Showing papers on "Myelitis published in 2013"
TL;DR: The patients showing relatively preserved cord with evidence of edema/myelitis with predominantly fluid collection in extradural space on MRI resolve on non-operative treatment, while the patients with extradural compression of mixed or granulomatous nature showing entrapment of spinal cord should be undertaken for early surgical decompression.
Abstract: The most dreaded neurological complications in TB spine occur in active stage of disease by mechanical compression, instability and inflammation changes, while in healed disease, these occur due to intrinsic changes in spinal cord secondary to internal salient in long standing kyphotic deformity. A judicious combination of conservative therapy and operative decompression when needed should form a comprehensive integrated course of treatment for TB spine with neurological complications. The patients showing relatively preserved cord with evidence of edema/myelitis with predominantly fluid collection in extradural space on MRI resolve on non-operative treatment, while the patients with extradural compression of mixed or granulomatous nature showing entrapment of spinal cord should be undertaken for early surgical decompression. The disease focus should be debrided with removal of pus caseous tissue and sequestra. The viable bone should only be removed to decompress the spinal cord and resultant gap should be bridged by bone graft. The preserved volume of spinal cord with edema/myelitis and wet lesion on MRI usually would show good neural recovery. The spinal cord showing myelomalacia with reduced cord volume and dry lesion likely to show a poor neural recovery. The internal kyphectomy is indicated for paraplegia with healed disease. These cases are bad risk for surgery and neural recovery. The best form of treatment of late onset paraplegia is the prevention of development of severe kyphosis in initial active stage of disease.
118 citations
TL;DR: AQP4 antibody–positive patients with single or recurrent attacks of optic neuritis, myelitis, or brain/brainstem disease not fulfilling the current criteria of NMO or NMOSD may not be uncommon, and they should also be included in the NMO spectrum.
Abstract: Objectives: To analyze aquaporin-4 (AQP4) antibody–positive patients who do not fulfill the current diagnostic criteria of neuromyelitis optica (NMO) and NMO spectrum disorders (NMOSD). Methods: We used a cell-based assay (CBA) with AQP4-transfected cells to detect AQP4 antibody in 298 consecutive patients with inflammatory CNS disorders seen at Tohoku University Hospital from 2007 to 2012. The patients were diagnosed as NMO, NMOSD, multiple sclerosis, or others using the respective current diagnostic criteria. The seropositive samples by CBA were also tested using a commercial ELISA. Results: Seventy-two patients were AQP4 antibody positive. Among them, 18.1% (13/72) did not meet the NMO or NMOSD criteria (7 with monophasic optic neuritis, 2 with attacks restricted to the brainstem, and 4 with myelitis with less than 3 vertebral segments) and 84.6% (11/13) of these had only a single attack. The ELISA results were negative in 38.4% (5/13) of those patients, and they had lower antibody titers by CBA than patients with NMO/NMOSD. Although these patients had a shorter follow-up and few attacks, they shared some clinical features with NMO/NMOSD patients such as onset age, female predominance, presence of other autoantibodies, severe optic neuritis attacks, centrally located spinal cord lesions, persisting hiccups, and nausea or vomiting episodes. Conclusions: AQP4 antibody–positive patients with single or recurrent attacks of optic neuritis, myelitis, or brain/brainstem disease not fulfilling the current criteria of NMO or NMOSD may not be uncommon, and they should also be included in the NMO spectrum.
97 citations
TL;DR: NMO patients had frequent occurrence of brainstem lesions and LETM lesions and the outcome included relapses, fragmentation and atrophy, and correlation was observed between spinal cord atrophy and neurological disability.
