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1.  Intramedullary non-specific inflammatory lesion of thoracic spine: A case report 
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.
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.
PMCID: PMC2817645  PMID: 20074378
2.  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.
PMCID: PMC3819895  PMID: 24251051
3.  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.
PMCID: PMC2775847  PMID: 19949655
Myelitis; Atopy; Toxocariasis
4.  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
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.
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.
The detection of VZV DNA in CSF by nested PCR assay and the evaluation of the antibody index to VZV had significant diagnostic value.
PMCID: PMC3656678  PMID: 23687496
Varicella zoster virus; Myelitis; Enzyme immunoassay; Antibody index; Nested polymerase chain reaction assay
6.  Patient with neuromyelitis optica and inflammatory demyelinating lesions comprising whole spinal cord from C2 level till conus: case report 
BMC Neurology  2009;9:56.
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.
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.
PMCID: PMC2773232  PMID: 19852774
7.  Medullary schistosomiasis 
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.
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.
PMCID: PMC4078447  PMID: 24991469
Differential diagnosis; medullary schistosomiasis; outcome
8.  Involvement of the cervical cord and medulla in posterior reversible encephalopathy syndrome 
Annals of Saudi Medicine  2011;31(1):90-92.
The posterior reversible encephalopathy syndrome (PRES) is characterized by patchy cortical and subcortical lesions in the distribution of the posterior circulation. The lesions are classically reversible. This syndrome has multiple etiologies, most of which cause acute hypertension. We present a case of PRES with involvement of the medulla and cervical cord (apart from the typical parieto-occipital lesions)-an extremely rare imaging manifestation of PRES. It is important to recognize the imaging findings of PRES in spinal cord, and avoid misdiagnosis as myelitis by proper clinical correlation. Typically patients with myelitis have a profound neurodeficit, while patients with spinal manifestations of PRES are asymptomatic. Involvement of the cord in PRES has probably been an underrecognized entity as spinal imaging is not routinely performed in posterior reversible encephalopathy syndrome.
PMCID: PMC3101734  PMID: 21245605
9.  Fulminant holocord intramedullary tubercular abscess with enigmatic presentation 
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.
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.
PMCID: PMC3622373  PMID: 23607054
Filum terminale; intramedullary; spinal tuberculosis; subarachnoidal; tubercular abscess
10.  Acute Myelitis in a Patient with Vogt-Koyanagi-Harada Disease: Case Report and Review of the Literature 
Vogt-Koyanagi-Harada (VKH) disease is characterized by bilateral granulomatous uveitis with neurologic, auditory, and dermatologic manifestations. However, acute myelitis complicating VKH disease has rarely been reported.
Case Report
A 50-year-old Chinese Han woman presented with difficulty walking, numbness on the left side of the body, and difficulty with urination. The patient was diagnosed with incomplete VKH disease and received corticosteroid treatment prior to the neurological presentation. Acute myelitis was diagnosed based on both clinical and spinal-cord MRI findings.
Clinicians should consider acute myelitis as a rare possible neurological manifestation in VKH disease patients, and early systemic administration of corticosteroids will suppress the acute inflammatory process and prevent recurrences. This report raises the possibility that VKH disease and acute myelitis share common pathogenic pathways.
PMCID: PMC3543912  PMID: 23346163
Vogt-Koyanagi-Harada disease; acute myelitis; pathogenesis
11.  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
12.  Transverse myelitis secondary to Melioidosis; A case report 
BMC Infectious Diseases  2012;12:232.
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.
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.
PMCID: PMC3511196  PMID: 23020820
Melioidosis; Transverse myelitis; Burkholderia pseudomallei; Flaccid paraplegia; Psoas abscess
13.  Toxocariasis Presenting as Encephalomyelitis 
Case Reports in Medicine  2011;2011:503913.
We describe a farmer who presented with a clinical picture of a transverse thoracic myelitis. MRI showed inflammatory lesions in brain and thoracic spinal cord. Toxocariasis was suspected because of eosinophilia in blood and cerebrospinal fluid, and this diagnosis was confirmed immunologically. He was successfully treated with antihelminthics in combination with corticosteroids. Neurotoxocariasis is rare and diagnosis can be difficult because of the different and atypical clinical manifestations. It should be considered in every case of central neurological syndrome associated with eosinophilia.
PMCID: PMC3099216  PMID: 21629809
14.  Idiopathic Transverse Myelitis and Neuromyelitis Optica: Clinical Profiles, Pathophysiology and Therapeutic Choices 
Current Neuropharmacology  2011;9(3):417-428.
