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1.  Antibody to Aquaporin 4 in the Diagnosis of Neuromyelitis Optica 
PLoS Medicine  2007;4(4):e133.
Background
Neuromyelitis optica (NMO) is a demyelinating disease of the central nervous system (CNS) of putative autoimmune aetiology. Early discrimination between multiple sclerosis (MS) and NMO is important, as optimum treatment for both diseases may differ considerably. Recently, using indirect immunofluorescence analysis, a new serum autoantibody (NMO-IgG) has been detected in NMO patients. The binding sites of this autoantibody were reported to colocalize with aquaporin 4 (AQP4) water channels. Thus we hypothesized that AQP4 antibodies in fact characterize NMO patients.
Methods and Findings
Based on these observations we cloned human water channel AQP4, expressed the protein in a eukaryotic transcription/translation system, and employed the recombinant AQP4 to establish a new radioimmunoprecipitation assay (RIPA). Indeed, application of this RIPA showed that antibodies against AQP4 exist in the majority of patients with NMO (n = 37; 21 positive) as well as in patients with isolated longitudinally extensive transverse myelitis (n = 6; six positive), corresponding to a sensitivity of 62.8% and a specificity of 98.3%. By contrast, AQP4 antibodies were virtually absent in 291 other participants, which included patients with MS (n = 144; four positive), patients with other inflammatory and noninflammatory neurological diseases (n = 73; one positive), patients with systemic autoimmune diseases (n = 45; 0 positive), and healthy participants (n = 29; 0 positive).
Conclusions
In the largest series reported so far to our knowledge, we quantified AQP4 antibodies in patients with NMO versus various other diseases, and showed that the aquaporin 4 water channel is a target antigen in a majority of patients with NMO. The newly developed assay represents a highly specific, observer-independent, and easily reproducible detection method facilitating clinically relevant discrimination between NMO, MS, and other inflammatory diseases.
A newly developed method to detect antibodies to the aquaporin 4 water channel can help discriminate between neuromyelitis optica, multiple sclerosis, and other inflammatory diseases.
Editors' Summary
Background.
Neuromyelitis optica (NMO or Devic syndrome) is a rare disease in which the immune system destroys the myelin (fatty material that insulates nerve fibers so that the body and the brain can communicate using electrical messages) in the optic nerve and spinal cord. Myelin destruction (demyelination) in these parts of the central nervous system (CNS) causes pain and swelling (inflammation) of the optic nerve (optic neuritis) and spinal cord (myelitis). The resultant disruption of communication along these nerves means that patients with NMO experience temporary or permanent blindness in one or both eyes that is preceded or followed by limb weakness or paralysis and loss of bladder and bowel control. These two sets of symptoms can occur many months apart and may happen once during a person's lifetime or recur at intervals. There is no cure for NMO, but corticosteroids or plasmapheresis reduce inflammation during acute attacks and, because NMO is an autoimmune disease (one in which the immune system attacks the body's own tissues instead of foreign organisms), long-term immunosuppression may prevent further attacks.
Why Was This Study Done?
There are many inflammatory/demyelinating diseases of the CNS with clinical symptoms similar to those of NMO. It is particularly hard to distinguish between NMO and multiple sclerosis, an autoimmune disease that involves widespread demyelination. Neurologists need to make a correct diagnosis before starting any treatment and usually use clinical examination and magnetic resonance imaging (to detect sites of inflammation) to help them in this task. Recently, however, a biomarker for NMO was identified. Many patients with NMO make autoantibodies (proteins that recognize a component of a person's own tissues) called NMO-IgGs. These recognize aquaporin 4 (AQP4), a protein that allows water to move through cell membranes. It is not known how often patients with NMO or other demyelinating diseases make antibodies to AQP4, so it is unclear whether testing for these antibodies would help in the diagnosis of NMO. In this study, the researchers have developed a new assay for antibodies to AQP4 and then quantified the antibodies in patients with NMO and other demyelinating diseases.
What Did the Researchers Do and Find?
The researchers made radioactively labeled AQP4 in a test tube, then incubated samples of this with serum (the liquid portion of blood), added small beads coated with protein A (a bacterial protein that binds to antibodies) and allowed the beads to settle. The amount of radioactivity attached to the beads indicates the amount of antibody to AQP4 in the original serum. The researchers used this radioimmunoprecipitation assay to measure antibodies to AQP4 in sera from 37 patients with NMO and from six with another neurological condition, longitudinally extensive transverse myelitis (LETM), which is characterized by large demyelinated lesions across the width of the spinal cord but no optic neuritis; these patients often develop NMO. They also measured antibodies to AQP4 in the sera of nearly 300 other people including patients with multiple sclerosis, other neurological conditions, various autoimmune diseases, and healthy individuals. Nearly two-thirds of the patients with NMO and all those with LETM made antibodies against AQP4; very few of the other study participants made these antibodies. In particular, only four of the 144 patients with multiple sclerosis made AQP4 antibodies.
What Do These Findings Mean?
These findings indicate that testing for antibodies to AQP4 could help neurologists distinguish between NMO and multiple sclerosis and between NMO and other demyelinating diseases of the CNS. In addition, the new radioimmunoprecipitation assay provides a standardized, high-throughput way to quantitatively test for these antibodies, whereas the indirect immune fluorescence assay for measurement of unspecific NMO-IgG is observer-dependent and nonquantitative. Although these findings need to be confirmed in more patients and the assay's reliability demonstrated in different settings, the measurement of antibodies to AQP4 by radioimmunoprecipitation may become a standard part of the differential diagnosis of NMO. Additional research will determine whether AQP4 is the only protein targeted by autoantibodies in NMO and whether this targeting is a critical part of the disease process.