Abstract: Neuromyelitis optica (NMO) includes transverse myelitis, optic neuritis and brain lesions. Recent studies have indicated that the brainstem is an important site of attack in NMO. Longitudinally extensive transverse myelitis (LETM) is an important component of the clinical diagnosis of NMO. The frequency of brainstem and LETM lesions, changes over time of LETM and the clinical consequences in the course of NMO have only been sparsely studied. The study was a population-based retrospective case series with clinical and magnetic resonance imaging (MRI) follow-up of 35 patients with definite NMO and a relapsing-remitting course. Brainstem lesions were observed in 25 patients, 18 in medulla oblongata (11 in area postrema). Lesions in the pons, mesencephalon and diencephalon occurred in 10, 7 and 7 patients, respectively. Lesions were symptomatic in medulla oblongata and pons, asymptomatic in mesencephalon and diencephalon. Brainstem lesions were observed significantly more often in anti-aquaporin-4 (AQP-4) antibody positive than in seronegative patients (p < 0.002). LETM was demonstrated by MRI of the spinal cord in 30/36 patients, 23/30 of whom had follow-up MRI of the spinal cord. Recurrent LETM was observed in five patients. In nine patients the LETM changed into multiple lesions during remission or treatment. Spinal cord atrophy was observed in 12/23 (52%) patients, correlating to Expanded Disability Status Scale (r = 0.88, p < 0.001). NMO patients had frequent occurrence of brainstem lesions and LETM. Brainstem lesions were associated with anti-AQP4 antibody positivity. LETM lesions differentiated over time and the outcome included relapses, fragmentation and atrophy. Correlation was observed between spinal cord atrophy and neurological disability.
83 citations
TL;DR: Diagnosis of VZV-induced neurological disease may require examination of cerebrospinal fluid (CSF), serum and/ or ocular fluids, and detection ofVZV IgG antibody in CSF is superior to detection of V zoster DNA inCSF to diagnose vasculopathy, recurrent myelopathy, and brainstem encephalitis.
Abstract: Varicella zoster virus (VZV) is an exclusively human neurotropic alphaherpesvirus Primary infection causes varicella (chickenpox), after which virus becomes latent in ganglionic neurons along the entire neuraxis With advancing age or immunosuppression, cell-mediated immunity to VZV declines and virus reactivates to cause zoster (shingles), which can occur anywhere on the body Skin lesions resolve within 1–2 weeks, while complete cessation of pain usually takes 4–6 weeks Zoster can be followed by chronic pain (postherpetic neuralgia), cranial nerve palsies, zoster paresis, meningoencephalitis, cerebellitis, myelopathy, multiple ocular disorders and vasculopathy that can mimic giant cell arteritis All of the neurological and ocular disorders listed above may also develop without rash Diagnosis of VZV-induced neurological disease may require examination of cerebrospinal fluid (CSF), serum and/ or ocular fluids In the absence of rash in a patient with neurological disease potentially due to VZV, CSF should be examined for VZV DNA by PCR and for anti-VZV IgG and IgM Detection of VZV IgG antibody in CSF is superior to detection of VZV DNA in CSF to diagnose vasculopathy, recurrent myelopathy, and brainstem encephalitis Oral antiviral drugs speed healing of rash and shorten acute pain Immunocompromised patients require intravenous acyclovir First-line treatments for post-herpetic neuralgia include tricyclic antidepressants, gabapentin, pregabalin, and topical lidocaine patches VZV vasculopathy, meningoencephalitis, and myelitis are all treated with intravenous acyclovir
76 citations
Journal Article•
TL;DR: A practical clinical approach to the diagnosis and acute management of patients with myelitis including clinical symptoms, the role of neuroimaging, and the utility of both CSF and serological studies in the management of these patients are discussed.
Abstract: Myelitis is a rare neurological disorder of the spinal cord that is caused by inflammation and can have devastating neurologic effects with up to two-thirds of patients having a moderate to severe degree of residual disability. Symptoms typically develop over hours or days and then worsen over a matter of days to weeks. Patients can present with sensory alteration, weakness, and autonomic dysfunction including bowel and bladder problems, temperature dysregulation, or even bouts of hypertension. Evaluation for compressive etiologies must be a priority as compressive myelopathy and transverse myelitis are often clinically indistinguishable and emergent surgical intervention is indicated in such cases. However, if neuroimaging and CSF studies indicate inflammation within the central nervous system, then a work-up for myelitis must include autoimmune, inflammatory, and infectious etiologies. Acute management of these patients is dictated by which etiology is suspected and rapid initiation of that treatment portends a more favorable patient outcome. This review will discuss a practical clinical approach to the diagnosis and acute management of patients with myelitis including clinical symptoms, the role of neuroimaging, and the utility of both CSF and serological studies in the management of these patients.
75 citations
TL;DR: CSF IL-6 and GFAP may be useful early diagnostic markers of NMOSD, according to data investigated in 13 NMO spectrum disorder patients at initial attacks and retrospectively.
