History Of Neuromyelitis Optica Research Articles

Secondary Hypogammaglobulinemia After Rituximab for Neuromyelitis Optica: A Case Report.

A 17-year-old male with history of neuromyelitis optica and seizures presented to the pulmonology clinic for evaluation of recurrent pneumonias. He had received rituximab for the past 6 years. Over the past 2 years, he experienced four episodes of pneumonia. In between these episodes, he would improve briefly but continued to have daily cough that was productive with yellow phlegm. He also had recurrent rhinitis and sinusitis despite multiple antibiotic courses. Review of chest X-rays revealed localized right middle lobe and right lower lobe infiltrates. An extensive workup was performed, including computed tomography (CT) of the chest and bronchoscopy to rule out congenital lesions of the right lung and foreign body aspiration. Chest CT showed right lower lobe bronchiectasis. Flexible bronchoscopy with bronchoalveolar lavage showed normal anatomy with thick mucus secretions in the right lower lobe. Immunologic evaluation was performed and revealed low levels of immunoglobulin (Ig)-G, IgM, and IgA, which had declined since initiation of rituximab. Lymphocyte subset testing was remarkable for low cluster of differentiation (CD)-19. He was referred to allergy and immunology and was initiated on immunoglobulin-replacement therapy (IGRT) for acquired hypogammaglobulinemia secondary to rituximab. There was marked clinical improvement after initiation of IGRT.

‘Spinal amaurosis’ (1841). On the early contribution of Edward Hocken to the concept of neuromyelitis optica

While the history of classical multiple sclerosis has been extensively studied, only little is known about the early history of neuromyelitis optica (Devic's syndrome). Here we discuss a forgotten report by Edward Octavius Hocken (1820-1845) published in The Lancet in 1841. Hocken's report is important from a historic point of view for two reasons. Firstly, apart from a French language report by Antoine Portal, no earlier case of spinal cord inflammation and amaurosis is known. Secondly and much more importantly, Hocken, who upon his untimely death at the age of just 25years was honoured by his contemporaries as a "precocious talent" of "very early reputation", in that article propagated the novel concept of 'spinal amaurosis', i.e. the concept of acute amaurosis and spinal cord disease being pathogenetically connected. Hocken's ideas predate Devic and Gault's seminal works on 'neuromyelitis optica' by more than 50years.

The history of neuromyelitis optica

The discovery of a novel serum autoantibody (termed NMO-IgG or AQP4-Ab) in a subset of patients in 2004 has revived interest in neuromyelitis optica (NMO). While the history of classical multiple sclerosis has been extensively studied, only little is known about the history of NMO. In the present article, we provide a comprehensive review of the early history of this rare but intriguing syndrome. We trace the origins of the concept of NMO in the 19th century medical literature and follow its evolution throughout the 20th and into the 21st century. Finally, we discuss recent proposals to revise the concept of NMO and explain why there is indeed a need for a more systematic and descriptive nomenclature.

Homonymous Hemimacular Thinning

A 42-year-old African American woman with a 5-year history of neuromyelitis optica was found to have an incongruous homonymous hemianopia. Optical coherence tomography (OCT) showed corresponding left homonymous hemimacular thinning. Magnetic resonance imaging of the brain demonstrated a demyelinating lesion in the left optic tract (OT) just anterior to the lateral geniculate nucleus and diffusion tensor magnetic resonance tractography confirmed axonal fiber loss in the left OT. This case illustrates the complementary and confirmatory roles of visual field testing, macular OCT, and neuroimaging in an OT lesion, which caused selective hemimacular thinning through retrograde degeneration.

Fulminant cerebral demyelination in neuromyelitis optica

A 43-year-old woman with a 2-year history of neuromyelitis optica (NMO; typical imaging [figure 1] and …

Neuromyelitis optica.

Whether neuromyelitis optica (NMO), the co-occurrence of myelitis and optic neuritis, is a variant of multiple sclerosis (MS) or a unique disease is controversial. Distinct neuropathological features and a fulminant clinical course argue in favor of NMO as a distinct disease. However, the combination of neurological impairments of myelitis and optic neuritis occurs in patients with several inflammatory disorders, including multiple sclerosis and collagen vascular diseases. NMO is also associated with certain infectious diseases. The fact that the NMO phenotype occurs in a variety of disease states suggests that NMO does not represent a specific clinical entity. To better understand NMO and its associations with recognized diseases, a systematic review of the literature using MEDLINE was conducted. The history of NMO, its nosology, associations with other diseases, and current concepts of its pathogenesis and treatment is reviewed in this article.

Demyelinating disease in a woman from tropical South America with features of multiple sclerosis and neuromyelitis optica: Clinical, protein chemistry and pathological findings

The case history of a patient who developed a demyelinating disease in tropical South America is described. The early history was suggestive of multiple sclerosis; the later history of neuromyelitis optica. Her life was prolonged by artificial respiration, but some anoxic episodes occurred. The problems of diagnosis and the geographical distribution of demyelinating diseases are discussed. The protein studies of the serum and cerebrospinal fluid suggest that the patient suffered from a kind of demyelinating disease not previously encountered in the Northern Hemisphere, in our experience, perhaps an intense variant of multiple sclerosis. At necropsy lesions in the grey matter were found and attributed to anoxia. In addition cystic lesions and lesions similar to plaques of multiple sclerosis were found. Wide-spread perivascular cuffing with small round cells was present even in areas showing no other manifestations of disease. Astrocytic fibres were infrequent. The spinal cord, in addition, showed severe destruction. Myelin sheaths similar to those of peripheral nerves were plentiful. The nature of the pathological process and its relationship to other demyelinating diseases are discussed.