Additional Information.
Please access these Web sites via the online version of this summary at http://dx.doi.org/10.1371/journal.pmed.0040133.
US National Institute of Neurological Disorders and Stroke has information for patients who have neuromyelitis optica, transverse myelitis, and multiple sclerosis
The Transverse Myelitis Association offers information and useful links for patients and their carers about transverse myelitis and neuromyelitis optica (in several languages, including English and Spanish)
Mayo Clinic information for patients on Devic's syndrome
Medline Plus encyclopedia pages discuss autoimmune disorders (in English and Spanish)
A brief overview of aquaporins is available from the University of Miami
The American MS Society has information on MS
doi:10.1371/journal.pmed.0040133
PMCID: PMC1852124  PMID: 17439296
2.  Intramedullary non-specific inflammatory lesion of thoracic spine: A case report 
Background
There are several non-neoplastic lesions which mimick intramedullary spinal cord neoplasm in their radiographic and clinical presentation. These can be classified as either infectious (TB, fungal, bacterial, parasytic, syphilis, CMV, HSV) and non-infectious (sarcoid, MS, myelitis, ADEM, SLE) inflammatory lesions, idiopathic necrotizing myelopathy, unusual vascular lesions and radiation myelopathy. Although biopsy may be indicated in many cases, an erroneous diagnosis of intramedullary neoplasm can often be eliminated pre-operatively.
Case description
the authors report a very rare case of intramedullary non-specific inflammatory lesion of unknown origin, without signs of infection or demyelinization, in a woman who showed no other evidence of systemic disease.
Conclusions
Intramedullary lesions that mimick a tumor can be various and difficult to interpret. Preoperative MRI does not allow a certain diagnosis because these lesions have a very similar signal intensity pattern. Specific tests for infective pathologies are useful for diagnosis, but histological examination is essential for establishing a certain diagnosis. In our case the final histological examination and the specific tests that we performed have not cleared our doubts regarding the nature of the lesion that remains controversial.
doi:10.1186/1477-7819-8-3
PMCID: PMC2817645  PMID: 20074378
3.  An Unusual Case of Subclinical Peripheral Neuropathy and Cervical Spondylosis in Atopic Myelitis 
Many cases of atopic myelitis have been reported in Japan; however very few were described in western countries. An 82-year-old woman with a past medical history of atopic dermatitis and asthma presented with progressive paresthesia (tingling) of both hands and tetraparesis. Before the onset of neurological symptoms, she complained of ichthyosis of both legs for 5 weeks. Magnetic resonance imaging demonstrated multisegmental degenerative arthritis, degenerative disc disease, and abnormal spinal cord signal intensity over several cervical segments, suggesting the diagnosis of myelitis. Total serum IgE level was elevated. Nerve conduction studies revealed asymmetric axonal sensorimotor neuropathy. The cerebrospinal fluid specimen showed lymphocytic pleocytosis and elevated protein level. Based on clinical, imaging, and laboratory findings, atopic myelitis was diagnosed. The diagnosis of atopic myelitis should be considered in myelopathy patients with history of atopy and elevated serum IgE levels.
doi:10.1155/2013/489451
PMCID: PMC3819895  PMID: 24251051
4.  Toxocariasis Might be an Important Cause of Atopic Myelitis in Korea 
Journal of Korean Medical Science  2009;24(6):1024-1030.
Atopic myelitis is defined as myelitis with atopic diasthesis but the cause is still unknown. Toxocariasis is one of the common causes of hyperIgEaemia that may lead to neurologic manifestations. The purpose of this study was to evaluate the sero-prevalence of Toxocara specific IgG Ab among the atopic myelitis patients. We evaluated the medical records of 37 patients with atopic myelitis whose conditions were diagnosed between March 2001 and August 2007. Among them, the 33 sera were analyzed for specific serum IgG Ab to Toxocara excretory-secretory antigens (TES). All of 37 patients had hyperIgEaemia. Specific IgE to D. pteronyssinus and D. farinae was detected in 22 (64.7%) and 34 (100%) patients, respectively, of the 34 patients. Thirty-one of 33 patients (93.9%) were found to be positive by TES IgG enzyme-linked immunosorbent assay (ELISA). Based on the image findings of eosinophilic infiltrations in the lung and liver, 8 patients had positive results. These results inferred that the prevalence of toxocariasis was high in patients with atopic myelitis. Our results suggest that toxocariasis might be an important cause of atopic myelitis and Toxocara ELISA is essential for evaluating the causes of atopic myelitis.
doi:10.3346/jkms.2009.24.6.1024
PMCID: PMC2775847  PMID: 19949655
Myelitis; Atopy; Toxocariasis
5.  Varicella Zoster Virus Myelitis in Two Elderly Patients: Diagnostic Value of Nested Polymerase Chain Reaction Assay and Antibody Index for Cerebrospinal Fluid Specimens 
Case Reports in Neurology  2013;5(1):81-90.