75 citations
TL;DR: In this study, the in vitro T cell proliferation and the production of Th1 cytokines were significantly lower in cell cultures from NMO patients, as compared with healthy individuals and a dominant Th17-related response in N MO patients that was directly proportional to neurological disability is indicated.
Abstract: Neuromyelitis optica (NMO), also known as Devic's disease, is an autoimmune, inflammatory disorder of the central nervous system (CNS) in which the immune system attacks myelin of the neurons located at the optic nerves and spinal cord, thus producing a simultaneous or sequential optic neuritis and myelitis. The objective of this study was evaluated the background T-cell function of patients suffering from neuromyelitis optica (NMO), an autoimmune disorder of the central nervous system. In our study, the in vitro T cell proliferation and the production of Th1 cytokines were significantly lower in cell cultures from NMO patients, as compared with healthy individuals. In contrast, a dominant Th17-like phenotype, associate with higher IL-23 and IL-6 production by LPS-activated monocytes, was observed among NMO patients. The release of IL-21 and IL-6 by polyclonally activated CD4+ T cells was directly correlated to neurological disability. In addition, the in vitro release of IL-21, IL-6 and IL-17 was significantly more resistant to glucocorticoid inhibition in NMO patients. In conclusion, the results indicate dominant Th17-related response in NMO patients that was directly proportional to neurological disability. Furthermore, our results can help to explain why NMO patients trend to be more refractory to corticoid treatment.
67 citations
TL;DR: The category of PINSs should be revised: most of the clinical variants have a poor prognosis and are not readily classifiable on the basis of current knowledge.
Abstract: Objectives: Postinfectious neurologic syndromes (PINSs) of the CNS include heterogeneous disorders, sometimes relapsing. In this study, we aimed to a) describe the spectrum of PINSs; b) define predictors of outcome in PINSs; and c) assess the clinical/paraclinical features that help differentiate PINSs from multiple sclerosis (MS). Methods: In this prospective cohort study, adult inpatients with PINSs underwent extensive diagnostic assessment and therapeutic protocols at inclusion and during a minimum 2-year follow-up. We compared them with newly diagnosed, treatment-naive patients with MS, also prospectively recruited. Results: The study sample comprised 176 patients with PINSs aged 59.9 ± 17.25 years (range: 18–80 years) divided into 2 groups: group 1 (CNS syndromes, 64%)—encephalitis, encephalomyelitis, or myelitis; and group 2 (CNS + peripheral nervous system [PNS] syndromes, 36%)—encephalomyeloradiculoneuritis or myeloradiculoneuritis. We observed the patients for 24 to 170 months (median 69 months). Relapses, almost invariably involving the spinal cord, occurred in 30.5%. PNS involvement was an independent risk factor for relapses (hazard ratio 2.8). The outcome was poor in 43% of patients; risk factors included older age, greater neurologic disability at onset, higher serum-CSF albumin percentage transfer, myelitis, and PNS involvement. Steroid resistance occurred in 30% of the patients, half of whom responded favorably to IV immunoglobulins. Compared with MS, PINSs were characterized by older age, lower tendency to relapse, and distinct CSF findings. Conclusions: The category of PINSs should be revised: most of the clinical variants have a poor prognosis and are not readily classifiable on the basis of current knowledge. PNS involvement has a critical role in relapses, which seem to affect the spine only.
54 citations
01 Aug 2013
TL;DR: Clinical and radiologic findings combined with serologic findings may permit classification of syndromes of transverse myelitis and acute disseminated encephalomyelitis in ways that may predict risk of relapse, type of relapse and prognosis.
Abstract: Purpose of review This review defines current clinical criteria for diagnosis, differential diagnosis, and clinical evaluation of acute disseminated encephalomyelitis, transverse myelitis, and neuromyelitis optica, and summarizes principles of treatment. Recent findings Consensus criteria for transverse myelitis and acute disseminated encephalomyelitis have been proposed. A specific biomarker, aquaporin-4 autoantibody, has been discovered for neuromyelitis optica that allows for early and accurate diagnosis even in the absence of cardinal findings of optic neuritis and myelitis. The antibody is pathogenic and is facilitating an understanding of the pathophysiology of neuromyelitis optica and development of antigen-specific treatments. Summary Clinical and radiologic findings combined with serologic findings may permit classification of syndromes of transverse myelitis and acute disseminated encephalomyelitis in ways that may predict risk of relapse, type of relapse, and prognosis. Treatment, especially to prevent relapse, is dependent on the specific disease context in which syndromes such as transverse myelitis occur.