Transverse myelitis is a focal inflammatory disorder of the spinal cord which may arise due to different etiologies. Transverse myelitis may be idiopathic or related/secondary to other diseases including infections, connective tissue disorders and other autoimmune diseases. It may be also associated with optic neuritis (neuromyelitis optica), which may precede transverse myelitis. In this manuscript we review the pathophysiology of different types of transverse myelitis and neuromyelitis optica and discuss diagnostic criteria for idiopathic transverse myelitis and risk of development of multiple sclerosis after an episode of transverse myelitis. We also discuss treatment options including corticosteroids, immunosuppressives and monoclonal antibodies, plasma exchange and intravenous immunoglobulins.
PMCID: PMC3151596  PMID: 22379456
Transverse myelitis; neuromyelitis optica; epidemiology; pathology; pathogenesis; treatment.
15.  Multilevel Intramedullary Spinal Neurocysticercosis With Eosinophilic Meningitis 
Archives of neurology  2004;61(5):770-772.
Cysticercal involvement of the spinal cord is a very rare form of neurocysticercosis. Intramedullary cysts are even less common.
To describe a novel presentation of multilevel intramedullary neurocysticercosis with eosinophilic meningitis.
Case report.
A 35-year-old man with a history of cerebral neurocysticercosis who presented with both cauda equina and Brown-Sequard syndromes associated with cerebrospinal fluid findings of eosinophilic meningitis.
Magnetic resonance imaging confirmed the multilevel intramedullary cord lesions. The patientwas treated medically with dexamethasone and albendazole and had a good recovery.
Intramedullary neurocysticercosis should be considered as a potentially treatable cause of multilevel spinal lesions with subacute meningitis.
PMCID: PMC3902854  PMID: 15148157
16.  Effective management of intractable neuropathic pain using an intrathecal morphine pump in a patient with acute transverse myelitis 
Transverse myelitis is a rare inflammatory myelopathy characterized by loss of motor and sensory function below the affected level of the spinal cord, and causes neurogenic bowel and bladder. Occasionally, it also causes neuropathic pain with spasticity. Traditional therapies for neuropathic pain are multiple, including multimodal analgesic regimens, antiepileptic or antidepressant medications, opioids, sympathetic blocks, and spinal cord stimulation. Persistent neuropathic pain can cause emotional distress by affecting sleep, work, recreation, and emotional well-being. Here we report the case of a patient suffering from intractable neuropathic pain following acute transverse myelitis that was not relieved by combinations of nonsteroidal anti-inflammatory, anti-epileptic, antidepressant, and opioid medications, or by acupuncture. Implantation of an intrathecal morphine pump controlled the pain successfully without side effects, and enabled the patient to embark on intensive rehabilitation. The patient’s muscle strength has improved significantly and the patient may soon be able to use a walker with minimal assistance.
PMCID: PMC3735335  PMID: 23935366
intrathecal morphine pump; neuropathic pain; rehabilitation; transverse myelitis
17.  Neuromyelitis Optica Immunoglobulin G in a Child 
Pediatric neurology  2006;35(5):370-372.
Neuromyelitis optica or Devic’s syndrome is an uncommon demyelinating disorder that preferentially attacks the spinal cord and optic nerves. Although it is well described in adults, childhood neuromyelitis optica has rarely been reported in the literature and is frequently misdiagnosed as severe multiple sclerosis. Recently, a serum immunoglobulin G test for neuromyelitis optica has become available which may clarify and accelerate the diagnosis. This report describes a child with recurrent myelitis and an elongated spinal cord lesion who was found to have positive neuromyelitis optica autoantibody. We believe that neuromyelitis optica autoantibody testing should be performed in cases of pediatric transverse myelitis with multiple vertical segments or recurrence.
PMCID: PMC2918874  PMID: 17074612
18.  Spinal stroke in older people secondary to dural arteriovenous fistula 
BMJ Case Reports  2011;2011:bcr0620114373.
A 61-year-old diabetic male developed weakness of both lower limbs while walking, 1 month go. When he was examined in hospital a hour later, it was found that he had total absence of movements in both legs, sensory loss of all modalities till umbilicus and had urinary retention. MRI spine demonstrated an intramedullary longitudinal T2 hyperintensity extending from upper thoracic cord till conus medullaris. A provisional diagnosis of transverse myelitis was made and started on corticosteroids. Partial improvement was noted over a 3 week period, after which he developed urinary infection, hyponatremia and sudden worsening of weakness. Repeat MRI spine with contrast raised the possibility of dural arteriovenous malformation leading to extensive spinal cord infarction, which was confirmed by MR angiogram.
