Recurrent Hypothalamic Dysfunction in Seropositive Neuromyelitis Optica

2020 ◽  
pp. 10.1212/CPJ.0000000000001012
Author(s):  
Mary Clare McKenna ◽  
Nuala McNicholas ◽  
Conor Fearon ◽  
David Bradley
Keyword(s):  
Spinal Cord ◽  
Central Nervous System ◽  
Neuromyelitis Optica ◽  
Clinical Manifestations ◽  
Inflammatory Disorder ◽  
Diverse Range ◽  
Hypothalamic Dysfunction ◽  
Inappropriate Secretion ◽  
Clinical Presentations ◽  
The Central Nervous System

Background:Neuromyelitis optica (NMO) is a rare autoimmune inflammatory disorder of the central nervous system1. Pathogenic aquaporin 4 (AQP4) antibodies are present in 65-88% of cases1. The majority of cases follow a relapsing course preferentially involving the optic nerves, spinal cord, brainstem, diencephalon or cerebral regions1, 2. Within the acute diencephalic clinical presentations, symptomatic hypothalamic lesions may have a diverse range of clinical manifestations including homeostatic dysfunction of neuroendocrine systems2-4. We report a case of recurrent hypothalamic dysfunction secondary to NMO manifesting as syndrome of inappropriate secretion of antidiuretic hormone (SIADH), thermal dysregulation, dysautonomia and disorder of alertness.

Neuromyelitis Optica

2017 ◽  
Author(s):  
Teri L. Schreiner ◽  
Jeffrey L. Bennett
Keyword(s):  
Multiple Sclerosis ◽  
Spinal Cord ◽  
Central Nervous System ◽  
Nervous System ◽  
Optic Neuritis ◽  
Neuromyelitis Optica ◽  
Water Channel ◽  
Transverse Myelitis ◽  
Inflammatory Disorder ◽  
The Central Nervous System

Neuromyelitis optica (NMO), or Devic’s disease is an inflammatory disorder of the central nervous system that preferentially affects the optic nerves and spinal cord. Initially considered a variant of multiple sclerosis (MS), NMO is now clearly recognized to have distinct clinical, radiographic, and pathologic characteristics. Historically, the diagnosis of NMO required bilateral optic neuritis and transverse myelitis; however, the identification of a specific biomarker, NMO-IgG, an autoantibody against the aquaporin-4 (AQP4) water channel, has broadened NMO spectrum disease to include patients with diverse clinical and radiographic presentations. This chapter addresses the diagnosis, pathophysiology, and management of the disease.

scholarly journals Differential diagnosis of neuromyelitis optica spectrum disorders

2017 ◽  
Vol 10 (7) ◽  
pp. 265-289 ◽  
Author(s):  
Sung-Min Kim ◽  
Seong-Joon Kim ◽  
Haeng Jin Lee ◽  
Hiroshi Kuroda ◽  
Jacqueline Palace ◽  
...  
Keyword(s):  
Differential Diagnosis ◽  
Neuromyelitis Optica ◽  
Clinical Manifestations ◽  
Inflammatory Disorder ◽  
Test Results ◽  
Early Disease ◽  
The Central Nervous System ◽  
Assay Methods ◽  
Spectrum Disorders ◽  
Disease Stages

Neuromyelitis optica spectrum disorder (NMOSD) is an inflammatory disorder of the central nervous system (CNS) mostly manifesting as optic neuritis and/or myelitis, which are frequently recurrent/bilateral or longitudinally extensive, respectively. As the autoantibody to aquaporin-4 (AQP4-Ab) can mediate the pathogenesis of NMOSD, testing for the AQP4-Ab in serum of patients can play a crucial role in diagnosing NMOSD. Nevertheless, the differential diagnosis of NMOSD in clinical practice is often challenging despite the phenotypical and serological characteristics of the disease because: (1) diverse diseases with autoimmune, vascular, infectious, or neoplastic etiologies can mimic these phenotypes of NMOSD; (2) patients with NMOSD may only have limited clinical manifestations, especially in their early disease stages; (3) test results for AQP4-Ab can be affected by several factors such as assay methods, serologic status, disease stages, or types of treatment; (4) some patients with NMOSD do not have AQP4-Ab; and (5) test results for the AQP4-Ab may not be readily available for the acute management of patients. Despite some similarity in their phenotypes, these NMOSD and NMOSD-mimics are distinct from each other in their pathogenesis, prognosis, and most importantly treatment. Understanding the detailed clinical, serological, radiological, and prognostic differences of these diseases will improve the proper management as well as diagnosis of patients.