45 citations
TL;DR: Clinical and spinal cord neuroimaging clues that can help distinguishing AQP4-IgG+ LETM patients are provided.
Abstract: Longitudinally extensive transverse myelitis (LETM) is a characteristic feature of Neuromyelitis Optica (NMO), but it can also occur in several other inflammatory diseases of the central nervous system (CNS). An IgG autoantibody that binds to aquaporin-4 (AQP4), the predominant water channel of the CNS, is a reliable biomarker of the NMO spectrum disorders, and if detected predicts the recurrence of the myelitis. In this study, we compared the clinical and neuroimaging characteristics of AQP4-IgG+ and AQP4-IgG- LETM patients. Thirty-seven first-ever LETM patients were retrospectively evaluated and divided into two groups according to the presence of AQP4 autoantibodies. AQP4-IgG was detected in the serum and in the cerebrospinal fluid of sixteen patients. The female to male ratio was higher in AQP4-IgG+ patients. Intractable nausea and vomiting and paroxysmal tonic spasms often accompanied the LETM in AQP4-IgG+ patients. T2-weighted spinal cord MRI revealed that inflammatory lesions extending into the brainstem and involving the central grey matter occurred more frequently in AQP4-IgG+ LETM patients. Hypointense lesions on T1-weighted spinal cord MRI were detected more frequently in the seropositive group, and their presence correlated with attack severity. In conclusion, this study provides clinical and spinal cord neuroimaging clues that can help distinguishing AQP4-IgG+ LETM patients.
42 citations
TL;DR: The current study describes the presence of Leishmania amastigotes in nervous tissue inciting radiculoneuritis, myelitis, and mild meningoencephalitis, suggesting a likely route by which L. infantum amastIGotes reach and affect the central nervous system parenchyma.
Abstract: A 4-year-old male Labrador Retriever dog was presented with a 10-day history of tetraplegia, depression, and absent postural reflexes. The cerebrospinal fluid was positive for Leishmania spp. DNA. At necropsy, a 2-cm long mass was observed adhered to C(7) and C(8) left spinal nerves. Microscopically, nerve fiber destruction together with mixed inflammatory infiltration was observed in the spinal nerves. Cervical spinal cord sections showed multifocal, diffuse granulomatous inflammation in the white matter. In the brain, perivascular infiltrates were observed in some areas together with subtle pallor of the parenchyma. Immunohistochemistry for Leishmania infantum confirmed the presence of amastigotes in the spinal nerves, spinal cord, brain parenchyma, and choroid plexuses. The current study describes the presence of Leishmania amastigotes in nervous tissue inciting radiculoneuritis, myelitis, and mild meningoencephalitis, suggesting a likely route by which L. infantum amastigotes reach and affect the central nervous system parenchyma.
TL;DR: Functional recovery in IATM is poorer in patients with urinary sphincter dysfunction at admission or LETM on MRI, and functional recovery in patients who fulfilled the current TMCWG criteria for definite and possible IatM will convert to MS.
Abstract: In 2002, the Transverse Myelitis Consortium Working Group (TMCWG) proposed the diagnostic criteria for idiopathic acute transverse myelitis (IATM) to delimit and unify this group of patients. This study aimed to describe the conversion rate to multiple sclerosis (MS) and variables associated with conversion, and to analyze functional outcome and prognostic factors associated with functional recovery in patients who fulfilled the current TMCWG criteria for definite and possible IATM. Eighty-seven patients diagnosed with IATM between 1989 and 2011 were retrospectively reviewed. Two patients with positive neuromyelitis optica IgG serum antibodies were excluded. Epidemiological, clinical, laboratory, magnetic resonance imaging (MRI) data and outcome of 85 patients were analyzed. Eleven (13%) patients converted to MS after a median follow-up of 2.9 years (interquartile range 1.0-4.8). Early-age onset of symptoms was related to conversion to MS. Only 9.4% of patients with IATM were unable to walk unassisted at the end of follow-up. Urinary sphincter dysfunction (odds ratio [OR] 3.37, 95% confidence interval [CI] 1.04-10.92) and longitudinally extensive transverse myelitis (LETM) on MRI (OR 12.34, 95% CI 3.38-45.00) were associated with a poorer outcome (Rankin ≥ 2). At least 13% of patients who fulfill the TMCWG criteria for definite and possible IATM will convert to MS. Functional recovery in IATM is poorer in patients with urinary sphincter dysfunction at admission or LETM on MRI.