PMCID: PMC3171046  PMID: 22688942
19.  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
20.  Acute Transverse Myelitis (Ascending Myelitis) as the Initial Manifestation of Japanese Encephalitis: A Rare Presentation 
Japanese encephalitis, an inflammatory brain disease prevalent in Southeast Asia, usually presented with fever, headache, convulsions, brain stem signs with pyramidal and extrapyramidal features, and altered sensorium. Acute transverse myelitis, as the initial manifestation of Japanese encephalitis, is an unusual manifestation and is seldom reported. We hereby report a case of 13-year-old adolescent boy who presented to us with fever and acute onset paraparesis with urinary retention initially, progressing to quadriparesis and then followed by headache and altered sensorium. Brain MRI revealed bilateral basal ganglia that were grossly swollen with vasogenic edema tracking along internal capsule and midbrain. Adjacent ventrolateral thalamus and internal capsule also showed mild abnormal intensities. Spinal screening showed abnormal cord intensities in entire cord with gross edema in cervical and conus regions. He had elevated IgM titres against JE virus in cerebrospinal fluid. The patient was treated conservatively along with intravenous methyl prednisolone for 5 days. He regained near normal power at 3 months in followup, but hesitancy, dysarthria, and slowness of movement still persisted. To conclude, a young boy presenting with ATM in an endemic region of JE, then a possibility of Japanese encephalitis, should be sought by clinicians as early use of immunomodulator shows survival benefit.
PMCID: PMC3621150  PMID: 23585973
21.  Spinal cord swelling with abnormal gadolinium-enhancement mimicking intramedullary tumors in cervical spondylosis patients: Three case reports and review of the literature 
Spinal cord swelling with abnormal gadolinium (Gd) enhancement is a rare preoperative radiological finding in patients with cervical spondylosis. In the presence of progressive myelopathy, timely surgical decompression can be curative.
Case presentation:
We report 3 patients with cervical spondylotic myelopathy. Preoperative magnetic resonance imaging (MRI) revealed spondylotic changes and intramedullary lesions in the cervical spine. We noted cervical cord swelling with high intensity on T2-weighted MRI and abnormal Gd-DTPA enhancement. Laminoplasty resulted in marked improvement of their neurological condition and postoperative MRI revealed gradual regression of the intramedullary lesions during the first year.
We posit that the intramedullary lesions in our patients were reflective of spinal cord edema with blood-brain-barrier disturbance in the cervical cord, possibly due to minor recurrent spinal cord injury and disturbed venous circulation. Spinal cord edema is a rare condition in patients with cervical spondylosis and an accurate diagnosis and timely surgery are necessary for cure. Therefore, this unusual condition must be considered in spondylosis patients manifesting as intramedullary lesions on MRI of the cervical spinal cord. Careful evaluation of the postoperative course can be used to confirm the diagnosis and help in selecting a subsequent therapeutic strategy.
PMCID: PMC3201080  PMID: 22028753
cervical spondylosis; gadolinium enhancement; intramedullary lesion; spinal cord edema; spinal cord swelling
22.  Longitudinally extensive transverse myelitis as the sole presentation of neuro-Behçet's disease responding to infliximab 
Isolated involvement of the spinal cord is an uncommon presentation of neuro-Behçet's disease (NBD) and it is associated with a poor prognosis for functional recovery.
A case report of an 18-year-old Turkish man who presented with a progressive paraparesis and bladder dysfunction secondary to a longitudinally extensive transverse myelitis as the sole presentation of NBD.
Examination revealed a spastic paraparesis and a T7 sensory level. Magnetic resonance imaging revealed multiple enhancing lesions throughout the thoracic cord and cerebrospinal fluid showed intense neutrophilia. On further enquiry a family history of Behçet's disease was elicited. The patient subsequently reported a history of recurrent oral ulceration and intermittent occular inflammation. A diagnosis of NBD was made and intravenous high-dose steroids commenced with poor response. In view of the poor prognosis for functional recovery associated with spinal NBD the patient was treated with infliximab, an anti-tumour necrosis factor-alpha monoclonal antibody, leading to excellent recovery of function.
Conclusion/clinical relevance
Early treatment with infliximab may facilitate a favourable functional recovery and should be considered in cases of NBD with spinal cord involvement.
PMCID: PMC3304557  PMID: 22333754
Behçet's disease; Infliximab; Myelitis; Spinal cord; Paraparesis
23.  Activation of Innate Immune Responses in the Central Nervous System during Reovirus Myelitis 
Journal of Virology  2012;86(15):8107-8118.
