Duane syndrome: A case report

Duane syndrome: A case report

Abstracts / Journal of the Neurological Sciences e629 (2013) e629–e678 Conclusion: Data shows that brain tumor impacts more on caregivers' mental hea...

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Abstracts / Journal of the Neurological Sciences e629 (2013) e629–e678

Conclusion: Data shows that brain tumor impacts more on caregivers' mental health. From 18 months after bereavement onward, caregivers reported a psychological well-being higher than the one experienced during the illness of their loved one. Even if grief elaboration is a long and painful experience caregivers seem to have elaborated the loss of their loved one and have restored interest in life. doi:10.1016/j.jns.2013.07.2337

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Abstract — WCN 2013 No: 2170 Topic: 36 — Other Topic Alginate oligosaccharide upregulates Ampk and induces mitochondrial biogenesis in H2O2-exposed Pc12 cells F. Khodagholi, S. Khoramian Tusi, F. Foolad. Neuroscience Research Center, Shahid Beheshti University of Medical Sciences, Tehran, Iran Background: Many studies show mitochondrial dysfunction in aging and some diseases such as Alzheimer's disease (AD). Mitochondrial biogenesis is a special defensive mechanism that many factors involved in it include: 1) AMPK and CREB as stimulater of PGC-1α; 2) NRF1 that is stimulated by PGC-1α; and 3) Tfam expression and initiation of mitochondrial DNA replication.

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Background: Vogt–Koyanagi–Harada syndrome is a rare disease defined as bilateral chronic granulomatous panuveitis that may be associated with CNS, auditory and integumentary manifestations. Objective: To describe 3 cases of Vogt–Koyanagi–Harada syndrome. Patients and methods: Case 1: A 27-year-old woman presented with recurrent bilateral anterior uveitis in the previous three months. In the last episode she received oral steroids with partial improvement of the visual acuity. One month later she complained of blurred vision and headache with photophobia. The examination revealed bilateral papilloedema. CSF had lymphocytic pleocytosis. She didn't have skin changes. Case 2: A 2-year-old woman presented with two-months of complaints of redness, blurred vision and discomfort in both eyes. She also had left tinnitus, severe headache, photophobia, and vomiting. On examination she had bilateral posterior uveitis with retinal detachment. One month later hair poliosis and achromatic patches in the face/chest were noticed. Case 3: A 25-year-old man complained of painful red eye, bilateral low visual acuity and headache with photophobia in the previous month. The examination disclosed posterior uveitis with retinal detachment. CSF showed lymphocytic pleocytosis. Two months later a hypopigmented lesion developed in the periocular area. Results: In the three cases other causes of uveomeningeal syndrome were ruled out and treated with high-dose systemic steroids. Methotrexate/azathioprine was additionally required because of recurrent ocular involvement. In all, inflammation decreased and vision was preserved enabling tapering of the steroid therapy. Conclusion: Early diagnosis of this syndrome is crucial because prompt administration of high-dose steroids may prevent permanent visual loss. Usually additional immunosuppression is needed with a steroid-sparing effect.

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doi:10.1016/j.jns.2013.07.2336

Objective: Effect of alginate oligosaccharide on mitochondrial biogenesis. Materials and methods: PC12 cells were pretreated by AOS for 12 h, then exposed to compound C (10 μM), AMPK inhibitor, for 12 h. Then, cells treated by H2O2 (150 μM) for different times. In addition, we compared the effects of enzymatically depolymerized unsaturated AOS with heat-degraded saturated AOS. The level of mentioned factors was determined by Western blotting and the mitochondrial number was measured by Mito Tracker Red fluorescent staining. Result: After H2O2 treatment, AMPK, PGC-1α, NRF1, Tfam and Cytochrome c increased and reached the highest level at 8 h after exposure, and then continually started to decrease. Also, phosphoCREB/CREB ratio increased but the highest level was at 12 h. Pretreatment with AOS caused a significant increase in the level of AMPK, PGC-1α, NRF1, Tfam, Cytochrome c and phosphor-CREB/CREB ratio in H2O2-induced cells and stayed higher than H2O2-treated cell till 24 h. In the group with compound C, we found a weaker effect of AOS on the induction of mitochondrial biogenesis. The results showed that heat-degraded saturated AOS has more effect on this pathway compared to enzymatically depolymerized unsaturated AOS. Conclusions: Mitochondrial biogenesis is involved in AOS-mediated protection.

