Background. Neuromyelitis optica (Devic's disease) is a severe autoimmune inflammatory disorder of the central nervous system. Epidemiological aspects of NMO have not been systemically reviewed. In this study we systematically reviewed and assessed the quality of studies reporting the incidence and/or prevalence of NMO across the world. Methods. A comprehensive literature search using MEDLINE, EMBASE, and Web of Science for the terms “Neuromyelitis optica,” “devic disease,” “incidence,” “prevalence,” and “epidemiology” was conducted on January 31, 2015. Study quality was assessed using an assessment tool based on recognized guidelines and designed specifically for this study. Results. A total of 216 studies were initially identified, with only 9 meeting the inclusion criteria. High level of heterogeneity amongst studies precluded a firm conclusion. Incidence data were found in four studies and ranged from 0.053 per 100,000 per year in Cuba to 0.4 in Southern Denmark. Prevalence was reported in all studies and ranged from 0.51 per 100,000 in Cuba to 4.4 in Southern Denmark. Conclusion. This review reveals the gaps that still exist in the epidemiological knowledge of NMO in the world. Published studies have different qualities and methodology precluding a robust conclusion. Future researches focusing on epidemiological features of NMO in different nations and different ethnic groups are needed.

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BackgroundCocaine abuse is associated with several mechanisms of brain injury including ischemic, hemorrhagic and metabolic. Recently two case reports of leukoencephalopathy in cocaine users implicated a commonly used cocaine adulterant, levamisole. One well-documented adverse effect of levamisole, when used alone as antihelminthic or immunomodulatory drug, is multifocal inflammatory leukoencephalopathy. Therefore, immune mechanisms may also contribute to cocaine-induced brain injury.Case presentationsTwo cocaine users with multifocal leukoencephalopathy, treated with steroids and plasmapheresis, are described. The first is a 25-year-old man who presented with unilateral motor and sensory impairment progressing to bilateral deficits, dysphagia, dysarthria and confusion over several days. Serial MRI showed increasing abnormal FLAIR signal lesions with patchy restricted diffusion and heterogenous enhancement deep in the right and left hemispheres, including periventricular white matter as well as in the pons and cerebellar peduncle. The second patient is a 41-year-old woman who presented with confusion and impaired balance. MRI showed bilateral periventricular FLAIR lesions with scattered restricted diffusion and subtle gadolinium enhancement of some of the lesions. She initially stabilized with supportive care only, but after further cocaine use was re-admitted six weeks later with marked neurological deterioration and MRI showed prominent worsening of the lesions. Both patients received steroid and plasma exchange and showed substantial improvement clinically and on imaging, which was sustained during out-patient follow-up.ConclusionMultifocal leukoencephalopathy associated with cocaine use may have an inflammatory/immune basis, possibly related to levamisole contamination, at least in some patients. Three cases, including the present two, have been described wherein good neurological improvement was seen in association with steroid treatment. However, in the absence of appropriate clinical trials, it remains unknown whether immunotherapy is truly beneficial for these patients.

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Table 1. Demographic and clinical features of patients. Number of patients: 95 (29 male and 66 female) Female/male: 2.27/1 Age of onset: 30±10 a (range: 8-68) Age of patients: 36.6 ± 11.0 (range: 8-68) Follow up duration: 6.3±4.5 years Course of the disease: Monophasic: 57 patients (60%) 20 males and 37 females (mean age: 38.1±11.4) Relapsing: 38 patients (40%) Nine males and 29 females (mean age: 34.2±10.1) Relapses: Relapse number/patient: 2.4±1.5 (range: 1-8) Annualized relapse rate (ARR): Total: 0.96 (Female ARR=1.14, male ARR=0.55, p=0.04

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A 26-year-old man with no substantial medical history presented to an academic emergency department in Winnipeg with a headache and associated neurologic symptoms. The patient reported that earlier in the day while driving a car, he had felt a sudden tingling and numbness in his right foot. This subsequently spread up the right side of his body to his right leg, right arm, and the right side of his face. The episode lasted about 10 minutes before the symptoms spontaneously resolved. Later the same day, a second episode of rightsided numbness occurred. This time, the symptoms were associated with difficulty speaking, prompting the patient to go to the emergency department.Between 45 and 60 minutes after the onset of symptoms, the patient had a moderate-to-severe throbbing headache. The speech and sensory problems resolved within 2 hours and 30 minutes, whereas the headache improved within 11 hours. The patient reported no history of migraine headaches. During the visit to the emergency department, computed tomography (CT) imaging of the brain, CT angiogram of the cervical and cranial arteries and unenhanced magnetic resonance imaging (MRI) of the brain done with diffusion weighted imaging and apparent diffusion coefficient mapping were all unremarkable. The patient returned home the same day with a diagnosis of migraine with aura.Two days later, the patient woke up early in the morning with numbness and tingling in his left foot, which gradually spread over several minutes to his left arm and the left side of his face, and was associated with a headache and a milder degree of speech impairment in the form of word-finding difficulty. No visual symptoms were reported. The patient again presented to the emergency department for further evaluation. Within 1 hour his neurologic deficits had improved, but he continued to complain of a severe bifrontal throbbing headache, with associated nausea, vomiting, photophobia and phonophobia. The headache was worse with standing and activity. The patient reported no fever, chills, sweats, neck stiffness or rash. A review of systems was otherwise negative. The patient lived in Winnipeg and reported no recent travel outside of Manitoba. He had no contact with animals or people who were ill. There was no history of tick exposure. However, the patient did report numerous recent mosquito bites.On physical examination, the patient was afebrile and hemodynamically stable, with a blood pressure of 165/97 mm Hg and a heart rate of 70 beats/min. Cardiac, respiratory and abdominal examinations were unremarkable. No nuchal rigidity or rash was seen. The neurologic examination showed blurring of the optic disk margins bilaterally. Basic electrolytes, serum creatinine and liver enzymes were within normal limits. The patient had a normal total white blood cell count, hemoglobin and platelet count. Additional CT imaging of the brain was unremarkable. Electroencephalography did not show any epileptiform discharges. A repeat MRI of the brain showed suspected leptomeningeal enhancement over both co...