Incidence and Mortality of Cerebral Venous Thrombosis in a Norwegian Population.

BACKGROUND AND PURPOSE: There are conflicting figures of the incidence of cerebral venous thrombosis (CVT). The incidence was previously estimated to around 0.5/100 000/y, but more recent studies have suggested 1 to 1.5/100 000/y. The purpose of this study was to explore the incidence and mortality of CVT in a Norwegian population. METHODS: A retrospective cross-sectional hospital population-based study conducted at Akershus University Hospital serving roughly 10% of the total Norwegian population. Patients were identified through chart reviews based on the relevant International Classification of Diseases(Tenth Revision) codes for new CVT cases in a 7-year period between January 1, 2011, and December 31, 2017. Only inhabitants living in the hospital's catchment area were included. RESULTS: Sixty-two patients aged 0 to 80 years were identified and included. The median age was 46 years and 53% were females. The overall incidence of CVT was 1.75 (95% CI, 1.36-2.23) per 100 000/y with no significant sex differences. The incidence for children and adolescents (<18 years, n=9) was lower than for adults (≥18 years, n=53); 1.08 (0.52-1.97) versus 1.96 (1.49-2.55) per 100 000/y per year, with the highest incidence for those >50 years with 2.10 (1.38-3.07)/100 000/y. Headache was the most prevalent symptom, reported in 83%, followed by nausea, motor deficits, and seizures observed in 45%, 32%, and 32% of the patients. Transverse sinuses and the jugular vein were the most frequent sites of thrombosis. In most patients (61%), thrombosis occurred in multiple sinuses/veins. Risk factors were found in 73% of the patients, and most of the patients had a combination of 2 or more risk factors. The 30-day and 1-year mortality rates were 3% and 6%. CONCLUSIONS: The incidence of CVT in this population was higher than previously reported. The mortality rate was similar to previous studies.

A young woman with seizures, visual impairment, and paralysis. / En ung kvinne med krampeanfall, synsforstyrrelser og lammelser.

BACKGROUND: Anti-MOG encephalomyelitis is a recently described demyelinating, autoimmune disease of the central nervous system, identified by antibodies against myelin oligodendrocyte glycoprotein (MOG). CASE PRESENTATION: A previously healthy 20-year-old woman was admitted to hospital after a seizure. MRI showed leptomeningeal enhancement and lumbar puncture revealed moderate pleocytosis but no evidence of infection. Over the following months, she experienced a series of neurological deficits including bladder dysfunction, loss of sensation in the lower extremities and genital area, impaired motor function of the legs and episodes of visual loss. All symptoms had MRI correlates in the medulla, brainstem, optic tract, thalami and corpus callosum. She responded excellently to corticosteroid treatment, but experienced relapses shortly after discontinuation of treatment. Repeated lumbar puncture revealed pleocytosis up to 475 ∙ 106 cells/l but there were no signs of intrathecal IgG synthesis or infection. Serum anti-MOG antibodies were detected two months after the initial episode. She has been treated with low dose corticosteroids in combination with rituximab for two years, without clinical or radiological relapse. INTERPRETATION: Symptoms and signs mimicking acute demyelinating encephalomyelitis and neuromyelitis optica are typical for anti-MOG encephalomyelitis. This case illustrates that the response to corticosteroids may be excellent but transient, and that the disease can be controlled with moderate immunosuppression.

Severe inflammatory disease activity 14 months after cessation of Natalizumab in a patient with Leber's optic neuropathy and multiple sclerosis - a case report.

BACKGROUND: Leber's hereditary optic neuropathy (LHON) co-occuring with multiple sclerosis-like disease (LHON-MS) is suggested to be a separate disease entity denoted Harding's disease. Little is known about the response to initiation and discontinuation of potent immunomodulatory treatment in LHON-MS. CASE PRESENTATION: We describe a LHON-MS patient with 27 years disease duration who developed severe disease activity peaking 14 months after discontinuation of natalizumab, with extensive new inflammatory lesions throughout the brain and in the spinal cord resembling immune inflammatory reconstitution syndrome. She had previously been clinically and radiologically stable on natalizumab treatment for 6 years, and before that only experienced subtle clinical activity during 9 years on interferon beta1a. CONCLUSION: This is the first report on severe exacerbation of inflammatory disease activity after discontinuation of natalizumab in LHON-MS, and suggests that late rebound activity can occur in these patients.

Extensive Multiple Sclerosis Reactivation after Switching from Fingolimod to Rituximab.

During treatment with fingolimod, B cells are redistributed from blood to secondary lymphoid organs, where they are protected from the effect of anti-CD20 and other cell-depleting therapies. We describe a multiple sclerosis patient who had almost complete depletion of B cells in blood during and shortly after treatment with fingolimod. He developed severe disease activity resembling immune reconstitution syndrome after switching from fingolimod to rituximab, with first dose being six weeks after fingolimod cessation. Following recommendations from the Swedish MS Association, rituximab treatment was started as one single dose of 1000 mg. In patients treated with fingolimod, pathogenic B cells may still be sequestered in secondary lymph nodes if this dose is given early. To deplete such B cells as they egress from the lymph nodes, we propose that a second dose of rituximab a few weeks after the first dose should be considered.

Diffuse alveolar hemorrhage during alemtuzumab infusion in a patient with multiple sclerosis: a case report.

BACKGROUND: Diffuse alveolar bleeding is a potentially life-threatening condition that can be induced by several drugs. Whereas fatal cases have been reported in patients treated for other indications, no report have so far been published in a patient with multiple sclerosis treated with alemtuzumab. CASE PRESENTATION: We report a case of alemtuzumab-induced diffuse alveolar bleeding in a 29 year old woman with relapsing remitting multiple sclerosis. The patient developed acute shortness of breath, chest pain on inspiration and haemoptysis following the second infusion of alemtuzumab during the first treatment cycle. Computed tomography showed bilateral alveolar opacities. Bronchoscopy and broncho-alveolar lavage showed persistently bloody return with no evidence of infection. The symptoms resolved completely without treatment and control computed tomography performed one week later showed total resolution of pulmonary infiltrates. CONCLUSION: This is the first published report of diffuse alveolar bleeding in a patient with multiple sclerosis treated with alemtuzumab. Four similar cases in patients treated for multiple sclerosis and several fatal cases in patients treated for other conditions are registered at the World Health Organization database of suspected adverse events (VIgiBase©), underscoring that this is a serious and possibly under-recognized complication of alemtuzumab which can also occur in the treatment of multiple sclerosis. The clinician should consider the possibility of diffuse pulmonary haemorrhage in patients with sudden onset of respiratory distress and haemoptysis following administration of alemtuzumab for multiple sclerosis.