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.
doi:10.1159/000350714
PMCID: PMC3656678  PMID: 23687496
Varicella zoster virus; Myelitis; Enzyme immunoassay; Antibody index; Nested polymerase chain reaction assay
6.  Acute transverse myelitis caused by Coxsackie virus B4 infection: a case report. 
Journal of Korean Medical Science  1998;13(4):449-453.
Acute transverse myelitis is a rare clinical manifestation of Coxsackie virus infection which cause acute and progressive debilitating illness associated with loss of spinal cord function in the affected patients. A 62 year-old female developed symptoms of rapidly progressive paraplegia with sensory loss. On spinal MRI, T2 sagittal image showed increased signal intensity with cord swelling at T11-L2 level and 8 folds or greater rise of Coxsackie virus B4 neutralizing antibody titers was observed in the CSF. There is only one previous report of acute transverse myelitis caused by Coxsackie virus B4 infection to our knowledge. The presence of specific viral antibody titers change in the CSF and a corresponding spinal cord lesion are sufficient to suggest a causal relationship between the virus and the illness. This article is a case report of an unusual acute transverse myelitis caused by Coxsackie virus B4 infection.
PMCID: PMC3054412  PMID: 9741555
7.  Toxoplasmosis of Spinal Cord in Acquired Immunodeficiency Syndrome Patient Presenting as Paraparesis: A Rare Entity 
Although brain has been the most common site for toxoplasma infection in acquired immunodeficiency syndrome patients, involvement of spinal cord by toxoplasma has been rarely found. Spinal cord toxoplasmosis can present as acute onset weakness in both lower limbs associated with sensory and bladder dysfunction. A presumptive diagnosis can be made in patients with CD4 count <100/mm3 based on a positive serum Toxoplasma gondii IgG antibodies, no recent prophylaxis against toxoplasmosis, intramedullary ring enhancing lesion in spinal cord supported by similar lesions in brain parenchyma. Institutions of antitoxoplasma treatment in such patients result in prompt clinical response and therefore avoiding the need of unnecessary invasive diagnostic tests. Here, we report a case of toxoplasmic myelitis in immunocompromised patient presenting as myelopathy who showed significant clinical improvement after starting antitoxoplasma treatment. Hence toxoplasmic myelitis should be considered in toxoplasma seropositive immunocompromised patients presenting as myelopathy and imaging studies showing ring enhancing intramedullary lesion.
doi:10.4103/0974-777X.145248
PMCID: PMC4265833  PMID: 25538456
Acquired immunodeficiency syndrome; Immunocompromised; Paraparesis; Spinal toxoplasmosis
8.  Acute myelitis with hyperIgEaemia and mite antigen specific IgE: atopic myelitis 
An occurrence of acute localised myelitis was recently seen in four adult patients with atopic dermatitis who had hyperIgEaemia and mite antigen specific IgE. The total and mite antigen specific IgE was therefore studied in serum samples from 19 consecutive patients with acute localised myelitis of unknown aetiology, 56patients with clinically definite multiple sclerosis, and 40 healthy controls. The total IgE concentration was significantly higher in acute localised myelitis (median=360 U/ml) than in multiple sclerosis (median=52 U/ml, p<0.0001) and the controls (median=85 U/ml, p=0.0002). The specific IgE to Dermatophagoides pteronyssinus was found more often in patients with acute localised myelitis (95%) than in patients with multiple sclerosis (34%, p<0.0001) and the controls (35%, p<0.0001) and the specific IgE to Dermatophagoides farinae was similar (acute localised myelitis 79%, multiple sclerosis 29% (p<0.0001), controls 30%, (p=0.0003). Atopic dermatitis coexisted more commonly in patients with acute localised myelitis (37%) than in patients with multiple sclerosis (0%, p<0.0001) and the controls (7.5%, p=0.0089). Therefore, acute localised myelitis with hyperIgEaemia, in which atopy to mite antigens seems to exist, may be a distinct subtype of allergic myelitis—that is, atopic myelitis.


PMCID: PMC2170101  PMID: 9598690
9.  A confounding coincidence: epidural anesthesia and paraplegia due to intramedullary tuberculoma in a patient who underwent cholecystectomy 
BMC Anesthesiology  2014;14(1):100.
Background
Paraplegia associated with epidural anesthesia or caused by intramedullary spinal tuberculoma is rare but catastrophic. We present a case of paraplegia following epidural anesthesia in a patient with an undiagnosed intramedullary spinal tuberculoma.
Case presentation
A 42-year-old man developed paraplegia after an open cholecystectomy under epidural anesthesia. Spinal cord infarction, acute transverse myelitis, and intramedullary neoplasms were ruled out by histopathologic examination, and intramedullary spinal tuberculoma at the T6–T7 level was identified. Despite surgical treatment and subsequent antituberculous therapy, the patient retained some disability attributable to the delay in diagnosis.
Conclusion
Physicians should be aware of coexisting disease as a cause of paraplegia following procedures using epidural anesthesia. Magnetic resonance imaging is the most sensitive diagnostic test, although it is still difficult to differentiate spinal cord infarction, myelitis, intramedullary spinal tuberculoma, and neoplasms from imaging features alone.
doi:10.1186/1471-2253-14-100
PMCID: PMC4237767  PMID: 25414594
Epidural anesthesia; Intramedullary spinal tuberculoma; Paraplegia
10.  Subacute transverse myelitis with Lyme profile dissociation 
Introduction: Transverse myelitis is a very rare neurologic syndrome with an incidence per year of 1-5 per million population. We are presenting an interesting case of subacute transverse myelitis with its MRI (magnetic resonance imaging) and CSF (cerebrospinal fluid) findings.