Reovirus infection of the murine spinal cord (SC) was used as a model system to investigate innate immune responses during viral myelitis, including the activation of glia (microglia and astrocytes) and interferon (IFN) signaling and increased expression of inflammatory mediators. Reovirus myelitis was associated with the pronounced activation of SC glia, as evidenced by characteristic changes in cellular morphology and increased expression of astrocyte and microglia-specific proteins. Expression of inflammatory mediators known to be released by activated glia, including interleukin-1β (IL-1β), tumor necrosis factor alpha (TNF-α), chemokine (C-C motif) ligand 5 (CCL 5), chemokine (C-X-C motif) ligand 10 (CXCL10), and gamma interferon (IFN-γ), was also significantly upregulated in the SC of reovirus-infected animals compared to mock-infected controls. Reovirus infection of the mouse SC was also associated with increased expression of genes involved in IFN signaling, including IFN-stimulated genes (ISG). Further, reovirus infection of mice deficient in the expression of the IFN-α/β receptor (IFNAR−/−) resulted in accelerated mortality, demonstrating that IFN signaling is protective during reovirus myelitis. Experiments performed in ex vivo SC slice cultures (SCSC) confirmed that resident SC cells contribute to the production of at least some of these inflammatory mediators and ISG during reovirus infection. Microglia, but not astrocytes, were still activated, and glia-associated inflammatory mediators were still produced in reovirus-infected INFAR−/− mice, demonstrating that IFN signaling is not absolutely required for these neuroinflammatory responses. Our results suggest that activated glia and inflammatory mediators contribute to a local microenvironment that is deleterious to neuronal survival.
PMCID: PMC3421655  PMID: 22623770
24.  Anti-AQP4 antibody in idiopathic acute transverse myelitis with recurrent clinical course: frequency of positivity and influence in prognosis 
The anti-aquaporin4 (anti-AQP4) antibody is specific for neuromyelitis optica (NMO), but is also found in limited forms. The presence of this antibody in acute transverse myelitis (ATM) has been associated with recurrence and conversion to NMO, but the influence on disability has not yet been described.
To describe the frequency of anti-AQP4 in ATM and analyze the influence in long-term prognosis.
Cross-sectional and retrospective study.
Consecutive ATM cases in a multiple sclerosis center in Rio de Janeiro, Brazil, from 2000 through 2009 were reviewed. Recurrent cases tested for anti-AQP4 were selected. ATM with magnetic resonance imaging spinal cord lesions extending over three or more vertebral segments was classified as longitudinally extensive transverse myelitis (LETM); Kurtzke scale was applied at last evaluation.
Outcome measures
Frequency of anti-AQP4; severity of spinal cord dysfunction at last follow-up.
Twenty six patients (21 female:5 male; 17 white:9 African descent) were studied. The first ATM occurred at 38.04 ± 12.7 years. The interval between the first and the second ATM was eight months (1–150) and the number of ATM varied from two to seven. After 40.5 months (12–192) of disease, the median Expanded Disability Status Scale (EDSS) score was three (0–9). Anti-AQP4 antibody was positive in 26.9%. LETM was found in 65.4%. LETM presented later onset, higher disability and higher positivity to anti-AQP4 (LETM 41.2% versus no-LETM 0%, P = 0.024). Dysfunction at long-term follow-up was similar in anti-AQP4 positive and negative cases.
The frequency of anti-AQP4 in recurrent ATM was 26.9%, increasing to 41.2% among LETM. Presence of the antibody had no influence on morbidity.
PMCID: PMC3425881  PMID: 22925751
Transverse myelitis; Demyelinating diseases; Neuromyelitis optica; Anti-AQP4 antibody; Disability; Multiple sclerosis; Disability; Paresis
25.  Transverse myelitis and acute HIV infection: a case report 
BMC Infectious Diseases  2014;14:149.
Most HIV infected patients will develop some sort of neurologic involvement of the disease throughout their lives, usually in advanced stages. Neurologic symptoms may occur in acute HIV infection but myelopathy in this setting is rare. Up until this date, only two cases of transverse myelitis as a manifestation of acute HIV infection have been reported in the literature. Therapeutic approach in these patients is not well defined.
Case presentation
A 35 year-old male Caucasian recently returned from the tropics presented to our hospital with urinary retention and acute paraparesis. After extensive diagnostic workup he was diagnosed with acute HIV infection presenting as transverse myelitis. Full neurologic recovery was observed without the use of anti-retroviral therapy.
Acute spinal cord disorders are challenging, as they present a wide array of differential diagnosis and may lead to devastating sequelae. Timely and rigorous diagnostic workup is of the utmost importance when managing these cases. Clinicians should be aware of the protean manifestations of acute HIV infection, including central nervous system involvement, and have a low threshold for HIV screening.
PMCID: PMC3995161  PMID: 24646059
Transverse myelitis; HIV; Acute infection

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