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Abstract — WCN 2013 No: 2420 Topic: 36 — Other Topic Bereavement in brain tumor: Psychological reactions in caregivers

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C.Y. Finocchiaroa,b, L. Rainoldia, A. Petruzzia, G. Simonettia, A. Silvania, E. Lampertia. aClinical Neurooncology Unit, Fondazione I.R.C.C.S. Istituto Neurologico ‘Carlo Besta’, Italy; bUniversità Vita-Salute San Raffaele, Milan, Italy

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Background: When brain tumor patients' conditions decline and death occurs, caregivers deal with the adaptation to a new life condition. Objective: We aim to describe changes in quality of life, anxiety and depression experienced by caregivers after bereavement of their loved one. Material and methods: The study involved 44 caregivers of brain tumor patients in two different periods. In order to evaluate the psychological features the following tools were provided: Hospital Anxiety and Depression Scale (HADS) and 36-Item Short-Form Health Survey (SF-36). First data collection (T1) occurred when caregivers, together with the patients, were attending the Neuro-oncology Unit of IRCCS Istituto Neurologico C. Besta, Milan, Italy. The same variables were detected in a period of time ranging from 18 months to 3 years after bereavement (T2). Results: Of the 44 caregivers involved, 4 could not be reached and 3 did not give consent to participate in the research. The 37 caregivers tested in T2 reported levels of anxiety and depression lower than those experienced in T1. In T2 higher scores were detected in psychological well-being scales (Social Functioning, Role Emotional, Mental Health) while scores related to physical health remained almost unchanged from T1 to T2.

doi:10.1016/j.jns.2013.07.2338

Abstract — WCN 2013 No: 2406 Topic: 36 — Other Topic Duane syndrome: A case report H. Akguna, M. Yucelb, S. Alayc, O. Ozc, F.C. Gundogand, Z. Odabasic, S. Demirkayac. aDepartment of Neurology, Etimesgut Military Hospital, Ankara, Turkey; bDepartment of Neurology, Kasimpasa Military Hospital, Istanbul, Turkey; cDepartment of Neurology, Gulhane Military Medical Academy, Ankara, Turkey; dDepartment of Ophthalmology, Gulhane Military Medical Academy, Ankara, Turkey

Abstracts / Journal of the Neurological Sciences e629 (2013) e629–e678

Abstract — WCN 2013 No: 1717 Topic: 36 — Other Topic Rhinocerebral mucormycosis: Clinical features and prognosis, two case reports and review of the literature A. Riahi, I. Bedoui, N. Touati, O. Ben Ouaday, M. Mansour, J. Zaouali, R. Mrissa. Department of Neurology, Military Hospital, Tunis, Tunisia

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Background: Rhinocerebral mucormycosis is a rare opportunistic fungal infection of the upper airways. Cerebral involvement is exceptional and life threatening. Objective: We report two cases of rhinocerebral mucormycosis and we discuss their clinical features and prognosis. Case report: Patient A, a 60 year old diabetic woman presented with fever, acute multiple right cranial nerve palsy (Garsin's syndrome). Few days later, a stroke with left side paralysis occurred. Cerebral-MRI showed right middle cerebral artery stroke with occlusion of the right carotid and a thickening of cavum mucosa. CSF analysis showed lymphocyte-type meningitis. Viral, bacterial and fungal serologies and cultures were negative. Cavum biopsy confirmed the diagnosis of mucormycosis. Patient B, a 69 year old man with history of mucosa-associated lymphoid tissue pulmonary lymphoma, has presented a left frontotemporal and retro-orbital headache with exophthalmia for one year. Cerebral-MRI showed liquid filling ethmoidal cells with a thickening of the left maxillary sinus mucosa. Facial CT-scan showed a left retroorbital tissular process with bone lysis. Biopsy confirmed the diagnosis of orbital mucormycosis. Discussion: Rhinocerebral mucormucosis is a life threatening infection and outcome is highly dependent on early diagnosis and treatment. Diagnosis is based on the biopsy. Both patients were treated by Amphotericin B, Fluconazole IV for 4 weeks. The outcome was good with clinical, biological and radiological improvement for patient B, while patient A had died of a massive pulmonary embolism. Conclusion: Our observations highlight the various clinical features of rhinocerebral mucormycosis. Some features like cranial nerve palsies and strokes should be considered of severe prognosis.