Case: A 46-year-old African-American woman presented with decreased sensation in the lower extremities which started three weeks ago when she had a 36-hour episode of sore throat. She reported numbness up to the level just below the breasts. Lyme disease antibodies total IgG (immunoglobulin G) and IgM (immunoglobulin M) in the blood was positive. Antinuclear antibody profile was within normal limits. MRI of the cervical spine showed swelling in the lower cervical cord with contrast enhancement. Cerebrospinal fluid was clear with negative Borrelia Burgdorferi IgG and IgM. Herpes simplex, mycoplasma, coxiella, anaplasma, cryptococcus and hepatitis B were all negative. No oligoclonal bands were detected. Quick improvement ensued after she was given IV Ceftriaxone for 7 days. The patient was discharged on the 8th day in stable condition. She continued on doxycycline for 21 days.
Conclusions: Transverse myelitis should be included in the differential diagnosis of any patient presenting with acute or subacute myelopathy in association with localized contrast enhancement in the spinal cord especially if flu-like prodromal symptoms were reported. Lyme disease serology is indicated in patients with neurological symptoms keeping in mind that dissociation in Lyme antibody titers between the blood and the CSF is possible.
PMCID: PMC2703261  PMID: 19675732
transverse myelitis; Lyme disease
11.  Radiation myelitis after hypofractionated radiotherapy with concomitant gefitinib 
We describe the case of a 71-year-old Caucasian female with primary disseminated non-small cell cancer of the lung, presented for palliative radiotherapy of metastatic spread to the 9th and 11th thoracic vertebrae without intramedullary growth. Palliative radiotherapy with daily fractions of 3 Gy and a cumulative dose of 36 Gy to thoracic vertebrae 8-12 was performed. The patient received concomitantly 250 mg gefitinib daily. After a latent period of 16 months, the patient developed symptoms of myelitis. Magnetic resonance imaging (MRI) did not reveal any bony or intraspinal tumor progression, but spinal cord signal alteration. No response to steroids was achieved. The neurological symptoms were progressive in August 2013 with the right leg being completely plegic. The left leg was incompletely paralyzed. Deep and superficial sensitivity was also diminished bilaterally. The patient was completely urinary and anally incontinent. Contrary to the clinical findings, a follow-up MRI (July 2013) showed amelioration of the former signal alterations in the spinal cord. The diagnosis of paraneoplastic myelopathy was refuted by a negative test for autologous antibodies. At the last clinical visit in May 2014, the neurological symptoms were stable. The last tumor-specific treatment the patient is receiving is erlotinib 125 mg/d.
We reviewed the literature and found no reported cases of radiation myelopathy after the treatment in such a setting. The calculated probability of such complication after radiotherapy alone is statistically measurable at the level of 0.02%. We suppose that gefitinib could also play a role in the development of this rare complication.
doi:10.1186/s13014-015-0334-7
PMCID: PMC4313465  PMID: 25631068
Radiation myelitis; Concomitant radiotherapy; Gefitinib
12.  IDIOPATHIC ACUTE TRANSVERSE MYELITIS IN CHILDREN: An analysis and discussion of MRI findings 
Background
There is lack of reported MRI studies of idiopathic acute transverse myelitis in children.
Objective
To describe the imaging features of idiopathic acute transverse myelitis in children..
Methods
We retrospectively analyzed the spinal MRI findings of children diagnosed with acute transverse myelitis . The anatomic regions, vertebral segmental length, gray or white matter involvement, cord expansion and gadolinium enhancement were examined.
Results
27 children were diagnosed with isolated monophasic acute transverse myelitis with a mean follow-up of 5.2 years. Two children later diagnosed with neuromyelitis optica were excluded from the pediatric ATM cohort. None of the patients had a subsequent diagnosis of multiple sclerosis. The mean age of onset was 9.5 years (0.5-16.9). Spinal MRIs were abnormal in 21 (78%). The mean interval between symptom onset and the MRI was 1.7 days (0-19 days). Central cord hyperintensity involving gray matter was seen in all patients. A majority (67%) of the patients demonstrated long segment lesions with a mean segment length of 6.4.
Conclusions
Central cord inflammation extending over three or more segments is the most common finding of idiopathic monophasic transverse myelitis in children. The risk of multiple sclerosis in children who experience isolated transverse myelitis as a first demyelinating event is low.
doi:10.1177/1352458510381393
PMCID: PMC3022414  PMID: 20858691
Acute transverse myelitis; MRI; demyelinating disease; pediatric onset multiple sclerosis; spinal cord inflammation
13.  Epstein-Barr virus myelitis and Castleman's disease in a patient with acquired immune deficiency syndrome: a case report 
Introduction
Few cases of Epstein-Barr virus myelitis have been described in the literature. Multi-centric Castleman's disease is a lymphoproliferative disorder that is well known for its associations with the human immunodeficiency virus, human herpes virus 8, and Kaposi's sarcoma. The concurrent presentation of these two diseases in a patient at the same time is extremely unusual.
