Neuroimmunology Reports最新文献

Background

Neurosarcoidosis is well recognized as a great imitator, however cerebral neurosarcoidosis is usually characterized by a limited spectrum of radiological features which does not include unilateral encephalitis. Truly unilateral encephalitides have a limited differential diagnosis. Most classically there are the infectious causes, predominantly HSV encephalitis, and the inflammatory causes. Rasmussen's syndrome, more common in children, is reported with presentation in adulthood and anti-MOG antibodies have been reported to cause a unilateral cortical inflammation too. However, most of these present acutely, in contrast to our patient who had a subacute presentation.

Case

We present a case of atypical neurosarcoidosis presenting with a hemiencephalitis. A subacute history of clinical and radiological deterioration is seen. Because the clinico-radiological picture was atypical for neurosarcoidosis a brain biopsy was performed which revealed granulomatous inflammation of the cortex without clear involvement of the meninges or vasculitis. Subsequent treatment included steroids, methotrexate and infliximab.

Conclusions

In conslusion, neurosarcoidosis is a challenging diagnosis because clinical manifestations and imaging findings can be resembled by several other diseases. This case highlights further variation in it's presentation.

Objective

To report a case of anti-IgLON5 disease unmasked by asymptomatic SARS-CoV-2 infection.

Background

Anti-IgLON5 disease is a clinically heterogeneous disease that shares features of both neurodegeneration and neuroinflammation. The onset can be insidious, posing diagnostic challenges and often resulting in treatment delay. Infectious trigger was rarely reported in this disease.

Case report

A 64-year-old male initially presented with 1-year history of progressive parasomnia and mild cognitive decline that precipitously worsened over the course of 1 month following asymptomatic SARS-CoV-2 infection, resulting in dysphagia, parkinsonism, weight loss and dependence on all activities of daily living. He was found to have high titer (1:3840) of anti-IgLON5 antibody in the serum, confirming the diagnosis of anti-IgLON5 disease.

Conclusion

Anti-IgLON5 disease as a potentially reversible cause of neurodegenerative syndrome in patients with atypical features. Timely diagnosis and treatment may improve clinical outcomes. It is also worth noting that symptoms precipitously worsened following SARS-CoV-2 infection. We suspect that a COVID-19-mediated immune activation response exacerbated the underlying autoimmune encephalitis process, unmasking his symptoms.

We report the case of a young woman who presented with chronic relapsing inflammatory optic neuritis (CRION) in association with HLA-B27 and silent brain lesions. The attacks were refractory to steroids but no further attacks occurred on treatment with mycophenolate mofetil. This is one of the few HLA-B27-associated optic neuritis cases reported in the literature, emphasizing the relation of HLA-B27 and inflammation of the brain and optic nerve, as well as the role mycophenolate mofetil might offer in stabilizing the disease.

Background

Aquaporin-4-antibody Neuromyelitis Optica Spectrum Disorder (AQP4-Ab NMOSD) is an immune-mediated inflammatory central nervous system disease. While AQP4 is widely expressed in other tissues, extra-CNS manifestations are rare, with most cases manifesting as hyperCKemia and myositis. Lung involvement manifesting as organising pneumonia in AQP4-Ab NMOSD has rarely been reported.

Case series

We present 3 patients with AQP4-Ab NMOSD who developed organising pneumonia at the onset of neurological disease. All 3 had area postrema syndrome and 2 developed longitudinally extensive transverse myelitis. The lung changes resolved spontaneously in 1 patient and after immunotherapy in the other 2 patients. We also tabulated the published cases to date in order to highlight the pertinent clinical and paraclinical features of this association.

Conclusion

The close temporal relationship between organising pneumonia and the onset of AQP4-Ab NMOSD suggests that AQP4-Ab autoimmunity is directly involved in the development of organising pneumonia, presumably from the increased susceptibility to AQP4-Ab induced complement-mediated injury in the peripheral tissues of these individuals in light of the current experimental evidence.

Background

Cerebrovascular involvement of neurosarcoidosis is a rare albeit increasingly recognized disorder requiring a multidisciplinary approach to diagnosis and management.

Case description

We present a case of systemic sarcoidosis with neurological involvement of brain parenchyma and intracranial vasculature, and discuss a step-wise approach to the diagnostic evaluation.

Conclusion

An inflammatory vasculitis should be considered in a patient with confirmed or highly suspected sarcoidosis with ischemic stroke. MR angiography with vessel-wall imaging should be pursued early in the evaluation to support this diagnosis. Cerebrovascular complications of sarcoidosis are often responsive to sarcoid-related immunotherapies.