Acute Disseminated Encephalomyelitis

2017 ◽  
Author(s):  
Benjamin M. Greenberg ◽  
Allen Desena
Keyword(s):  
Spinal Cord ◽  
Central Nervous System ◽  
Nervous System ◽  
Supportive Care ◽  
Acute Disseminated Encephalomyelitis ◽  
Autoimmune Response ◽  
Inflammatory Disorder ◽  
The Central Nervous System ◽  
The Brain

Acute disseminated encephalomyelitis (ADEM) is a rare inflammatory disorder of the central nervous system (CNS) that can be fatal or lead to long-term disability. Various triggers have been identified in children and adults, which presumably cause an autoimmune response targeting myelin. The resulting inflammation causes demyelination and edema of the brain, spinal cord, and optic nerves. Depending on which portion of the CNS is affected, patients will experience a variety of symptoms including weakness, numbness, ataxia, encephalopathy, and seizures. Treatment is currently focused on reducing the amount of inflammation and supportive care.

Neuromyelitis Optica Spectrum Disorders

2019 ◽  
pp. 109-129
Author(s):  
A. Sebastian López-Chiriboga ◽  
Brian G. Weinshenker
Keyword(s):  
Spinal Cord ◽  
Central Nervous System ◽  
Neuromyelitis Optica ◽  
Inflammatory Diseases ◽  
Treatment Options ◽  
Neuromyelitis Optica Spectrum Disorders ◽  
Future Prospects ◽  
Mortality And Morbidity ◽  
The Central Nervous System ◽  
Spectrum Disorders

Neuromyelitis optica spectrum disorders (NMOSD) are inflammatory diseases of the central nervous system, traditionally diagnosed in patients with inflammatory attacks restricted to the optic nerves and spinal cord. The chapter considers the epidemiology, pathophysiology and immunopathogenesis of NMOSD. The clinical presentation and radiographic features are reviewed and the prognosis of patients with NMOSD is considered. The mortality and morbidity of untreated NMOSD is much greater than those of MS. Treatment options are summarized and finally future prospects of research are considered.

Brainstem manifestations in neuromyelitis optica: a multicenter study of 258 patients

2013 ◽  
Vol 20 (7) ◽  
pp. 843-847 ◽  
Author(s):  
L Kremer ◽  
M Mealy ◽  
A Jacob ◽  
I Nakashima ◽  
P Cabre ◽  
...  
Keyword(s):  
Spinal Cord ◽  
Central Nervous System ◽  
Nervous System ◽  
Hearing Loss ◽  
Neuromyelitis Optica ◽  
Multicenter Study ◽  
High Frequency ◽  
Caucasian Population ◽  
The Central Nervous System ◽  
Optic Nerve Involvement

Background: Neuromyelitis optica (NMO) is a severe autoimmune disease of the central nervous system characterized by spinal cord and optic nerve involvement. Brainstem manifestations have recently been described. Objective: To evaluate the time of occurrence, the frequency and the characteristics of brainstem symptoms in a cohort of patients with NMO according to the ethnic background and the serologic status for anti-aquaporin-4 antibodies (AQP4-abs). Methods: We performed a multicenter study of 258 patients with NMO according to the 2006 Wingerchuk criteria and we evaluated prospectively the frequency, the date of onset and the duration of various brainstem signs in this population. Results: Brainstem signs were observed in 81 patients (31.4%). The most frequently observed signs were vomiting (33.1%), hiccups (22.3%), oculomotor dysfunction (19.8%), pruritus (12.4%), followed by hearing loss (2.5%), facial palsy (2.5%), vertigo or vestibular ataxia (1.7%), trigeminal neuralgia (2.5%) and other cranial nerve signs (3.3%). They were inaugural in 44 patients (54.3%). The prevalence was higher in the non-Caucasian population (36.6%) than in the Caucasian population (26%) ( p<0.05) and was higher in AQP4-ab-seropositive patients (32.7%) than in seronegative patients (26%) (not significant). Conclusions: This study confirms the high frequency of brainstem symptoms in NMO with a majority of vomiting and hiccups. The prevalence of these manifestations was higher in the non Caucasian population.

An uncommon cause of vertigo: Neuromyelitis optica spectrum disorder

2021 ◽  
pp. 014556132110533
Author(s):  
Kuan-Ling Lin ◽  
Ching-Yu Yang ◽  
Wen-Ko Su
Keyword(s):  
Spinal Cord ◽  
Central Nervous System ◽  
Nervous System ◽  
Neuromyelitis Optica ◽  
Spectrum Disorder ◽  
Atypical Presentation ◽  
Neuromyelitis Optica Spectrum Disorder ◽  
Positive Serum ◽  
The Central Nervous System ◽  
Aquaporin 4 Antibody

Neuromyelitis optica spectrum disorder (NMOSD) is an uncommon antibody-mediated disease of the central nervous system. Its classic presentation includes long segments of spinal cord inflammation, optic neuritis with or without intractable vomiting, and hiccups. Here, we described a case of a 39-year-old woman with an atypical presentation of vertigo, which was finally diagnosed as NMOSD by a positive serum aquaporin-4 antibody.