TL;DR: The immunophenotypic and clonal analysis of CSF cells might be helpful in the differential diagnosis between encephalitis and lymphoproliferative diseases.
Abstract: Herpesvirus infections of the central nervous system (CNS) are associated with encephalitis/myelitis and lymphoproliferative diseases in immunocompromised individuals. As of now, data of herpesvirus-associated CNS diseases in transplant recipients is limited. Hence, in this prospective study, we investigated the incidence of herpesvirus-associated CNS diseases and explored the diagnosis of these diseases in 281 allogeneic hematopoietic stem cell transplantation (allo-HSCT) recipients. Herpesvirus-DNA and cerebrospinal fluid (CSF) cells were sampled from 58 recipients with herpesvirus-associated diseases or with unexplainable CNS manifestations. Results showed that 23 patients were diagnosed as herpesvirus-associated CNS diseases, including 15 Epstein-Barr virus (EBV)-associated diseases (4 encephalitis and 11 lymphoproliferative diseases), 5 herpes simplex virus type 1 encephalitis, 2 cytomegalovirus encephalitis/myelitis and 1 varicella zoster virus encephalitis. The median time of diseases onset was 65 (range 22-542) days post-transplantation. The 3-year cumulative incidence of herpesvirus-associated encephalitis/myelitis and post-transplant lymphoproliferative disorder (PTLD) was 6.3% ±1.9% and 4.1% ±1.2%, respectively. Of the evaluable cases, CSF cells mainly consisted of CD19+CD20+ B cells (7/11) and had clonal rearrangement of immunoglobulin genes (3/11) in patients with CNS-PTLD. On the contrary, in patients with encephalitis/myelitis, CSF cells were comprised of different cell populations and none of the gene rearrangement was detected. Herpesvirus-associated CNS diseases are common in the early stages of allo-HSCT, wherein EBV is the most frequent causative virus. The immunophenotypic and clonal analysis of CSF cells might be helpful in the differential diagnosis between encephalitis and lymphoproliferative diseases.
TL;DR: A marked to moderate clinical improvement post-PE accompanied by a lack of radiologic resolution of the active lesion is not indicative of poor prognosis.
TL;DR: The AQP4-Ab assay can facilitate the early diagnosis of NMO and prevent limited NMO from being misdiagnosed as MS and probably detect the subclinical optic nerve involvement in subgroups of NMOSD.
Abstract: Objective:Our aim was to evaluate the utility of aquaporin-4 antibodies (AQP4-Ab) in patients with neuromyelitis optica spectrum disorders (NMOSD).Methods:The clinical and radiological characteristics of 78 patients with NMOSD and 22 with multiple sclerosis (MS), who were tested for AQP4-Ab by a cell-based assay, were assessed.Results:The mean time interval between symptom onset and development of optic neuritis and myelitis was 39.9 months in neuromyelitis optica (NMO). About 40% of patients with limited NMO would have fulfilled the diagnostic criteria for MS in the absence of the antibody assay results. In patients with longitudinally extensive transverse myelitis, positive AQP4-Ab assay results were associated with the poor response to acute steroid treatment and asymptomatic visual evoked potential abnormality. Presence of either painful tonic spasm associated with myelitis or severe disability at onset had high specificity and relatively high sensitivity in differentiating NMOSD with AQP4-Ab from MS....
TL;DR: A case of a 36-year-old female patient with a rare genetic disorder (ANE1: Acute Necrotizing Encephalopathy due to a RANBP2 mutation) who presented with an acute quadriplegia and was found to carry an autosomal-dominant missense mutation in the RAN BP2 gene predisposing for ANE is presented.
