Bilateral Optic Neuropathy Mimicking Neuromyelitis Optica in the Setting of Intracranial Germinoma

2021 ◽  
pp. 10.1212/CPJ.0000000000001101
Author(s):  
Khaled M S Abdalla ◽  
Madhura Tamhankar ◽  
Aparna M. Prabhu
Keyword(s):  
Neuromyelitis Optica ◽  
Optic Neuropathy ◽  
Rare Condition ◽  
Optic Chiasm ◽  
Acute Disseminated Encephalomyelitis ◽  
Inadequate Response ◽  
Intracranial Germinoma ◽  
Common Etiology ◽  
Cranial Neuropathies ◽  
Post Infectious

Bilateral optic neuropathy is uncommon in adults with a prevalence varying from 19% -50% of all optic neuropathies [1]. The most common etiology is Neuromyelitis Optica (NMO) or Devic’s disease. Other less frequent etiologies include myelin-oligodendrocyte-glycoprotein (MOG) antibody, syphilis, meningioma, post-vaccinal, post-infectious (chicken pox, human herpes virus 6), acute disseminated encephalomyelitis and idiopathic. Sequential optic neuropathy is seen in multiple sclerosis, but bilateral simultaneous optic neuropathy is rare [2]. When optic neuropathy involves posterior nerve segments, long segments and the optic chiasm, NMO should be suspected. If there is involvement of the hypothalamus, basal meninges, other cranial neuropathies, sarcoidosis must be considered [3]. Primary intracranial germinoma is a rare condition and should be included in the differential diagnosis, especially if there are atypical features and inadequate response to standard treatment [4].

Aquaporin-4- und Myelin-Oligodendrozyten-Glykoprotein-Antikörper-assoziierte Optikusneuritis: Diagnose und Therapie

2020 ◽  
Vol 237 (11) ◽  
pp. 1290-1305
Author(s):  
Brigitte Wildemann ◽  
Solveig Horstmann ◽  
Mirjam Korporal-Kuhnke ◽  
Andrea Viehöver ◽  
Sven Jarius
Keyword(s):  
Neuromyelitis Optica ◽  
Optic Neuropathy ◽  
Aquaporin 4 ◽  
Phase Iii ◽  
Neuromyelitis Optica Spectrum Disorders ◽  
Interferon Β ◽  
Off Label ◽  
Diagnostik Und Therapie ◽  
Spectrum Disorders ◽  
Diagnose Und Therapie

ZusammenfassungDie Optikusneuritis (ON) ist vielfach die erste Manifestation einer AQP4-Antikörper-vermittelten NMOSD (AQP4: Aquaporin-4, NMOSD: Neuromyelitis-optica-Spektrum-Erkrankung, Engl.: neuromyelitis optica spectrum disorders) oder einer Myelin-Oligodendrozyten-Glykoprotein-Antikörper-assoziierten Enzephalomyelitis (MOG-EM; auch MOG antibody associated disorders, MOGAD). Für beide Erkrankungen wurden in den vergangenen Jahren internationale Diagnosekriterien und Empfehlungen zu Indikation und Methodik der serologischen Testung vorgelegt. Seit Kurzem liegen zudem Ergebnisse aus 4 großen, internationalen Phase-III-Studien zur Behandlung der NMOSD vor. Mit dem den Komplementfaktor C5 blockierenden monoklonalen Antikörper Eculizumab wurde 2019 erstmalig ein Medikament zur Langzeitbehandlung der NMOSD, die bislang vornehmlich Off-Label mit Rituximab, Azathioprin und anderen Immunsuppressiva erfolgt, auf dem europäischen Markt zugelassen. Für die erst vor wenigen Jahren erstbeschriebene MOG-EM stehen inzwischen Daten aus mehreren retrospektiven Studien zur Verfügung, die eine Wirksamkeit von Rituximab und anderen Immunsuppressiva in der Schubprophylaxe auch in dieser Indikation nahelegen. Viele der zur Therapie der MS zugelassenen Medikamente sind entweder unwirksam oder können, wie z. B. Interferon-β, eine Verschlechterung des Krankheitsverlaufes bewirken. Beide Erkrankungen werden im Akutstadium mit hochdosierten Glukokortikoiden und Plasmapherese oder Immunadsorption behandelt. Diese Behandlung sollte möglichst rasch nach Symptombeginn eingeleitet werden. Insbesondere die MOG-EM ist durch eine oft ausgeprägte Steroidabhängigkeit gekennzeichnet, die ein langsames Ausschleichen der Steroidtherapie erfordert, und schließt viele Fälle der bislang meist als „idiopathisch“ klassifizierten „chronic relapsing inflammatory optic neuropathy“ (CRION) ein. Unbehandelt kann sowohl die NMOSD- als auch die MOG-EM-assoziierte ON zu schweren, persistierenden und oft bilateralen Visuseinschränkungen bis hin zur Erblindung führen. Beide Erkrankungen verlaufen meist relapsierend. Neben den Sehnerven sind häufig das Myelon sowie der Hirnstamm und, vor allem bei NMO-Patienten, das Dienzephalon betroffen; supratentorielle Hirnläsionen im kranialen MRT sind, anders als früher gedacht, kein Ausschlusskriterium, sondern häufig. In der vorliegenden Arbeit geben wir einen Überblick über Klinik, Diagnostik und Therapie dieser beiden wichtigen Differenzialdiagnosen der MS-assoziierten und idiopathischen ON.