An acute onset of paraparesis in 65 years old Croatian woman – a case report

2018 ◽  
Author(s):  
V. Kosta
Keyword(s):  
Spinal Cord ◽  
Central Nervous System ◽  
Nervous System ◽  
Case Report ◽  
Neuromyelitis Optica ◽  
Demyelinating Disease ◽  
Acute Onset ◽  
Spinal Cord Lesions ◽  
The Central Nervous System ◽  
High Doses

Neuromyelitis optica (NMO, Devic`sdisease) is a rare inflammatory, demyelinating disease of the central nervous system that predominantly affects the spinal cord and optic nerves. Seropositivity for NMO-IgG (aquaporin 4antybodies) and longitudinally extensive spinal cord lesions (3 or more segments) are characteristics of NMO. We described a 65-year old woman with an acute onset of paraparesis that was not recognized as NMO at the beginning. The diagnosis was made three months later when she was readmitted because of the relapse.Despite the treatment with high doses of methylprednisolone, plasmapheresis and immunoglobulins her condition stayed unchanged – she was paraplegic and incontinent.

Neuromyelitis Optica

2013 ◽  
Author(s):  
Aaron E. Miller ◽  
Teresa M. DeAngelis
Keyword(s):  
Multiple Sclerosis ◽  
Spinal Cord ◽  
Central Nervous System ◽  
Nervous System ◽  
Neuromyelitis Optica ◽  
Autoimmune Disorder ◽  
Therapeutic Approaches ◽  
New Concepts ◽  
The Central Nervous System ◽  
Spectrum Disorders

Neuromyelitis optica (NMO), a chronic inflammatory, demyelinating autoimmune disorder of the central nervous system with a predilection for the optic nerves and spinal cord, has long been confused with classical multiple sclerosis. In this chapter, we review the important clinical and radiographic distinctions of NMO and NMO spectrum disorders, and summarize promising new concepts in pathophysiology and therapeutic approaches.

scholarly journals Acute Respiratory Failure due to Neuromyelitis Optica Treated Successfully with Plasmapheresis

2016 ◽  
Vol 2016 ◽  
pp. 1-3
Author(s):  
Massa Zantah ◽  
Timothy B. Coyle ◽  
Debapriya Datta
Keyword(s):  
Spinal Cord ◽  
Central Nervous System ◽  
Nervous System ◽  
Respiratory Failure ◽  
Acute Respiratory Failure ◽  
Neuromyelitis Optica ◽  
Cervical Spinal Cord ◽  
High Dose ◽  
Pulse Dose ◽  
The Central Nervous System

Neuromyelitis Optica (NMO) is a demyelinating autoimmune disease involving the central nervous system. Acute respiratory failure from cervical myelitis due to NMO is known to occur but is uncommon in monophasic disease and is treated with high dose steroids. We report a case of a patient with NMO who developed acute respiratory failure related to cervical spinal cord involvement, refractory to pulse dose steroid therapy, which resolved with plasmapheresis.

scholarly journals Neuromyelitis optica with brain stem involvement in a middle-aged Ethiopian woman: a case report and review of literature

2021 ◽  
Vol 15 (1) ◽  
Author(s):  
Rodas Asrat Kassu ◽  
Hailu Abera Mulatu ◽  
Sisay Gizaw ◽  
Henok Fisseha ◽  
Amir Musema ◽  
...  
Keyword(s):  
Spinal Cord ◽  
Central Nervous System ◽  
Nervous System ◽  
Case Report ◽  
Optic Neuritis ◽  
Neuromyelitis Optica ◽  
Oculomotor Nerve ◽  
Oculomotor Nerve Palsy ◽  
Optic Nerves ◽  
The Central Nervous System

Abstract Introduction Neuromyelitis optica is a demyelinating disease of the central nervous system that predominantly affects the optic nerves and spinal cord. In neuromyelitis optica, white blood cells and antibodies primarily attack the optic nerves and the spinal cord, but may also attack the brain. Brainstem manifestation has been described recently. So far, neuromyelitis optica is very rare in Ethiopia and there were only two case reports, but this is the first case report of neuromyelitis optica with brainstem involvement. Case presentation A 47-year-old Addis Ababa woman presented to Saint Paul’s Hospital Millennium Medical College with a history of visual loss of 7 years and bilateral lower limb weakness of 4 days duration. She had bilateral oculomotor nerve palsy. Her past medical history showed systemic hypertension for 18 years and dyslipidemia for 1 year. The objective evaluation of the patient revealed right optic nerve atrophy suggesting optic neuritis and flaccid paraplegia with sensory level at the fourth thoracic vertebra. Diagnostic work-up using electromyography and spinal magnetic resonance imaging revealed demyelinating anterior visual pathway dysfunction and signs of extensive cervicothoracic transverse myelitis from the third cervical to lower thoracic vertebrae, respectively. Then a diagnosis of neuromyelitis optica was established. After treatment with high-dose systemic steroid followed by azathioprine, the patient was stable for several months with significant improvement of vision and lower-extremity weakness with no relapse of symptoms. Conclusion The case described here is a rare inflammatory demyelinating disorder of the central nervous system occurring in East Africa. It reminds clinicians to suspect neuromyelitis optica in a patient who presented with unexplained recurrent optic neuritis to make a timely diagnosis and prevention of permanent neuronal damage. Neuromyelitis optica can also be associated with oculomotor nerve involvement.

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