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Background: Duane's syndrome is characterized with a defect in the nucleus of the sixth cranial nerve and/or a developmental defect of the sixth cranial nerve axon. It was first described in 1879 by Heuck in a patient with a serious eye movement restriction and globe retraction. Alexander Duane reported a series of 54 cases in 1905 and the syndrome was named after him. Heterochromia can be seen in Duane's syndrome. Objective: In this report we would like to present a rare case of Duane syndrome with heterochromia. Patients and methods: A 38-year-old male patient presented to our outpatient clinic with complaints of double vision which he had since childhood. He stated that his complaints increased especially when looking to the left. The color of his eyes had been different since birth. His right eye was green, and his left eye brown. On the neurological exam left sided sixth cranial nerve palsy was present. Other neurological findings were normal. Results: On his brain MRI and brainstem thin slice MRI, the intracysternal segment of the sixth cranial nerve was visualized on the right but not on the left side. Conclusion: Because of its rare nature and the absent left sixth cranial nerve on the brain MRI we found this case worth reporting.

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doi:10.1016/j.jns.2013.07.2339

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Abstract — WCN 2013 No: 2413 Topic: 36 — Other Topic Avicenna's description of Willis circle

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A. Zargarana,b, A. Karimia, S.A. Ahmadic, A. Borhani-Haghighid. a Student Research Committee, Shiraz University of Medical Sciences, Shiraz, Iran; bDepartment of Traditional Pharmacy, Shiraz University of Medical Sciences, Shiraz, Iran; cResearch Office for the History of Persian Medicine, Shiraz University of Medical Sciences, Shiraz, Iran; dDepartment of Neurology, Shiraz University of Medical Sciences, Shiraz, Iran

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Background and objective: Willis circle is an interconnection between anterior and posterior, and right and left cerebral circulations. It supplies brain and neighboring structures. Although this circle was noticed briefly and incompletely by ancient doctors, it is described completely by Thomas Willis in the book of Cerebri Anatome in 1664. In this study, the description of Avicenna, who was one of the most important scientists of the medieval period, about Willis circle is investigated. It can be important to shed light on a part of history of neurology. Method: Arabic and Persian versions of Canon on Medicine, Avicenna's medical encyclopedia were studied and his point of view about Willis circle was compared with current and also ancient terms. Results: Avicenna (980–1037) was one of the great scholars in medieval time. He wrote about this circle in the third volume of the Canon, in the section about head diseases. According to Avicenna's description, Willis circle was well known. Persian scholars can dissect in the ancient and medieval era. With regard to this, they had good information about human bodies and progress the previous Greco–Roman knowledge. Conclusion: There is a gap between the knowledge of the medieval Persians and current era. It was acquired in the Renaissance, when western scholars refused the medieval knowledge, created the new paradigm of medicine and became the leadership of science. It seems that many of the eastern medieval findings were forgotten in this transmission. Therefore many well known subjects in the medieval era were rediscovered by western scientists after 16th century AD. doi:10.1016/j.jns.2013.07.2340

doi:10.1016/j.jns.2013.07.2341

Abstract — WCN 2013 No: 2210 Topic: 36 — Other Topic An unusual case of subacute sclerosing panencephalitis (SSPE) with cognitive decline and simultanagnosia

Z. Ozdemir, A. Soysal, G. Gul, N. Behrem Gayir, B. Ciftci Kavaklioglu. Bakirkoy Prof. Dr. Mazhar Osman Mental Health and Neurological Diseases Training and Research Hospital, Istanbul, Turkey Objective: To introduce a case of subacute sclerosing panencephalitis (SSPE) presenting with cognitive decline, hemiparesis, simultanagnosia, and diagnostic MRI findings. Case report: A 21-year-old male patient was admitted to our hospital with hemiparesis, hesitant speech and progressive cognitive decline in the absence of myoclonus. His sister defined that the patient had an epileptic seizure one month ago and then developed disorientation. Neurological examination revealed prolongation of reaction time, disorientation of time and person, slight hemiparesis on the right side, hyperactive deep tendon reflexes and simultanagnosia. T2-weighted and FLAIR MR images showed extensive gray matter changes involving both temporal and parieto-occipital regions that are more prominent on the left side. EEG revealed recurrent periodic sharp and slow paroxysms. Extensive serum and BOS analysis for metabolic and infectious diseases revealed negative results. A diagnosis of SSPE was confirmed by elevated serum and BOS measles IgG titers.