Background

Temporal Arteritis is the most common panarteritis in adults and typically affects elderly people, particularly women. This occlusive vasculitis is associated with at least 51 symptoms and signs and can result in bilateral blindness and potentially death from aortic root dilatation and rupture.

Case Report

This report demonstrates a case of Temporal Arteritis with bilateral, rapidly progressive arteritic anterior ischaemic optic neuropathy despite recommended high dose oral steroid therapy, with right eye blindness only recognised after left visual loss developed. The addition of tocilizumab was required for resolution of the symptoms.

Conclusions

It is essential to examine thoroughly all the parameters of visual function in a patient who has lost vision. Such an examination, especially in patients with TA, consists as a minimum of visual acuity, pupil reactions, confrontation fields and fundi in both eyes in every patient.

Introduction

Visual impairment due to optic neuritis is one of the most common manifestations of multiple sclerosis (MS); however progressive visual loss without relapse is rare. Furthermore, the association of progression independent of relapse activity (PIRA) with visual impairment has not been largely explored. We report a rare case of MS associated with progressive visual loss, independent of relapse, with detailed follow-up data of visual acuity, critical flicker fusion frequency (CFF), and visual-evoked potential (VEP), which provided supporting evidence for monitoring visual function.

Case report

A 44-year-old Japanese woman with MS presented with decreased visual acuity in both eyes. Her symptoms progressed slowly without relapse and did not respond to intravenous methylprednisolone or plasma exchange. Her CFF and VEP also showed gradual exacerbation. Magnetic resonance imaging revealed no new lesions of MS. Visual impairment was correlated with physical worsening, suggesting progression independent of relapse. After ofatumumab was initiated, her visual acuity mildly improved.

Conclusion

We describe a patient with visual impairment that was considered the PIRA. Impairment of visual function, in addition to impaired walking ability and cognitive function, should be considered when assessing disability progression in MS. CFF and P100 latency of VEP may aid in the early diagnosis of PIRA, potentially leading to timely treatment.

Background

Dimethyl fumarate (DMF) is an oral disease modifying therapy (DMT) approved for the treatment of relapsing-remitting multiple sclerosis (RRMS). DMF is effective at reducing relapses, and decreasing disease activity. Reports of rebound activity following DMF discontinuation are rare.

Case report

We report the case of a 63-year old woman with RRMS, showing an extensive radiological and clinical rebound after DMF discontinuation. She started DMF in 2014 at which time her EDSS was 1.5. She was clinically stable until 2019, when she noted the onset of progressive left leg weakness but without signs of radiological activity on brain MRI. In 2020, she experienced further worsening and MRI showed one new brain lesion. She was treated with oral steroids with partial recovery. She self-discontinued DMF in February 2021. Approximately 5 months after discontinuation she developed severe left side weakness and her brain MRI showed 34 enhancing lesions. She was treated with another course of oral steroids with clinical benefit and her DMT was switched to oral cladibrine.

Conclusions

This case is unusual because MS disease rebound is uncommon after DMF discontinuation and because disease activity of this magnitude with over 30 gadolinium enhancing lesions is rare in people with MS over the age of 60. Our case stresses that a reactivation of MS activity including a large number of enhancing lesions can occur in older individuals, despite a presumably senescent immune system. Monitoring for disease activity before and after discontinuing a DMT is important, regardless of the patient's age.

Background

MOG antibody disease presents along a spectrum that includes acute disseminated encephalomyelitis, transverse myelitis, and optic neuritis. CLIPPERS is a rare condition that may complicate an accurate MOGAD diagnosis. This is in part due to overlapping clinical and imaging features with MOGAD.

Case report

Here we report a case of a 63-year-old woman with relapsing optic neuritis due to MOGAD that was initially concerning for CLIPPERS. The patient was seropositive for MOG-ab and responded well to high dose corticosteroid therapy which was tapered over 9-months.

Conclusion

This case underscores the importance of recognizing the overlap in clinical presentation that may occur between MOGAD and CLIPPERS despite both conditions having distinct biological origins. CLIPPERS criteria and the exclusion of alternative causes can help distinguish between the two. A MOG-ab titer should be used to screen MOGAD as a CLIPPERS mimicker. Antibody testing, clinical imaging, steroid responsiveness, history of present illness, and the extent of existing disability may provide a complete diagnostic picture.