Case Presentation
We describe the case of a 43-year-old Caucasian man with acquired immune deficiency syndrome who presented with fever, weight loss and diffuse lymphadenopathy, and was diagnosed with multi-centric Castleman's disease. He presented three weeks later with lower extremity weakness and urinary retention, at which time cerebrospinal fluid contained lymphocytic pleocytosis and elevated protein. Magnetic resonance imaging demonstrated abnormal spinal cord signal intensity over several cervical and thoracic segments, suggesting the diagnosis of myelitis. Our patient was ultimately diagnosed with Epstein-Barr virus myelitis, as Epstein-Barr virus DNA was detected by polymerase chain reaction in the cerebrospinal fluid.
Conclusion
To the best of our knowledge, this is the first case of multi-centric Castleman's disease followed by acute Epstein-Barr virus myelitis in a human immunodeficiency virus-infected patient. Clinicians caring for human immunodeficiency virus-infected patients should be vigilant about monitoring patients with increasing lymphadenopathy, prompting thorough diagnostic investigations when necessary.
doi:10.1186/1752-1947-5-209
PMCID: PMC3128854  PMID: 21615962
14.  Clinically Isolated Syndromes Suggestive of Multiple Sclerosis: An Optical Coherence Tomography Study 
PLoS ONE  2012;7(3):e33907.
Background
Optical coherence tomography (OCT) is a simple, high-resolution technique to quantify the thickness of retinal nerve fiber layer (RNFL), which provides an indirect measurement of axonal damage in multiple sclerosis (MS). This study aimed to evaluate RNFL thickness in patients at presentation with clinically isolated syndromes (CIS) suggestive of MS.
Methodology
This was a cross-sectional study. Twenty-four patients with CIS suggestive of MS (8 optic neuritis [ON], 6 spinal cord syndromes, 5 brainstem symptoms and 5 with sensory and other syndromes) were prospectively studied. The main outcome evaluated was RNFL thickness at CIS onset. Secondary objectives were to study the relationship between RNFL thickness and MRI criteria for disease dissemination in space (DIS) as well as the presence of oligoclonal bands in the cerebrospinal fluid.
Principal Findings
Thirteen patients had decreased RNFL thickness in at least one quadrant. Mean RNFL thickness was 101.67±10.72 µm in retrobulbar ON eyes and 96.93±10.54 in unaffected eyes. Three of the 6 patients with myelitis had at least one abnormal quadrant in one of the two eyes. Eight CIS patients fulfilled DIS MRI criteria. The presence of at least one quadrant of an optic nerve with a RNFL thickness at a P<5% cut-off value had a sensitivity of 75% and a specificity of 56% for predicting DIS MRI.
Conclusions
The findings from this study show that axonal damage measured by OCT is present in any type of CIS; even in myelitis forms, not only in ON as seen up to now. OCT can detect axonal damage in very early stages of disease and seems to have high sensitivity and moderate specificity for predicting DIS MRI. Studies with prospective long-term follow-up would be needed to establish the prognostic value of baseline OCT findings.
doi:10.1371/journal.pone.0033907
PMCID: PMC3309007  PMID: 22448279
15.  Transverse myelitis secondary to Melioidosis; A case report 
BMC Infectious Diseases  2012;12:232.
Background
Melioidosis has become an emerging infection in Sri Lanka; a country which is considered non endemic for it. Paraplegia due to Burkholderia pseudomallei is a very rare entity encountered even in countries where the disease is endemic. There are no reported cases of transverse myelitis due to melioidosis in Sri Lankan population thus we report the first case.
Case presentation
A 21 year old farmer presented with sudden onset bi lateral lower limb weakness, numbness and urine retention. Examination revealed flaccid areflexic lower limbs with a sensory loss of all modalities and a sensory level at T10 together with sphincter involvement. MRI of the thoracolumbar spine showed extensive myelitis of the thoracic spine complicating left psoas abscess without definite extension to the spinal cord or cord compression. Burkholderia pseudomallei was isolated from the psoas abscess pus cultures and the diagnosis of melioidosis was confirmed with high titers of Burkholderia pseudomallei antibodies and positive PCR. He was treated with high doses of IV ceftazidime and oral cotrimoxazole for one month with a plan to continue cotrimoxazole and doxycycline till one year. Patient’s general condition improved but the residual neurological problems persisted.
Conclusion
The exact pathogenesis of spinal cord melioidosis is not quite certain except in the cases where there is direct microbial invasion, which does not appear to be the case in our patient. We postulate our patient’s presentation could be due to ischemia of the spinal cord following septic embolisation or thrombosis of spinal artery due to the abscess nearby. A neurotrophic exotoxin causing myelitis or post infectious immunological demyelination is yet another possibility. This emphasizes the necessity of further studies to elucidate the exact pathogenesis in this type of presentations.
Health care professionals in Sri Lanka, where this is an emerging infection, need to improve their knowledge regarding this disease and should have high degree of suspicion to make a correct and a timely diagnosis to reduce the morbidity and mortality due to Burkholderia pseudomallei infection. It is highly likely that this infection is under diagnosed in developing countries where diagnostic facilities are minimal. Therefore strategies to improve the awareness and upgrade the diagnostic facilities need to be implemented in near future.
doi:10.1186/1471-2334-12-232
PMCID: PMC3511196  PMID: 23020820
Melioidosis; Transverse myelitis; Burkholderia pseudomallei; Flaccid paraplegia; Psoas abscess
16.  Patient with neuromyelitis optica and inflammatory demyelinating lesions comprising whole spinal cord from C2 level till conus: case report 
BMC Neurology  2009;9:56.