Abstract: The term "acute transverse myelitis (ATM)" comprises various non-traumatic disorders that eventually can be associated with a focal myelopathy. Patients characteristically present with an acutely occurring paraparesis/plegia and require a comprehensive and timely diagnostic work up for the initiation of an appropriate treatment. We present a case of a 36-year-old female patient with a rare genetic disorder (ANE1: Acute Necrotizing Encephalopathy due to a RANBP2 mutation) who presented with an acute quadriplegia. Following an acute pulmonal infection, she rapidly (< 24 h) developed a severe quadriplegia (total motor score 38) with some facial sensory symptoms (perioral hypoesthesia). Magnetic resonance imaging (MRI) revealed a combination of longitudinal extensive transverse myelitis and symmetrical thalamic lesions. A work-up for infectious and systemic diseases was negative; specifically, no findings related to multiple sclerosis, neuromyelitis optica or vascular disorders. After empirical high dose steroid treatment and rehabilitation therapy, the patient gained almost normal gait and upper limb function. She was found to carry an autosomal-dominant missense mutation in the RANBP2 gene predisposing for ANE. Gene segregation was confirmed in other family members that had been affected by other episodes of acute steroid-responsive encephalopathies. We propose that a redefined diagnostic workup of ATM might include ANE1, as the frequency of this rare disorder might be underestimated.
TL;DR: In patients with extensive myelitis who are seronegative for anti-AQP4 antibodies, and after other classical causes have been excluded, the hypothesis of atypical anti-NMDAR-Ab encephalitis should also be considered.
Abstract: Background
Encephalitis with anti-N-methyl-D-aspartate receptor antibodies (anti-NMDAR-Ab) is a rapid-onset encephalitis including psychosis, seizures, various movement disorders and autonomic system disturbances.
TL;DR: OCT appears to be a more sensitive test than VA or VF for monitoring ophthalmological function in NMO and it seems to be helpful for the detection of infra-clinical episodes in patients with a past history of optic neuritis.
Abstract: Background:Neuromyelitis optica (NMO) is an inflammatory disease associated with optic neuritis and myelitis. Recently, several studies showed that optical coherence tomography (OCT) could be an in...
TL;DR: A right-sided peripheral deafness due to a non-keratinizing type II nasopharyngeal carcinoma with infiltration of the tuba auditiva had been known for 15 years and was reported on a patient with NMO-IgG/AQP4-IGG-positive NMO and hearing loss.
Abstract: In 2004, Lennon et al. [7, 9] discovered a novel, pathogenic serum autoantibody reactivity [termed neuromyelitis optica (NMO)-IgG]. Aquaporin-4 (AQP4), the target antigen of NMO-IgG, is expressed in the central nervous system (CNS) as well as in numerous other tissues outside the CNS. Over the last few years an expanding spectrum of clinical syndromes associated with NMO-IgG/AQP4-IgG has been described, which includes, in addition to optic neuritis and myelitis, intractable vomiting and hiccups, oculomotor dysfunction (internuclear ophthalmoplegia, abducens nerve palsy, nystagmus), dysphagia, symptomatic narcolepsy, hyperthermia, central endocrinopathies, central hypotension, posterior reversible encephalopathy, and, in particular in children, a wide range of encephalitic manifestations including seizures [6, 11]. High levels of AQP4 are also expressed in the inner ear and the cochlear nerve, and, accordingly, deafness is a typical and consistent result of AQP4 loss as shown in transgenic mice [10, 12, 15]. Here we report on a patient with NMO-IgG/AQP4-IgG-positive NMO and hearing loss. The 51-year-old man with a 2-year history of NMO was transferred to the neurology department because of acute left-sided hearing loss; serological testing revealed antibodies to AQP4 [3, 5]. A right-sided peripheral deafness due to a non-keratinizing type II nasopharyngeal carcinoma with infiltration of the tuba auditiva had been known for 15 years. The patient had been under treatment with mycophenolate-mofetil (1,500 mg/day) for 15 months. At the ENT department, a peripheral cause was ruled out by audiometry. Physical, imaging [cranial magnetic resonance imaging (MRI), abdominal and thoracic computed tomography], blood and cerebrospinal fluid (FACS, paraneoplastic autoantibodies, neurotropic viruses) examinations did not reveal any signs of infection or tumor. Assuming a clinical attack in the context of NMO, the patient was treated with prednisolone (100 mg/day), after which he significantly improved within 10 days. Steroids were reduced to a maintenance dose of 10 mg while continuing mycophenolate-mofetil. At follow-up 4 weeks later, the patient reported left-sided hearing to be normal. This was confirmed by audiometry. AQP4 is expressed at high levels in the central part of the cochlear nerve as well as by supporting epithelial cells (Hensen’s, Claudius, and inner sulcus cells) in the organ of Corti [10, 15]. In the organ of Corti, AQP4 may facilitate rapid osmotic-driven water fluxes in the sensory epithelial cells, which are subject to large K? fluxes during mechano-electric signal transduction and thus contribute to the volume and ion-homeostasis at these sites [10]. It was shown that transgenic mice lacking AQP4 are regularly deaf, as demonstrated by auditory brain stem response measurement [10]. Presbycusis was shown to be characterized by a decrease in AQP4 expression in an animal model [2]. In patients with NMO, AQP4 loss has been demonstrated to occur early in lesion formation, to precede astrocyte loss and to be reversible in some lesions; [4, 13, 14] S. Jarius B. Wildemann Division of Molecular Neuroimmunology, Department of Neurology, University of Heidelberg, Heidelberg, Germany
TL;DR: A case of varicella-zoster virus (VZV) myelitis in a woman with relapsing-remitting multiple sclerosis receiving natalizumab, a humanized monoclonal antibody that induces an immunosuppression localized to the CNS is reported.
