Disseminated inflammation of the central nervous system associated with acute hepatitis E: a case report.

BACKGROUND: Hepatitis E infection affects over 20 million people worldwide. Reports of neurological manifestations are largely limited to the peripheral nervous system. We report a middle-aged genotype 3c male patient with acute hepatitis E virus (HEV) infection and severe neurological deficits with evidence of multiple disseminated inflammatory lesions of the central nervous system. CASE PRESENTATION: A 42-year-old male patient presented to our emergency department with musculoskeletal weakness, bladder and bowel retention, blurred vision and ascending hypoesthesia up to the level of T8. Serology showed elevated liver enzymes and positive IgM-titers of hepatitis E. Analysis of cerebrospinal fluid (CSF) showed mild pleocytosis and normal levels of glucose, lactate and protein. HEV-RNA-copies were detected in the CSF and stool. Within 3 days after admission the patient became paraplegic, had complete visual loss and absent pupillary reflexes. MRI showed inflammatory demyelination of the optic nerve sheaths, multiple subcortical brain regions and the spinal cord. Electrophysiology revealed axonal damage of the peroneal nerve on both sides with absent F-waves. Treatment was performed with methylprednisolone, two cycles of plasma exchange (PLEX), one cycle of intravenous immunoglobulins (IVIG) and ribavirin which was used off-label. Liver enzymes normalized after 1 week and serology was negative for HEV-RNA after 3 weeks. Follow-up MRI showed progressive demyelination and new leptomeningeal enhancement at the thoracic spine and cauda equina 4 weeks after admission. Four months later, after rehabilitation was completed, repeated MRI showed gliotic transformation of the spinal cord without signs of an active inflammation. Treatment with rituximab was initiated. The patient remained paraplegic and hypoesthesia had ascended up to T5. Nevertheless, he regained full vision. CONCLUSIONS: Our case indicates a possible association of acute HEV infection with widespread disseminated central nervous system inflammation. Up to now, no specific drugs have been approved for the treatment of acute HEV infection. We treated our patient off-label with ribavirin and escalated immunomodulatory therapy considering clinical progression and the possibility of an autoimmune response targeting nerve cell structures. While response to treatment was rather limited in our case, detection of HEV in patients with acute neurological deficits might help optimize individual treatment strategies.

Parapapillary choroidal microvasculature in retrobulbar optic neuritis: An optical coherence tomography angiography study.

PURPOSE: To compare superficial and deep vascular characteristics of the optic disc in retrobulbar optic neuritis using optical coherence tomography angiography (OCT-A). METHODS: Nineteen patients with unilateral non-infectious retrobulbar neuritis were included in the study. The contralateral eyes of each patient were served as controls. OCT-A scans of the optic discs were performed in a 4.5 × 4.5 mm rectangular area, while macular OCT-A scans were performed in a 6 × 6 mm rectangular area. Various parameters, including radial peripapillary capillary (RPC) density, peripapillary retinal nerve fibre layer (pRNFL) thickness, cup volume, rim area, disc area, cup-to-disc (c/d) area ratio, and vertical and horizontal c/d ratios were automatically obtained using the instrument software. The density for superficial capillary plexus (SCP) and deep capillary plexus (DCP) were assessed using macular OCT-A. Parapapillary choroidal microvascular (PPCMv) density was calculated using MATLAB software. RESULTS: Parafoveal inferior, perifoveal total and inferior SCP densities were significantly decreased in eyes with optic neuritis when compared with contralateral control eyes in OCT-A measurements (respectively, p = 0.027, p = 0.041, p = 0.045). The axial lengths, (p = 0.72), vertical and horizontal cup-disc ratios, and disc area, cup-disc areas, cup volumes, and pRNFL thicknesses between the groups were similar (for each, p>0.05). CONCLUSIONS: This study demonstrated for the first time that patients with retrobulbar optic neuritis had decreased SCP densities, though it did not cause any changes in PPCMv density.