Background
Neuromyelitis optica (NMO) is an idiopathic, severe, inflammatory demyelinating disease of the central nervous system, that causes severe optic neuritis and myelitis attacks. Early discrimination between multiple sclerosis (MS) and NMO is important, as optimum treatment for both diseases may differ considerably.
Case Presentation
We report a case of a patient who initially presented as longitudinally extensive transverse myelitis (LETM), having spastic upper extremities diparesis and spastic paraplegia, C2/C3 sensory level and urinary incontinence, as well as extensive inflammatory spinal cord lesions from C2 level to conus. After 5 months the patient had another attack of transverse myelitis, had electrophysiological findings consistent with optic neuritis, was seropositive for NMO-IgG (aquaporin-4 IgG) and thus fulfilled NMO diagnostic criteria. Following treatment of disease attacks with pulse corticosteroid therapy and intravenous immunoglobulins, we included oral azathioprine in a combination with oral prednisone in the therapy. Since there was no significant clinical improvement, we decided to use cyclophosphamide therapy, which resulted in good clinical improvement and gradual decrease of cord swelling.
Conclusion
In this NMO case report we wanted to emphasize the extensiveness of inflammatory spinal cord changes in our patient, from C2 level to conus. In the conclusion it is important to say that accurate, early diagnosis and distinction from MS is critical to facilitate initiation of immunosuppressive therapy for attack prevention.
doi:10.1186/1471-2377-9-56
PMCID: PMC2773232  PMID: 19852774
17.  The Frequency of Anti-Aquaporin-4 Ig G Antibody in Neuromyelitis Optica and Its Spectrum Disorders at a Single Tertiary Referral Center in Malaysia 
Background. In the past the occurrence of neuromyelitis optica in Malaysia was thought to be uncommon and the frequency of anti-aquaporin-4 Ig G antibody was unknown. Objective. To evaluate the frequency of anti-aquaporin-4 Ig G antibody (Anti-AQP4 antibody) amongst patients with neuromyelitis optica (NMO) and its spectrum disorders (NMOSD) and the differences between the seropositive and seronegative groups. Methods. Retrospectively, 96 patients with NMO/high risk syndromes for NMOSD (HRS-NMOSD) were identified out of 266 patients with idiopathic inflammatory demyelinating disease from a single center hospital based registry. Anti-AQP4 seropositivity was found in 38/48 (79.2%) with NMO, 12/21 (57.1%) with brain involvement at high risk for NMOSD, 12/15 (80%) with transverse myelitis (i.e., 11/15 with relapsing transverse myelitis and one with monophasic transverse myelitis), and 3/7 (42.8%) with relapsing optic neuritis. Sixty-five out of 96 patients, that is, 67.7%, with NMO/HRS for NMOSD were seropositive. Seropositivity was significantly associated with female gender, a higher number of mean relapses, that is, 5.15 ± 4.42 versus 2.10 ± 1.68, longer length of spinal cord lesions, that is, 6.6 ± 4.9 versus 2.9 ± 2.5, vertebral bodies, higher EDSS, 4.5 ± 2.4 versus 2.4 ± 2.6, presence of paroxysmal tonic spasms, and blindness (unilateral/bilateral); P < 0.001. Longitudinally extensive cord lesions (contiguous or linear), presence of lesions in the cervical and thoracic regions, and involvement of the central gray matter or holocord regions on axial scans, were also significantly associated with seropositivity; P < 0.001. Conclusion. NMO and HRS for NMOSD are present in larger numbers than previously thought in Malaysia. More than 2/3rds are seropositive. Seropositive and seronegative NMO/NMOSD have differences that are useful in clinical practice.
doi:10.1155/2014/568254
PMCID: PMC4274866  PMID: 25548676
18.  Contrasting disease patterns in seropositive and seronegative neuromyelitis optica: A multicentre study of 175 patients 
Background
The diagnostic and pathophysiological relevance of antibodies to aquaporin-4 (AQP4-Ab) in patients with neuromyelitis optica spectrum disorders (NMOSD) has been intensively studied. However, little is known so far about the clinical impact of AQP4-Ab seropositivity.
Objective
To analyse systematically the clinical and paraclinical features associated with NMO spectrum disorders in Caucasians in a stratified fashion according to the patients' AQP4-Ab serostatus.
Methods
Retrospective study of 175 Caucasian patients (AQP4-Ab positive in 78.3%).
Results
Seropositive patients were found to be predominantly female (p < 0.0003), to more often have signs of co-existing autoimmunity (p < 0.00001), and to experience more severe clinical attacks. A visual acuity of ≤ 0.1 during acute optic neuritis (ON) attacks was more frequent among seropositives (p < 0.002). Similarly, motor symptoms were more common in seropositive patients, the median Medical Research Council scale (MRC) grade worse, and MRC grades ≤ 2 more frequent, in particular if patients met the 2006 revised criteria (p < 0.005, p < 0.006 and p < 0.01, respectively), the total spinal cord lesion load was higher (p < 0.006), and lesions ≥ 6 vertebral segments as well as entire spinal cord involvement more frequent (p < 0.003 and p < 0.043). By contrast, bilateral ON at onset was more common in seronegatives (p < 0.007), as was simultaneous ON and myelitis (p < 0.001); accordingly, the time to diagnosis of NMO was shorter in the seronegative group (p < 0.029). The course of disease was more often monophasic in seronegatives (p < 0.008). Seropositives and seronegatives did not differ significantly with regard to age at onset, time to relapse, annualized relapse rates, outcome from relapse (complete, partial, no recovery), annualized EDSS increase, mortality rate, supratentorial brain lesions, brainstem lesions, history of carcinoma, frequency of preceding infections, oligoclonal bands, or CSF pleocytosis. Both the time to relapse and the time to diagnosis was longer if the disease started with ON (p < 0.002 and p < 0.013). Motor symptoms or tetraparesis at first myelitis and > 1 myelitis attacks in the first year were identified as possible predictors of a worse outcome.