Abstract: We report a case of varicella-zoster virus (VZV) myelitis in a woman with relapsing-remitting multiple sclerosis (RRMS) receiving natalizumab, a humanized monoclonal antibody that induces an immunosuppression localized to the CNS.
TL;DR: A case of varicella-zoster virus (VZV) myelitis in a patient with MS treated with natalizumab (NTZ) is studied to stress another manifestation of VZV infection in NTZ-treated patients.
Abstract: Yeung et al.1 studied a case of varicella-zoster virus (VZV) myelitis in a patient with MS treated with natalizumab (NTZ). We wanted to stress another manifestation of VZV infection in NTZ-treated patients. A patient with MS recently came to …
TL;DR: Neuromyelitis optica spectrum disorder can initially present as meningoencephalitis mimicking intracranial infection, and the characteristic MRI imaging is quite critical for differentiation.
Abstract: Two patients presented with initial symptoms of headache and fever, and two weeks later had disturbance of consciousness. Cerebrospinal fluid (CSF) showed pleocytosis >500×106/L. Magnetic resonance imaging (MRI) showed multiple brain lesions at sites of high aquaporin-4 (AQP-4) expression. Case 1 presented optic neuritis four years after the first attack and case 2 had symptoms of myelitis three weeks after headache. Serum AQP-4 antibody was positive in both cases, and the diagnosis of neuromyelitis optica spectrum disorder (NMOSD) was made. Accordingly, NMOSD can initially present as meningoencephalitis mimicking intracranial infection, and the characteristic MRI imaging is quite critical for differentiation.
TL;DR: Autophagy caused by 125I radiation was mainly as an attempt of cell survival at an early stage, but it would be a self-destructive process and promoted the process of apoptosis and necrosis radiated by125I for more than 72 hours.
Abstract: Radiation myelitis is the most serious complication in clinical radiotherapy for spinal metastases. We previously showed that 125I brachytherapy induced apoptosis of spinal cord neurons accompanied by autophagy. In this study, we further investigated the mechanism by which 125I radiation triggered autophagy in neural cells. We found that autophagy induced by 125I radiation was involved in endoplasmic reticulum (ER) stress and mainly dependent on PERK-eIF2α pathway. The expressions of LC3II, ATG12 and PI3K were significantly suppressed in PERK knockout neural cells. Meanwhile, the expressions of phosphorylated-Akt s473 and caspase3/8 all significantly increased in neural cells transfected with a PERK siRNA and which enhanced apoptosis of neurons after 125I radiation. The results were consistent with that by MTT and Annexin-FITC/PT staining. In annimal model of banna pigs with radiation myelitis caused by 125I brachytherapy, we have successfully decreased PERK expression by intrathecal administration of the lentivirus vector. The apoptosis rate was significantly higher than that in control group and which deteriorated radiation myelitis of banna pigs. Thus, autophagy caused by 125I radiation was mainly as an attempt of cell survival at an early stage, but it would be a self-destructive process and promoted the process of apoptosis and necrosis radiated by 125I for more than 72 hours. The study would be useful and helpful to maximize efficiency of radiation therapy in clinical therapy.
TL;DR: A 12-year-old boy is described with acute transverse myelitis and Guillain–Barré syndrome associated with CSD, a benign, self-limiting condition associated with Bartonella henselae.