Optic Neuritis Associated or Not with TNF Antagonists in Patients with Inflammatory Bowel Disease.

BACKGROUND AND AIMS: Acute optic neuritis [ON] is an inflammatory condition affecting the optic nerve. Clinicians should suspect optic neuritis in cases of painful and rapidly progressive loss of central visual field. This condition may be associated with a multitude of diseases, and mostly with multiple sclerosis [MS] where it may present as an initial symptom. The literature reports that optic neuritis and MS occur in patients with inflammatory bowel disease [IBD] before and after the era of anti-tumour necrosis factor-α [TNFα] drugs. At the present moment, there is little consensus for managing this complication, currently treated with corticosteroids and discontinuation of the causative agents. METHODS: We collected cases through a retrospective multicentre European Crohn's and Colitis Organisation CONFER [COllaborative Network For Exceptionally Rare case reports] project. We also performed a comprehensive retrospective search of the available literature on this topic. RESULTS: We report herein 12 new cases of ON, including 10 under anti-TNF therapy, collected through the CONFER project. We also compare characteristics of ON associated or not with anti-TNFα agents. CONCLUSIONS: The exceptional and current observation of distant family history of MS in 17% of our patients who developed ON, despite the small number and the lack of a control arm, might be an important signal that should be taken into account in our therapeutic strategies in the future.

Neurological Manifestations of Brucellosis in an Indian Population.

Brucellosis is a zoonotic disease causing serious public health problems in countries of the Middle-East and developing countries like India. Neurobrucellosis is one of the devastating complications of this re-emerging zoonosis. The objective of this review was to identify the neurological manifestations of Brucellosis in an Indian population and bring into light the effective modalities used for treating neurobrucellosis. A systematic review of the scientific literature reported in accordance with the preferred reporting items for systematic reviews and meta-analysis (PRISMA) guidelines was conducted. Three databases (PubMed, IndMed, and ScienceDirect) were used to analyze retrospectively case reports of sufficient quality for data extraction (from the last 15 years, 2002-2016), and relevant literature was reviewed. Most of the cases had a definite history of exposure to Brucella through occupational contact with cattle, drinking raw milk, or living near unhygienic abattoir or even trips to epidemic areas outside India. The common presentations include fever, meningitis, brisk deep-tendon reflexes, extensor plantars, sensory deficit usually below the twelfth thoracic vertebral level, weakness of lower limbs, ocular signs of papilledema, and retrobulbar neuritis. The usual systemic findings associated were hepatosplenomegaly and weight-loss. Neurobrucellosis needs to be kept in mind in the differential diagnosis of fever of unknown origin involving neurological symptoms and systemic involvement. Prognosis is good if there is a combination of antibiotics, each with different mechanisms of action given in full dose. Suitable measures for its prevention are also suggested.

Orbital and optic nerve complications of inflammatory bowel disease.

BACKGROUND AND AIMS: Extraintestinal manifestations of inflammatory bowel disease (IBD) can involve the orbit and the optic nerve. Although these manifestations are rare, they can be particularly serious as they can lead to permanent loss of vision. The aim of the review is to present the existing literature on IBD-related optic nerve and orbital complications. METHODS: A literature search identified the publications reporting on incidence, clinical features and management of IBD patients with optic nerve and orbital manifestations. RESULTS: Posterior scleritis and orbital inflammatory disease (orbital pseudotumor) are the most commonly encountered entities affecting the structures of the orbit. On the other hand, the optic nerve of IBD patients can be affected by conditions such as optic (demyelinating) neuritis ("retrobulbar" neuritis), or ischaemic optic neuropathy. Other neuro-ophthalmic manifestations that can be encountered in patients with IBD are related to increased intracranial pressure or toxicity secondary to anti tumour necrosis factor (anti-TNF) agents. CONCLUSIONS: IBD-related optic nerve and orbital complications are rare but potentially vision-threatening. Heightened awareness and close cooperation between gastroenterologists and ophthalmologists are warranted.