Conclusion
This study provides an overview of the clinical and paraclinical features of NMOSD in Caucasians and demonstrates a number of distinct disease characteristics in seropositive and seronegative patients.
doi:10.1186/1742-2094-9-14
PMCID: PMC3283476  PMID: 22260418
Neuromyelitis optica; Devic disease; Devic syndrome; longitudinally extensive transverse myelitis; recurrent optic neuritis; NMO-IgG; aquaporin-4 (AQP4) antibody; epidemiology; clinical features; magnetic resonance imaging; cerebrospinal fluid
19.  Tuberculosis of spine: neurological deficit 
European Spine Journal  2012;22(Suppl 4):624-633.
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.
doi:10.1007/s00586-012-2335-7
PMCID: PMC3691413  PMID: 22565802
Tuberculosis of spine; Neurological complication; Early onset paraplegia; Late onset paraplegia
20.  Medical Management for Intractable Pain Arising From Primary Sjögren Syndrome Involving Both Brain and Spinal Cord: A Case Report 
Annals of Rehabilitation Medicine  2014;38(4):568-574.
Primary Sjögren syndrome, which involves lesions in both the brain and spinal cord, is rarely reported. Related symptoms, such as intractable pain due to central nervous system involvement, are very rare. A 73-year-old woman diagnosed with primary Sjögren syndrome manifested with subacute encephalopathy and extensive transverse myelitis. She complained of severe whole body neuropathic pain. Magnetic resonance imaging demonstrated a non-enhancing ill-defined high intensity signal involving the posterior limb of the both internal capsule and right thalamus on a T2 fluid-attenuated inversion recovery image. Additionally, multifocal intramedullary ill-defined contrast-enhancing lesion with cord swelling from the C-spine to L-spine was also visible on the T2-weighted image. Her intractable pain remarkably improved after administration of concomitant oral doses of gabapentin, venlafaxine, and carbamazepine.
doi:10.5535/arm.2014.38.4.568
PMCID: PMC4163599  PMID: 25229038
Sjögren's syndrome; Extensive transverse myelitis; Neuropathic pain
21.  Schistosomal myeloradiculopathy due to Schistosoma mansoni: Report on 17 cases from an endemic area 
Background:
After malaria, schistosomiasis is the second most prevalent tropical disease. The prevalence of oviposition in CNS of infected persons varies from 0.3 to 30%. The conus medullaris is a primary site of schistosomiasis, either granulomatous or acute necrotizing myelitis.
Objective:
To report the clinical, radiological, and laboratory results of spinal cord schistosomiasis (SCS) and to design proper therapeutic regimens.
Materials and Methods:
Seventeen patients (13 males and four females) with SCS were enrolled between 1994 and 2009 at Mansoura University Hospitals. Their median age at diagnosis was 19 years (13-30 years). Independent neurological, radiological, and laboratory assessments were performed for both groups, excluding pathological confirmation that was done earlier in eight patients (Group 1). In the group 2 (nine patients), indirect hemagglutination (IHA) test for bilharziasis in blood and cerebrospinal fluid (CSF) was performed. Higher positive titer in CSF than serum indicated SCS plus induction of antibilharzial and corticosteroid protocols for 12 months with a three-year follow-up.
Results:
Rate of neurological symptoms of granulomatous intramedullary cord lesion was assessed independently in 16 cases and acute paraparesis in one case. All patients in group 2 had positive IHA against Schistosoma mansoni with median CSF and serum ranges 1/640 and 1/320, respectively. Seven patients (41.18%) had complete recovery, eight patients (47.06%) showed partial recovery, and no response was reported in two patients (11.76%) (P = 0.005). There was no recorded mortality in the current registry.
Conclusions:
Rapid diagnosis of SCS with early medical therapies for 12 months is a crucial tool to complete recovery.
doi:10.4103/0972-2327.82796
PMCID: PMC3141472  PMID: 21808472
Myelopathy; radiculopathy; Schistosoma mansoni
22.  Medullary schistosomiasis 
Background:
Schistosomal infestation of the central nervous system is a rare cause of cord compression, although a predominant one in endemic areas.
Case Description:
A 38-year-old male, native of Ivory Coast, with a history of 1 month of progressive paraparesis, neurogenic bladder, diminished deep tendon reflexes of the lower limbs, and sensory level. The magnetic resonance imaging (MRI) showed a medullary lesion at D4-D5 level, suggestive of an intramedullary tumor. Laminotomy of D3 to D5 and excision of a grayish white lesion according to a preliminary histopathologic review suggestive of a high grade glioma. Definitive histopathology review established the diagnosis of medullary schistosomiasis.