scholarly journals Clinical presentation and immunological features of Post-Malaria Neurologic Syndrome: a case report and review of literature

2020 ◽  
Vol 19 (1) ◽  
Author(s):  
Nadia Castaldo ◽  
Carlo Tascini ◽  
Paola Della Siega ◽  
Maddalena Peghin ◽  
Davide Pecori
Keyword(s):  
Unmet Need ◽  
Specific Treatment ◽  
Rare Condition ◽  
Acute Disseminated Encephalomyelitis ◽  
Intravenous Immunoglobulins ◽  
Neurological Involvement ◽  
Systematic Research ◽  
High Dose ◽  
Microbiological Analysis ◽  
Randomized Controlled Studies

Abstract Background Malaria still represents a major health threat, in terms of both morbidity and mortality. Complications of malaria present a diversified clinical spectrum, with neurological involvement leading to the most serious related-conditions. The authors recently encountered a case of a 60-year old Italian man presenting with confusion, language disturbances and Parkinson-like syndrome 3 weeks after complete remission from severe Plasmodium falciparum cerebral malaria. Chemical and microbiological analysis revealed aseptic meningitis, diffuse encephalitis and abnormal immune-activation. Re-infection and recrudescence of infection were excluded. Further analysis excluded paraneoplastic and autoimmune causes of encephalitis. A diagnosis of Post-Malaria Neurological Syndrome (PMNS) was finally formulated and successfully treated with high dose of steroids. Methods A systematic research of current literature related to PMNS was performed. Results 151 cases of PMNS were included, the majority of which occurred after severe P. falciparum infections. Four main clinical pattern were identified: 37% of the cases presented as “classical” PMNS, 36% presented as delayed cerebellar ataxia (DCA), 18% resembled acute inflammatory demyelinating polyneuropathy (AIDP), and 8% presented as acute disseminated encephalomyelitis (ADEM)-like form. Differentiation between different forms was not always simple, as clinical and radiological findings frequently overlap. Overall, in almost all of the tested cases, cerebrospinal fluid was found pathological; EEG revealed nonspecific encephalopathy in 30% of classical PMNS and 67% ADEM; imaging tests were found abnormal in 92% of ADEM-like forms. Pathogenesis remains unclear. An autoimmune mechanism is the most corroborated pathogenic hypothesis. Overall, the majority of PMNS cases revert without specific treatment. In most severe forms, high dose steroids, intravenous immunoglobulins, and plasmapheresis have been shown to improve symptoms. Conclusions PMNS is a disabling complication of malaria. The overall incidence is not known, due to frequent misdiagnosis and under-reporting. Pathogenesis is not also fully understood, but rapid response to immune-modulating treatment along with similarities to auto-immune neurological disease, strongly support a dysregulated immunological genesis of this condition. The lack of randomized controlled studies regarding therapeutic approaches is a major unmet need in this setting. A systematic collection of all the PMNS cases would be desirable, in order to increase awareness of this rare condition and to prospectively investigate the most appropriate management.

