidemiology[ojrd.biomedcentral.com]
  • Epidemiology International data Although it is likely underreported, the incidence of Boerhaave syndrome is relatively rare, with an estimated incidence of 3.1 per 1,000,000 persons per year. [4] Among esophageal perforations, aproximately 15% are spontaneous[emedicine.medscape.com]
Sex distribution
Age distribution

Pathophysiology

  • "Hermansky-Pudlak Syndrome: Background, Pathophysiology, Epidemiology". 13 June 2017. "Hermansky-Pudlak Syndrome". Hermansky–Pudlak Syndrome. University of Washington, Seattle. 1993. Retrieved 24 November 2008.[en.wikipedia.org]
  • In 1988 he highlighted the clinical importance of the syndrome, identifying insulin resistance as the central pathophysiologic feature( 2 ).[clinchem.aaccjnls.org]
  • Diagnostic considerations in mediastinal emphysema: a pathophysiologic-roentgenologic approach to Boerhaave's syndrome and spontaneous pneumomediastinum. Am J Roentgenol Radium Ther Nucl Med. 1972 Jul. 115(3):495-511. [Medline]. Rozycki GS.[emedicine.medscape.com]
  • .— Fibromyalgia syndrome: an overview of pathophysiology, diagnosis and management. Oman Med J, 2012, 27, 192-195. 3. Beighton P, De Paepe A, Steinmann B, et al.— EhlersDanlos syndrome : revised nosology, Villefranche, 1997.[invisibleillnessforum.com]
  • Jahan F, Nanji K, Qidwai W, Qasim R (2012) Fibromyalgia syndrome: an overview of pathophysiology, diagnosis and management. Oman Med J 27: 192-195.[omicsonline.org]

Prevention

  • "Treatment and prevention are key. There is, of course, tremendous interest in new and more effective ways to treat and prevent seizures, which is extremely important.[epilepsy.com]
  • Symptoms - Reynolds Neri Hermann syndrome Large head Retarded development Skin changes Heart defects Facial anomalies Causes - Reynolds Neri Hermann syndrome Hydrocephalus Acromegaly Proteus syndrome Prevention - Reynolds Neri Hermann syndrome Not supplied[checkorphan.org]
  • A boy receives ivermectin to prevent river blindness. Some think the drug may also prevent nodding syndrome.[sciencemag.org]
  • Página 310 - A randomized trial comparing ticlopidine hydrochloride with aspirin for the prevention of stroke in high-risk patients. ‎[books.google.es]
  • Locations Specialties & Treatments Adult Physical Colorectal Cancer - Prevention Cryotherapy Depo-Provera Shots Ear Irrigation Knee Injections Mammogram Mole Removal Pre-Op for OUTSIDE surgeon School Physical Skin Care Sports Physicals Stitches - Not[my.clevelandclinic.org]

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