Abstract: Cat scratch disease (CSD) is a benign, self-limiting condition associated with Bartonella henselae. Neurological manifestations are uncommon. Acute transverse myelitis and Guillain–Barre syndrome have been reported rarely with CSD. This report describes a 12-year-old boy with acute transverse myelitis and Guillain–Barre syndrome associated with CSD.
TL;DR: In both cases, although the conventional single polymerase chain reaction (PCR) assays all showed negative results, the original nested PCR assay detected VZV DNA in the cerebrospinal fluid (CSF) specimen collected on admission and the evaluation of the antibody index to VzV had significant diagnostic value.
Abstract: Background: Myelitis is one of the rarest neurological complications of the varicella zoster virus (VZV) infection. Focal muscle weakness with or without sensory disturbance occurs in approximately 5% of the cases after acute VZV infection, with complete recovery in 50-70%. Case Presentation: This report describes two rare cases of elderly patients with VZV myelitis secondary to dermatomal zoster rash. Patient 1 was a 79-year-old woman who developed paraplegia, numbness and decreased sensation in the left arm and below thoracic (Th)-10 after sacral zoster. Spinal cord MRI showed a high-signal-intensity lesion at the cervical spinal nerve 2 on a T2-weighted image. Patient 2 was a 73-year-old man who developed right flaccid leg weakness and urinary retention after right dorsal Th 5-8 zoster. Spinal cord MRI showed a high-signal-intensity lesion at Th 3-4 on a T2-weighted image. In both cases, although the conventional single polymerase chain reaction (PCR) assays all showed negative results, the original nested PCR assay detected VZV DNA in the cerebrospinal fluid (CSF) specimen collected on admission. In addition, the anti-VZV IgG antibody by enzyme immunoassay and antibody index were elevated in the CSF specimens during the clinical courses of both patients. On the basis of these findings, both patients were diagnosed with VZV myelitis and were treated with high-dose acyclovir and corticosteroid. This combined treatment was appropriate and effective for the improvement of their functional outcomes. Conclusion: The detection of VZV DNA in CSF by nested PCR assay and the evaluation of the antibody index to VZV had significant diagnostic value.
TL;DR: This article reviews the features that should prompt consideration of diseases that mimic acquired demyelinating syndromes and multiple sclerosis using vignettes to highlight unusual clinical and radiologic features.
TL;DR: The spectrum of disease in vulnerable patient groups, such as transplant and chemotherapy recipients, patients infected with the human immunodeficiency virus, and international travelers, is discussed and clinical, epidemiologic, and initial management strategies for important spinal cord infections are discussed.
TL;DR: A case of MAC-related IRIS of the CNS is reported in an HIV-infected patient who presented with meningoencephalitis and myelitis 2 months after discontinuation of antiMAC therapy, when he had achieved prolonged suppression of HIV replication and restoration of CD4 counts to >100 cells/μL for 1 year.
Abstract: Disseminated Mycobacterium avium complex (MAC) infection involves the central nervous system (CNS) less frequently than tuberculosis, and MAC-related immune reconstitution inflammatory syndrome (IRIS) of the CNS in AIDS patients is even more rarely described. We report a case of MAC-related IRIS of the CNS in an HIV-infected patient who presented with meningoencephalitis and myelitis 2 months after discontinuation of antiMAC therapy, when he had achieved prolonged suppression of HIV replication and restoration of CD4 counts to >100 cells/μL for 1 year. Cases of MAC-related IRIS of the CNS reported in the literature are reviewed.
TL;DR: While rare, herpes simplex or herpes zoster reactivation is a complication of spine surgery that must be considered in the face of new-onset focal neurological symptoms in a dermatome pattern without a structural cause, even without a rash.
Abstract: Herpes simplex or herpes zoster reactivation after spinal surgery is rarely reported. This case report and review of the literature describes patients in whom this reactivation occurs to clarify the diagnosis and management. In addition to reporting their case, the authors reviewed case reports and series published between 1980 and 2012 found through a PubMed search. Herpes reactivation is generally confined to a vesicular rash that can be treated with acyclovir. However, occasional dissemination has occurred and has led to myelitis or encephalitis. Atypical presentations led to delays in diagnosis, delayed treatment, and poor neurological outcome. While rare, herpes simplex or herpes zoster reactivation is a complication of spine surgery that must be considered in the face of new-onset focal neurological symptoms in a dermatome pattern without a structural cause, even without a rash.