Leber Hereditary Optic Neuropathy Mimicking Neuromyelitis Optica

2011 ◽  
Vol 31 (3) ◽  
pp. 265-268 ◽  
Author(s):  
Collin M McClelland ◽  
Gregory P Van Stavern ◽  
Alex C Tselis
Keyword(s):  
Neuromyelitis Optica ◽  
Optic Neuropathy ◽  
Leber Hereditary Optic Neuropathy ◽  
Hereditary Optic Neuropathy

First Presentation of an Acquired Demyelinating Syndrome

2017 ◽  
Vol 16 (03) ◽  
pp. 164-170
Author(s):  
Rachel Gottlieb-Smith ◽  
Amy Waldman
Keyword(s):  
Multiple Sclerosis ◽  
Central Nervous System ◽  
Nervous System ◽  
Differential Diagnosis ◽  
Optic Neuritis ◽  
Clinical Features ◽  
Neuromyelitis Optica ◽  
Transverse Myelitis ◽  
Acute Disseminated Encephalomyelitis ◽  
The Central Nervous System

AbstractAcquired demyelinating syndromes (ADS) present with acute or subacute monofocal or polyfocal neurologic deficits localizing to the central nervous system. The clinical features of distinct ADS have been carefully characterized including optic neuritis, transverse myelitis, and acute disseminated encephalomyelitis. These disorders may all be monophasic disorders. Alternatively, optic neuritis, partial transverse myelitis, and acute disseminated encephalomyelitis may be first presentations of a relapsing or polyphasic neuroinflammatory disorder, such as multiple sclerosis or neuromyelitis optica. The clinical features of these disorders and the differential diagnosis are discussed in this article.

Atlanto-Occipital Decompression of Vertebral Artery for a Variant of Bow Hunter's Syndrome: 2-Dimensional Operative Video

2021 ◽  
Author(s):  
Nickalus R Khan ◽  
Turki Elarjani ◽  
Stephanie H Chen ◽  
Laszlo Miskolczi ◽  
Sheryl Strasser ◽  
...  
Keyword(s):  
Vertebral Artery ◽  
Rare Condition ◽  
Head Rotation ◽  
Treatment Modalities ◽  
Spinal Instability ◽  
Vertebrobasilar Insufficiency ◽  
Common Etiology ◽  
Neurologically Intact ◽  
Hunter's Syndrome ◽  
Visual Abnormalities

Abstract Rotational vertebral artery (VA) occlusion syndrome, also known as bow hunter's syndrome, is an uncommon variant of vertebrobasilar insufficiency typically occurring with head rotation.1-3 The most common presenting symptom is dizziness (76.8%), followed by visual abnormalities and syncope (50.4% and 40.4%, respectively).2 Osteophytic compression due to spinal spondylosis has been shown to be the most common etiology (46.2%), with other factors, such as a fibrous band, muscular compression, or spinal instability, being documented.1,2 Treatment is dependent on the level and site of VA compression with anterior, anterolateral, or posterior approaches being described.1,4 We present the case of a 72-yr-old male with osteophytic compression of the V3 segment of the vertebral artery at the occipital-cervical junction. The patient underwent a C1 hemilaminectomy and removal of osteophytic compression from the occipital-cervical joint. The patient had complete resolution of compression of his vertebral artery on postoperative imaging and remained neurologically intact following the procedure. We review the literature on this topic, the technical nuances of the procedure performed, and review the different treatment modalities available for this rare condition.1-11  The patient consented to the procedure and to publication of their image.

scholarly journals Teaching NeuroImages: Leber hereditary optic neuropathy masquerading as neuromyelitis optica

Neurology ◽  
2017 ◽  
Vol 90 (1) ◽  
pp. e94-e95 ◽  
Author(s):  
Roman Kassa ◽  
Flavius Raslau ◽  
Charles Smith ◽  
Padmaja Sudhakar
Keyword(s):  
Neuromyelitis Optica ◽  
Optic Neuropathy ◽  
Leber Hereditary Optic Neuropathy ◽  
Hereditary Optic Neuropathy