Conclusion:
Schistosomal myeloradiculopathy should be considered in patients presenting with cord compression or features of transverse myelitis, especially in patients from endemic areas or low social economic settlements.
doi:10.4103/2152-7806.132235
PMCID: PMC4078447  PMID: 24991469
Differential diagnosis; medullary schistosomiasis; outcome
23.  Fulminant holocord intramedullary tubercular abscess with enigmatic presentation 
Background:
Intramedullary and subarachnoidal tubercular abscesses are rare forms of spinal tuberculosis as compared with extradural collections secondary to vertebral tuberculosis.
Case Description:
We herein present a 33-year-old, apparently healthy male patient who presented clinically as transverse myelitis, with a lesion at detected at conus cauda, developing fulminant holocord intramedullary tubercular abscess, treated with surgical evacuation and much later with anti-tubercular drugs. Atypical clinical, serological, imaging findings in addition to lack of knowledge of occurrence of fulminant intramedullary tuberculosis led to the delay in starting anti-tubercular treatment.
Conclusion:
Early diagnosis requires a high index of suspicion, search for a primary focus of tubercular infection, investigation with magnetic resonance imaging (MRI) of spinal cord, biopsy, and confirmation with microscopy and culture, even in immunocompetent individuals. Early diagnosis, prompt treatment with surgical evacuation of abscess, and anti-tubercular drugs can lead to a good neurological recovery.
doi:10.4103/2152-7806.109506
PMCID: PMC3622373  PMID: 23607054
Filum terminale; intramedullary; spinal tuberculosis; subarachnoidal; tubercular abscess
24.  Differentiation of localization of spinal hemangioblastomas based on imaging and pathological findings 
European Spine Journal  2011;20(8):1377-1384.
Intramedullary + extramedullary hemangioblastomas with largely extramedullary growth are rare and often incorrectly assigned as intradural-extramedullary tumors preoperatively. Preoperative evaluation of the precise tumor location is important for total resection of tumor and improving the surgical outcome. The aim of this study was to provide the first identification of the key differences among of preoperative MR images of hemangioblastomas in different locations and to correlate these with pathological findings. The subjects were 26 patients with surgery for spinal hemangioblastoma in our department, including 6 with an intramedullary tumor who were complicated with von Hippel Lindau disease. Intramedullary, intramedullary + extramedullary, and intradural-extramedullary tumors were present in 22, 3 and 1 cases, respectively. Sagittal MR images showed that intramedullary and intramedullary + extramedullary tumors gave intramedullary T2 high intensity areas (HIAs) spreading toward the craniocaudal sides of the tumor, whereas such findings were absent for the intradural-extramedullary tumor. All the tumors showed strong contrast on axial images, with focal enhancement of hemangioblastomas limited to the intramedullary region (focal type); smooth boundary lines between the spinal cord and the extramedullary tumor (smooth type); and a snowman sign for intramedullary + extramedullary tumors, which provided a key characteristic for differentiating intramedullary + extramedullary tumors from those limited to the extramedullary region. In pathological findings, the Ki67 activity was less than 1% for intramedullary and intradural-extramedullary tumors, but 18–25% in all cases with an intramedullary + extramedullary tumor. In conclusion, on preoperative MRI, a change in the intramedullary HIAs spreading the craniocaudal sides of the tumor on sagittal T2 weighted image (T2WI) and a snowman sign on contrast axial T1WI may be important for differentiation among spinal hemangioblastomas in different locations. Pathologically, we found that intramedullary + extramedullary hemangioblastoma has high cell proliferative activity, which may suggest that enlargement of this tumor occurs faster than that of intramedullary hemangioblastoma.
doi:10.1007/s00586-011-1814-6
PMCID: PMC3175843  PMID: 21528401
Spinal hemangioblastoma; Exophytic intramedullary hemangioblastoma; Differentiation of localization; MRI findings; Pathological findings
25.  Febrile "migrating" eosinophilic cellulitis with hepatosplenomegaly: adult toxocariasis – a case report 
Cases Journal  2008;1:356.
Background
Eosinophilic cellulitis (Wells' syndrome) is a polyetiologic clinical entity with still obscure pathogenesis. Clinically overt toxocariasis is uncommon in adults, yet helminthozoonoses, including toxocariasis have been occasionally implicated in the pathogenesis of eosinophilic cellulitis.
Case representation
A 55-year-old female patient presented with a skin biopsy verified recurring febrile eosinophilic cellulitis, blood eosinophilia (42%), slight anaemia (Hct 35%), hepatosplenomegaly and positive specific anti-Toxocara canis antibodies. Toxocariasis-associated eosinophilic cellulitis was diagnosed. Already two weeks after treatment with thiabendazole the skin lesions resolved, T. canis antibody titre normalized eight months after treatment and no recurrences of eosinophilic cellulitis have been observed (for meanwhile three years).
Conclusion
The clinical characteristics (relapsing skin lesions, fever, hepatosplenomegaly), the laboratory features (blood eosinophilia, modest anemia, positive T. canis serology) and the clinical course after treatment, all support a causal relationship between Toxocara infection and the disease of this patient. We propose that in this context eosinophilic cellulitis must be interpreted as the leading symptom of a "skin-predominant" form of overt adult toxocariasis out of a spectrum of toxocariasis-associated febrile, "migrating-relapsing", organotropic eosinophilic inflammatory syndromes.
doi:10.1186/1757-1626-1-356
PMCID: PMC2621125  PMID: 19038064

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