Multiple Sclerosis and Related Disorders

2014 ◽  
Author(s):  
J William Lindsey
Keyword(s):  
Multiple Sclerosis ◽  
Differential Diagnosis ◽  
Neuromyelitis Optica ◽  
Subacute Sclerosing Panencephalitis ◽  
Large Diameter ◽  
Transverse Myelitis ◽  
Acute Disseminated Encephalomyelitis ◽  
Demyelinating Diseases ◽  
Pathologic Features ◽  
Relapsing Remitting

Multiple sclerosis (MS) is a relatively common cause of neurologic symptoms and disability in young adults. The distinguishing pathologic features of MS are loss of myelin and inflammation in the central nervous system (CNS). The myelin sheath is essential for rapid conduction of nerve signals along large-diameter axons. Oligodendrocytes produce and maintain myelin in the CNS, and Schwann cells produce and maintain myelin in the peripheral nerves. In addition to MS, there are a number of related disorders causing demyelination, inflammation, or both in the CNS. This chapter discusses MS and related disorders, including neuromyelitis optica, optic neuritis, acute disseminated encephalomyelitis, transverse myelitis, Behçet syndrome, neurosarcoidosis, inherited demyelinating diseases (leukodystrophies, cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy [CADASIL]), and virus-induced demyelination (progressive multifocal leukoencephalopathy, subacute sclerosing panencephalitis). The section on MS covers epidemiology, etiology/genetics, pathogenesis, diagnosis, differential diagnosis, management, and prognosis. Figures include organization of the microenvironment of larger-diameter axons, typical magnetic resonance imaging findings in MS and neuromyelitis optica, postgadolinium images of the cervical spine in MS, and an approach to treatment of relapsing-remitting MS. Tables list MS and related disorders, distribution of neurologic deficits at the onset of MS, differential diagnosis of MS, disease-modifying therapies for relapsing-remitting MS, and selected leukodystrophies, as well as diagnostic criteria and selected symptomatic therapies for MS. This review contains 3 highly rendered figures, 7 tables, and 82 references.

Multiple Sclerosis and Related Disorders

2015 ◽  
Author(s):  
J William Lindsey
Keyword(s):  
Multiple Sclerosis ◽  
Differential Diagnosis ◽  
Neuromyelitis Optica ◽  
Subacute Sclerosing Panencephalitis ◽  
Large Diameter ◽  
Transverse Myelitis ◽  
Acute Disseminated Encephalomyelitis ◽  
Demyelinating Diseases ◽  
Pathologic Features ◽  
Relapsing Remitting

Multiple sclerosis (MS) is a relatively common cause of neurologic symptoms and disability in young adults. The distinguishing pathologic features of MS are loss of myelin and inflammation in the central nervous system (CNS). The myelin sheath is essential for rapid conduction of nerve signals along large-diameter axons. Oligodendrocytes produce and maintain myelin in the CNS, and Schwann cells produce and maintain myelin in the peripheral nerves. In addition to MS, there are a number of related disorders causing demyelination, inflammation, or both in the CNS. This chapter discusses MS and related disorders, including neuromyelitis optica, optic neuritis, acute disseminated encephalomyelitis, transverse myelitis, Behçet syndrome, neurosarcoidosis, inherited demyelinating diseases (leukodystrophies, cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy [CADASIL]), and virus-induced demyelination (progressive multifocal leukoencephalopathy, subacute sclerosing panencephalitis). The section on MS covers epidemiology, etiology/genetics, pathogenesis, diagnosis, differential diagnosis, management, and prognosis. Figures include organization of the microenvironment of larger-diameter axons, typical magnetic resonance imaging findings in MS and neuromyelitis optica, postgadolinium images of the cervical spine in MS, and an approach to treatment of relapsing-remitting MS. Tables list MS and related disorders, distribution of neurologic deficits at the onset of MS, differential diagnosis of MS, disease-modifying therapies for relapsing-remitting MS, and selected leukodystrophies, as well as diagnostic criteria and selected symptomatic therapies for MS.   This chapter contains 3 highly rendered figures, 7 tables, 82 references, 1 teaching slide set, and 5 MCQs.

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